Nutricia Product Reference Guide

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NUTRICIA PRODUCT REFERENCE GUIDE FOR CLINICIANS Updated as of February 2019 Nutricia Contacts How to Contact Nutricia • Nutricia is a Global Health Company My Nutricia sales representative(s): Nutricia’s Customer Service: • Nutricia Leads the Development and Use of Advanced Medical Nutrition • Phone: (800) 365-7354 for Specialized Care Name:...... • Fax: (301) 795-2301 • Nutricia Products and Services Transform the Daily Lives of Millions of Phone:...... • Email: [email protected] Infants, Children, Adults and Aging Seniors • Live Chat: www.Neocate.com (M-F 8:30 am – 7 pm ET) Email:...... This guide is a convenient reference for clinicians to the full range of Nutricia’s Nutricia’s Nutrition Services: medical nutritional products. Nutricia strives to develop quality products that • Phone: (800) 365-7354 – Option 2 offer choice and flexibility to today’s patients to enhance diet acceptance. • Email: [email protected] Please note that all of our products require medical supervision. For additional Name:...... information, please call our Nutrition Services team or visit our web site at • Live Chat: www.Neocate.com (M-F 8:30 am – 5 pm ET) www.Nutricia-NA.com. Our staff is comprised of individuals with the highest Phone:...... level of expertise ready to offer support and reliability to our customers. We look forward to hearing from you. Nutricia Metabolics: Email:...... • Phone: (800) 605-0410 • Email: [email protected] Consumers and Patients: Nutricia products are available for convenient home delivery directly from Nutricia North America. Consumers can call our toll free number or visit one of Internet: Name:...... our websites. See Resources for a list of our websites. For information on Nutricia products, reimbursement,* and other helpful resources, visit www.Nutricia-NA.com. Consumers can also purchase Nutricia products through retail pharmacies. Phone:...... For a complete listing of our websites, see Resources. Pharmacies can special order Nutricia products from their wholesaler or * For reimbursement information, please see Resources. directly from Nutricia North America. Email:......

Ordering Information...... 2-3 Complete Amino Acid Mix...... 180 Periflex Advance...... 44 Contents by Product Indications...... 6-8 Complex Essential MSD Drink Mix...... 80 PKU Periflex Early Years...... 40 Complex Junior MSD Drink Mix...... 78 PKU Periflex Junior Plus...... 42 GI/Allergy...... 9-38 Complex MSD Amino Acid Blend...... 82 Periflex LQ...... 46 Mixing Instructions...... 22-23 Diff-Stat...... 166 PhenylAde40 Drink Mix...... 56 Household Measurements...... 24 Duocal...... 176 PhenylAde60 Drink Mix...... 58 PhenylAde Amino Acid Blends...... 60 Volume Prescription...... 25 Essential Amino Acid Mix...... 182 Fiber-Stat...... 168 PhenylAde Essential Drink Mix...... 54 Dilution Charts...... 26-37 GA-1 Anamix Early Years...... 94 PhenylAde GMP Drink Mix...... 48 Update on Monitoring Micronutrients...... 38 GlutarAde Amino Acid Blend...... 100 PhenylAde GMP MIX-IN...... 52 Metabolics...... 39-140 GlutarAde Essential GA-1 Drink Mix...... 98 PhenylAde GMP READY...... 50 GlutarAde Junior GA-1 Drink Mix...... 96 PhenylAde PheBLOC LNAA...... 62 Household Measurements: Metabolics...... 128-129 HCU Anamix Early Years...... 102 Phlexy-10 Tablets...... 72 Mixing Instructions: Metabolics...... 130-140 HCU Anamix Next...... 104 Phlexy-10 Drink Mix...... 74 Low Protein Foods...... 141-142 HCU Lophlex LQ...... 108 Phlexy-Vits...... 184 Ketogenic Diet...... 143-154 IVA Anamix Early Years...... 88 PKU Lophlex LQ...... 68 IVA Anamix Next...... 90 PKU Lophlex Powder...... 70 Mixing Instructions: KetoCal...... 152 KetoCal 2.5:1 LQ...... 150 Polycal...... 178 Volume & Ordering Guide...... 153 KetoCal 3:1 Powder...... 144 Pro-Stat MAX...... 160 Specialized Adult Nutrition...... 155-170 KetoCal 4:1 LQ...... 148 Pro-Stat Renal Care...... 162 Pro-Stat Sugar Free...... 156 Other Medical Nutritionals...... 171-188 KetoCal 4:1 Powder...... 146 Lanaflex...... 64 Pro-Stat Sugar Free AWC...... 158 Resources...... 189-198 Liquigen...... 174 Single Amino Acids...... 186 Low Protein Foods...... 142 SOD Anamix Early Years...... 126 MMA/PA Anamix Early Years...... 110 TYR Anamix Early Years...... 116 MMA/PA Anamix Next...... 112 TYR Anamix Next...... 118 TYR Lophlex GMP MIX-IN...... 122 GI/Allergy Metabolics Low Protein Foods Ketogenic Diet Specialized Adult Nutrition Other Nutritionals Monogen...... 172 MSUD Anamix Early Years ...... 76 TYR Lophlex LQ...... 120 UCD Anamix Junior...... 124 page 9-38 page 39-140 page 141-142 page 143-154 page 155-170 page 171-188 MSUD Lophlex LQ...... 86 MSUD Maxamum...... 84 UTI-Stat...... 164 Neocate Infant DHA/ARA...... 12 XLeu Maxamum...... 92 Neocate Junior...... 16-19 XMet Maxamum...... 106 Neocate Nutra...... 14 XMet, XCys Maxamaid...... 127 Neocate Splash...... 20 XMTVI Maxamum...... 114 4 Neocate Syneo Infant...... 10 XPhe Maxamum...... 66 5 CONTENTS – By Indication and Diet Need*

Allergies, Cow Milk and Multiple Food Allergies Food Protein-Induced Enterocolitis Syndrome (FPIES) Inborn Errors of Metabolism Malabsorption Neocate Infant DHA/ARA...... 12 Neocate Junior...... 16-19 Nutricia Metabolic Formulas...... 40-127 Duocal...... 176 Neocate Nutra...... 14 Neocate Splash...... 20 Low Protein Foods...... 141 Neocate Nutra...... 14 Neocate Junior...... 16-19 Neocate Syneo Infant...... 10 Liquigen...... 174 Neocate Junior...... 16-19 Neocate Splash...... 20 Gastrointestinal Health (Adult) Monogen...... 172 Neocate Splash...... 20 Neocate Syneo Infant...... 10 Fiber-Stat...... 168 Duocal...... 176 Liquigen...... 174 Complete Amino Acid Mix...... 180 Diff-Stat...... 166 Polycal...... 178 Complete Amino Acid Mix...... 180 Complete Amino Acid Mix...... 180 Amino Acid-Based Products Gastroesophageal Reflux (GER) Maple Syrup Urine Disease Neocate Infant DHA/ARA...... 12 Essential Amino Acid Mix...... 182 Complex Junior MSD Drink Mix...... 78 Neocate Nutra...... 14 Phlexy-Vits...... 184 Neocate Nutra...... 14 Neocate Junior...... 16-19 Complex Essential MSD Drink Mix...... 80 Neocate Junior...... 16-19 Single Amino Acids...... 186 Complex MSD Amino Acid Blend...... 82 Neocate Splash...... 20 Isovaleric Acidemia Neocate Splash...... 20 Neocate Syneo Infant...... 10 MSUD Anamix Early Years...... 76 Neocate Syneo Infant...... 10 IVA Anamix Early Years...... 88 MSUD Maxamum...... 84 Nutricia Metabolic Formulas...... 40-127 Glutaric Acidemia Type I IVA Anamix Next...... 90 MSUD Lophlex LQ...... 86 Complete Amino Acid Mix...... 180 GlutarAde Junior GA-1 Drink Mix...... 96 XLeu Maxamum...... 92 GlutarAde Essential GA-1 Drink Mix...... 98 Modular Diets Essential Amino Acid Mix...... 182 Ketogenic Diet Duocal...... 176 Single Amino Acids...... 186 GlutarAde Amino Acid Blend...... 100 GA-1 Anamix Early Years...... 94 KetoCal 2.5:1 LQ...... 150 Liquigen...... 174 Chylothorax KetoCal 3:1 Powder...... 144 Polycal...... 178 Liquigen...... 174 Homocystinuria/Hypermethioninemia KetoCal 4:1 LQ...... 148 Complete Amino Acid Mix...... 180 Monogen...... 172 HCU Anamix Early Years...... 102 KetoCal 4:1 Powder...... 146 Essential Amino Acid Mix...... 182 HCU Anamix Next...... 104 Critical Illness Requiring Increased Protein Liquigen...... 174 Phlexy-Vits...... 184 XMet Maxamum...... 106 Long Chain Fatty Acid Oxidation Disorders Pro-Stat MAX...... 160 HCU Lophlex LQ...... 108 Dialysis Liquigen...... 174 Pro-Stat Renal Care...... 162 Monogen...... 172 Eosinophilic Esophagitis (EoE) Low Protein Diets Neocate Nutra...... 14 Low Protein Foods...... 141 Neocate Junior...... 16-19 Duocal...... 176 Neocate Splash...... 20 Low Serum Protein Levels Neocate Syneo Infant...... 10 Pro-Stat MAX...... 160 Complete Amino Acid Mix...... 180 Pro-Stat Sugar Free AWC...... 158 Pro-Stat Sugar Free...... 156 Pro-Stat Renal Care...... 162

*Not a complete listing 6 7 Phenylketonuria Short Bowel Syndrome PKU Periflex Early Years...... 40 Liquigen...... 174 PKU Periflex Junior Plus...... 42 Neocate Nutra...... 14 Periflex LQ...... 46 Neocate Junior...... 16-19 Periflex Advance...... 44 Neocate Splash...... 20 PhenylAde40 Drink Mix...... 56 Complete Amino Acid Mix...... 180 PhenylAde60 Drink Mix...... 58 Sulphite Oxidase Deficiency PhenylAde Amino Acid Blends...... 60 SOD Anamix Early Years...... 126 PhenylAde Essential Drink Mix...... 54 XMet, XCys Maxamaid...... 127 PhenylAde GMP Drink Mix...... 48 PhenylAde GMP MIX-IN...... 52 Tyrosinemia PhenylAde GMP READY...... 50 TYR Anamix Early Years...... 116 PhenylAde PheBLOC LNAA...... 62 TYR Anamix Next...... 118 XPhe Maxamum Powder...... 66 TYR Lophlex GMP MIX-IN...... 122 PKU Lophlex LQ...... 68 TYR Lophlex LQ...... 120 PKU Lophlex Powder...... 70 Unintentional Muscle Loss Phlexy-10 Tablets...... 72 Pro-Stat MAX...... 160 Phlexy-10 Drink Mix...... 74 Pro-Stat Sugar Free...... 156 Lanaflex...... 64 Pro-Stat Sugar Free AWC...... 158 Propionic/Methylmalonic Acidemia Pro-Stat Renal Care...... 162 MMA/PA Anamix Early Years...... 110 Urea Cycle Disorders MMA/PA Anamix Next...... 112 UCD Anamix Junior...... 124 XMTVI Maxamum...... 114 Essential Amino Acid Mix...... 182 Protein-Energy Malnutrition (PEM) Urinary Tract Health Pro-Stat MAX...... 160 UTI-Stat...... 164 Pro-Stat Sugar Free AWC...... 158 Wound/Pressure Injury Management Pro-Stat Sugar Free...... 156 Pro-Stat MAX...... 160 Pro-Stat Renal Care...... 162 Pro-Stat Sugar Free AWC...... 158 Pro-Stat Sugar Free...... 156 Pro-Stat Renal Care...... 162

8 Tobey GI/ALLERGY Diagnosed with multiple food allergies

TM Find more information and resources on these and all of our products at www.NutriciaLearningCenter.com 9 10 GI/ALLERGY • • • Fat • • • • • • • • Main Features: * 2014;75:343-51. 3. Candy, etal. Pediatr Res.2018;83:677-86. Pediatr1. Burks,etal. AllergyImmunol.2015;26:316-22. 2.Harvey, etal.Pediatr Res.

Halal Hypoallergenic short- and long-chain fructooligosaccharides ** short- and long-chain fructooligosaccharides • • Displacement 0.71mL/g Potential RenalSoluteLoad**: 176 mOsm/L Energy distribution: Free water**:90.0% Osmolality**: 360mOsm/kg Prebiotic blendofscFOSandlcFOS* Probiotic Backed by 3clinicaltrials,clinicallyshownto: Carbohydrate Protein

Help balancethegutmicrobiotaof Support growthanddevelopment Effectively resolve foodallergysymptoms food-allergic infants (MCT33%/LCT 67%) Bifidobacterium breve

containing ironandnucleotides. with prebioticsandprobioticsspecificallydesignedforfood-allergicinfants, exemptA nutritionallycomplete,powderedaminoacid-based infantformula Neocate 43.5% 45.1% 11.4% 3 M-16V Atstandard concentration, 20kcal/fl oz ® 1,2 Syneo

1 111436 Code Product Ordering/Reimbursement Information: office todetermine availability. WIC For guidanceonmonitoringmicronutrients, pleasesee page38. please seetheendofthissection. For detailson • • • • • • Indications:

® Related GIandallergicconditions: Multiple foodallergies Cow milkallergy Infant ‡

Contact your stateWIC Gastroesophageal reflux Eosinophilic esophagitis(EoE) Food protein-inducedenterocolitis syndrome(FPIES) 49735-0114-36 Reimbursement scoop size,householdmeasures, directed by ahealthcare professional. venous catheterorpost-pyloric feedingtubeunless Not recommended foruseininfantswith a central B4161 Code Packaging HCPCS

information see information For morereimbursement 4 x400g(14.1oz) Product andadministration, Resources of April2018 Updated as 1900 Calories Per Can .

Nutrition Information: Inositol, mg Choline, mg Vitamin C,mg Biotin, mcg Pantothenic Acid,mcg Folic Acid,mcg Niacin, mcg Vitamin B Vitamin B Riboflavin, mcg Thiamin, mcg Vitamin K,mcg Vitamin E,IU(mg Vitamin D Vitamin A,IU(mcgRE) Vitamins SolubleFiber, g Carbohydrate, g ARA,mg DHA,mg LinoleicAcid,mg Fat, g Protein Equivalent,g Calories Nutrients a -Linolenic Acid,mg

6 12 3

, mcg , IU(mcg) , mcg a -TE) 352(8.8) Per 100g 1352(406) 2900 4900 3348 396 108 540 540 475 100 540 95.4 51.6 19.0 64.0 42.5 53.5 77.0 77.0 23.0 13.5 6.5 (4.4) 1.3 4.5 Per 100kcal 285(85.5) 1032 114 110 611 705 114 83.4 22.7 20.1 10.9 13.5 11.3 16.2 16.2 74.1 (1.9) 1.4 (0.93) 0.27 4.0 9.0 0.95 4.8 2.8 Chloride, mg Potassium, mg Sodium, mg Selenium, mcg Chromium, mcg Molybdenum, mcg Iodine, mcg Copper, mcg Manganese, mcg Zinc, mg Iron, mg Magnesium, mg Phosphorus, mg Calcium, mg Minerals

Per 100g 378 515 185 400 190 388 549 14.2 10.4 11.0 98.0 50.0 5.2 7.1 Per 100kcal 108 116 79.6 38.9 20.6 84.2 40.0 10.5 81.7 3.0 2.2 2.3 1.1 1.5 ** ASourceof Docosahexaenoic Acid(DHA) * ASourceofArachidonic Acid(ARA) Cyanocobalamin. Sodium Molybdate,D-Biotin, VitaminD Chloride, Phylloquinone, SodiumSelenite, Tocopherols, Folic Acid,Chromium Tocopherol, Potassium Iodide,Mixed Riboflavin, VitaminAAcetate,DL-alpha Pyridoxine Hydrochloride, CupricSulfate, Sulfate, ThiaminChlorideHydrochloride, M-16V, Ascorbyl Palmitate, Manganese Tocopheryl Acetate, 5’-Monophosphate DisodiumSalt,DL-alpha Calcium D-Pantothenate, Guanosine Salt, Adenosine5’-Monophosphate, Inosine 5’-Monophosphate Disodium Cytidine 5’-Monophosphate, Niacinamide, 5’-Monophosphate DisodiumSalt, Sulfate, ZincL-Carnitine, Uridine L-Ascorbic Acid,Taurine, Ferrous M. alpina Tricalcium Citrate, MagnesiumL-Aspartate, Chloride, CholineBitartrate, L-Methionine, L-Cystine, L-Tryptophan, Magnesium L-Histidine, L-Alanine, SodiumChloride, L-Phenylalanine, L-Tyrosine, L-Serine, Glycine, L-Isoleucine, L-Threonine, of Mono-andDiglycerides (CITREM), L-Proline, L-Valine, CitricAcidEsters Tripotassium Citrate, L-Glutamine, Dibasic, L-Leucine, L-Lysine Acetate, L-Arginine, L-Aspartate, CalciumPhosphate Carbonate, CalciumGlycerophosphate, Phosphate, Tricalcium Phosphate,Calcium of eachthefollowing:Dipotassium (4%) andInulin(0.5%),lessthan2% Fructooligosaccharides (Oligofructose Sunflower Oil(4%),Canola(4%)), (8%), HighOleicSunflowerOil(6%), Vegetable Oil(MediumChainTriglycerides Corn SyrupSolids(51%),Refined Ingredients: Oil*, C. cohnii Bifidobacterium breve Oil**, M-Inositol, 3 ,

11 Neocate Syneo Infant Syneo Neocate 12 GI/ALLERGY • • • • • • • • • • Main Features: Hypoallergenic 168 mOsm/L Fat Carbohydrate Protein 340 mOsm/kg support Neocate DHA andARAat1:1ratio 4.8 mgper100kcals Displacement Halal &Kosher Pareve Potential RenalSoluteLoad: Energy distribution: Free water(at0.67kcal/mL): 90.3% Osmolality (at0.67kcal/mL): Over 100publications andcounting DHA at0.35%fattyacids Nucleotides, presentinhumanmilk (MCT 33%/LCT 67%)

containing ironandnucleotides. exemptA nutritionallycomplete,powderedaminoacid-based infantformula Neocate 45.7% 43.1% 11.2% 0.70 mL/g

® InfantDHA/ARA For guidanceonmonitoringmicronutrients, pleasesee page38. please seetheendofthissection. For detailson • • • Indications:

12595 Code Product Ordering/Reimbursement Information: Related GIandallergicconditions Multiple foodallergies Cow milkallergy office todetermine availability. WIC ‡ :

Contact your stateWIC

49735-0125-95 Reimbursement scoop size,householdmeasures, B4161 Code Packaging HCPCS

information see information For morereimbursement 4 x400g(14.1oz) Product andadministration, Resources of April2018 1932 Per Calories Can Updated as

2900 4900 3565 419 108 540 540 483 100 540 95.4 51.6 19.0 64.0 42.5 52.0 81.6 81.6 24.5 13.5 1.3 6.8 (4.6) Per 100kcal 280(84.1) 1014 110 110 600 738 112 86.8 22.4 19.8 10.7 13.3 10.8 16.9 16.9 0.27 3.9 8.8 5.1 2.8 1.4 (0.94) Chloride, mg Potassium, mg Sodium, mg Selenium, mcg Chromium, mcg Molybdenum, mcg Iodine, mcg Copper, mcg Manganese, mcg Zinc, mg Iron, mg Magnesium, mg Phosphorus, mg Calcium, mg Minerals Per 100g 386 525 189 100 410 200 397 561 14.5 10.6 11.3 51.0 5.3 7.3 Per 100kcal 109 116 79.9 39.1 20.7 84.9 41.4 10.6 82.2 3 2.2 2.3 1.1 1.5 ** ASource ofDocosahexaenoic Acid (DHA) * ASourceofArachidonic Acid(ARA) D-Biotin, Vitamin D Sodium Selenite, Molybdate, Acid, ChromiumChloride, Phylloquinone, Potassium Iodide, Mixed Tocopherols, Folic Vitamin AAcetate,DL-alpha Tocopherol, Hydrochloride, CupricSulfate, Riboflavin, Chloride Hydrochloride, Pyridoxine Palmitate, ManganeseSulfate,Thiamin DL-alpha Tocopheryl Acetate,Ascorbyl 5’-Monophosphate DisodiumSalt, Calcium D-Pantothenate, Guanosine Salt, Adenosine5’-Monophosphate, Inosine 5’-Monophosphate Disodium Cytidine 5’-Monophosphate, Niacinamide, 5’-Monophosphate DisodiumSalt, Zinc Sulfate,L-Carnitine, Uridine Acid, Taurine, Ferrous Sulfate, C. Cohnii Magnesium L-Aspartate, L-Methionine, Tricalcium Citrate, L-Tryptophan, CholineBitartrate, Chloride, L-Cystine, MagnesiumChloride, L-Serine, L-Histidine, L-Alanine, Sodium L-Threonine, L-Phenylalanine, L-Tyrosine, (CITREM), L-Valine, Glycine, L-Isoleucine, Acid EstersofMono-andDiglycerides Acetate, L-Glutamine, L-Proline, Citric L-Leucine, Tripotassium Citrate, L-Lysine Glycerophosphate, L-Arginine L-Aspartate, Phosphate, CalciumCarbonate, Dipotassium Phosphate,Tricalcium following: CalciumPhosphateDibasic, (4%)), andlessthan2%ofeachthe Oil (6%),Sunflower(5%),Canola Triglycerides (8%),HighOleicSunflower Vegetable Oil(Medium Chain Corn SyrupSolids(54%),Refined Ingredients: Oil**, M-Inositol,L-Ascorbic 3 , Cyanocobalamin. M. Alpina Oil*,

13 ARA / DHA Infant Neocate 14 GI/ALLERGY • • • • • • • • Main Features: nutrition. Notforbottle ortubefeed. Note: Notsuitable as solesourceof Fat Carbohydrate Energy distribution: and preservatives Free fromartificialflavors, colors Good sourceofvitaminD, zincandiron High incalcium Milk proteinandglutenfree amino acids Made with100%non-allergenic Hypoallergenic Smooth, spoonableconsistency Protein (MCT 4%/LCT 96%)

57% 36% 7% The first and only amino acid-based semi-solid medical foodforchildrenThe firstandonlyaminoacid-basedsemi-solidmedical Neocate and infantsover 6monthsofage.

please seetheendofthissection. For detailson • • • Indications: 66739 Code Product Ordering/Reimbursement Information: office todetermine availability. WIC Related GIandallergicconditions: Multiple foodallergies Cow milkallergy

® • • • •

Nutra Gastroesophageal reflux Malabsorption Eosinophilic esophagitis(EoE) Short bowelsyndrome(SBS) ‡ :

Contact your stateWIC 49735-016739 Reimbursement scoop size,householdmeasures, B4161 Code Packaging HCPCS

information see information For morereimbursement 4 x400g(14.1oz) Product andadministration, Resources 1888 Per Calories Can .

Nutrition Information: Inositol, mg Choline, mg Vitamin C,mg Biotin, mcg Pantothenic Acid,mcg Folic Acid,mcg Niacin, mcg Vitamin B Vitamin B Riboflavin, mcg Thiamin, mcg Vitamin K,mcg Vitamin E,IU(mg Vitamin D Vitamin A,IU(mcgRE) Vitamins Carbohydrate, g Fat, g Protein Equivalent,g Calories Nutrients Linoleic Acid,mg 12 6 3 , mcg , IU(mcg) , mcg a T.E.) 172(4.3) Per 100g 649(195) 3222 1400 1100 250 250 318 472 175 49.0 45.0 23.0 27.5 18.5 67.4 18.8 Serving 3.4 (2.3) 9.1 0.6 8.2 Per Suggested (37 gPowder) 1192 240(72.2) 518 407 118 63.6(1.6) 92.5 18.1 16.7 10.2 92.5 24.9 1.3 (0.84) 8.5 3.4 0.22 6.8 7.0 3.0

Chloride, mg Potassium, mg Sodium, mg Selenium, mcg Chromium, mcg Molybdenum, mcg Iodine, mcg Copper, mcg Manganese, mcg Zinc, mg Iron, mg Magnesium, mg Phosphorus, mg Calcium, mg Minerals Per 100g <20 310 310 340 668 44.0 38.0 11.3 10.0 41.4 21.0 Serving 8.8 4.1 5.9 Per Suggested (37 gPowder) <10 115 115 126 247 16.3 14.1 15.3 4.2 3.7 3.3 1.5 2.2 7.8

Cyanocobalamin. Phylloquinone, D-Biotin,VitaminD Sodium Selenite,Molybdate, Chromium Chloride,Folic Acid, Vitamin AAcetate,Potassium Iodide, Pyridoxine Hydrochloride, Riboflavin, Sulfate, ThiaminChlorideHydrochloride, Niacinamide, ManganeseSulfate,Cupric Acetate, CalciumD-Pantothenate, L-Carnitine, DL-Alpha Tocopheryl Ferrous Sulfate,Taurine, ZincSulfate, Acetate, M-Inositol,L-Ascorbic Acid, Magnesium L-Aspartate, Magnesium L-Methionine, CholineBitartrate, L-Alanine, L-Cystine, L-Tryptophan, L-Tyrosine, L-Serine, L-Histidine, L-Threonine, L-Phenylalanine, L-Proline, L-Valine, Glycine, L-Isoleucine, CAEM (AnEmulsifier), L-Glutamine, L-Aspartate, L-Leucine, L-Lysine Acetate, less thaneachofthefollowing:L-Arginine Phosphate Tribasic (2%),contains2%or Sunflower (3%)),Sugar(9%),Calcium High OleicSunflower(6%),Canola(3%), (Non-Hydrogenated Coconut(6%), (19%), Refined Vegetable Oil(18%) Corn SyrupSolids(42%),RiceStarch Ingredients:

3 , 15 Nutra Neocate 16 GI/ALLERGY • • • • • • • • • • Main Hypoallergenic Fat Carbohydrate Protein 5 great-tastingflavors Halal &Kosher Pareve Displacement: 0.69mL/g distribution: Energy Free water (at1kcal/mL): 85.6% Osmolality (at1kcal/mL): 600mOsm/kg Standard dilution=1kcal/mL Also available withprebioticfiberin Nutritionally complete 100% non-allergenicfreeaminoacids (MCT 35%/LCT 65%) Feature s: for individualsover theageof1. medicalfoodA nutritionallycomplete,powderedaminoacid-based (without prebiotics) Neocate

45% 42.6% 12.4%

® Junior, Unflavored

For guidanceonmonitoringmicronutrients, pleasesee page38. please seetheendofthissection. For detailson • • • Indications:

11790 Code Product Ordering/Reimbursement Information: enterocolitissyndrome(FPIES) Related GIandallergicconditions: Multiple foodallergies Cow milkallergy

• • • • • office todetermine availability. WIC

Gastroesophageal reflux Malabsorption Food protein-induced Eosinophilic esophagitis(EoE) Short bowelsyndrome(SBS) ‡ :

Contact your stateWIC scoop size,householdmeasures, 49735-0117-90 Code Reimbursement

B4161 Code HCPCS

information see information For morereimbursement 4 x400g(14.1oz) Packaging Product 14-18 years 9-13 years 4-8 years 1-3 years & minerals: + 25key vitamins 100% DRIs Meets orexceeds andadministration, Resources of May 2018 Upgraded as § forprotein 2550 kcal 1565 kcal 1370 kcal 930 kcal 1916 Per Can Calories .

Inositol, mg Choline, mg Vitamin C,mg Biotin, mcg Pantothenic Acid,mg Folic Acid,mcg Niacin, mg Vitamin B Vitamin B Riboflavin, mg Thiamin, mg Vitamin K,mcg Vitamin E,IU(mg Vitamin D Vitamin A,IU(mcgRE) Vitamins Carbohydrate, g LinoleicAcid,mg Fat, g Protein Equivalent,g Calories Nutrients Nutrition Information:

6 12 3 , mg , IU(mcg) , mcg a T.E.) 380(9.5) Per 100g 956(287) 3912 105 143 143 479 100 10.0 (6.7) 44.6 14.4 19.2 51.0 24.0 14.8 0.48 1.9 4.3 0.96 0.48 1.9 Per 100kcal 200(60.0) 818 21.9 29.9 29.9 10.7 79.4 (2.0) 0.10 2.1 (1.4) 9.3 3.0 0.40 0.90 0.20 0.10 4.0 5.0 3.1 0.40 Chloride, mg Potassium, mg Sodium, mg Selenium, mcg Chromium, mcg Molybdenum, mcg Iodine, mcg Copper, mcg Manganese, mg Zinc, mg Iron, mg Magnesium, mg Phosphorus, mg Calcium, mg Minerals Per 100g 364 653 239 530 382 565 19.2 18.0 21.5 85.2 76.5 0.62 4.7 7.4 Per 100kcal 136 111 118 76.1 50.0 17.8 16.0 79.8 4.0 3.8 4.5 0.13 0.98 1.5

D Selenite, Phylloquinone, D-Biotin,Vitamin Chloride, SodiumMolybdate, Folic Acid,Potassium Iodide,Chromium Hydrochloride, VitaminAAcetate, Chloride Hydrochloride, Pyridoxine Cupric Sulfate,Riboflavin, Thiamin D-Pantothenate, ManganeseSulfate, Ascorbyl Palmitate, Niacinamide,Calcium Sulfate, DL-Alpha Tocopheryl Acetate, Taurine, Ferrous Sulfate, L-Carnitine, Zinc L-Ascorbic Acid,Sunflower Lecithin, Magnesium Chloride,M-Inositol, Chloride, CalciumL-Tryptophan, L-Methionine, L-Arginine, Sodium Magnesium Hydrogen Phosphate, L-Phenylalanine, CholineBitartrate, Carbonate, L-Proline, L-Cystine, L-Histidine, Citrate, Trisodium Citrate, Calcium Maltodextrin, L-Isoleucine, Tripotassium (CITREM), L-Valine, Tricalcium Phosphate, Acid EstersofMono-andDiglycerides L-Tyrosine, DipotassiumPhosphate,Citric L-Leucine, L-Lysine Acetate,L-Threonine, following: L-Glutamine, Glycine, L-Alanine, L-Serine, andlessthan2%ofeachthe Sunflower Oil,CanolaOil), Kernel and/or CoconutOil),HighOleic Oil (MediumChainTriglycerides (Palm Corn SyrupSolids,Refined Vegetable Ingredients:

3 , Cyanocobalamin.

Neocate Junior, Unflavored Unflavored Junior, Neocate 17 prebiotics) (without 18 GI/ALLERGY • mL/g (mOsm/kg) Fat Carbohydrate Protein Energy distribution: Displacement, Free water Osmolality • • • • • • Main

Hypoallergenic

†Standard dilution=1 kcal/mL (MCT35%/LCT 65%) vanilla, strawberry, chocolate&tropical gut health Contains prebioticfibertohelpsupporttotal Contains Also available withoutprebioticfiberinunflavored Available in5great-tastingflavors: unflavored, Nutritionally complete freeaminoacids 100% non-allergenic Feature † †

with prebiotics Unflavored

43.9% 43.6% 12.5% 84.3% 0.74 620 s: Strawberry 43.9% 43.6% 12.5% 85.2% 0.70 610

43.9% 43.6% 12.5% 85.2% Vanilla 0.70 620 660670

Chocolate 43.0% 44.7% 12.3% 84.8% Tropical 41.9% 45.5% 12.6% 84.8% food withprebioticfiberforindividuals over theageof1. A nutritionally complete, powdered amino acid-based medical Neocate

12124 -Tropical 12690 -Chocolate 86456 -Strawberry 60627 -Vanilla 12912 -Unflavored Code Product Ordering/Reimbursement Information: For guidanceonmonitoringmicronutrients, pleasesee page38. please seetheendofthissection. For detailson • • • Indications: office todetermine availability. WIC

enterocolitissyndrome(FPIES) Related GIandallergicconditions: Multiple foodallergies Cow milkallergy

• • • • • ‡

Gastroesophageal reflux Malabsorption Food protein-induced Eosinophilic esophagitis(EoE) Short bowel :

Contact your stateWIC ® Junior scoop size,householdmeasures, 49735-0121-24 49735-0126-90 49735-0164-56 49735-0106-27 49735-0129-12 Code Reimbursement syndrome (SBS)

B4161 B4161 B4161 B4161 B4161 Code HCPCS information see information For morereimbursement 4 x400g(14.1oz) 4 x400g(14.1oz) 4 x400g(14.1oz) 4 x400g(14.1oz) 4 x400g(14.1oz) Packaging Product 14-18 years 9-13 years 4-8 years 1-3 years & minerals: + 25key vitamins 100% DRIs Meets orexceeds andadministration, Resources Upgraded asof § forprotein 2550 kcal 1565 kcal 1370 kcal late 2018 930 kcal 1804 1840 1888 1888 1888 Per Can Calories

Information: Nutrition Chloride, Potassium, Sodium, Selenium, mg Chromium, mg Molybdenum, mg Iodine, mcg Copper, mcg Manganese, mcg Zinc, mcg Iron, mcg Magnesium, mg Phosphorus, mg Calcium, mg Minerals mg Inositol, mg Choline, mg Vitamin C,mg Biotin, mg Pantothenic Acid,mg mg Folic Acid,mcg Niacin, mcg Vitamin B Vitamin B Riboflavin, mg mg Thiamin, Vitamin K,mcg Vitamin E,IU(mg Vitamin D mg Vitamin A,IU(mcgR.E.) Vitamins PrebioticFiber, g Carbohydrate, g LinoleicAcid,mg Fat, Protein Equivalent,g Calories Nutrients g **Strawberry 51.5

12 6 3 , mg , IU(mcg) , mcg

α -T.E.) 380(9.5) Unflavored Per 100g 3750 956(287) 364 77.1 653 138 239 50.6 530 112 382 80.9 565 120 105 22.2 143 30.3 143 472 100 Vanilla, Strawberry, 19.2 4.1 18.0 3.8 21.5 4.6 85.2 18.1 76.5 16.2 44.6 14.4 3.1 19.2 51.4** 23.0 4.9 14.8 10.0 (6.7) 0.62 0.13 4.7 1.0 7.4 1.6 1.9 4.3 0.91 0.96 0.48 0.10 1.9 1.9 0.40 0.48 0.10 202(60.8) (withprebiotics) Per 100kcal 794 30.3 10.9 80.5 (2.0) 9.4 0.40 0.20 4.1 0.40 3.1 2.1 (1.4)

Chocolate 956(287) 380(9.5) Per 100g 3590 364 653 239 530 382 565 105 143 460 143 19.2 18.0 21.5 85.2 76.5 14.4 22.0 44.6 19.2 51.4 14.2 10.0 (6.7) 0.62 4.7 7.4 4.3 0.48 1.9 0.96 1.9 1.9 0.48 208(62.4) Per 100kcal 142 115 123 100 780 82.6(2.1) 79.1 52.0 18.5 16.6 83.0 22.8 31.1 31.1 11.2 4.2 3.9 4.7 0.13 1.0 1.6 3.1 0.93 0.10 4.8 9.7 0.41 0.21 4.2 0.41 3.1 0.41 0.10 2.2 (1.5) 956(287) 380(9.5) 3420 Per 100g 653 145 530 118 565 125 451 100 364 80.7 239 53.0 382 84.7 105 23.3 143 31.7 143 85.2 18.9 76.5 17.0 19.2 4.3 18.0 4.0 21.5 4.8 14.4 3.2 21.0 4.7 44.6 19.2 51.4 14.2 10.0 (6.7) 0.62 0.14 4.7 1.0 7.4 1.6 4.3 0.95 0.48 0.11 1.9 1.9 0.42 0.96 1.9 0.48 0.11 Tropical 212(63.6) Per 100kcal 758 31.7 11.4 84.3 (2.1) 9.9 0.42 0.21 4.3 0.42 3.1 2.2 (1.5) Acesulfame Potassium. Acesulfame Potassium. Citric Acid, andlessthan 2%of Tropical and lessthan2% ofSucralose. Chocolate Citric Acid. the following:ArtificialFlavor, Sucralose, Strawberry following: ArtificialFlavor, Sucralose. Vanilla Vitamin D Sodium Selenite,Phylloquinone, D-Biotin, Chromium Chloride,SodiumMolybdate, Acetate, Folic Acid,Potassium Iodide, Pyridoxine Hydrochloride, VitaminA Riboflavin, ThiaminChlorideHydrochloride, Manganese Sulfate,Cupric Niacinamide, CalciumD-Pantothenate, Tocopheryl Acetate,Ascorbyl Palmitate, Sulfate, L-Carnitine, ZincSulfate,Dl-Alpha Sunflower Lecithin, Taurine, Ferrous Chloride, M-Inositol,L-Ascorbic Acid, Calcium Chloride,L-Tryptophan, Magnesium L-Methionine, L-Arginine, SodiumChloride, Bitartrate, MagnesiumHydrogen Phosphate, L-Histidine, L-Phenylalanine, Choline Calcium Carbonate,L-Proline, L-Cystine, Tripotassium Citrate, Trisodium Citrate, Phosphate, Maltodextrin, L-Isoleucine, Diglycerides (CITREM), Tricalcium L-Valine, CitricAcidEstersofMono-and Phosphate, Fructooligosaccharides, Inulin, L-Threonine, L-Tyrosine, Dipotassium L-Alanine, L-Leucine, L-Lysine Acetate, of thefollowing:L-Glutamine, Glycine, Oil), L-Serine, andlessthan2%ofeach Sunflower Oil,Canola Kernel and/or CoconutOil),HighOleic Oil (MediumChainTriglycerides (Palm Corn SyrupSolids,Refined Vegetable Ingredients:

also contains less than 2% of the also containslessthan2%ofthe alsocontains: Artificial Flavor, 3 alsocontains:ArtificialFlavors, , Cyanocobalamin. also contains less than 2% of alsocontainslessthan2%of

19 Prebiotics with Junior Neocate 20 GI/ALLERGY • • • • • • • • • • Main Features: Free water:84.6% 790 740 670 Osmolality (mOsm/kg): Fat Carbohydrate Protein Energy distribution: Energy density=1kcal/mL Unflavored Orange-Pineapple, Grape, Tropical Fruit, Available in4great-tastingflavors: Gluten andcaseinfree boxes (for oral and/or tubefeeding) Convenient ready-to-feed8fl oz drink Nutritionally complete 100% non-allergenicfreeaminoacids Hypoallergenic (MCT 35%/LCT 65%) (Orange-Pineapple, Tropical Fruit) (Grape) (Unflavored)

46% 42% 12%

food forindividualsover theageof1. The firstandonlynutritionallycomplete,ready-to-feedaminoacid-basedmedical Neocate

® 84451 -Unflavored 122437 -Tropical Fruit 122435 -Grape 122436 -Orange Pineapple Code Product Ordering/Reimbursement Information: For detailsonadministration, please seetheendofthissection. • • • Indications: Splash office todetermine availability. WIC enterocolitissyndrome(FPIES) Related GIandallergicconditions: Multiple foodallergies Cow milkallergy

• • • • •

Gastroesophageal reflux Malabsorption Food protein-induced Eosinophilic esophagitis(EoE) Short bowelsyndrome(SBS) ‡ :

Contact your stateWIC Code Reimbursement 49735-0144-51 49735-0124-37 49735-0124-35 49735-0124-36

Code HCPCS B4161 orB4153 B4161 orB4153 B4161 orB4153 B4161 orB4153 information see information For morereimbursement 14-18 years 9-13 years 4-8 years 1-3 years & minerals: + 25key vitamins 100% DRIs Meets orexceeds Packaging Product 27 x237mL(8floz) 27 x237mL(8floz) 27 x237mL(8floz) 27 x237mL(8floz) Resources § forprotein 2550 mL 1565 mL 1370 mL 930 mL .

Per Box Calories 237 237 237 237

Nutrition Information: Vitamin B Vitamin B Riboflavin, mg Thiamin, mg Vitamin K,mcg Vitamin E,IU(mg Vitamin D Vitamin A,IU(mcgR.E.) Vitamins Carbohydrate, g (Unflavored) LinoleicAcid,mg Fat, g Protein Equivalent,g Calories Nutrients Inositol, mg Choline, mg Vitamin C,mg Biotin, mcg Pantothenic Acid,mg Folic Acid,mcg Niacin, mg 12 6 3 , mg , IU(mcg) , mcg a 769(691)325(292) T.E.) Per drinkbox 190(4.7) (237 mL) 474(142) 237 52.1 24.9 12.1 70.9 22.0 70.9 0.24 5.0 (3.3) 0.47 0.31 9.5 7.1 7.1 0.95 2.1 0.95 200(60.0) 100 100 mL 10.5 29.9 29.9 80.0 (2.0) 22.0 Per 0.10 2.1 (1.4) 0.20 0.13 4.0 5.1 3.0 9.3 3.0 0.40 0.90 0.40

Chloride, mg Potassium, mg Sodium, mg Selenium, mcg Chromium, mcg Molybdenum, mcg Iodine, mcg Copper, mcg Manganese, mg Zinc, mg Iron, mg Magnesium, mg Phosphorus, mg Calcium, mg Minerals Per drinkbox (237 mL) 180 325 119 237 190 280 10.7 42.2 37.9 9.5 9.0 0.31 2.3 3.6 137 100 118 100 mL 76.0 50.0 80.0 17.8 16.0 Per 4.5 4.0 3.8 0.13 0.98 1.5

Ferrous Lactate. than 2%of:Artificial Flavors, Sucralose, Orange-Pineapple Potassium, Ferrous Lactate. 2% of:ArtificialFlavor, Acesulfame Tropical Fruit Lactate. Artificial Flavors, Sucralose, Ferrous Grape Ferrous Sulfate. Unflavored Vitamin D Molybdate, Phylloquinone, D-Biotin, Folic Acid,Potassium Iodide,Sodium Palmitate, Pyridoxine Hydrochloride, Chloride Hydrochloride, VitaminA Riboflavin, ChromiumChloride,Thiamin Sulfate, SodiumSelenite,Cupric Calcium D-Pantothenate, Manganese Tocopheryl Acetate,Niacinamide, L-Carnitine, ZincSulfate,Dl-Alpha Acid, M-Inositol,L-Cystine, Taurine, L-Tyrosine, CelluloseGum,L-Ascorbic L-Tryptophan, CalciumChloride, Magnesium Acetate,CholineBitartrate, L-Serine, L-Alanine, Potassium Chloride, L-Methionine, L-Threonine, L-Histidine, L-Valine, Glycine, L-Isoleucine, N-Acetyl Diglycerides, Trisodium Citrate, L-Proline, Mono- AndDiglycerides, Mono-And Diacetyl Tartaric AcidEstersOf Tripotassium Citrate, L-Phenylalanine, Microcrystalline Cellulose,L-Leucine, Acid, L-Arginine, L-Lysine L-Aspartate, Calcium Glycerophosphate, Malic Than 2%ofeachthefollowing: Coconut Oil),CanolaAndLess (Modified Palm Kernel And/Or Oil, MediumChainTriglycerides Vegetable Oils(High OleicSunflower Water, Maltodextrin, Sugar, Refined Ingredients: alsocontainslessthan2%of: 3 , Cyanocobalamin. alsocontainslessthan2%of: alsocontainslessthan

alsocontains less 21 Splash Neocate 22 GI/ALLERGY than 24hours. Donotfreeze. Liquids: a refrigerator) and usewithinoneweek. Packets: store inacool,dryplace (notarefrigerator). SyneoInfant). Usewithinonemonth (twoweeksforNeocate Cans: STORAGE: • • • • • • • • DIRECTIONS FORPREPARATION ANDUSE:

Household measuresarenotrecommended formeasuringpowderedNutriciaproducts. up to Tube feedinghangtime: or stirformulaimmediatelybeforeuse. (2-4ºC) andkept nolongerthan post-pyloric feedingtubeunlessdirectedorsupervisedby ahealthcareprofessional. • • condition ofthepatient.Intendedforuseundermedicalsupervision. All scoopsshouldbeunpacked andlevel. Useascalethatmeasuresingrams accurate forthe most results. Discard formulaleftinbottleorcup Once formulaispreparedordrinkbox is opened,formulashouldbestoredinarefrigerator at35-40ºF If taken orally, formulasforbeyond 1year ofagearebestserved chilled. Do notboilformulaoruseamicrowave toprepareorwarmformula. All formulascanbetaken orally ortubefed.Notforparenteral use. Intake andmedical tobedeterminedbyweight ahealthcareprofessionalandisdependentontheage,body NeocateSyneoInfantisnotrecommendedforuseininfantswithacentral venous or catheter NeocateNutra isasemi-solidmedicalfoodandnotforbottleortubefeeding. Store unopenedcansatroomtemperature. Avoid extreme temperatures. resealcanand Onceopened, Storeunopened productinacool, dryplace.Once opened,storeinarefrigerator andkeep nolonger 4 hours Store unopenedpackets atroomtemperature. (not dryplace in acool, Onceopened, resealandstore , atwhichtimeunusedformulashouldbediscarded. Nutriciarecommendsformulacanbesafelykept atroomtemperature for 24 hours 1 hour fromthetimeofpreparation Shake liquids). (timeofopeningfor after feedingstarts.

Shake wellbefore use. NEOCATE SPLASH Do notwarmprepared product.Notsuitableasasolesource ofnutrition. discarded ifnotused withinonehour. Neocate Nutra canbeaddedtocooked notaddtovery dishes.Do hotfoods. Neocate Nutra should bepreparedimmediatelypriorto feeding.Any remaining shouldbe prepared product cooled, boiledwaterbeusedwhenpreparing theproductforinfants. 3. Stir, orcover andshake, untilthepowderhasdissolved. 2. AddtheprescribedamountofNeocateJuniortowater. 1. Measuretherequiredamountofwarmorcoolwaterintoacontainer. To make astandarddilution(30kcal/fl oz),add1unpacked, level fluid ounceofwater. scoopofpowdertoeach NEOCATE JUNIOR the useofsterilizedbottles. (40°C) may lessenthebenefits oftheprobioticcultures. *For NeocateSyneo Infant:Mixingwithwaterwarmerthan104°F(40°C) formulaabove orwarming 104°F Only usethescoopprovided. To make astandarddilution(20cal/floz),add1unpacked level fluidounceofwater. scoopofpowdertoeach NEOCATE SYNEOINFANT ANDNEOCATE INFANT DHA/ARA DIRECTIONS FORPREPARATION ANDUSE: consistency, addmoreorlesswaterasrequired. 3. 2. 1. 3. 2. 1.

Gradually add the required amount of water while stirring withacleanspoon. Mixuntilthickened. Towhile water alterthe of amount required the add Gradually 24 hours.Oncefeedingbegins,usewithin1hourordiscard. Add therecommendednumberofscoops ofNeocateNutra toacleanbowl. Wash handsthoroughlyandcleanthepreparation area. Cap bottle;shake well.Feed immediatelyORrefrigerate immediatelyat35-40°F(2-4°C) usewithin and Add 1unpacked level scoopofpowdertoeach1flozwater.* Wash handsthoroughlyandcleanpreparation area. serving size can be prepared by adding 1 fl oz of water to 4 scoops of powder (18.5 g). It is recommended that serving sizecanbepreparedby adding1flozofwater to4scoopsofpowder(18.5g).Itisrecommendedthat The suggestedservingis8scoops(37g) of powderplus2flozwatertogive aspoonableconsistency. Asmaller NEOCATE NUTRA We waterforpreparation recommendcooled,boiled and Refer toproductlabelsforfullpreparation instructions Not for bottle or tubefeeding Not forbottle .

23 Instructions Mixing GI/Allergy 24 GI/ALLERGY † so arepreferable tohouseholdmeasures. North America recommends using agramscaleforgreatest accuracy. The scoops Nutriciaprovidesare validatedforusewithNutricia formulas, approximations onlyandhavebeenprovided forconvenience:results canvarysignificantly based ontheindividual,device, andmethod.Nutricia Household measuresarebased onstandardUS,dryhouseholdmeasures. Household Measures US measures: 1cup =240mL;½ cup=120mL; ⅓cup=80 mL;¼cup = 60mL;1 1 cup 1/2 cup 1/3 cup 1/4 cup 1 Tbsp 1 tsp 1/2 tsp 1 scoop Neocate Junior, Vanilla, Strawberry, andTropical 1 cup 1/2 cup 1/3 cup 1/4 cup 1 Tbsp 1 tsp 1/2 tsp 1 scoop Neocate Junior, Unflavored (withoutPrebiotics) 1 cup 1/2 cup 1/3 cup 1/4 cup 1 Tbsp 1 tsp 1/2 tsp 1 scoop Neocate SyneoInfant Measure 121 g 120 g 130 g Measure Weight 60.5 g 40.3 g 30.3 g 60.0 g 40.0 g 30.0 g 65.0 g 43.3 g 32.5 g 7.6 g 2.5 g 1.3 g 7.5 g 2.5 g 1.3 g 7.3 g 8.1 g 2.7 g 1.4 g 4.7 g 7.3 g

Neocate MixingTool andHouseholdMeasuresCalculator

Visit NutriciaLearningCenter.com † All measuresareleveland unpacked. These valuesare Neocate Junior, Chocolate 1 cup 1/2 cup 1/3 cup 1/4 cup 1 Tbsp 1 tsp 1/2 tsp 1 scoop 1 cup 1/2 cup 1/3 cup 1/4 cup 1 Tbsp 1 tsp 1/2 tsp 1 scoop 1 cup 1/2 cup 1/3 cup 1/4 cup 1 Tbsp 1 tsp 1/2 tsp 1 scoop Neocate InfantDHA/ARAandNutra Neocate JuniorwithPrebiotics,Unflavored

Tbsp = 15mL;1tsp =5mL;½ tsp =2.5 mL for the *

125 g 129 g 126 g Weight 62.5 g 41.7 g 31.3 g 64.5 g 43.0 g 32.3 g 63 g 42 g 31.5 g 7.8 g 2.6 g 1.3 g 7.5 g 8.1 g 2.7 g 1.3 g 7.7 g 7.9 g 2.6 g 1.3 g 4.6 g *

* Upgraded as of Upgraded asof 2018 Number of cases permonth (30 days) arerounded upto thenearest whole number. Volume Prescription 40 oz 36 oz 32 oz 28 oz 24 oz 20 oz Volume/ Total Vol/can DHA/ARA Neocate Infant Total Vol/can Infant Neocate Syneo Total Servings/can 4 Servings 3 Servings 2 Servings 1 Serving Servings/day 20 floz 24 floz 28 floz 32 floz 36 floz 40 floz day

Neocate Neocate Neocate JuniorUnflavored Syneo Syneo Infant (# cans/month) 10 11 13 6 8 9 20 kcal/fl oz (# cans/month) 97 floz 95 floz

DHA/ARA Neocate Neocate 19 17 16 14 12 10 Infant Infant 10 11 12 6 7 9 30 kcal/fl oz

(# cans/month) Neocate Neocate Syneo Syneo Infant (# cans/month) (# cases/month) 10 11 13 14 7 8 22 kcal/fl oz 4 scoopsNutra (88kcal) 6 5 3 2 (withoutprebiotics) 86 floz 88 floz

Neocate SyneoInfantandDHA/ARA

5 5 4 4 3 3 DHA/ARA Neocate Neocate 21 Servings Infant Infant 10 11 12 14 7 8

Neocate Neocate (# cases/month) Syneo Syneo Infant (# cans/month) 11 12 14 15 8 9 24 kcal/fl oz (# cans/month) 79 floz 81 floz Neocate Nutra Neocate Junior, all otherflavors

Volume/day 2 2 1 1 DHA/ARA Neocate Neocate 20 18 16 14 12 10 Infant Infant 10 12 13 15 7 9 30 kcal/fl oz

Neocate Neocate Syneo Syneo Infant (# cans/month) 10 11 13 15 16 (# cases/month) 8 26 kcal/fl oz 74 floz 73 floz

DHA/ARA Neocate Neocate 5 5 4 4 3 3 Infant Infant 10 11 13 15 16 8

(# cans/month) Neocate Neocate Syneo Syneo Infant (# cans/month) 11 12 14 16 18 9 8 scoopsNutra (175kcal) 28 kcal/fl oz 12 (# boxes/month) 68 floz 69 floz 9 6 3

DHA/ARA Neocate Neocate 152 137 122 107 Infant Infant 10 Servings 92 76 Neocate Splash 10 12 14 16 17 9 30 kcal/fl oz

(# cases/month) Neocate Neocate Syneo Syneo Infant (# cases/month) (# cans/month) 11 13 15 17 19 9 30 kcal/fl oz 63 floz 64 floz

3 2 1 3

6 6 5 4 4 3 DHA/ARA Neocate Neocate Infant Infant 11 13 15 17 19 9

25 Measurements Household GI/Allergy 26 GI/ALLERGY Neocate Chloride, mg Potassium, mg Sodium,mg Selenium,mcg Chromium,mcg Molybdenum,mcg Iodine,mcg Copper, mcg Manganese,mcg Zinc,mg Iron,mg Magnesium,mg Phosphorus,mg Calcium,mg Minerals Inositol,mg Choline,mg VitaminC,mg Biotin,mcg Pantothenic Acid,mcg Folic Acid,mcg Niacin,mcg VitaminB VitaminB Riboflavin, mcg Thiamin,mcg VitaminK,mcg VitaminE,IU VitaminD VitaminA,IU Vitamins LinoleicAcid,mg SolubleFiber, g Carbohydrate, g Fat,g ProteinEquivalent,g Calories Dilution Nutrients per100mL α -Linolenic Acid, mg

12 6 3 , mcg , IU , mcg ® Syneo ® Infant 20 kcal/fl oz 407 688 190 470 49.4 75.8 66.7 55.6 53.1 72.3 26 13.8 56.1 26.7 54.5 77.1 15.2 13.4 75.8 96.8 0.2 2.0 1.5 1.5 0.7 1.0 7.0 7.2 2.7 9.0 6.0 0.9 0.6 7.5 3.2 1.9

Nutrient DilutionChart 22 kcal/fl oz 448 756 209 517 107 54.3 83.4 73.3 61.1 58.4 79.5 28.6 15.1 61.8 29.3 59.9 84.8 16.7 14.7 83.4 0.2 2.2 1.6 1.7 0.8 1.1 7.7 8.0 2.9 9.9 6.6 1.0 0.7 8.3 3.6 2.1 24 kcal/fl oz 488 825 116 228 564 59.3 90.9 80 66.7 10.8 63.7 86.7 31.2 16.5 67.4 32.0 65.3 92.5 18.2 16.1 90.9 0.2 2.4 1.8 1.9 0.9 1.2 8.4 8.7 3.2 7.2 1.1 0.8 9.0 3.9 2.3 26 kcal/fl oz 529 894 126 247 611 100 64.2 98.5 86.7 72.3 11.7 69.0 94.0 33.8 17.9 73.0 34.7 70.8 19.7 17.4 98.5 0.2 2.6 1.9 2.0 0.9 1.3 9.1 9.4 3.5 7.8 1.2 0.8 9.8 4.2 2.5 28 kcal/fl oz 106 570 963 136 266 658 101 108 106 69.2 93.3 77.8 12.6 10.1 10.5 74.3 36.4 19.3 78.6 37.3 76.2 21.2 18.7 1.0 0.3 2.8 2.0 2.2 1.4 9.8 3.7 8.4 1.3 0.9 4.5 2.7 30 kcal/fl oz 1032 114 100 108 116 611 114 145 285 705 74.1 83.4 10.5 10.9 13.5 11.3 79.6 38.9 20.6 84.2 40.0 81.7 22.7 20.1 0.3 1.1 3.0 2.2 2.3 1.5 4.0 9.0 1.4 0.9 4.8 2.8 Neocate Chloride, mg Potassium, mg Sodium,mg Selenium,mcg Chromium,mcg Molybdenum,mcg Iodine,mcg Copper, mcg Manganese,mcg Zinc,mg Iron,mg Magnesium,mg Phosphorus,mg Calcium,mg Minerals Inositol,mg Choline,mg VitaminC,mg Biotin,mcg Pantothenic Acid,mcg Folic Acid,mcg Niacin,mcg VitaminB VitaminB Riboflavin, mcg Thiamin,mcg VitaminK,mcg VitaminE,IU VitaminD VitaminA,IU Vitamins LinoleicAcid,mg Carbohydrate, g Fat,g ProteinEquivalent,g Calories Dilution Nutrients per100mL α -Linolenic Acid, mg

12 6 3 , mcg , IU , mcg ® InfantDHA/ARA 20 kcal/fl oz 400 676 187 492 74.5 48.6 66.7 57.9 53.3 72.5 26.1 13.8 56.6 27.6 54.8 77.4 14.9 13.2 74.5 95.2 0.18 2.0 1.5 1.6 0.73 1.0 7.0 7.1 2.6 8.8 5.9 0.94 7.2 3.4 1.9

Nutrient DilutionChart 22 kcal/fl oz 440 744 105 205 541 82.0 53.4 73.3 63.7 58.6 79.7 28.7 15.2 62.2 30.4 60.3 85.2 16.4 14.5 82.0 0.2 2.2 1.6 1.7 0.8 1.1 7.7 7.8 2.9 9.7 6.5 1.0 7.9 3.7 2.0 24 kcal/fl oz 480 812 114 224 590 89.4 58.3 80.0 69.5 63.9 87.0 31.3 16.6 67.9 33.1 65.8 92.9 17.9 15.8 10.6 89.4 0.22 2.4 1.8 1.9 0.88 1.2 8.4 8.5 3.1 7.0 1.1 8.6 4.1 2.2 26 kcal/fl oz 101 520 879 124 243 640 96.9 63.2 86.7 75.3 69.3 94.2 33.9 17.9 73.6 35.9 71.2 19.4 17.1 11.5 96.9 0.23 2.6 1.9 2.0 0.95 1.3 9.2 9.3 3.4 7.6 1.2 9.3 4.4 2.4 28 kcal/fl oz 104 101 108 560 947 104 133 261 689 68.0 93.3 81.0 74.6 36.5 19.3 79.2 38.6 76.7 20.9 18.4 10.0 12.4 10.0 0.25 2.8 2.0 2.2 1.0 1.4 9.9 3.7 8.2 1.3 4.7 2.6 30 kcal/fl oz 1014

112 100 109 116 600 112 143 280 738

72.9 86.8 79.9 39.1 20.7 84.9 41.4 10.6 82.2 22.4 19.8 10.7 13.3 10.8 0.27 3.0 2.2 2.3 1.1 1.5 3.9 8.8 1.4 5.1 2.8 27 Charts Dilution GI/Allergy 28 GI/ALLERGY Neocate Chloride, mg Potassium, Sodium, Selenium, Chromium, Molybdenum, mcg mg mg Iodine, Copper, mg Manganese, mcg Zinc, mg mcg Iron, Magnesium, mcg mg Phosphorus, mg Calcium, Minerals mg mg Inositol, Choline, Vitamin C,mg mg Biotin, mcg mg Pantothenic Acid,mg mg Folic Acid,mcg Niacin, mg Vitamin B Vitamin B Riboflavin, mg Thiamin, Vitamin K,mcg Vitamin E,IU Vitamin D mg Vitamin A,IU Vitamins LinoleicAcid,mg Fat, Carbohydrate, g Protein Equivalent,g Calories Dilution g Nutrients per100mL

12 6 3 , mg , IU , mcg ® Junior, Unflavored 20 kcal/fl oz 133 545 53.0 66.7 50.7 90.9 33.3 11.9 73.8 10.6 53.2 78.6 14.6 19.9 19.9 0.07 0.26 2.7 2.5 3.0 0.09 0.65 1.0 6.2 2.0 0.26 0.60 0.13 0.07 2.7 1.4 3.3 7.1 2.1 22 kcal/fl oz 100 146 599 58.3 73.3 55.8 36.6 13.0 81.1 11.7 58.5 86.5 16.1 21.9 21.9 0.07 0.29 2.9 2.8 3.3 0.09 0.72 1.1 6.8 2.2 0.29 0.66 0.15 0.07 2.9 1.5 3.7 7.8 2.3 Nutrient DilutionChart 24 kcal/fl oz 109 160 653 63.5 80.0 60.8 40.0 14.2 88.5 12.8 63.8 94.4 17.5 23.9 23.9 0.08 0.32 3.2 3.0 3.6 0.10 0.78 1.2 7.4 2.4 0.32 0.72 0.16 0.08 3.2 1.6 4.0 8.5 2.5 (without prebiotics) 27 kcal/fl oz 123 106 180 735 71.5 90.0 68.4 45.0 16.0 99.6 14.4 71.8 19.7 26.9 26.9 0.09 0.36 3.6 3.4 4.0 0.12 0.88 1.4 8.4 2.7 0.36 0.81 0.18 0.09 3.6 1.8 4.5 9.6 2.8 30 kcal/fl oz 100 120 136 164 111 133 118 142 200 818 79.4 76.1 50.0 59.9 17.8 21.3 16.0 19.2 79.7 21.9 26.3 29.9 35.8 29.9 10.7 0.10 0.12 0.40 0.48 4.0 4.8 3.8 4.5 4.5 0.13 0.16 0.98 1.5 1.9 9.3 3.0 0.40 0.90 0.20 0.10 0.12 4.0 2.0 5.0 6.0 3.1 36 kcal/fl oz 239 980 95.3 91.3 95.7 35.8 12.8 11.2 5.4 1.2 3.6 0.48 1.1 0.24 4.8 2.5 3.7 40 kcal/fl oz 1089 106 133 150 101 106 266 182 204 148 166 157 177 39.8 14.2 66.6 74.9 23.7 26.7 21.3 24.0 29.2 32.9 39.8 44.8 12.4 0.13 0.15 0.53 0.59 6.0 1.3 4.0 0.53 1.2 0.27 5.3 2.7 4.1 5.3 6.0 5.0 5.6 0.17 0.19 2.1 2.3 0.13 0.15 6.7 7.5 45 kcal/fl oz 1225 119 114 120 299 44.8 16.0 14.0 6.7 1.5 4.5 0.59 1.3 0.30 6.0 3.1 4.6 Neocate Chloride, mg Potassium, Sodium, Selenium, Chromium, Molybdenum, mcg mg mg Iodine, Copper, mg Manganese, mcg Zinc, mcg Iron, Magnesium, mcg mg Phosphorus, mg Calcium, mg Minerals mg mg Inositol, Choline, Vitamin C,mg mg Biotin, mcg mg Pantothenic Acid,mg mg Folic Acid,mcg Niacin, mg Vitamin B Vitamin B Riboflavin, mg Thiamin, Vitamin K,mcg Vitamin E,IU Vitamin D mg Vitamin A,IU Vitamins LinoleicAcid,mg Fat, PrebioticFiber, g Carbohydrate, g Protein Equivalent,g Calories g Dilution Nutrients per100mL

12 6 3 , mg , IU , mcg ® JuniorwithPrebiotics,Unflavored 20 kcal/fl oz 135 530 53.7 66.7 51.4 92.2 33.8 12.0 74.9 10.8 54.0 79.8 14.8 20.2 20.2 0.07 0.27 2.7 2.5 3.0 0.09 0.66 1.0 6.3 2.0 0.27 0.61 0.14 0.07 2.7 1.4 3.2 0.27 7.3 2.1 22 kcal/fl oz 101 148 583 59.0 73.3 56.6 37.1 13.2 82.3 11.9 59.4 87.8 16.3 22.2 22.2 0.07 0.30 3.0 2.8 3.3 0.10 0.73 1.1 6.9 2.2 0.30 0.67 0.15 0.07 3.0 1.6 3.6 0.30 8.0 2.3 Nutrient DilutionChart 24 kcal/fl oz 111 162 636 64.4 80.0 61.7 40.5 14.4 89.8 13.0 64.7 95.8 17.8 24.2 24.2 0.08 0.32 3.3 3.1 3.6 0.11 0.80 1.3 7.6 2.4 0.32 0.73 0.16 0.08 3.3 1.7 3.9 0.32 8.7 2.5 27 kcal/fl oz 125 101 108 182 715 72.5 90.0 69.4 45.6 16.2 14.6 72.8 20.0 27.3 27.3 0.09 0.36 3.7 3.4 4.1 0.12 0.90 1.4 8.5 2.7 0.36 0.82 0.18 0.09 3.7 1.9 4.4 0.36 9.8 2.8 30 kcal/fl oz 100 120 138 166 112 135 120 144 202 794 80.5 77.1 50.6 60.8 18.1 21.7 16.2 19.4 80.9 22.2 26.7 30.3 36.4 30.3 10.9 0.10 0.12 0.40 0.48 4.1 4.9 3.8 4.6 4.6 0.13 0.16 1.0 1.2 1.6 1.9 9.4 3.1 0.40 0.91 0.20 0.10 0.12 4.1 2.1 4.9 5.8 0.40 3.1 36 kcal/fl oz 243 953 96.6 92.5 97.1 11.3 36.4 13.1 5.5 3.7 0.48 1.1 0.24 4.9 2.5 0.48 3.8 40 kcal/fl oz 1059 107 133 150 184 208 150 168 160 180 103 108 270 67.5 76.0 24.1 27.1 21.6 24.3 29.7 33.4 40.4 45.4 12.6 40.4 14.5 0.14 0.15 0.54 0.60 5.4 6.1 5.1 5.7 0.18 0.20 1.3 1.5 2.1 2.4 0.14 0.15 6.5 7.3 6.1 4.1 0.54 1.2 0.27 5.4 2.8 0.54 4.2 45 kcal/fl oz 1192

121 116 121 304

14.2 45.4 16.3 6.8 4.6 0.60 1.4 0.31 6.1 3.2 0.60 4.7 29 Charts Dilution GI/Allergy 30 GI/ALLERGY ~0.71 mL water. scale provides greatestaccuracy. 1level,unpackedscoop yields4.7gramspowder andprovides22.33kcal. 1gramprovides4.75kcal anddisplaces † * This chartassumes1floz=30mL. Water volumesareexact,but final volumeofpreparedformulamayvaryslightly. 33.0 29.0 25.0 21.0 17.0 14.5 11.0 8.0 5.5 4.5 fl oz 32.0 28.0 24.0 20.0 18.0 16.0 11.0 8.0 5.0 3.0 fl oz Neocate Scoops shouldbeleveland unpacked.WhilethescoopsNutriciaprovidesarevalidated forusewithNutriciaformul Volume of Volume of Water* Water* 20 kcal/fl oz(Standarddilution) 990 870 750 630 510 435 330 240 165 135 960 840 720 600 540 480 330 240 150 mL mL 90 Neocate PowderNeocate PowderNeocate scoops scoops 22 kcal/fl oz 37 32 28 23 19 16 12 32 28 24 20 18 16 11 Amount of Amount of 9 6 5 8 5 3 ® Syneo 173.9 150.4 131.6 108.1 150.4 131.6 112.8 grams grams 89.3 75.2 56.4 42.3 28.2 23.5 94.0 84.6 75.2 51.7 37.6 23.5 14.1 † †

37.1 1115 32.6 975 28.1 845 23.6 705 19.1 575 16.3 490 12.3 370 35.6 1065 31.1 935 26.7 800 22.2 665 20.0 600 17.8 535 12.2 365 fl oz fl oz Final Volume*Final Final Volume*Final Approximate Approximate 9.0 270 6.2 185 5.1 150 9.0 270 5.5 165 3.3 100 ® Infant mL mL

32.0 28.0 24.0 21.0 17.0 14.0 9.0 6.0 3.0 1.5 fl oz 30.0 28.0 24.0 21.0 18.0 14.0 11.5 10.5 9.0 6.5 fl oz Volume of Volume of Water* Water* 960 840 720 630 510 420 270 180 900 840 720 630 540 420 345 315 270 195 Dilution Charts mL mL 90 45 Neocate PowderNeocate PowderNeocate scoops scoops 26 kcal/fl oz 24 kcal/fl oz 43 37 32 28 23 19 12 37 34 29 26 22 17 14 13 11 Amount of Amount of 8 8 4 2 202.1 173.9 150.4 131.6 108.1 173.9 159.8 136.3 122.2 103.4 grams grams 89.3 56.4 37.6 79.9 65.8 61.1 51.7 37.6 18.8 9.4 † †

Final Volume*Final Volume*Final Approximate Approximate 36.8 1105 32.1 965 27.6 825 24.1 725 19.6 585 16.1 485 10.3 310 34.1 1025 31.8 955 27.2 815 23.9 715 20.4 615 15.9 475 13.1 390 11.9 360 10.2 305 fl oz fl oz 6.9 205 7.4 220 3.4 105 1.7 50 mL mL

32.0 28.0 24.0 20.0 17.0 14.0 11.5 9.5 5.0 2.5 fl oz 32.0 27.0 24.0 20.0 17.0 13.0 11.0 9.0 7.5 5.5 fl oz Volume of Volume of Water* Water* 960 840 720 600 510 420 345 285 150 960 810 720 600 510 390 330 270 225 165 mL mL 75 as, weighingpowderusing agram Neocate PowderNeocate PowderNeocate scoops scoops 30 kcal/fl oz 28 kcal/fl oz 51 44 38 32 27 22 18 15 47 39 35 29 25 19 16 13 11 Amount of Amount of 8 8 4 239.7 206.8 178.6 150.4 126.9 103.4 221 183 165 136 118 grams grams 84.6 70.5 37.6 89.3 75.2 61.1 51.7 37.6 18.8 † †

Final Volume*Final Volume*Final Approximate Approximate 37.7 1130 32.9 985 28.2 845 23.6 705 20.0 600 16.4 495 13.5 405 11.2 335 37.2 1115 31.3 940 27.9 835 23.2 695 19.8 595 15.1 455 12.8 385 10.4 315 fl oz fl oz 5.9 175 8.7 260 6.4 190 2.9 90 mL mL

~0.70 mL water. scale provides greatestaccuracy. 1level,unpackedscoop yields4.6gramspowder andprovides22.22kcal. 1gramprovides4.83kcal anddisplaces † * This chartassumes1floz=30mL. Water volumesareexact,but final volumeofpreparedformulamayvaryslightly. 31.0 29.0 25.0 22.0 18.0 15.0 11.0 7.0 5.0 3.0 fl oz Neocate 32.5 29.0 25.5 22.0 18.0 14.5 11.0 8.0 5.5 4.5 fl oz Scoops shouldbeleveland unpacked.WhilethescoopsNutriciaprovidesarevalidated forusewithNutriciaformul Volume of Volume of Water* Water* 20 kcal/fl oz(Standarddilution) 930 870 750 660 540 450 330 210 150 975 870 765 660 540 435 330 240 165 135 mL mL 90 Neocate PowderNeocate PowderNeocate scoops scoops 22 kcal/fl oz 31 29 25 22 18 15 11 36 32 28 24 20 16 12 Amount of Amount of 3 7 5 9 6 5 ® InfantDHA/ARA 142.6 133.4 115.0 101.2 165.6 147.2 128.8 110.4 grams grams 82.8 69.0 50.6 13.8 32.2 23.0 92.0 73.6 55.2 41.4 27.6 23.0 † †

34.3 1030 32.1 963 27.7 831 24.4 731 19.9 598 16.6 498 12.2 365 36.4 1091 32.4 973 28.5 855 24.6 737 20.1 604 16.2 487 12.3 369 fl oz fl oz Final Volume*Final Final Volume*Final Approximate Approximate 3.3 100 7.8 233 5.5 166 9.0 269 6.1 184 5.0 151 mL mL

30.0 27.5 24.5 21.0 18.0 14.0 10.5 7.5 4.5 3.0 fl oz 32.0 28.5 24.5 21.5 18.0 14.0 10.5 6.5 5.0 2.5 fl oz Volume of Volume of Water* Water* 900 825 735 630 540 420 315 225 135 960 855 735 645 540 420 315 195 150 Dilution Charts mL mL 90 75 Neocate PowderNeocate PowderNeocate scoops scoops 26 kcal/fl oz 24 kcal/fl oz 40 37 33 28 24 19 14 10 39 35 30 26 22 17 13 Amount of Amount of 4 3 6 8 6 184.0 170.2 151.8 128.8 110.4 179.4 161 138 119.6 101.2 grams grams 18.4 13.8 87.4 64.4 46.0 27.6 78.2 59.8 36.8 27.6 † †

Final Volume*Final Volume*Final Approximate Approximate 34.3 1029 31.5 944 28.0 841 24.0 720 20.6 617 16.0 481 12.0 360 36.2 1086 32.3 968 27.7 832 24.3 729 20.4 611 15.8 475 11.9 357 fl oz fl oz 3.4 103 2.8 85 8.6 257 5.1 154 7.4 221 5.6 169 mL mL

31.0 28.0 24.0 20.5 17.0 14.0 10.0 7.0 5.0 2.5 fl oz 31.5 28.0 24.0 20.5 17.0 14.5 11.0 9.0 7.5 5.5 fl oz Volume of Volume of Water* Water* 930 840 720 615 510 420 300 210 150 945 840 720 615 510 435 330 270 225 165 mL mL 75 as, weighingpowderusing agram Neocate PowderNeocate PowderNeocate scoops scoops 30 kcal/fl oz 28 kcal/fl oz 49 44 38 32 27 22 16 11 46 41 35 30 25 21 16 13 11 Amount of Amount of 8 4 8 225.4 202.4 174.8 147.2 124.2 101.2 211.6 188.6 161.0 138.0 115.0 grams grams 73.6 50.6 36.8 18.4 96.6 73.6 59.8 50.6 36.8 † †

Final Volume*Final Volume*Final Approximate Approximate 36.3 1088 32.7 982 28.1 842 23.9 718 19.9 597 16.4 491 11.7 352 36.4 1093 32.4 972 27.8 833 23.7 712 19.7 591 16.8 503 12.7 382 10.4 312 fl oz fl oz 8.2 245 5.9 176 2.9 88 8.7 260 6.4 191

mL mL

31 Charts Dilution GI/Allergy 32 GI/ALLERGY (without prebiotics) Neocate 28 20 10 8 4.5 Water Volume* 28 22 14 9 6.5 Water Volume* 30 21 14 10 7 Water Volume* 30 22 16 11 8 Water Volume* Visit www.NutriciaLearningCenter forprintable dilution charts and theNeocate Mixing Tool. 4.79 kcal anddisplaces ~0.69mL water. using a gramscaleprovides greatest accuracy. 1level,unpacked scoopyields7.3grams powderandprovides35.0 kcal.1gramprovides † *This chartassumes 1floz=30mL. Water volumesare exact,butfinalvolumeof preparedformulamayvary slightly. Scoops should belevelandunpacked.While thescoopsNutriciaprovides arevalidatedforuse withNutriciaformul fl oz fl oz fl oz fl oz 840 600 300 240 135 840 660 420 270 195 900 630 420 300 210 900 660 480 330 240 mL mL mL mL ® Junior, Unflavored NeocatePowder NeocatePowder NeocatePowder NeocatePowder scoops scoops scoops scoops 27 kcal/fl oz 24 kcal/fl oz 22 kcal/fl oz 20 kcal/fl oz 25 18 22 17 11 21 15 10 19 14 10 9 7 4 7 5 7 5 7 5

grams grams grams grams 183 131 161 124 153 110 139 102 65.7 51.1 29.2 80.3 51.1 36.5 73.0 51.1 36.5 73.0 51.1 36.5 † † † †

FinalVolume* FinalVolume* FinalVolume* FinalVolume* 32.2 23.0 11.5 31.7 24.9 15.8 10.2 33.5 23.5 15.7 11.2 33.2 24.4 17.7 12.2 fl oz fl oz fl oz fl oz 9.2 5.2 7.3 7.8 8.8 Dilution Charts 1005 965 690 345 275 155 950 745 475 305 220 705 470 335 235 995 730 530 365 265 mL mL mL mL

Water Volume* 25 19 11 8.5 5.5 Water Volume* 26 19 12 7 3.5 Water Volume* 26 20 10.5 6.5 4 Water Volume* 28 21 10 7 4 fl oz fl oz fl oz fl oz

750 570 330 255 165 780 570 360 210 105 780 600 315 195 120 840 630 300 210 120 30 kcal/fl oz(Standarddilution) mL mL mL mL NeocatePowder NeocatePowder NeocatePowder NeocatePowder

scoops scoops scoops scoops 45 kcal/fl oz 40 kcal/fl oz 36 kcal/fl oz 41 31 18 14 37 27 17 10 32 25 13 28 21 10 9 5 8 5 7 4

grams grams grams grams 299 226 131 102 270 197 124 234 183 204 153 65.7 73.0 36.5 94.9 58.4 36.5 73.0 51.1 29.2 Upgraded asof2018

† † † †

as, weighing powder FinalVolume* FinalVolume* FinalVolume* FinalVolume* 31.9 24.2 14.0 10.9 32.2 23.5 14.9 31.4 24.2 12.7 32.7 24.5 11.7 fl oz fl oz fl oz fl oz 7.0 8.7 4.3 7.8 4.8 8.2 4.7

210 260 130 235 145 245 140 955 725 420 325 965 705 445 940 725 380 980 735 350 mL mL mL mL Visit www.NutriciaLearningCenter for printabledilution charts andtheNeocate Mixing Tool. 4.72 kcal anddisplaces ~0.74mL water. using agram scale providesgreatestaccuracy. 1level,unpacked scoopyields7.7grams powderandprovides36.3 kcal.1gramprovides † *This chartassumes 1floz=30mL. Water volumesare exact,butfinalvolumeof preparedformulamayvary slightly. Scoops should belevelandunpacked.While thescoopsNutriciaprovides arevalidatedforuse withNutriciaformul Neocate 28 21 10.5 7 3.5 28 21 10.5 8 4 29 23 19 16 11.5 29 21 13 8 6.5 Water Volume* Water Volume* Water Volume* Water Volume* fl oz fl oz fl oz fl oz 840 630 315 210 105 840 630 315 240 120 870 690 570 480 345 870 630 390 240 195 ® mL mL mL mL JuniorwithPrebiotics,Unflavored scoops scoops scoops scoops Neocate Powder Neocate Powder Neocate Powder Neocate Powder 27 kcal/fl oz 24 kcal/fl oz 22 kcal/fl oz 20 kcal/fl oz 24 18 21 16 20 16 13 11 18 13 9 6 3 8 6 3 8 8 5 4

185 139 162 123 154 123 100 139 100 grams grams grams grams 84.7 69.3 46.2 23.1 61.6 46.2 23.1 61.6 61.6 38.5 30.8 † † † †

fl oz fl oz fl oz fl oz 32.6 24.4 32.0 24.0 32.8 26.0 21.5 18.1 32.4 23.5 12.2 12.0 13.0 14.5 Final Volume* Final Volume* Final Volume* Final Volume* 8.1 4.1 9.1 4.6 9.0 7.3 Dilution Charts

975 735 960 720 985 780 645 545 975 705 365 245 120 360 275 135 390 435 270 220 mL mL mL mL 24 19 13 10.5 8 25 20 15 10 5 26 19 13 9 6.5 28 21 10 7 4

Water Volume* Water Volume* Water Volume* Water Volume* fl oz fl oz fl oz fl oz 30 kcal/fl oz(Standarddilution) 720 570 390 315 240 750 600 450 300 150 780 570 390 270 195 840 630 300 210 120 mL mL mL mL

scoops scoops scoops scoops Neocate Powder Neocate Powder Neocate Powder Neocate Powder 45 kcal/fl oz 40 kcal/fl oz 36 kcal/fl oz 39 31 21 17 13 35 28 21 14 32 23 16 11 27 21 10 7 8 7 4

as, weighing powder Upgraded asoflate2018 300 239 162 131 100 270 216 162 108 246 177 123 208 162 grams grams grams grams 53.9 84.7 61.6 77.0 53.9 30.8 † † † †

fl oz fl oz fl oz fl oz 31.4 24.9 17.0 13.7 10.5 31.6 25.3 19.0 12.7 32.1 23.4 16.0 11.1 33.1 24.9 11.9 Final Volume* Final Volume* Final Volume* Final Volume* 6.3 8.0 8.3 4.7

940 745 510 410 315 950 760 570 380 960 700 480 335 995 750 355 190 240 250 145 mL mL mL mL 33 Charts Dilution GI/Allergy 34 GI/ALLERGY Neocate Visit www.NutriciaLearningCenter for printabledilution charts and theNeocate Mixing Tool. displaces gram scaleprovides greatestaccuracy. 1level,unpacked scoopyields7.3grams powderandprovides34.5 kcal.1gramprovides4.72 kcaland † *This chartassumes 1floz=30mL. Water volumesare exact,butfinalvolumeof preparedformulamayvary slightly. Scoops should belevelandunpacked.While thescoopsNutriciaprovides arevalidatedforuse withNutriciaformul 5 Water Volume* 29 22 14 8.5 5.5 Water Volume* 30 22 17 14 11 28 21 19 10 5.5 28 20 10 7.5 Water Volume* Water Volume* fl oz fl oz fl oz fl oz ~0.70 150 870 660 420 255 165 900 660 510 420 330 840 630 570 300 165 840 600 300 225 mL ® mL mL mL mL Junior, Vanilla &Strawberry water. Neocate Powder scoops scoops scoops scoops Neocate Powder Neocate Powder Neocate Powder 27 kcal/fl oz 24 kcal/fl oz 22 kcal/fl oz 20 kcal/fl oz 21 16 10 19 14 11 25 19 17 22 16 4 6 4 9 7 9 5 8 6

153 117 139 102 183 139 124 161 117 grams grams grams grams 29.2 73 43.8 29.2 80.3 65.7 51.1 65.7 36.5 58.4 43.8 †

† † †

Final Volume* fl oz fl oz fl oz fl oz 32.6 24.7 15.7 33.2 24.4 18.9 15.5 12.2 32.2 24.2 21.9 11.5 31.7 22.7 11.4 Final Volume* Final Volume* Final Volume* 5.7 9.5 6.2 6.4 8.5 Dilution Charts 170 975 740 470 285 185 995 730 565 465 365 970 725 655 345 190 950 680 340 255 mL mL mL mL

25 19 12 6 3 26 20 13 9 5.5 Water Volume* 27 22 15 9.5 5.5 Water Volume* 28 21 16 7 4 Water Volume* Water Volume* fl oz fl oz fl oz fl oz 30 kcal/fl oz(Standarddilution) 750 570 360 180 780 600 390 270 165 810 660 450 285 165 840 630 480 210 120 mL mL mL mL 90 Neocate Powder scoops scoops scoops scoops Neocate Powder Neocate Powder Neocate Powder 45 kcal/fl oz 40 kcal/fl oz 36 kcal/fl oz 42 32 20 10 38 29 19 13 34 28 19 12 29 21 16 8 7 7 4 5

Upgraded asoflate2018 307 234 146 277 212 139 248 204 139 212 153 117 grams grams grams grams 73 94.9 58.4 87.6 51.1 51.1 29.2 36.5 †

as, weighing powder usinga † † †

Final Volume* fl oz fl oz fl oz fl oz 32.2 24.5 15.4 32.4 24.9 16.2 11.2 32.8 26.8 18.2 11.5 32.9 24.6 18.7 Final Volume* Final Volume* Final Volume* 7.7 6.9 6.7 8.2 4.7 3.9

230 205 200 245 140 965 735 460 975 750 485 335 985 805 545 345 990 735 560 115 mL mL mL mL Visit www.NutriciaLearningCenter for printabledilution charts and theNeocate Mixing Tool. 4.60 kcal anddisplaces ~0.70mL water. using agram scale providesgreatestaccuracy. 1level,unpacked scoopyields7.5grams powderandprovides34.5 kcal.1gramprovides † *This chartassumes 1floz=30mL. Water volumesare exact,butfinalvolumeof preparedformulamayvary slightly. Scoops should belevelandunpacked.While thescoopsNutriciaprovides arevalidatedforuse withNutriciaformul Neocate 29 21 15.5 10 5.5 28 20 15 7.5 5 29 21 14 8.5 5.5 29 20 17 14 11 Water Volume* Water Volume* Water Volume* Water Volume* fl oz fl oz fl oz fl oz 870 630 465 300 165 840 600 450 225 150 870 630 420 255 165 870 600 510 420 330 ® mL mL mL mL Junior, Chocolate scoops scoops scoops scoops Neocate Powder Neocate Powder Neocate Powder Neocate Powder 27 kcal/fl oz 24 kcal/fl oz 22 kcal/fl oz 20 kcal/fl oz 26 19 14 22 16 12 21 15 10 19 13 11 9 5 6 4 6 4 9 7

195 143 105 165 120 158 113 143 grams grams grams grams 90.0 75.0 97.5 82.5 67.5 37.5 45.0 30.0 45.0 30.0 67.5 52.5 † † † †

fl oz fl oz fl oz fl oz 33.6 24.3 18.0 31.9 22.8 17.1 32.7 23.6 15.8 32.3 22.3 18.9 11.6 15.6 12.2 Final Volume* Final Volume* Final Volume* Final Volume* 6.4 8.6 5.7 9.6 6.2 Dilution Charts

1005 730 540 955 685 515 980 710 475 970 670 570 345 190 255 170 285 185 465 365 mL mL mL mL 25 20 16 9.5 6.5 26 20 13 7.5 5.5 28 21 11 7 5.5 28 21 10 7 4

Water Volume* Water Volume* Water Volume* Water Volume* fl oz fl oz fl oz fl oz 30 kcal/fl oz(Standarddilution) 750 600 480 285 195 780 600 390 225 165 840 630 330 210 165 840 630 300 210 120 mL mL mL mL

scoops scoops scoops scoops Neocate Powder Neocate Powder Neocate Powder Neocate Powder 45 kcal/fl oz 40 kcal/fl oz 36 kcal/fl oz 42 34 27 16 11 38 29 19 11 36 27 14 29 21 10 8 9 7 7 4

as, weighing powder Upgraded asoflate2018 315 255 203 120 285 218 143 270 203 105 218 158 grams grams grams grams 82.5 82.5 60.0 67.5 52.5 75.0 52.5 30.0 † † † †

fl oz fl oz fl oz fl oz 32.4 26.0 20.7 12.3 32.7 25.1 16.3 34.3 25.7 13.5 33.1 24.9 11.8 Final Volume* Final Volume* Final Volume* Final Volume* 8.4 9.4 6.9 8.6 6.7 8.3 4.7

1030 970 780 620 370 980 750 490 770 405 990 740 355 255 285 205 255 200 245 140 mL mL mL mL 35 Charts Dilution GI/Allergy 36 GI/ALLERGY Neocate Visit www.NutriciaLearningCenter for printabledilution charts and theNeocate Mixing Tool. displaces gram scaleprovides greatestaccuracy. 1level,unpacked scoopyields7.3grams powderandprovides32.9 kcal.1gramprovides4.51 kcaland † *This chartassumes 1floz=30mL. Water volumesare exact,butfinalvolumeof preparedformulamayvary slightly. Scoops should belevelandunpacked.While thescoopsNutriciaprovides arevalidatedforuse withNutriciaformul 6 Water Volume* 28 21 12 8 4 Water Volume* 30 22 12 7.5 4.5 32 21 16 10.5 8.5 28 23 12 8.5 Water Volume* Water Volume* fl oz fl oz fl oz fl oz ~0.70 180 840 630 360 240 120 900 660 360 225 135 960 630 480 315 255 840 690 360 255 mL ® mL mL mL mL Junior, Tropical water. Neocate Powder scoops scoops scoops scoops Neocate Powder Neocate Powder Neocate Powder 27 kcal/fl oz 24 kcal/fl oz 22 kcal/fl oz 20 kcal/fl oz 21 16 20 15 30 20 15 10 23 19 10 5 9 6 3 8 5 3 8 7

153 117 146 110 219 146 110 168 139 grams grams grams grams 36.5 65.7 43.8 21.9 58.4 36.5 21.9 73.0 58.4 73.0 51.1 †

† † †

Final Volume* fl oz fl oz fl oz fl oz 31.6 23.7 13.5 33.4 24.5 13.4 37.1 24.4 18.5 12.2 31.9 26.2 13.7 Final Volume* Final Volume* Final Volume* 6.8 9.0 4.5 8.3 5.0 9.9 9.7 Dilution Charts 1000 1110 205 945 710 405 270 135 735 400 250 150 730 555 365 295 955 785 410 290 mL mL mL mL

26 18 13 8.5 4.5 26 19 13 10.5 6.5 Water Volume* 26 20 12 6 3 Water Volume* 29 21 11 7 4 Water Volume* Water Volume* fl oz fl oz fl oz fl oz 30 kcal/fl oz(Standarddilution) 780 540 390 255 135 780 570 390 315 195 780 600 360 180 870 630 330 210 120 mL mL mL mL 90 Neocate Powder scoops scoops scoops scoops Neocate Powder Neocate Powder Neocate Powder 45 kcal/fl oz 40 kcal/fl oz 36 kcal/fl oz 46 32 23 15 40 29 20 16 10 35 27 16 31 21 11 8 8 7 4 4

Upgraded asoflate2018 336 234 168 110 292 212 146 117 256 197 117 226 153 grams grams grams grams 58.4 73.0 58.4 80.3 51.1 29.2 29.2 †

as, weighing powder usinga † † †

Final Volume* fl oz fl oz fl oz fl oz 33.8 23.4 16.9 11.0 32.8 23.9 16.4 13.2 31.9 24.6 14.7 34.2 24.6 12.9 Final Volume* Final Volume* Final Volume* 5.9 8.2 7.4 8.2 4.7 3.7

1015 1025 175 245 220 245 140 700 505 330 985 715 490 395 960 735 440 735 385 110 mL mL mL mL and displaces ~0.69 mL water. Scoops shouldbelevel andunpacked. 1level, unpackedscoop yields7.3 gramspowder andprovides 35.0kcal. 1gramprovides 4.79kcal †Nutricia North America recommends usingascaleforgreatest accuracy. Dilutionsare moreaccurateusingweighed powdervs.scoops. call foraneven numberofNeocateSplash drinkboxesandthose that callforaddingwater *This chartassumes 1floz=~29.6mL. 1drinkbox=8floz237 mL Neocate

4 drinkboxes 2 drinkboxes 4 drinkboxes 3 drinkboxes 2 drinkboxes 4 drinkboxes 2 drinkboxes Neocate SplashVolume* Neocate SplashVolume* Neocate SplashVolume* 1 drinkbox fl oz fl oz fl oz 3.5 5.5 19 21 11 7 5 Add NeocateJunior, Unflavored ® Splash Concentration Charts– 948 570 1180.3 474 210 105 948 711 474 237 165 948 630 474 330 150 17.3 mL mL mL Neocate JuniorPowder Neocate JuniorPowder Neocate JuniorPowder 45 kcal/fl oz 40 kcal/fl oz 35 kcal/fl oz scoops scoops scoops 18 12 + 9 4 2 9 6 3 2 6 4 3 2

131 grams grams grams 87.6 65.7 29.2 14.6 65.7 43.8 21.9 14.6 43.8 29.2 21.9 14.6 † † †

Approx. FinalVolume* Approx. FinalVolume* Approx. FinalVolume* 34.1 35.1 21.2 17.6 25.6 17.1 33.1 22.0 650 16.5 11.5 340 fl oz fl oz fl oz 5.2 7.8 230 3.9 115 8.5 5.9 175 1008 1039 625 155 519 756 504 252 978 489 mL mL mL andstandard dilutionis30kcal/floz,or 1kcal/mL.Inrecipesthat don’t

Neocate Splash Neocate Splash Neocate Splash Neocate Splash Drink boxes Drink boxes Drink boxes Drink boxes 6 5 4 3 2 5 4 3 2 1 6 5 4 3 2 5 4 3 2 1 , mL volumesareroundedtothe nearest15mL. Dilution Charts–AddWater Volume ofwater Volume ofwater Volume ofwater Volume ofwater 17 480 14 405 11 330 19 570 15 450 12 330 fl oz fl oz fl oz fl oz 5 150 4 120 3 2.5 75 1.5 45 9 270 7 210 6 180 4 120 2 60 8.5 240 5.5 165 8 240 4 120 27 kcal/fl oz 24 kcal/fl oz 22 kcal/fl oz 20 kcal/fl oz 90

mL mL mL mL Approx. FinalVolume* Approx. FinalVolume* Approx. FinalVolume* Approx. FinalVolume* 53 44 35 1038 26.5 17.5 49 39 30 20 10 65 54 43 32.5 21.5 59 47 36 24 12 fl oz fl oz fl oz fl oz

1572 1305 1455 1158 1902 1590 1278 1755 1398 1041

786 519 891 594 297 951 639 714 357 mL mL mL mL 37 Charts Dilution GI/Allergy 38 GI/ALLERGY allergies, andrelated dietisindicated. GIandallergicconditions forwhichanaminoacid-based management ofcowmilkallergy, fordietary ** Neocateisanaminoacid-basedformula multiplefoodprotein systemic conditions,ofteninconjunctionwithtubefeedingand/ hydrocephalus); respiratory (lungdisease,tracheostomy, aspiration); cardiac (congenitaldisease); heart andother esophageal atresia, tracheoesophageal fistula);neurological conditions(suchasseizures, intraventricular hemorrhage, multiple systems,includingcongenitalgastrointestinal anomaliesandGIsurgeries (suchasnecrotizing enterocolitis, * Aretrospective review identifiedcasestudiesof patientsonprimaryorsolesource covering diagnoses nutritionwith prematurity. Neocate** formulasare intendedforuseunder medicalsupervision. multiple diagnosesandintestinaldisease,* withtubefeedingand/or especiallyincombination ahistoryof Neocate isusedasaprimaryorsolesource complex ofnutritionforpatientswith systemicdiseaseinvolving Serum levels ofmicronutrients, shouldberoutinely phosphorus, particularly monitored byclinicianswhen Update onMonitoringMicronutrients or ahistoryofprematurity. METABOLICS

Sarah Diagnosed with PKU

Find more information and resources on these and all of our products at www.NutriciaLearningCenter.com 3939 PKU Periflex Early Years

Nutrition Information: Ingredients: Per 10 g Per 10 g Corn Syrup Solids, Refined Vegetable ® Nutrients Per 100 g Protein Equivalent Vitamins Per 100 g Protein Equivalent Oils (High Oleic Sunflower, Soy, PKU Periflex Early Years Calories 473 350 Vitamin A, IU (mcg RE) 1305.(392) 967.(290) Non-hydrogenated Coconut, Canola, Protein Equivalent,g 13.5 10.0 Vitamin D3, IU (mcg) 348.(8.7) 258.(6.4) Sunflower), Galacto-oligosaccharides A phenylalanine-free powdered infant formula with iron, for the dietary Fat, g 23.0 17.0 Vitamin E, IU (mg α-TE) 10.9 (7.3) 8.1 (5.4) (GOS) Dried Syrup (from milk), L-Arginine management of phenylketonuria (PKU). Contains DHA and ARA, and a unique Saturated, g 7.5 5.6 Vitamin K, mcg 38.0 28.1 L-Aspartate, L-Leucine, L-Lysine Acetate, blend of prebiotic fiber. Monounsaturated, g 9.4 7.0 Thiamin, mcg 640 474 L-Tyrosine, L-Glutamine, L-Proline, Polyunsaturated, g 5.0 3.7 Riboflavin, mcg 500 370 Tricalcium Phosphate, L-Valine, CAEM DHA, mg 70.0 51.9 Vitamin B6, mcg 500 370 (an emulsifier), Glycine, L-Isoleucine, ARA, mg 140 104 Vitamin B12, mcg 1.2 0.89 L-Threonine, L-Serine, L-Histidine, Main Features: Indications: Linoleic Acid,mg 3500 2593 Niacin, mcg 2200 1630 L-Alanine, Potassium Chloride, Fructo- Carbohydrate, g 53.0 39.3 Folic Acid, mcg 55.0 40.7 oligosaccharides (FOS), Tripotassium • Phenylalanine-free powdered infant • Phenylketonuria Fiber, g 5.3 3.9 Pantothenic Acid, mcg 2800 2074 Citrate, Sodium Phosphate Dibasic, GOS, g 4.7 3.5 Biotin, mcg 18.2 13.5 L-Cystine, M. Alpina Oil*, L-Tryptophan, formula with iron FOS, g 0.55 0.41 For details on administration and household measurements, please Vitamin C, mg 49.0 36.3 Choline Bitartrate, L-Methionine, • Includes DHA & ARA Amino Acids, g Choline, mg 91.0 67.4 Magnesium Acetate, Magnesium see the end of this section. L-Alanine 0.62 0.46 • Contains a prebiotic fiber blend Inositol, mg 98.0 72.6 Aspartate, C. Cohnii Oil**, Sodium Chloride, L-Arginine 1.09 0.81 Minerals M-Inositol, L-Ascorbic Acid, Ferrous • Unflavored powder L-Aspartic Acid 1.02 0.76 Calcium, mg 371 275 Sulfate, Taurine, Zinc Sulfate, L-Carnitine, Ordering/Reimbursement Information: L-Cystine 0.41 0.30 • Contains a balanced mixture of all other Phosphorus, mg 270 200 DL-alpha Tocopheryl Acetate, Calcium Product Reimbursement HCPCS Product Calories L-Glutamine 1.28 0.95 Magnesium, mg 52.5 38.9 D-Pantothenate, Niacinamide, Ascorbyl essential and non-essential amino acids, Code Code Code* Packaging Per Can Glycine 0.96 0.71 Iron, mg 7.3 5.4 Palmitate, Manganese Sulfate, Cupric carbohydrates, fat, vitamins, minerals 90164 49735-0101-64 B4162 6 x 400 g (14.1 oz) 1892 L-Histidine 0.63 0.47 Zinc, mg 5.2 3.9 Sulfate, Thiamin Chloride Hydrochloride, and trace elements L-Isoleucine 0.96 0.71 Manganese, mcg 390 289 Pyridoxine Hydrochloride, Riboflavin, L-Leucine 1.65 1.2 Copper, mcg 390 289 Vitamin A Acetate, DL-alpha Tocopherol, • Provides 13.5 g of protein equivalent L-Lysine 1.11 0.82 Iodine, mcg 82.5 61.1 WIC‡: Contact your state WIC For more reimbursement Mixed Tocopherols, Potassium Iodide, per 100 g of powder L-Methionine 0.26 0.19 Molybdenum, mcg 10.9 8.1 Chromium Chloride, Folic Acid, office to determine availability. information see Resources. • Osmolality (at 0.7 kcal/mL): L-Phenylalanine Nil Added Nil Added Chromium, mcg 12.5 9.3 Phylloquinone, Sodium Selenite, Sodium L-Proline 1.1 0.81 Selenium, mcg 14.1 10.4 Molybdate, D-Biotin, Vitamin D3, 380 mOsm/kg L-Serine 0.72 0.53 Sodium, mg 171 127 Cyanocobalamin Based on dilution of 5 g powder L-Threonine 0.81 0.6 Potassium, mg 454 336 (1 unpacked level scoop) to 1 fl oz L-Tryptophan 0.31 0.23 Chloride, mg 321 238 Contains: Soy Oil, Coconut Oil, Milk. L-Tyrosine 1.44 1.07 Metabolics (30 mL) water L-Valine 1.05 0.78 * A source of Arachidonic Acid (ARA) • Displacement: 0.75 mL/g L-Carnitine 0.01 0.01 ** A source of Docosahexaenoic Acid Taurine 0.02 0.01 (DHA) Contains GOS/FOS 0.8 g per 100 mL 40 * S9435 = For individuals who submit their bills to insurance on their own. 41 PKU Periflex Junior Plus

Nutrition Information: Ingredients: Per 10 g Per 10 g Corn Syrup Solids, Refined Vegetable Oils ® Nutrients Per 100 g Protein Equivalent Vitamins Per 100 g Protein Equivalent (High Oleic Sunflower, Canola, Medium PKU Periflex Junior Plus Calories (flavors) 385.(377) 138.(135) Vitamin A, IU (mcg RE) 1399.(420) 500.(150) Chain Triglycerides (Palm Kernel and/or Protein Equivalent, g 28 10 Vitamin D , IU (mcg) 840.(21.0) 300.(7.5) Coconut Oil), Sunflower), L-Alanine, Glycine, A phenylalanine-free, powdered medical food for the dietary 3 Fructo-oligosaccharides, L-Glutamine, Fat, g 12.5 4.5 Vitamin E, IU (mg α-TE) 12.5 (8.4) 4.5 (3) Inulin, Corn Starch, L-Tyrosine, Calcium management of phenylketonuria (PKU) in children over 1 year of age. Saturated, g 2 0.71 Vitamin K, mcg 14 5 Phosphate Dibasic, L-Lysine Acetate, Monounsaturated, g 8.2 2.9 Thiamin, mg 0.93 0.33 L-Threonine, Microcrystalline Cellulose, L-Leucine, L-Proline, Artificial Flavor, Gum Polyunsaturated, g 1.8 0.64 Riboflavin, mg 0.73 0.26 Arabic, L-Cystine, L-Arginine, L-Valine, DHA, mg 180 64.3 Vitamin B6, mg 0.73 0.26 L-Isoleucine, Tripotassium Citrate, Trisodium Citrate, L-Serine, Calcium Chloride, Carbohydrate, g (flavors) 43.2 (41.2) 15.4 (14.7) 12 Main Features: Indications: Vitamin B , mcg 1.3 0.46 L-Methionine, L-Histidine, Maltodextrin, Fiber, g 11.2 4 Niacin, mg 2.0 0.71 Choline Bitartrate, Magnesium Hydrogen Soluble 8 2.9 Folic Acid, mcg 210 75 Phosphate, Magnesium L-Aspartate, CAEM • Phenylalanine free • Phenylketonuria (an emulsifier), *, L-Tryptophan, Insoluble 3.2 1.1 C. Cohnii Oil • Contains a balanced mixture of all Pantothenic Acid, mg 4.2 1.5 Tricalcium Citrate, L-Ascorbic Acid, Soy Amino Acids, g Biotin, mcg 11.2 4 Fiber, M-Inositol, Taurine, Ferrous Sulfate, other essential and non-essential For details on administration and household measurements, please L-Alanine 4.76 1.7 Vitamin C, mg 42 15 Sunflower Lecithin, L-Carnitine, Zinc amino acids, carbohydrate (including see the end of this section. Sulfate, DL-alpha Tocopheryl Acetate, L-Arginine 1.74 0.62 Choline, mg 224 80 Calcium D-Pantothenate, Manganese a multi-fiber blend), fat (including L-Aspartic Acid 0.42 0.15 Inositol, mg 56 20 Sulfate, Niacinamide, Cupric Sulfate, DHA), vitamins, minerals and trace Ordering/Reimbursement Information: L-Cystine 1.9 0.68 Minerals Thiamin Chloride Hydrochloride, Pyridoxine Product Reimbursement HCPCS Product Calories Hydrochloride, Riboflavin, Vitamin A elements L-Glutamine 3.36 1.2 Calcium, mg 980 350 Acetate, Ascorbyl Palmitate, Folic Acid, Code Code Code* Packaging Per Can • Age-appropriate DRI formulation Glycine 4.59 1.6 Phosphorus, mg 641 229 Potassium Iodide, Mixed Tocopherols, 89477 - Plain 49735-0194-77 B4162 6 x 400 g (14.1 oz) 1540 L-Histidine 0.7 0.25 Sodium Molybdate, Sodium Selenite, • Provides 28 g protein equivalent and Magnesium, mg 113 40.4 Chromium Chloride, DL-alpha Tocopherol, 89478 - Vanilla 49735-0194-78 B4162 6 x 400 g (14.1 oz) 1508 L-Isoleucine 1.4 0.50 Iron, mg 10 3.6 Vitamin D , Phylloquinone, D-Biotin, 12.5 g fat per 100 g of powder 3 89474 - Berry 49735-0194-74 B4162 6 x 400 g (14.1 oz) 1508 L-Leucine 2.24 0.80 Zinc, mg 4.9 1.8 Cyanocobalamin • Available in plain, vanilla, berry and 89476 - Orange 49735-0194-76 B4162 6 x 400 g (14.1 oz) 1508 L-Lysine 1.85 0.66 Manganese, mg 0.92 0.33 Berry version also contains: Natural Flavor, Malic Acid, Artificial Sweetener: Sucralose, orange L-Methionine 0.73 0.26 Copper, mcg 560 200 Artificial Color (Beet Red). L-Phenylalanine Nil Added Nil Added Iodine, mcg 139 49.6 • Osmolality (at 1.1 kcal/mL): WIC‡: Contact your state WIC For more reimbursement Vanilla version also contains: Artificial L-Proline 2.24 0.80 Molybdenum, mcg 25.5 9.1 Sweetener: Sucralose. 1340 mOsm/kg office to determine availability. information see Resources. Based on dilution of 36 g powder L-Serine 1.2 0.43 Chromium, mcg 10.2 3.6 Orange version also contains: Citric (approx 4 scoops) to 3.5 fl oz L-Threonine 2.52 0.90 Selenium, mcg 28.2 10.1 Acid, Natural Flavor, Artificial Sweetener: Metabolics L-Tryptophan 0.42 0.15 Sodium, mg 280 100 Sucralose, Artificial Colors (Beta Carotene, (100 mL) water Beet Red). L-Tyrosine 2.66 1.0 Potassium, mg 445 159 • Displacement: 0.72 mL/g L-Valine 1.68 0.60 Chloride, mg 406 145 Contains: Soy, Coconut Oil L-Carnitine 0.02 0.01 42 * S9435 = For individuals who submit their bills to insurance on their own. *A source of Docosahexaenoic Acid (DHA) 43 Taurine 0.05 0.02 Periflex Advance

Nutrition Information: Ingredients: ® Per 10 g Per 10 g Corn Syrup Solids, High Oleic Safflower Periflex Advance Nutrients Per 100 g Protein Equivalent Vitamins Per 100 g Protein Equivalent Oil, Canola Oil, L-Lysine, L-Aspartate, Calories (orange) 385 (369) 110 (105.4) Vitamin A, IU (mcg RE) 1951.(586) 557.4 (167.4) L-Glutamine, L -Leucine, L-Tyrosine, A phenylalanine-free, powdered medical food for the dietary management of Protein Equivalent, g 35 10 Vitamin D3, IU (mcg) 292.(7.3) 83.4 (2.1) L-Proline, L -Arginine, L-Valine, Glycine, phenylketonuria (PKU) in older children and adults, including pregnant women Fat, g 12.6 3.6 Vitamin E, IU (mg α-TE) 16.2 (10.9) 4.6 (3.1) L-Isoleucine, Magnesium Hydrogen Phosphate, L-Threonine, and 2% or and women of child-bearing age. Saturated, g 1.4 0.4 Vitamin K, mcg 71.8 20.5 less of each of the following: L-Serine, Monounsaturated, g 7.9 2.3 Thiamin, mg 0.8 0.2 Disodium Hydrogen Phosphate, Polyunsaturated, g 2.7 0.8 Riboflavin, mg 0.85 0.2 L-Histidine, L-Alanine, Potassium Main Features: Indications: Linoleic Acid, mg 2064 590 Vitamin B6, mg 1.4 0.4 Chloride, Choline Hydrogen Tartrate, • Phenylalanine free • Phenylketonuria α-Linolenic Acid, mg 535 153 Vitamin B12, mcg 2.7 0.8 L-Cystine, L-Tryptophan, Sugar, Carbohydrate, g (orange) 33 (29) 9.4 (8.3) Niacin, mg 5 1.4 L-Methionine, Fractionated Coconut Oil, • Contains a balanced mixture of all other For details on administration and household measurements, Amino Acids, g Folic Acid, mcg 430 122.9 L-Lysine Acetate, CAEM (An Emulsifier), essential and non-essential amino acids, Tripotassium Citrate, Taurine, L-Ascorbic please see the end of this section. L-Alanine 1.4 0.40 Pantothenic Acid, mg 4.3 1.2 carbohydrate, fat, vitamins, minerals Acid, M-Inositol, Ferrous Sulfate, L-Arginine 2.6 0.74 Biotin, mcg 21.4 6.1 L-Carnitine, Zinc Sulfate, DL-Alpha and trace elements L-Aspartic Acid 2.5 0.71 Vitamin C, mg 54.3 15.5 Tocopherol Acetate, Manganese Sulfate, • Periflex Advance provides 35 g of L-Cystine 0.96 0.27 Choline, mg 376 107.4 Niacinamide, Calcium D-Panthothenate, protein equivalent and 12.6 g of fat Glycine 2.5 0.71 Inositol, mg 70 20 Cupric Sulfate, Pyridoxine Hydrochloride, per 100 g of powder Ordering/Reimbursement Information: L-Histidine 1.5 0.43 Minerals Thiamin Chloride Hydrochloride, Riboflavin, Vitamin A Acetate, Folic Acid, • Meets protein requirements in lower Product Reimbursement HCPCS Product Calories L-Isoleucine 2.4 0.69 Calcium, mg 1035 296 Code Code Code* Packaging Per Can Potassium Iodide, Chromium Chloride, volume when compared to L-Leucine 4.0 1.14 Phosphorus, mg 995 284 Sodium Selenite, Sodium Molybdate, 118305 - Unflavored 49735-0183-05 B4157 or B4162 6 x 454 g (1 lb) 1748 L-Lysine 3.1 0.89 Magnesium, mg 268 76.6 Periflex Junior Plus Phylloquinone, D-Biotin, Vitamin D3, 118307 - Orange 49735-0183-07 B4157 or B4162 6 x 454 g (1 lb) 1675 L-Methionine 0.64 0.18 Iron, mg 12.9 3.7 • Pleasant taste, smell and texture Cyanocobalamin. L-Phenylalanine Nil Added Nil Added Zinc, mg 7.9 2.3 Unflavored version also contains: • Available in unflavored and orange L-Proline 2.8 0.80 Manganese, mg 1.6 0.5 Tricalcium Citrate and 2% or less of • Osmolality (mOsm/kg): WIC‡: Contact your state WIC For more reimbursement L-Serine 1.8 0.51 Copper, mcg 720 206 Tricalcium Phosphate. Dilution Unflavored Orange office to determine availability. information see Resources. L-Threonine 1.9 0.54 Iodine, mcg 115 32.9 Orange flavored version also contains: 1 to 5 830 890 L-Tryptophan 0.78 0.22 Molybdenum, mcg 32.4 9.3 Artificial Flavor, Citric Acid, Tricalcium Phosphate and 2% or less of each of the 1 to 4 1060 1100 L-Tyrosine 3.5 1.00 Chromium, mcg 22.8 6.5

Metabolics following: Tricalcium Citrate, Guar Gum, • Displacement: 0.6 mL/g L-Valine 2.5 0.71 Selenium, mcg 50.8 14.5 Xanthan Gum, Artificial Sweetener: L-Carnitine 0.03 0.01 Sodium, mg 490 140 Sucralose, Artificial Colors (Beet Red, Taurine 0.12 0.03 Potassium, mg 613 175 Beta Carotene). * S9435 = For individuals who submit their bills to insurance on their own. 44 L-Glutamine 4.3 1.23 Chloride, mg 490 140 Contains: Coconut Oil. 45 Periflex LQ

Nutrition Information: Ingredients: ® Per 10 g Per 10 g Periflex LQ Nutrients Per 250 mL Protein Equivalent Vitamins Per 250 mL Protein Equivalent Water, Sugar, and less than 2% of each of Calories 160 107 Vitamin A, IU (mcg RE) 833 (250) 555.(167) the following: Calcium Glycerophosphate, A phenylalanine-free, ready-to-drink medical food for the dietary Protein Equivalent, g 15 10 Vitamin D3, IU (mcg) 130 (3.3) 87 (2.2) High Oleic Sunflower Oil, L-Lysine management of phenylketonuria (PKU) in children and adults, Fat, g 5 3.3 Vitamin E, IU (mg α-TE) 7 (4.8) 4.7 (3.2) Acetate, Canola Oil, L-Leucine, L-Tyrosine, Artificial Flavor, L-Proline, L-Arginine, including pregnant women and women of child-bearing age. Saturated, g 0.75 0.5 Vitamin K, mcg 30.8 21 L-Valine, Glycine, L-Aspartic Acid, Monounsaturated, g 3 2 Thiamin, mg 0.35 0.23 L-Isoleucine, Micro-Crystalline Cellulose, Polyunsaturated, g 1 0.7 Riboflavin, mg 0.38 0.25 Citric Acid, L-Threonine, L-Serine, Carbohydrate, g 13.8 9.2 Vitamin B6, mg 0.6 0.4 Magnesium Acetate, Tripotassium Amino Acids, g Vitamin B , mcg 1.2 0.8 Citrate, L-Histidine, L-Alanine, L-Cystine, Main Features: Indications: 12 L-Alanine 0.68 0.45 Niacin, mg 2.1 1.4 Choline Hydrogen Tartrate, N-Acetyl, L-Methionine, Sodium Chloride, • Phenylalanine free • Phenylketonuria L-Arginine 1.28 0.85 Folic Acid, mcg 184 123 L-Tryptophan, Trisodium Citrate, L-Aspartic Acid 1.2 0.80 Pantothenic Acid, mg 1.9 • Contains a balanced mixture of other 1.3 Soybean Oil, L-Ascorbic Acid, Diacetyl amino acids, carbohydrate, vitamins For details on administration, please see the end of this L-Cystine 0.48 0.32 Biotin, mcg 9.3 6.2 Tartaric Acid Esters of Monoglycerides, Glycine 1.23 and minerals section. 0.82 Vitamin C, mg 23.3 16 Mono- and Diglycerides, Guar Gum, L-Histidine 0.73 0.49 Choline, mg 161 107 Xanthan Gum, Artificial Sweetener: • Can easily be used along with or in L-Isoleucine 1.13 0.75 Inositol, mg 30 20 Acesulfame Potassium, Taurine, place of PKU Periflex Junior Plus or L-Leucine 1.95 1.30 Minerals M-Inositol, Ferrous Sulphate, Tricalcium Phosphate, Carnitine, Zinc Advance Ordering/Reimbursement Information: L-Lysine 1.5 1.00 Calcium, mg 400 267 Sulfate, DL-Alpha Tocopherol Acetate, • No measuring or mixing Product Reimbursement HCPCS Product Calories Per L-Methionine 0.33 0.22 Phosphorus, mg 288 192 Code Code Code* Packaging Container Niacinamide, Manganese Sulphate, L-Phenylalanine Nil Added Nil Added Magnesium, mg 114 76 Calcium-D-Pantothenate, Cupric Sulfate, • Great addition to any daily PKU 80177 - Orange 49735-0125-51 B4162 or 18 x 250 mL (8.5 fl oz) 160 L-Proline 1.38 0.92 Iron, mg 5.5 3.7 Pyridoxine Hydrochloride, Thiamin diet plan Créme B4157 L-Serine 0.85 0.57 Zinc, mg 3.5 2 Chloride Hydrochloride, Riboflavin, • Periflex LQ provides 15 g of protein 80176 - Berry 49735-0125-24 B4162 or 18 x 250 mL (8.5 fl oz) 160 L-Threonine 0.95 0.63 Manganese, mg 0.7 0.5 Vitamin A Acetate, Potassium Iodide, Créme B4157 Chromium Chloride, Sodium Selenite, equivalent and 5 g of fat per 250 mL L-Tryptophan 0.38 0.25 Copper, mcg 310 207 Sodium Molybdate, Phylloquinone, container L-Tyrosine 1.73 1.15 Iodine, mcg 49 33 ‡ D-Biotin, Cholecalciferol, Folic Acid, • Available in orange créme and WIC : Contact your state WIC For more reimbursement L-Valine 1.25 0.83 Molybdenum, mcg 14 9 Cyanocobalamin. office to determine availability. information see Resources. L-Carnitine 0.01 0.01 Chromium, mcg 9.8 7 berry créme flavors Contains: Soybean Oil. Taurine 0.08 0.05 Selenium, mcg 21.8 15 Metabolics • Osmolality (at 0.64 kcal/mL): L-Glutamine Nil Added Nil Added Sodium, mg 210 140 Orange 1080 mOsm/kg Potassium, mg 263 175 Berry 1030 mOsm/kg Chloride, mg 210 140 46 • Free water: 84% * S9435 = For individuals who submit their bills to insurance on their own. 47 PhenylAde GMP Drink Mix PhenylAde® GMP Drink Mix Nutrition Information: Ingredients: Per 10 g Protein Equivalent Per 10 g Protein Equivalent Whey Protein Concentrate* (FROM MILK), A phenylalanine-low, powdered medical food containing a balanced mix Nutrients Per 100 g (1 scoop/pouch) Vitamins Per 100 g (1 scoop/pouch) Refined Vegetable Oils (Canola, Safflower, of glycomacropeptide (GMP), essential and non-essential amino acids, Calories 396 132 Vitamin A, IU (mcg RE) 2040.(765) 680.(255) Coconut and Soybean), Modified Corn Protein Equivalent, g 30.0 10.0 Starch, Dextrin, Maltodextrin, L-Tyrosine, carbohydrates (including fiber), fat (including docosahexaenoic acid Vitamin D3, IU (mcg) 900.(22.5) 300.(7.5) Phenylalanine, mg 46.0 15.3 Vitamin E, IU (mg α-TE) 22.8 (15.3) 7.6.(5.1) Tricalcium Phosphate. Contains 2 % or less (DHA)), vitamins, minerals and trace elements. Fat, g 12.0 4.0 Vitamin K, mcg 72 24 of the following: L-Glycine, Magnesium Saturated, g 2.8 0.93 Thiamin, mg 1.8 0.6 Phosphate, L-Leucine, L-Histidine, Monounsaturated, g 6.3 2.1 Riboflavin, mg 1.8 0.6 L-Arginine, Potassium Chloride, L-Cystine, Indications: Choline Bitartrate, Dicalcium Phosphate, Main Features: Polyunsaturated, g 2.5 0.84 Vitamin B6, mg 1.7 0.56 L-Tryptophan, C. Cohnii Oil**, Glucose DHA, mg 155 51.6 Vitamin B12, mcg 3.6 1.2 • Low in Phenylalanine • Phenylketonuria Syrup Solids, Potassium Citrate, Ascorbyl Carbohydrate, g 42.0 14.0 Niacin, mg 15.0 5.0 • Suitable for individuals with PKU Palmitate, Mixed Tocopherols, Silicon For details on administration and household measurements, please Fiber, g 4.5 1.5 Folic Acid, mcg 360 120 Dioxide, Rosemary Extract, Sodium over 1 year of age see the end of this section. Amino Acids, g Pantothenic Acid, mg 4.8 1.6 Chloride, Ascorbic Acid, Taurine, Inositol, • Complete with essential vitamins L-Alanine 1.3 0.44 Biotin, mcg 36 12 Mannitol, Vitamin E Acetate, Ferrous and minerals L-Arginine 1.4 0.47 Vitamin C, mg 72 24 Sulfate, L-Carnitine, Glycerol Mono and Ordering/Reimbursement Information: 100 • Contains a source of DHA L-Aspartic acid 1.9 0.63 Choline, mg 300 Distearate, High Oleic Sunflower Oil, L-Cystine 0.93 0.31 Inositol, mg 60 20 Sodium Polyphosphate, Sunflower Lecithin, • 1.5 grams of soluble fiber per each Product Reimbursement HCPCS Product Calories Per L-Glutamic acid 4.1 1.4 Minerals Niacinamide, Zinc Oxide, Sodium Ascorbate, Code Code Code* Packaging Can/Pouch 10 g PE serving L-Glycine 2.2 0.75 Calcium, mg 1050 350 Vitamin D3, Vitamin A Palmitate, Copper • PhenylAde GMP Drink Mix provides CANS L-Histidine 1.5 0.49 Phosphorus, mg 945 315 Gluconate, Manganese Sulfate, D-Calcium 6832 - Vanilla 49735-0168-32 B4157 or B4162 4 x 400 g (14.1 oz) 1584 L-Isoleucine 2.3 0.76 Magnesium, mg 252 84 Pantothenate, D-Biotin, Beta-Carotene, 30 g of protein equivalent and 12 g Thiamin Hydrochloride, Pyridoxine POUCHES L-Leucine 2.3 0.78 Iron, mg 13.2 4.4 of fat per 100 g of powder Hydrochloride, Riboflavin, Potassium 68324 - Vanilla 49735-0183-04 B4157 or B4162 16 x 33.3 g (1.2 oz) 132 L-Lysine 1.3 0.44 Zinc, mg 10.2 3.4 • Available in vanilla and original flavor Iodide, Vitamin K1, Sodium Selenite, Folic 114116 - Original 49735-0141-16 B4157 or B4162 16 x 33.3 g (1.2 oz) 132 L-Methionine 0.44 0.15 Manganese, mg 1.6 0.52 Acid, Vitamin B12, Chromium Chloride, • Available in cans and pouches L-Phenylalanine 0.05 0.02 Copper, mcg 1200 400 Sodium Molybdate. • Osmolality (at 0.73 kcal/mL): ‡ L-Proline 2.6 0.86 Iodine, mcg 135 45 WIC : Contact your state WIC For more reimbursement Original version also contains: Corn L-Serine 1.6 0.54 Molybdenum, mcg 54 18 460 mOsm/kg office to determine availability. information see Resources. Syrup Solids and 2% or less of Sugar, L-Threonine 3.7 1.2 12 Based on dilution of 33.3 g powder Chromium, mcg 36 Natural Flavors. 16 (1 scoop or 1 pouch) to 5 fl oz L-Tryptophan 0.72 0.24 Selenium, mcg 48 * S9435 = For individuals who submit their bills to insurance on their own. L-Tyrosine 3.7 1.2 Sodium, mg 465 155 Vanilla version also contains: Sugar, (150 mL) water Natural and Artificial Flavors. Metabolics L-Valine 1.8 0.61 Potassium, mg 1047 349 • Displacement: 0.9 mL/g L-Carnitine 0.04 0.01 Chloride, mg 569 190 Contains: Milk, Soybean Oil, Coconut Oil. Taurine 0.09 0.03 *Contains 15.3 mg of phenylalanine/10 g protein equivalent 48 49 **A source of Docosahexaenoic Acid (DHA) PhenylAde GMP READY PhenylAde® GMP READY Nutrition Information: Ingredients: Nutrients Per 8.5 fl oz/ 250 mL Vitamins Per 8.5 fl oz/ 250 mL Water, Whey Protein Concentrate* (Milk), A ready-to-drink medical food, low in phenylalanine containing a mix of glycomacropeptide Calories 110 Vitamin A, IU (mcg RE) 665 (200) Sugar, Refined Vegetable Oils (Canola (GMP), essential and non-essential amino acids, carbohydrates, fat (including docosahexaenoic Protein Equivalent, g 10 Vitamin D3, IU (mcg) 250 (6.3) Oil, High Oleic Sunflower Oil), L-Leucine, Calcium Glycerophosphate, L-Tyrosine, acid (DHA) and eicosapentaenoic acid (EPA)), vitamins, minerals and trace elements. Phenylalanine, mg 18 Vitamin E, IU (mg α-TE) 5 (3.3) Fat, g 4 Vitamin K, mcg 17.5 Maltodextrin, Cellulose Gum, Calcium Saturated, g 0.40 Thiamin, mg 0.40 Phosphate Dibasic, L-Arginine, Magnesium Acetate, L-Histidine, L-Valine, L-Cystine, Trans, g 0 Riboflavin, mg 0.40 Choline Bitartrate, Artificial Flavor, Monounsaturated, g 2.4 Vitamin B6, mg 0.35 L-Tryptophan, Potassium Chloride, Calcium Main Features: Vitamin B12, mcg 0.70 Indications: Polyunsaturated, g 1.2 Lactate, Microcrystalline Cellulose, C. Cohnii Niacin, mg 3.5 • Low in Phenylalanine DHA, mg 60 Oil**, Citric Acid Esters Of Mono- And • Phenylketonuria Folic Acid, mcg 50 • Suitable for individuals with PKU EPA, mg 15 Diglycerides (CITREM), Sodium Chloride, Pantothenic Acid, mg 1.3 † For details on administration, please see the end of this section. Carbohydrate, g 8.5 L-Ascorbic Acid, Schizochytrium Sp. Oil , over 1 year of age Fiber, g 0.93 Biotin, mcg 7.8 Taurine, M-Inositol, Sucralose††, Ferrous • Complete with essential vitamins Sugars, g 7.5 Vitamin C, mg 15.5 Lactate, L-Carnitine, Zinc Sulfate, Dl-Alpha and minerals Amino Acids, g Choline, mg 93.8 Tocopheryl Acetate, Niacinamide, Copper Ordering/Reimbursement Information: • Contains a source of DHA & EPA L-Alanine 0.40 Inositol, mg 20.3 Gluconate, Calcium D-Pantothenate, Minerals and Trace Elements Manganese Sulfate, Thiamin Hydrochloride, • No measuring or mixing L-Arginine 0.49 Product Reimbursement HCPCS Product Calories Per L-Aspartic acid 0.59 Calcium, mg 325 Pyridoxine Hydrochloride, Riboflavin, Code Code Code* Packaging Container • PhenylAde GMP READY provides L-Cystine 0.29 Phosphorus, mg 275 Vitamin A Palmitate, Mixed Tocopherols, 139686 49735-0196-86 B4157 or B4162 18 x 8.5 fl oz (250 mL) 110 Sunflower Lecithin, Potassium Iodide, 10 g of protein equivalent and 4 g of L-Glutamic acid 1.3 Magnesium, mg 58.5 Folic Acid, Ascorbyl Palmitate, Chromium L-Glycine 0.07 Iron, mg 3 fat per 250 mL container Chloride, Sodium Molybdate, Sodium Zinc, mg 2.2 • Available in Plain (neutral) flavor L-Histidine 0.40 Selenite, Phylloquinone, D-Biotin, WIC‡: Contact your state WIC For more reimbursement Manganese, mg 0.23 • Osmolality (at 0.73 kcal/mL): L-Isoleucine 0.72 Cholecalciferol, Cyanocobalamin. office to determine availability. information see Resources. L-Leucine 1.5 Copper, mcg 250 Contains: Milk. 460 mOsm/kg L-Lysine 0.43 Iodine, mcg 36.5 • Free water: 93% L-Methionine 0.13 Molybdenum, mcg 12.5 * Contains 18 mg of phenylalanine per 10 g * S9435 = For individuals who submit their bills to insurance on their own. L-Phenylalanine 0.02 Chromium, mcg 7.5 protein equivalent L-Proline 0.79 Selenium, mcg 12.5 ** A source of docosahexaenoic acid (DHA) Sodium, mg 146 L-Serine 0.51 † A source of docosahexaenoic acid (DHA) L-Threonine 1.1 Potassium, mg 185 and eicosapentaenoic acid (EPA)

Metabolics Metabolics L-Tryptophan 0.24 Chloride, mg 100 †† Contains 22.5 mg of sucralose per L-Tyrosine 1.0 250 mL of product L-Valine 0.87 L-Carnitine 0.01 50 Taurine 0.03 51 PhenylAde GMP Mix-In

Nutrition Information: Ingredients: Per 10 g Protein Per 10 g Protein Whey Protein Concentrate* (FROM ® Equivalent Equivalent MILK), L-Tyrosine, Glycine, L-Leucine, PhenylAde GMP Mix-In Nutrients Per 100 g (1 scoop/pouch) Minerals Per 100 g (1 scoop/pouch) L-Histidine, L-Arginine, Corn Syrup Calories 334 42 Calcium, mg 32 4 Solids, L-Cystine. Contains 2 % or A powdered medical food, low in phenylalanine containing a balanced Protein Equivalent, g 80 10 Phosphorus, mg 320 40 less of the following: L-Tryptophan, mix of glycomacropeptide (GMP), essential and non-essential amino Phenylalanine, mg 46.0 15.3 Potassium, mg 1160 145 Refined Vegetable Oils (Canola, acids, some carbohydrates and fat. Fat, g <0.5 <0.5 Sodium, mg 960 120 Safflower, Coconut and Soybean), Carbohydrate, g 2.7 <0.5 Silicon Dioxide, Taurine, Modified Amino Acids, g Corn Starch, Maltodextrin, L-Alanine, g 3.5 0.44 L-Carnitine, Ascorbyl Palmitate, L-Arginine, g 3.8 0.47 Rosemary Extract, Mixed Tocopherols, Main Features: Indications: L-Aspartic acid, g 5 0.63 Tricalcium Phosphate. • Low in Phenylalanine L-Cystine, g 2.5 0.31 *Contains 15.3 mg of • Phenylketonuria phenylalanine/10 g protein equivalent • Suitable for individuals with PKU L-Glutamic acid, g 11 1.4 Glycine, g 5.9 0.75 For details on administration and household measurements, please Contains: Milk, Soybean Oil, Coconut over 1 year of age L-Histidine, g 3.9 0.49 Oil. • Provides 80 g protein equivalent per see the end of this section. L-Isoleucine, g 6 0.76 100 g of powder L-Leucine, g 6.2 0.78 • Mixes easily with water, flavored L-Lysine, g 3.5 0.44 Note: Must be combined with an Ordering/Reimbursement Information: appropriate vitamin & mineral drinks or other PKU formula L-Methionine, g 1.2 0.15 L-Phenylalanine, g 0.12 0.02 supplement when taken as the sole • Available in cans and pouches Product Reimbursement HCPCS Product Calories L-Proline, g 6.8 0.86 source of PKU Medical Food. Code Code Code* Packaging Per Can/Pouch • Displacement: 0.4 mL/g L-Serine, g 4.3 0.54 CAN L-Threonine, g 9.7 1.2 135426 49735-0154-26 B4155 24 x 200 g (28.2 oz) 668 L-Tryptophan, g 1.9 0.24 POUCHES L-Tyrosine, g 9.8 1.2 116130 49735-0111-61 B4155 20 x 12.5 g (0.44 oz) 42 L-Valine, g 4.8 0.61 L-Carnitine, g 0.08 0.01 Taurine, g 0.24 0.03 WIC‡: Contact your state WIC For more reimbursement office to determine availability. information see Resources. Metabolics Metabolics

* S9435 = For individuals who submit their bills to insurance on their own.

52 53 PhenylAde Essential Drink Mix

® Nutrition Information: Ingredients: PhenylAde Essential Drink Mix Per 10 g Protein Per 10 g Protein Corn Syrup Solids, Modified Corn Starch, Equivalent Equivalent Canola Oil, Maltodextrin, Safflower Oil, A phenylalanine-free, powdered medical food for the dietary Nutrients Per 100 g (1 scoop/pouch) Vitamins Per 100 g (1 scoop/pouch) Dextrin, L-Asparagine Monohydrate, management of phenylketonuria (PKU) in children and adults, including Calories 393 157 Vitamin A, IU (mcg RE) 1700 (637) 680 (255) L-Glutamine, L-Tyrosine, L-Lysine Acetate, (25% as beta-carotene) L-Alanine, L-Leucine, L-Serine. Contains pregnant women and women of child-bearing age. Protein Equivalent, g 25 10 2% or less of the following: L-Arginine, Fat, g 13 5 Vitamin D3, IU (mcg) 600 (15) 240 (6) Monocalcium Phosphate, L-Valine, Dicalcium Main Features: Saturated, g 3 1.2 Vitamin E, IU (mg α-TE) 19 (12.7) 7.6 (5.1) Phosphate, Sodium Citrate, Glycine, L-Proline, Monounsaturated, g 6.8 2.7 Vitamin K, mcg 61 24 Potassium Citrate, Coconut Oil, Soybean • Phenylalanine free Oil, L-Threonine, L-Isoleucine, Potassium Indications: Polyunsaturated, g 2.8 1.1 Thiamin, mg 1.5 0.6 Chloride, L-Histidine, L-Methionine, Choline • Complete with essential vitamins Linoleic Acid, mg 1936 774 Riboflavin, mg 1.5 0.6 Bitartrate, L-Tryptophan, Flaxseed Oil, • Phenylketonuria Magnesium Oxide, L-Cystine, Ascorbyl and minerals α-Linolenic Acid, mg 725 290 Vitamin B6, mg 1.4 0.56 Palmitate, Mixed Tocopherols, Rosemary Vitamin B12, mcg 3.0 1.2 • 1.5 grams of soluble fiber For details on administration and household measurements, please Carbohydrate, g 46 18 Extract, Silicon dioxide, Tricalcium Phosphate, Niacin, mg 17 6.8 per serving see the end of this section. Fiber, g 3.9 1.5 Sodium Chloride, Taurine, Ascorbic Acid, Amino Acids, g Folic Acid, mcg 300 120 Inositol, Ferric Orthophosphate, Vitamin E • PhenylAde Essential Drink Mix L-Alanine 2.32 0.93 Pantothenic Acid, mg 4 1.6 Acetate, L-Carnitine, Niacinamide, Calcium Phosphate, Zinc Oxide, Copper Gluconate, provides 25 g of protein equivalent Ordering/Reimbursement Information: Biotin, mcg 30 12 L-Arginine 1.97 0.79 Vitamin K1, Vitamin D3, Vitamin A Palmitate, L-Asparagine 3.49 1.40 Vitamin C, mg 60 24 D-Calcium Pantothenate, Manganese and 13 g of fat per 100 g of powder Product Reimbursement HCPCS Product Calories Per Code Code Code* Packaging Can/Pouch L-Cystine 0.34 0.14 Choline, mg 210 84 Sulfate, Mono- and Diglycerides, D-Biotin, • Available in chocolate, vanilla, Beta-carotene, Pyridoxine Hydrochloride, L-Glutamine 3.89 1.56 Inositol, mg 50 20 orange creme, strawberry, CANS Riboflavin, Thiamin Mononitrate, Potassium Minerals and unflavored 9501 - Chocolate 00847-0950-12 B4157 or B4162 4 x 454 g (1 lb) 1784 Glycine 1.52 0.61 Iodide, Sodium Selenite, Folic Acid, Vitamin L-Histidine 0.63 0.25 Calcium, mg 822 329 B12, Chromium Chloride, Sodium Molybdate. 9502 - Vanilla 00847-0950-22 B4157 or B4162 4 x 454 g (1 lb) 1784 • Available in cans and pouches L-Isoleucine 1.04 0.42 Phosphorus, mg 788 315 9503 - Orange Creme 00847-0950-32 B4157 or B4162 4 x 454 g (1 lb) 1784 Contains: Coconut Oil, Soybean Oil. • Osmolality (at 0.65 kcal/mL): L-Leucine 2.24 0.90 Magnesium, mg 210 84 800 mOsm/kg 9504 - Strawberry 00847-0950-42 B4157 or B4162 4 x 454 g (1 lb) 1784 L-Lysine 1.82 0.73 Iron, mg 11 4.4 Chocolate flavored version also contains: 9508 - Unflavored 00847-0950-82 B4157 or B4162 4 x 454 g (1 lb) 1784 Sugar, Natural and Artificial Flavor, and less Based on dilution of 40 g powder L-Methionine 0.61 0.24 Zinc, mg 10 4 than 2% of the following: FD&C Red#40, POUCHES (1 scoop or 1 pouch) to 7 fl oz L-Phenylalanine Nil Added Nil Added Manganese, mg 1.3 0.52 FD&C Yellow#5, FD&C Blue #2. 95014 - Chocolate 00847-0950-14 B4157 or B4162 16 x 40 g (1.4 oz) 157 L-Proline 1.52 0.61 Copper, mcg 1000 400 (210 mL) water Vanilla flavored version also contains: Sugar, 95024 - Vanilla 00847-0950-24 B4157 or B4162 16 x 40 g (1.4 oz) 157 L-Serine 2.24 0.90 Iodine, mcg 100 40 Natural and Artificial Flavor, and less than 2% of the following: Titanium Dioxide. • Displacement: 0.75 mL/g 95034 - Orange Creme 00847-0950-34 B4157 or B4162 16 x 40 g (1.4 oz) 157 L-Threonine 1.15 0.46 Molybdenum, mcg 45 18 L-Tryptophan 0.58 0.23 Chromium, mcg 30 12 Orange Creme flavored version also 95044 - Strawberry 00847-0950-44 B4157 or B4162 16 x 40 g (1.4 oz) 157 contains: Sugar, Natural and Artificial Flavor, L-Tyrosine 2.69 1.08 Selenium, mcg 40 16 Metabolics Metabolics 95084 - Unflavored 00847-0950-84 B4157 or B4162 16 x 40 g (1.4 oz) 157 and less than 2% of the following: FD&C L-Valine 1.7 0.67 Sodium, mg 388 155 Yellow #6. Potassium, mg 872 349 WIC‡: Contact your state WIC For more reimbursement L-Carnitine 0.04 0.01 Strawberry flavored version also contains: Chloride, mg 475 190 office to determine availability. information see . Taurine 0.08 0.03 Sugar, Natural and Artificial Flavor, and less Resources than 2% of the following: FD&C Red #40, 54 FD&C Blue #1. 55 * S9435 = For individuals who submit their bills to insurance on their own. PhenylAde40 Drink Mix

Nutrition Information: Ingredients: Per 10 g Protein Per 10 g Protein ® Corn Syrup Solids, Sugar, L-Glutamine, PhenylAde 40 Drink Mix Equivalent Equivalent Nutrients Per 100 g (1 pouch) Vitamins Per 100 g (1 pouch) L-Asparagine Monohydrate, L-Serine, A phenylalanine-free, powdered medical food for the dietary Calories 330 83 Vitamin A, IU (mcg RE) 1557 (584) 389 (146) L-Alanine, L-Tyrosine, L-Lysine Monohydrochloride, L-Leucine, Protein Equivalent, g 40 10 (25% as beta-carotene) management of phenylketonuria (PKU) in children and adults, including L-Arginine, L-Valine, Citric Acid, Fat, g 2 0.5 Vitamin D , IU (mcg) 347 (8.7) 87 (2.2) pregnant women and women of child-bearing age. 3 Tricalcium Phosphate, Glycine, L-Proline, Vitamin E, IU (mg -TE) 15.4 (10.3) 3.9 (2.6) Saturated, g 0.48 0.12 α Natural Flavor. Contains 2% or less of Monounsaturated, g 1.1 0.28 Vitamin K, mcg 60 15 the following: L-Threonine, L-Isoleucine, Main Features: Indications: Polyunsaturated, g 0.40 0.10 Thiamin, mg 1.4 0.35 Magnesium Phosphate, L-Methionine, Carbohydrate, g 38 9.5 Riboflavin, mg 1.3 0.32 Sodium Citrate, L-Histidine, Dipotassium • Phenylalanine free • Phenylketonuria Amino Acids, g Vitamin B6, mg 1.5 0.38 Phosphate, Modified Corn Starch, • Complete with essential vitamins L-Alanine 3.85 0.96 Vitamin B12, mcg 2.8 0.7 Canola Oil, Maltodextrin, Safflower and minerals For details on administration and household measurements, please L-Arginine 3.27 0.82 Niacin, mg 16.4 4.1 Oil, Choline Bitartrate, Potassium see the end of this section. L-Asparagine 5.04 1.26 Folic Acid, mcg 468 117 Chloride, L-Tryptophan, L-Cystine, • Only 0.5 gram of fat and 83 calories L-Cystine 0.55 0.14 Pantothenic Acid, mg 5.8 1.5 Sodium Chloride, Coconut Oil, Soybean per serving L-Glutamine 6.34 1.59 Biotin, mcg 35 8.8 Oil, Taurine, Silicon Dioxide, Inositol, • PhenylAde40 Drink Mix provides Glycine 2.47 0.62 Vitamin C, mg 75 18.9 Ascorbic Acid, L-Carnitine, Ascorbyl Palmitate, Mixed Tocopherols, Rosemary L-Histidine 1.02 0.26 Choline, mg 284 71 40 g of protein equivalent and 2 g of Ordering/Reimbursement Information: Extract, Ferric Orthophosphate, Vitamin L-Isoleucine 1.64 0.41 Inositol, mg 86 22 fat per 100 g of powder E Acetate, Zinc Sulfate, Niacinamide, Product Reimbursement HCPCS Product Calories Minerals • Available in citrus and unflavored L-Leucine 3.64 0.91 Sucralose, D-Calcium Pantothenate, Code Code Code* Packaging Per Pouch L-Lysine 2.98 0.75 Calcium, mg 894 224 single serve pouches Vitamin K1, Manganese Sulfate, 95404 - Unflavored 00847-0954-04 B4157 or B4162 20 x 25 g (0.88 oz) 83 L-Methionine 1.08 0.27 Phosphorus, mg 809 202 Vitamin A Palmitate, D-Biotin, Vitamin • Osmolality (at 0.63 kcal/mL): 95414 - Citrus 00847-0954-14 B4157 or B4162 20 x 25 g (0.88 oz) 83 L-Phenylalanine Nil Added Nil Added Magnesium, mg 209 52 D3, Beta-carotene, Copper Sulfate, 1110 mOsm/kg L-Proline 2.47 0.62 Iron, mg 12 3 Pyridoxine Hydrochloride, Potassium Based on dilution of 25 g powder L-Serine 4.08 1.02 Zinc, mg 8 2 Iodide, Thiamin Mononitrate, Riboflavin, Sodium Selenite, Folic Acid, Vitamin B12, (1 pouch) to 4 fl oz (120 mL) water WIC‡: Contact your state WIC For more reimbursement L-Threonine 1.92 0.48 Manganese, mg 1.8 0.45 L-Tryptophan 0.61 0.15 Copper, mcg 869 217 Chromium Chloride, Sodium Molybdate. office to determine availability. information see Resources. • Displacement: 0.48 mL/g L-Tyrosine 3.78 0.95 Iodine, mcg 133 33 Contains: Coconut Oil, Soybean Oil. L-Valine 2.74 0.69 Molybdenum, mcg 40 10 * S9435 = For individuals who submit their bills to insurance on their own. L-Carnitine 0.06 0.01 Chromium, mcg 27 6.7 Citrus flavored version also contains: Metabolics Metabolics Taurine 0.12 0.03 Selenium, mcg 56 13.9 less than 2% of the following: FD&C Sodium, mg 424 106 Yellow #5. Potassium, mg 776 194 Chloride, mg 1322 331 56 57 Ingredients: PhenylAde60 Drink Mix Nutrition Information: Unflavored: L-Asparagine Monohydrate, L-Glutamine, Sugar, L-Tyrosine, L-Lysine Per 10 g Protein Per 10 g Protein Acetate, L-Alanine, L-Leucine, L-Serine, ® Equivalent Equivalent L-Arginine, L-Valine, Tricalcium Phosphate, PhenylAde 60 Drink Mix Nutrients Per 100 g (1 scoop/pouch) Vitamins Per 100 g (1 scoop/pouch) Glycine, L-Proline, L-Threonine, Corn Syrup Solids, L-Isoleucine, Magnesium Phosphate. Calories 294 49 Vitamin A, IU (mcg RE) 2331 (874) 389 (146) Contains 2% or less of the following: A phenylalanine-free, powdered medical food for the dietary Protein Equivalent, g 60 10 (25% as beta-carotene) Sodium Citrate, L-Histidine, L-Methionine, Fat, g 1 0.17 Vitamin D , IU (mcg) 520 (13) 87 (2.2) L-Tryptophan, Dipotassium Phosphate, management of phenylketonuria (PKU) in children and adults, including 3 Potassium Chloride, Choline Bitartrate, Soy pregnant women and women of child-bearing age. Saturated, g <0.5 <0.1 Vitamin E, IU (mg α-TE) 23 (15.4) 3.8 (2.6 Lecithin, L-Cystine, Sodium Chloride, Silicon Monounsaturated, g <0.5 <0.1 Vitamin K, mcg 90 15 Dioxide, Modified Corn Starch, Taurine, Polyunsaturated, g <0.5 <0.1 Thiamin, mg 2.1 0.35 Canola Oil, Maltodextrin, Safflower Oil, Ascorbic Acid, Inositol, L-Carnitine, Ferric Carbohydrate, g 11 2 Riboflavin, mg 1.9 0.32 Orthophosphate, Vitamin E Acetate, Coconut Amino Acids, g Vitamin B6, mg 2.3 0.38 Oil, Soybean Oil, Zinc Sulfate, Niacinamide, Sucralose, D-Calcium Pantothenate, Vitamin Main Features: Indications: L-Alanine 5.56 0.93 Vitamin B12, mcg 4.2 0.7 K1, Ascorbyl Palmitate, Mixed Tocopherols, • Phenylalanine free • Phenylketonuria L-Arginine 4.72 0.79 Niacin, mg 25 4.1 Rosemary Extract, Manganese Sulfate, L-Asparagine 8.36 1.39 Folic Acid, mcg 700 117 Vitamin A Palmitate, D-Biotin, Vitamin D3, Beta-carotene, Copper Sulfate, Pyridoxine • Complete with essential vitamins L-Cystine 0.81 0.13 Pantothenic Acid, mg 8.7 1.5 and minerals For details on administration and household measurements, please Hydrochloride, Potassium Iodide, Thiamin see the end of this section. L-Glutamine 9.31 1.55 Biotin, mcg 53 8.8 Mononitrate, Riboflavin, Sodium Selenite, • Only 49 calories per serving Glycine 3.63 0.61 Vitamin C. mg 113 19 Folic Acid, Vitamin B12, Chromium Chloride, Sodium Molybdate. • PhenylAde60 Drink Mix provides L-Histidine 1.51 0.25 Choline, mg 425 71 L-Isoleucine 2.49 0.42 Inositol, mg 129 22 Contains: Soy, Coconut Oil. 60 g of protein equivalent and 1 g of Ordering/Reimbursement Information: fat per 100 g of powder L-Leucine 5.37 0.90 Minerals Vanilla: L-Asparagine Monohydrate, L-Lysine 4.36 0.73 Calcium, mg 1300 217 L-Glutamine, Sugar, L-Tyrosine, L-Lysine • Available in vanilla and unflavored Product Reimbursement HCPCS Product Calories Per L-Methionine 1.45 0.24 Phosphorus, mg 1194 199 Acetate, L-Alanine, L-Leucine, L-Serine, Code Code Code* Packaging Can/Pouch L-Arginine, L-Valine, Tricalcium Phosphate, Magnesium, mg 310 52 • Available in cans or single serve CANS L-Phenylalanine Nil Added Nil Added Glycine, L-Proline, L-Threonine, Natural and L-Proline 3.63 0.61 Iron, mg 18 3 Artificial Flavor, L-Isoleucine, Magnesium pouches 9560 - Unflavored 00847-0956-02 B4157 or B4162 4 x 454 g (1 lb) 1335 Phosphate. Contains 2% or less of the Zinc, mg 12 2 • Osmolality (at 0.49 kcal/mL): 9562 - Vanilla 00847-0956-22 B4157 or B4162 4 x 454 g (1 lb) 1335 L-Serine 5.37 0.90 following: Sodium Citrate, L-Histidine, L-Threonine 2.76 0.46 Manganese, mg 2.7 0.45 L-Methionine, L-Tryptophan, Dipotassium 1256 mOsm/kg POUCHES L-Tryptophan 1.38 0.23 Copper, mcg 1300 217 Phosphate, Potassium Chloride, Choline 95604 - Unflavored 00847-0956-04 B4157 or B4162 30 x 16.7 g (0.59 oz) 49 Bitartrate, Soy Lecithin, L-Cystine, Sodium Based on dilution of 16.7 g powder L-Tyrosine 6.45 1.08 Iodine, mcg 180 30 95624 - Vanilla 00847-0956-24 B4157 or B4162 30 x 16.7 g (0.59 oz) 49 Chloride, Taurine, Ascorbic Acid, Inositol, (1 scoop or 1 pouch) to 3 fl oz L-Valine 4.01 0.67 Molybdenum, mcg 60 10 Silicon Dioxide, L-Carnitine, Ferric Chromium, mcg 40 6.7 Orthophosphate, Sucralose, Vitamin (90 mL) water L-Carnitine 0.09 0.01 E Acetate, Zinc Sulfate, Niacinamide, ‡ Selenium, mcg 83 14 • Displacement: 0.54 mL/g WIC : Contact your state WIC For more reimbursement Taurine 0.18 0.03 D-Calcium Pantothenate, Vitamin K1, Metabolics Metabolics office to determine availability. information see Resources. Sodium, mg 600 100 Manganese Sulfate, Vitamin A Palmitate, D-Biotin, Vitamin D3, Beta-carotene, Copper Potassium, mg 1162 194 Sulfate, Pyridoxine Hydrochloride, Potassium * S9435 = For individuals who submit their bills to insurance on their own. Chloride, mg 900 150 Iodide, Thiamin Mononitrate, Riboflavin, Sodium Selenite, Folic Acid, Vitamin B12, 58 Chromium Chloride, Sodium Molybdate. 59 Contains: Soy. PhenylAde Amino Acid Blends Nutrition Information: Ingredients: Amino Acid Blend Per 10 g Protein MTE Amino Acid Blend Amino Acid Blend Equivalent Per 10 g Protein Equivalent L-Asparagine Monohydrate, L-Glutamine, PhenylAde® Amino Acid Blends Nutrients Per 100 g (1 scoop/pouch) Nutrients Per 100 g (1 scoop/pouch) L-Tyrosine, L-Lysine Acetate, L-Alanine, Calories 323 40 Calories 312 40 L-Leucine, L-Serine, L-Arginine, L-Valine, Protein Equivalent, g 81 10 Protein Equivalent, g 78 10 Glycine, L-Proline, L-Threonine, A phenylalanine-free, powdered medical food for the dietary management of Fat, g 0 0 Fat, g 0 0 L-Isoleucine, L-Histidine, L-Methionine, phenylketonuria (PKU) in children and adults, including pregnant women and Carbohydrate, g 0 0 Carbohydrate, g 0 0 L-Tryptophan, L-Cystine, Silicon Dioxide, women of child-bearing age. Amino Acids, g Amino Acids, g Taurine and L-Carnitine. L-Alanine 7.46 0.93 L-Alanine 7.23 0.93 L-Arginine 6.33 0.79 L-Arginine 6.13 0. 79 MTE Amino Acid Blend L-Asparagine 11.22 1.40 L-Asparagine 10.87 1.40 Indications: L-Asparagine Monohydrate, L-Glutamine, L-Cystine 1.08 0.14 L-Cystine 1.05 0. 14 Main Features: L-Tyrosine, L-Lysine Acetate, L-Alanine, L-Glutamine 12.5 1.55 L-Glutamine 12.11 1.55 • Phenylketonuria L-Leucine, L-Serine, L-Arginine, L-Valine, Glycine 4.87 0.61 Glycine 4.72 0. 61 • Phenylalanine free Glycine, L-Proline, L-Threonine, L-Histidine 2.03 0.25 L-Histidine 1.96 0. 25 • Only 40 calories per serving For details on administration and household measurements, please L-Isoleucine, Dicalcium Phosphate, L-Isoleucine 3.34 0.42 L-Isoleucine 3.24 0. 42 see the end of this section. L-Histidine, L-Methionine, L-Tryptophan, • Available in cans and single-serve L-Leucine 7.20 0.90 L-Leucine 6.98 0. 90 L-Cystine, Magnesium Phosphate L-Lysine 5.84 0.73 L-Lysine 5.67 0. 73 pouches (Dibasic), Silicon Dioxide, Taurine, L-Methionine 1.95 0.24 L-Methionine 1.89 0. 24 MTE Amino Acid Blend L-Carnitine, Calcium Citrate, Chromium L-Phenylalanine Nil Added Nil Added L-Phenylalanine Nil Added Nil Added Chloride, Sodium Selenite, Sodium • Contains added calcium, L-Proline 4.87 0.61 L-Proline 4.72 0. 61 Ordering/Reimbursement Information: Molybdate. phosphorus, magnesium, L-Serine 7.20 0.90 L-Serine 6.98 0. 90 L-Threonine 3.70 0.46 L-Threonine 3.59 0. 46 chromium, molybdenum, Product Reimbursement HCPCS Product Calories Per L-Tryptophan 1.85 0.23 and selenium Code Code Code* Packaging Can/Pouch L-Tryptophan 1.79 0. 23 Note: Must combine with an appropriate CANS L-Tyrosine 8.65 1.08 L-Tyrosine 8.39 1.08 vitamin & mineral supplement when • 100 g of powder provides L-Valine 5.38 0.67 9500 00847-0950-00 B4155 4 x 454 g (1 lb) 1466 L-Valine 5.22 0. 67 taken as the sole source of amino acid 78 g of protein equivalent L-Carnitine 0.12 0.01 L-Carnitine 0. 11 0.01 9596 - MTE 00847-0959-60 B4155 4 x 454 g (1 lb) 1421 protein. • Displacement: 0.47 mL/g Taurine 0.24 0.03 Taurine 0. 24 0.03 POUCHES Minerals Amino Acid Blend 95004 00847-0950-04 B4155 30 x 12.4 g (0.44 oz) 40 Calcium, mg 570 73 • 100 g of powder provides 95964 - MTE 00847-0959-64 B4155 30 x 12.8 g (0.45 oz) 40 Chloride, mg Trace Trace 81 g of protein equivalent Chromium, mcg 23 3 Magnesium, mg 123 15.8 • Displacement: 0.48 mL/g ‡ For more reimbursement Metabolics Metabolics WIC eligible in all States. Molybdenum, mcg 23 3 information see . Resources Phosphorus, mg 570 73 Sodium, mg Trace Trace * S9435 = For individuals who submit their bills to insurance on their own. Selenium, mcg 26 3.3 60 61 PhenylAde PheBLOC LNAA

Nutrition Information: Ingredients: ® ™ Per Tablet Per 5 Tablets Per 10 Tablets Per Pouch Per 2 Pouches Tablet Ingredients: PhenylAde PheBLOC LNAA Nutrients (750mg) (3,750mg) (7,500mg) Nutrients (3g Powder) (6g Powder) L-Tyrosine, Calcium Calories 3 15 30 Calories 10 20 Carbonate, L-Tryptophan, A phenylalanine-free medical food containing the remaining essential Protein Equivalent, g 0.44 2.2 4.4 Protein Equivalent, g 2.2 4.4 L-Methionine, amino acids (large neutral amino acids and lysine) for the dietary Fat, g 0.02 0.1 0.2 Fat, g 0 0 Microcrystalline Cellulose, management of phenylketonuria (PKU) in individuals over 12 years of age. Carbohydrate, g 0.2 1 2 Carbohydrate, g 0.34 0.68 L-Arginine, L-Threonine, Minerals Minerals L-Valine, L-Isoleucine, L-Histidine, L-Leucine, Calcium, mg 43.5 218 435 Sodium, mg Trace Trace L-Lysine Monohydrochloride, Sodium, mg 0.82 4.1 8.2 Chloride, mg 28 56 Hydroxypropyl Amino Acids, g Main Features: Indications: Chloride, mg 5.5 28 55 Methylcellulose, Stearic L-Arginine 0.17 0.34 • Phenylketonuria Amino Acids, g Acid, Croscarmellose • Phenylalanine free L-Arginine 0.03 0.17 0.34 L-Histidine 0.16 0.31 Sodium, Magnesium • Provides full compliment of Precautions: L-Histidine 0.03 0.16 0.31 L-Isoleucine 0.16 0.32 Stearate and Silicon Dioxide. L-Leucine 0.15 0.31 • Not for use when planning or during pregnancy L-Isoleucine 0.03 0.16 0.32 essential amino acids (without L-Leucine 0.03 0.15 0.31 L-Lysine 0.12 0.23 phenylalanine) • Seek medical advice before using with serotonergic L-Lysine 0.02 0.12 0.23 L-Methionine 0.25 0.50 Powder Ingredients: L-Tyrosine, Maltodextrin, medications L-Methionine 0.05 0.25 0.50 L-Phenylalanine Nil Added Nil Added • Simplifies diet management L-Tryptophan, L-Methionine, L-Phenylalanine Nil Added Nil Added Nil Added L-Threonine 0.16 0.33 L-Arginine, L-Threonine, • Suitable for those currently using For details on administration, please see the end of this section. L-Threonine 0.03 0.16 0.33 L-Tryptophan 0.31 0.61 sapropterin and prefer L-Valine, L-Isoleucine, L-Tryptophan 0.06 0.31 0.61 L-Tyrosine 0.98 1.97 L-Histidine, L-Leucine and non-liquid PKU formula L-Tyrosine 0.20 0.98 1.97 L-Valine 0.16 0.32 L-Lysine Monohydrochloride • An option for late-diagnosed or Ordering/Reimbursement Information: L-Valine 0.03 0.16 0.32 those who have never been Product Reimbursement HCPCS Product on-diet Code Code Code* Packaging Calories • Available in tablets or powder TABLETS 95501 00847-0955-01 B9998 550 x 750 mg (0.03 oz) 1650/pack POUCHES 95504 00847-0955-04 B4155 110 x 3 g (0.11 oz) 1100/case Visit NutriciaLearningCenter.com for Metabolics Metabolics Metabolics guidelines on the usage of PhenylAde PheBloc LNAA in the dietary * S9435 = For individuals who submit their bills to insurance on their own. management of PKU. 62 63 Lanaflex

Nutrition Information: Ingredients: ® Lanaflex Per Sachet Per Sachet Sugar, Calcium Phosphate, Artificial Nutrients (15.8 g) Per 100 g Minerals (15.8 g) Per 100 g Flavors, L-Histidine, L-Tryptophan, A phenylalanine-free powdered medical food containing the remaining essential Calories 40 253 Calcium, mg 474 2998 L-Tyrosine, Tricalcium Phosphate, amino acids (large neutral amino acids and lysine) for the dietary management of Protein Equivalent, g 5.2 33 Phosphorus, mg 456 2888 Magnesium Acetate, L-Lysine Acetate, Fat, g 0.16 1 N-Acetyl-L-Methionine, L-Leucine, phenylketonuria (PKU) in individuals over 12 years of age. Magnesium, mg 122 775 Carbohydrate g 4.4 28 Iron, mg 5.9 37.6 L-Methionine, L-Threonine, L-Valine, Amino Acids, g Zinc, mg 3.7 23.2 L-Isoleucine, Choline Bitartrate, Guar L-Histidine 0.92 5.85 Manganese, mg 0.73 4.6 Gum, Soy Lecithin, Citric Acid, L-Ascorbic L-Isoleucine 0.46 2.92 Copper, mcg 332 2100 Acid, M-Inositol, Ferrous Sulfate, Artificial Sweetener (Acesulfame Potassium), L-Leucine 0.46 2.92 Iodine, mcg 52.3 331 DL-Alpha-Tocopheryl Acetate, Zinc Main Features: Indications: L-Lysine 0.46 2.92 Molybdenum, mcg 14.9 94.2 Sulfate, Nicotinamide, Manganese L-Methionine 0.92 5.85 • Phenylalanine free • Phenylketonuria Chromium, mcg 10.6 66.9 Sulfate, Calcium-D-Pantothenate, Cupric L-Threonine 0.46 2.92 Selenium, mcg 23.1 146 • Contains lysine and other large neutral Sulfate, Pyridoxine Hydrochloride, Precautions: L-Tryptophan 0.92 5.85 Sodium, mg 4.9 31 Riboflavin, Vitamin A Acetate, Folic amino acids L-Tyrosine 0.92 5.85 Potassium, mg 3.5 22 Acid, Artificial Color (Beta Carotene), • Contains carbohydrate, vitamins, minerals • Not for use when planning or during pregnancy L-Valine 0.46 2.92 Chloride, mg 0.25 1.6 Potassium Iodide, Chromium Chloride, and trace elements Vitamins Sodium Selenite, Sodium Molybdate, • Seek medical advice before using with serotonergic Vitamin A IU, (mcg RE) 892 (268) 5644 (1695) Phylloquinone, D-Biotin, Cholecalciferol, medications Cyanocobalamin. • One sachet of Lanaflex provides Vitamin D3 IU, (mcg) 135 (3.4) 852 (21.3) 6 g of amino acids equivalent to Vitamin E IU, (mg α-TE) 7.4 (5) 46.9 (31.5) Contains: Soy. 5.2 g of protein For details on administration, please see the end of this section. Vitamin K, mcg 32.7 207 • Available in orange flavor Thiamin, mg 0.36 2.3 Riboflavin, mg 0.38 2.4 • Osmolality (mOsm/kg): Vitamin B6, mg 0.65 4.1 Dilution Ordering/Reimbursement Information: Vitamin B12, mcg 1.3 8 1 to 4 1350 Product Reimbursement HCPCS Product Calories Niacin, mg 2.2 14.2 Code Code Code* Packaging Per Sachet Folic Acid, mcg 197 1246 12643 49735-0126-43 B4157 40 x 15.8 g (0.6 oz) 40 Pantothenic Acid, mg 1.9 12.2 Visit NutriciaLearningCenter.com for Biotin, mcg 9.8 61.9 guidelines for the usage of Lanaflex in Vitamin C, mg 25.1 159 Metabolics Metabolics the dietary management of PKU. Choline, mg 165 1045 Inositol, mg 32.1 203 * S9435 = For individuals who submit their bills to insurance on their own. 64 65 XPhe Maxamum

Nutrition Information: Ingredients: ® Per 10 g Per 10 g XPhe Maxamum Nutrients Per 100 g Protein Equivalent Vitamins Per 100 g Protein Equivalent Sugar, L-Arginine L-Aspartate, L-Lysine Calories 305 76 Vitamin A, IU (mcg RE) 2228 (669) 557 (167) Acetate, L-Glutamine, L-Leucine, A phenylalanine-free powdered medical food for the dietary management of Protein Equivalent, g 40 10 Vitamin D3, IU (mcg) 332 (8.3) 83 (2.1) L-Tyrosine, Calcium Glycerophosphate, phenylketonuria (PKU) in older children and adults, including pregnant women and Fat, g <1 <0.25 Vitamin E, IU (mg α-TE) 18.3 (12.3) 4.6 (3.1) L-Proline, L-Valine, Glycine, L-Isoleucine, L-Threonine, Tripotassium Citrate, women of child-bearing age. Carbohydrate, g 34 8.5 Vitamin K, mcg 82 21 Magnesium Hydrogen Phosphate, and 2% Amino Acids, g Thiamin, mg 0.91 0.23 or less of each of the following: L-Serine, L-Alanine 1.6 0.40 Riboflavin, mg 0.97 0.24 L-Histidine, L-Alanine, Calcium Phosphate L-Arginine 3 0.75 Vitamin B6, mg 1.6 0.4 Dibasic, Choline Bitartrate, L-Cystine, Main Features: Indications: L-Aspartic Acid 2.8 Tricalcium Phosphate, Sodium Chloride, 0.70 Vitamin B12, mcg 3.1 0.8 N-Acetyl L-Methionine, Trisodium Citrate, • Phenylalanine free • Phenylketonuria L-Cystine 1.1 0.28 Niacin, mg 5.7 1.4 Glycine 2.8 0.70 Folic Acid, mcg 491 123 L-Tryptophan, Soy Lecithin, Magnesium L-Aspartate, Taurine, L-Ascorbic Acid, • Contains a balanced mixture of all other L-Histidine 1.7 0.43 Pantothenic Acid, mg 4.9 1.2 M-Inositol, Ferrous Sulfate, L-Carnitine, essential and non-essential amino acids, L-Isoleucine 2.7 0.68 Biotin, mcg 24.5 6.1 For details on administration and household measurements, please Zinc Sulfate, DL-Alpha Tocopheryl carbohydrate, vitamins, minerals and L-Leucine 4.6 1.15 Vitamin C, mg 62 16 Acetate, Niacinamide, Manganese trace elements see the end of this section. L-Lysine 3.5 0.88 Choline, mg 430 108 Sulfate, Calcium D-Pantothenate, Cupric • Age-appropriate DRI formulation L-Methionine 0.73 0.18 Inositol, mg 80 20 Sulfate, Pyridoxine Hydrochloride, L-Phenylalanine Nil Added Nil Added Minerals Sodium Selenite, Thiamin Chloride • XPhe Maxamum provides 40 g of Ordering/Reimbursement Information: Hydrochloride, Riboflavin, Vitamin A L-Proline 3.2 0.80 Calcium, mg 1183 296 protein equivalent per 100 g of powder Product Reimbursement HCPCS Product Calories Per Acetate, Folic Acid, Potassium Iodide, L-Serine 2 Phosphorus, mg 1137 284 Code Code Code* Packaging Can/Pouch 0.50 Chromium Chloride, Sodium Molybdate, • Available in unflavored and orange flavor L-Threonine 2.2 0.55 Magnesium, mg 306 77 Phylloquinone, D-Biotin, Vitamin D , CANS 3 L-Tryptophan 0.89 0.22 Iron, mg 14.7 3.7 Cyanocobalamin. • Contains <1 g of fat per 10 g PE thereby 118324 - Orange 49735-0183-24 B4162 or B4157 6 x 454 g (1 lb) 1385 allowing greater flexibility in modifying L-Tyrosine 4 1.00 Zinc, mg 9 2 118323 - Unflavored 49735-0183-23 B4162 or B4157 6 x 454 g (1 lb) 1385 Orange flavored version also contains: energy intake L-Valine 2.9 0.73 Manganese, mg 1.8 0.5 Citric Acid, and 2% or less of each of POUCHES L-Carnitine 0.039 0.01 Copper, mcg 820 205 • Available in cans or pouches the following: Artificial Flavor, Artificial 12312 - Orange 49735-0123-02 B4162 or B4157 30 x 50 g (1.8 oz) 153 Taurine 0.14 0.04 Iodine, mcg 131 33 Sweetener: Acesulfame Potassium, • Osmolality (mOsm/kg): 12311 - Unflavored 49735-0123-01 B4162 or B4157 30 x 50 g (1.8 oz) 153 L-Glutamine 4.9 1.23 Molybdenum, mcg 37 9 Artificial Colors (Beta Carotene, Beet Red). Dilution Unflavored Orange Chromium, mcg 26 7 Contains: Soy 1 to 7 770 790 WIC‡: Contact your state WIC For more reimbursement Selenium, mcg 58 15 Metabolics Metabolics 1 to 5 1090 1140 office to determine availability. information see Resources. Sodium, mg 560 140 • Displacement: 0.6 mL/g Potassium, mg 700 175 * S9435 = For individuals who submit their bills to insurance on their own. Chloride, mg 560 140 66 67 Ingredients: PKU Lophlex LQ Nutrition Information: Water, and less than 2% of each of the ® Per Pouch Per 10 g Per Pouch Per 10 g following: L-Leucine, Citric Acid, L-Proline, PKU Lophlex LQ Nutrients (125 mL) Protein Equivalent Vitamins (125 mL) Protein Equivalent L-Arginine, Glycine, L-Tyrosine, L-Lysine Acetate, L-Valine, L-Aspartic Acid, Calories 120 60 Vitamin A, IU (mcg RE) 949 (285) 475 (143) A phenylalanine-free*, ready-to-drink medical food containing amino acids, L-Isoleucine, Dicalcium Phosphate, L-Alanine, Protein Equivalent, g 20 10 Vitamin D3, IU (mcg) 320 (8) 160 (4) L-Serine, L-Threonine, L-Histidine, L-Lysine, vitamins and minerals and DHA, based on fruit juice (from concentrate) with Fat, g 0.44 0.2 Vitamin E, IU (mg α-TE) 4.8 (3.2) 2.4 (1.6) L-Aspartate, Maltodextrin, Magnesium natural flavor; for the dietary management of proven phenylketonuria (PKU) in DHA, mg 150 75 Vitamin K, mcg 24.9 12.5 Acetate, L-Cystine, Choline Bitartrate, individuals 4 years and older, including pregnant women and women of Carbohydrate, g 9.3 4.7 Thiamin, mg 0.54 0.27 L-Tryptophan, Sugar, C. Cohnii Oil**, N-Acetyl child-bearing age. Fiber, g 0.5 0.25 Riboflavin, mg 0.5 0.25 L-Methionine, Microcrystalline Cellulose, L-Ascorbic Acid, Taurine, L-Methionine, Guar Amino Acids, g Vitamin B , mg 0.58 0.29 6 Gum, Sunflower Lecithin, Xanthan Gum, L-Alanine 1.16 0.58 Vitamin B12, mcg 1.8 0.90 M-Inositol, Potassium Sorbate (preservative Main Features: L-Arginine 2 1 Niacin, mg 7.1 3.6 to delay spoilage), Ferrous Lactate, Cellulose Indications: L-Aspartic Acid 1.75 0.88 Folic Acid, mcg 120 60 Gum, Artificial Sweetener: Acesulfame • Phenylalanine free • Phenylketonuria L-Cystine 0.51 0.26 Pantothenic Acid, mg 1.8 0.9 Potassium, L-Carnitine, Sodium Benzoate (preservative to delay spoilage), Zinc Sulfate, • Low volume; provides 20 g PE in just Glycine 1.88 0.94 Biotin, mcg 53.4 26.7 L-Histidine 0.79 0.40 Vitamin C, mg 17.8 8.9 Niacinamide, DL-alpha Tocopheryl Acetate, 4.2 fl oz (125 mL) Calcium D-Pantothenate, Manganese For details on administration, please see the end of this section. L-Isoleucine 1.24 0.62 Choline, mg 153 76.5 • Convenient ready-to-drink Sulfate, Cupric Sulfate, Thiamin Chloride L-Leucine 2.13 1.1 Inositol, mg 40.6 20.3 Hydrochloride, Pyridoxine Hydrochloride, • Made with real fruit juice from L-Lysine 1.63 0.82 Minerals Vitamin A Palmitate, Riboflavin, Folic Acid, concentrate Ordering/Reimbursement Information: L-Methionine 0.34 0.17 Calcium, mg 319 160 Potassium Iodide, Ascorbyl Palmitate, Mixed L-Phenylalanine Trace* Trace* Phosphorus, mg 244 122 Tocopherols, Sodium Molybdate, D-Biotin, • Contains a balanced mixture of Product Reimbursement HCPCS Product Calories Code Code Code* Packaging Per Pouch L-Proline 2 1 Magnesium, mg 96.3 48.2 Sodium Selenite, Chromium Chloride, other essential and non-essential amino Phylloquinone, Vitamin D , Cyanocobalamin. 86051 49735-0161-30 B4157 and 30 x 4.2 fl oz 120 L-Serine 1.09 0.55 Iron, mg 5.1 2.6 3 acids, carbohydrates, vitamins and Juicy Orange B4162 (125 mL) L-Threonine 1.04 0.52 Zinc, mg 3.8 1.9 **A Source Of Docosahexaenoic Acid (DHA) minerals 86021 49735-0161-29 B4157 and 30 x 4.2 fl oz 120 L-Tryptophan 0.41 0.21 Manganese, mg 0.56 0.28 Juicy Orange flavored version also contains: • Provides 150 mg of DHA per pouch Mixed Berry Blast B4162 (125 mL) L-Tyrosine 1.88 0.94 Copper, mcg 563 282 Orange Juice from Concentrate (45.5%), 86055 49735-0195-35 B4157 and 30 x 4.2 fl oz 120 L-Valine 1.38 0.69 Iodine, mcg 56.3 28.2 Corn Syrup Solids, and less than 2% of • Available in juicy orange, mixed berry Juicy Tropical B4162 (125 mL) L-Carnitine 0.01 0.01 Molybdenum, mcg 25 12.5 the following: Natural Flavor, Artificial blast and juicy tropical flavors Sweetener: Sucralose. Taurine 0.1 0.05 Chromium, mcg 10.8 5.4 ‡ • Osmolality (at 0.96 kcal/mL): WIC : Contact your state WIC For more reimbursement Selenium, mcg 26.8 13.4 Mixed Berry Blast flavored version also

Metabolics Metabolics office to determine availability. information see . 2460 mOsm/kg Resources Sodium, mg <25 <12.5 contains: Apple Juice from Concentrate (34.1%), Grape Juice from Concentrate (6.9%), * S9435 = For individuals who submit their bills to insurance on their own. Potassium, mg 90 45 Blackcurrant Juice from Concentrate (2.5%), *May contain traces of phenylalanine (PHE) from fruit juice (from concentrate). Chloride, mg <25 <12.5 and less than 2% of the following: Elderberry 68 Juice from Concentrate (0.6%), Natural 69 Flavor, Concentrate (Apple, Blackcurrant, Radish), Artificial Sweetener: Sucralose. Juicy Tropical flavored version also contains: PKU Lophlex Powder Nutrition Information: Ingredients: Nutrients Per 100 g Per sachet (14.3 g) Vitamins Per 100 g Per sachet (14.3 g) Berry: L-Lysine L-Aspartate, L-Leucine, Calories 286 41 Vitamin A, IU (mcg RE) 3896 (1170) 557 (167) L-Tyrosine, L-Proline, L-Arginine, Calcium ® Phosphate Tribasic, L-Valine, Glycine, PKU Lophlex Powder Protein Equivalent, g 70 10 Vitamin D3, IU (mcg) 580 (14.5) 82.9 (2.1) L-Isoleucine, L-Threonine, Magnesium Fat, g 0.2 0.03 Vitamin E, IU (mg α-TE) 32 (21.5) 4.6 (3.1) Hydrogen Phosphate, L-Glutamine, L-Serine, Carbohydrate, g 1 0.14 Vitamin K, mcg 143 20.4 L-Histidine, L-Alanine, Citric Acid, L-Cystine, A low volume, phenylalanine-free, powdered medical food for the dietary N-Acetyl L-Methionine, Choline Bitartrate, management of phenylketonuria (PKU) in older children and adults, including Amino Acids, g Thiamin, mg 1.6 0.23 L-Tryptophan, Calcium Phosphate Monobasic, L-Alanine 3.3 0.47 Riboflavin, mg 1.7 0.24 Guar Gum, Artificial Flavor, Soy Lecithin, pregnant women and women of child-bearing age. L-Ascorbic Acid, M-Inositol, Artificial L-Arginine 5.8 0.83 Vitamin B6, mg 2.8 0.4 Sweetener: Acesulfame Potassium*, Taurine, L-Aspartic Acid 5.4 0.77 Vitamin B12, mcg 5.5 0.79 Ferrous Sulfate, L-Carnitine, Zinc Sulfate, L-Cystine 2.2 0.31 Dl-Alpha Tocopheryl Acetate, Niacinamide, Niacin, mg 10 1.4 Manganese Sulfate, Calcium D-Pantothenate, Glycine 5.1 0.73 Folic Acid, mcg 860 123 Cupric Sulfate, Pyridoxine Hydrochloride, Main Features: L-Histidine 3.3 0.47 Pantothenic Acid, mg 8.6 1.2 Thiamin Chloride Hydrochloride, Riboflavin, Indications: Vitamin A Acetate, Folic Acid, Beet Juice (For • Phenylalanine free L-Isoleucine 5.1 0.73 Biotin, mcg 43 6.1 Color), Potassium Iodide, Chromium Chloride, • Phenylketonuria L-Leucine 8.7 1.24 Vitamin C, mg 110 15.7 Sodium Selenite, Sodium Molybdate, • Low volume and low calorie Phylloquinone, D-Biotin, Vitamin D3, L-Lysine 5.9 0.84 Choline, mg 720 103 Cyanocobalamin. • Contains vitamins, minerals, and For details on administration, please see the end of this section. L-Methionine 1.4 0.2 Inositol, mg 140 20 Orange L-Lysine L-Aspartate, L-Leucine, trace elements L-Phenylalanine Nil Added Nil Added Minerals L-Tyrosine, L-Proline, L-Arginine, Calcium L-Proline 6.2 0.89 Calcium, mg 2070 296 Phosphate Tribasic, L-Valine, Glycine, • Age-appropriate DRI formulation L-Isoleucine, Magnesium Hydrogen L-Serine 3.8 0.54 Phosphorus, mg 1990 285 • Lophlex provides 10 g of protein Phosphate, L-Threonine, L-Glutamine, Ordering/Reimbursement Information: L-Threonine 4.3 0.61 Magnesium, mg 535 76.5 L-Serine, L-Histidine, L-Alanine, Citric Acid, equivalent per sachet L-Tryptophan 1.7 0.24 Iron, mg 25.7 3.7 L-Cystine, N-Acetyl L-Methionine, Choline Product Reimbursement HCPCS Product Calories Bitartrate, L-Tryptophan, Calcium Phosphate • Available in berry and orange flavors Code Code Code* Packaging Per Sachet L-Tyrosine 7.8 1.12 Zinc, mg 15.8 2.3 Monobasic, Artificial Flavor, Guar Gum, Soy L-Valine 5.6 0.8 Manganese, mg 3.1 0.44 Lecithin, L-Ascorbic Acid, M-Inositol, Artificial • Osmolality (mOsm/kg): 12169 - Berry 49735-0121-69 B4157 or 30 x 14.3 g (0.5 oz) 42 Sweetener: Acesulfame Potassium*, Taurine, B4162 L-Carnitine 0.06 0.01 Copper, mcg 1430 204 Dilution Orange Berry Ferrous Sulfate, L-Carnitine, Zinc Sulfate, 12167 - Orange 49735-0121-67 B4157 or 30 x 14.3 g (0.5 oz) 42 Taurine 0.11 0.02 Iodine, mcg 229 32.7 Dl-Alpha Tocopheryl Acetate, Niacinamide, 1 to 5 1390 1340 L-Glutamine 3.95 0.56 Manganese Sulfate, Calcium D-Pantothenate, B4162 Molybdenum, mcg 65 9.3 Cupric Sulfate, Pyridoxine Hydrochloride, Chromium, mcg 46 6.6 Thiamin Chloride Hydrochloride, Riboflavin, Vitamin A Acetate, Beet Juice (For Color), ‡ Selenium, mcg 101 14.4 WIC : Contact your state WIC For more reimbursement Beta Carotene (For Color), Folic Acid, Sodium, mg <20 <3 Metabolics Metabolics office to determine availability. information see Resources. Potassium Iodide, Chromium Chloride, Potassium, mg <40 <6 Sodium Selenite, Sodium Molybdate, Phylloquinone, D-Biotin, Vitamin D3, Chloride, mg <1 <1 Cyanocobalamin. * S9435 = For individuals who submit their bills to insurance on their own. Both Flavors 70 Contains: Soy. 71 Phlexy-10 Tablets

Nutrition Information: Ingredients: Phlexy-10® Tablets Per 10 Per 12 Microcrystalline Cellulose, L-Leucine, Nutrients Tablets Tablets L-Tyrosine, L-Lysine Hydrochloride, Calories 41 49 L-Proline, L-Arginine, L-Valine, L-Aspartic A phenylalanine-free medical food in tablet form for the dietary Protein Equivalent, g 8.3 10.0 Acid, Pregelatinized Corn Starch, Glycine, management of phenylketonuria (PKU) in individuals over 8 years of Fat, g 0.38 0.46 L-Isoleucine, L-Threonine, L-Glutamine, age, including pregnant women and women of child-bearing age. Saturated, g 0.36 0.43 L-Serine, L-Alanine, L-Histidine, Magnesium Carbohydrate, g 3.7 4.3 Stearate, L-Cystine, Hydroxypropyl Methyl Fiber, g 3.0 3.5 Cellulose, L-Tryptophan, Acacia Gum, Amino Acids, g L-Methionine, Stearic Acid, Glycerol, L-Alanine 0.41 0.49 Taurine, L-Carnitine. L-Arginine 0.72 0.86 Main Features: Indications: L-Aspartic Acid 0.68 0.82 L-Cystine 0.27 0.32 • Phenylalanine free • Phenylketonuria L-Glutamic Acid Nil Added Nil Added • Every 12 tablets provide 10 g of Glycine 0.64 0.77 L-Histidine 0.41 0.49 protein equivalent For details on administration, please see the end of this section. L-Isoleucine 0.64 0.77 • A great alternative to PKU liquid L-Leucine 1.09 1.3 consumption L-Lysine 0.74 0.89 L-Methionine 0.17 0.20 Ordering/Reimbursement Information: L-Phenylalanine Nil Added Nil Added L-Proline 0.77 0.92 Product Reimbursement HCPCS Product Code Code Code* Packaging Calories L-Serine 0.47 0.56 L-Threonine 0.54 0.65 11951 - Tablets 49735-0019-51 A9270 75/bottle 285/bottle L-Tryptophan 0.22 0.26 L-Tyrosine 0.97 1.2 L-Valine 0.7 0.84 WIC‡: Contact your state WIC For more reimbursement L-Carnitine 0.01 0.01 office to determine availability. information see Resources. Taurine 0.01 0.02 L-Glutamine 0.50 0.60 Metabolics Metabolics

72 * S9435 = For individuals who submit their bills to insurance on their own. 73 Phlexy-10 Drink Mix

® Nutrition Information: Ingredients: Phlexy-10 Drink Mix Per 20 g Phlexy-10 Drink Mix (blackcurrant/ Pouch apple): Sucrose, L-Lysine Aspartate, A phenylalanine-free powdered medical food for the dietary Nutrients L-Leucine, L-Tyrosine, Artificial Flavors, management of phenylketonuria (PKU) in children and adults, Calories 69 L-Proline, L-Arginine, L-Valine, L-Isoleucine, including pregnant women and women of child-bearing age. Protein Equivalent, g 8.33 Glycine, Malic Acid, L-Threonine, Fat, g Nil Added L-Glutamine, Corn Syrup Solids, L-Serine, Saturated, g Nil Added L-Histidine, L-Alanine, L-Cystine, N-Acetyl Monounsaturated, g Nil Added L-Methionine, L-Tryptophan, Soy Lecithin, Polyunsaturated, g Nil Added Taurine, L-Carnitine, Artificial Sweetener: Acesulfame Potassium, Artificial Color. Main Features: Indications: Carbohydrate, g 8.8 Fiber, g Nil Added Phlexy-10 Drink Mix (tropical surprise): • Phenylalanine free • Phenylketonuria Amino Acids, g Sucrose, L-Lysine Aspartate, Artificial L-Alanine 0.41 Flavors, L-Leucine, L-Tyrosine, L-Proline, • Available in black currant/apple or L-Arginine, L-Valine, Citric Acid, Glycine, For details on administration, please see the end of this section. L-Arginine 0.72 tropical surprise flavor L-Aspartic Acid 0.68 L-Isoleucine, L-Threonine, L-Glutamine, Corn Syrup Solids, L-Serine, L-Histidine, • Each pouch provides 8.33 g PE L-Cystine 0.27 L-Alanine, L-Cystine, N-Acetyl L-Methionine, Glycine 0.64 • Convenient, pre-measured single L-Tryptophan, Soy Lecithin, Artificial L-Histidine 0.41 serve pouches Sweetener: Acesulfame Potassium, Taurine, Ordering/Reimbursement Information: L-Isoleucine 0.64 L-Carnitine, Artificial Color. • Osmolality (mOsm/kg): L-Leucine 1.09 Product Reimbursement HCPCS Product Contains: Soy. Dilution Blackcurrant- Tropical Code Code Code* Packaging Calories L-Lysine 0.74 L-Methionine 0.17 Must combine with an appropriate vitamin Apple Surprise 11467 - Drink Mix 49735-0114-67 B4157 or 30 x 20 g (0.7 oz) 69/pouch and mineral supplement when taken as the (Blackcurrant-Apple) B4162 L-Phenylalanine Nil Added 1 to 5 1172 1140 L-Proline 0.77 sole source of amino acid protein. 11910 - Drink Mix 49735-0119-10 B4157 or 30 x 20 g (0.7 oz) 69/pouch L-Serine 0.47 (Tropical Surprise) B4162 L-Threonine 0.54 L-Tryptophan 0.22 WIC‡: Contact your state WIC For more reimbursement L-Tyrosine 0.97 L-Valine 0.7 office to determine availability. information see Resources. L-Carnitine 0.007

Metabolics Metabolics Taurine 0.014 L-Glutamine 0.5 * S9435 = For individuals who submit their bills to insurance on their own.

74 75 MSUD Anamix Early Years

Ingredients: ® Nutrition Information: MSUD Anamix Early Years Per 10 g Per 10 g Corn Syrup Solids, Refined Vegetable Nutrients Per 100 g Protein Equivalent Vitamins Per 100 g Protein Equivalent Oils (High Oleic Sunflower, Soy, A valine-, leucine-, and isoleucine-free powdered infant formula with iron, for the Calories 473 350 Vitamin A, IU (mcg RE) 1305.(392) 967.(290) Non-Hydrogenated Coconut, Canola, dietary management of maple syrup urine disease (MSUD). Contains DHA and Protein Equivalent,g 13.5 10.0 Vitamin D3, IU (mcg) 348.(8.7) 258.(6.4) Sunflower), Galacto-oligosaccharides ARA, and a unique blend of prebiotic fiber. Fat, g 23.0 17.0 Vitamin E, IU (mg α-TE) 10.9 (7.3) 8.1 (5.4) (GOS) Dried Syrup (from milk), L-Lysine Saturated, g 7.5 5.6 Vitamin K, mcg 38.0 28.1 L-Glutamate, L-Proline, L-Arginine, Monounsaturated, g 9.4 7.0 Thiamin, mcg 640 474 Glycine, L-Aspartic Acid, Tricalcium Polyunsaturated, g 5.0 3.7 Riboflavin, mcg 500 370 Phosphate, L-Threonine, CAEM (an Main Features: Indications: DHA, mg 70.0 51.9 Vitamin B6, mcg 500 370 emulsifier), L-Tyrosine, L-Phenylalanine, • Maple syrup urine disease (MSUD) ARA, mg 140 104 Vitamin B12, mcg 1.2 0.89 L-Serine, L-Histidine, L-Alanine, Potassium • Valine-, leucine-, and isoleucine-free Linoleic Acid,mg 3500 2593 Niacin, mcg 2200 1630 Chloride, Fructo-oligosaccharides powdered infant formula with iron Carbohydrate, g 53.0 39.3 Folic Acid, mcg 55.0 40.7 (FOS), Tripotassium Citrate, L-Cystine, For details on administration and household measurements, please • Includes DHA & ARA Fiber, g 5.3 3.9 Pantothenic Acid, mcg 2800 2074 L-Tryptophan, Sodium Phosphate Dibasic, see the end of this section. GOS, g 4.7 3.5 Biotin, mcg 18.2 13.5 Magnesium Acetate, L-Methionine, • Contains a prebiotic fiber blend FOS, g 0.55 0.41 Vitamin C, mg 49.0 36.3 M. Alpina Oil*, Choline Bitartrate, C. Cohnii • Unflavored powder Amino Acids, g Choline, mg 91.0 67.4 Oil**, L-Glutamine, Sodium Chloride, L-Alanine 0.82 0.61 Inositol, mg 98.0 72.6 M-Inositol, L-Ascorbic Acid, L-Glutamic • Contains a balanced mixture of all L-Arginine 1.4 1.0 Minerals Acid, Taurine, Ferrous Sulfate, Zinc other essential and non-essential L-Aspartic Acid 1.2 0.89 Calcium, mg 371 275 Sulfate, L-Carnitine, DL-alpha Tocopheryl amino acids, carbohydrates, fat, Ordering/Reimbursement Information: L-Cystine 0.52 0.39 Phosphorus, mg 270 200 Acetate, Calcium D-Pantothenate, L-Glutamic Acid 1.6 1.2 Magnesium, mg 52.5 38.9 Niacinamide, Ascorbyl Palmitate, vitamins, minerals and trace elements Product Reimbursement HCPCS Product Calories L-Glutamine 0.13 0.10 Iron, mg 7.3 5.4 Manganese Sulfate, Cupric Sulfate, Code Code Code* Packaging Per Can • Provides 13.5 g of protein equivalent Glycine 1.29 0.96 Zinc, mg 5.2 3.9 Thiamine Chloride Hydrochloride, per 100 g of powder 90168 49735-0101-68 B4162 6 x 400 g (14 oz) 1892 L-Histidine 0.8 0.59 Manganese, mcg 390 289 Pyridoxine Hydrochloride, Riboflavin, L-Isoleucine Nil Added Nil Added Copper, mcg 390 289 Vitamin A Acetate, DL-alpha Tocopherol, • Osmolality (at 0.7 kcal/mL): L-Leucine Nil Added Nil Added Iodine, mcg 82.5 61.1 Mixed Tocopherols, Potassium Iodide, ‡ 380 mOsm/kg WIC : Contact your state WIC For more reimbursement L-Lysine 1.45 1.1 Molybdenum, mcg 10.9 8.1 Chromium Chloride, Folic Acid, Based on dilution of 5 g powder office to determine availability. information see Resources. L-Methionine 0.34 0.25 Chromium, mcg 12.5 9.3 Phylloquinone, Sodium Selenite, Sodium L-Phenylalanine 0.94 0.70 (1 unpacked level scoop) to 1 fl oz Selenium, mcg 14.1 10.4 Molybdate, D-Biotin, Vitamin D3, L-Proline 1.56 1.2 Sodium, mg 171 127 Cyanocobalamin. (30 mL) water L-Serine 0.96 0.71 Potassium, mg 454 336 Contains: Soy Oil, Coconut Oil, Milk. • Displacement: 0.75 mL/g L-Threonine 1.05 0.78 Chloride, mg 321 238 L-Tryptophan 0.42 0.31 *A source of Arachidonic Acid (ARA) Metabolics Metabolics L-Tyrosine 0.94 0.70 Visit NutriciaLearningCenter.com * S9435 = For individuals who submit their bills to insurance on their own. L-Valine Nil Added Nil Added **A source of Docosahexaenoic Acid (DHA) for guidelines for the nutritional L-Carnitine 0.01 0.01 Taurine 0.02 0.01 Contains GOS/FOS 0.8 g per 100 mL 76 management of MSUD. 77 Complex Junior MSD Drink Mix

Nutrition Information: Ingredients: Nutrients Per 100 g Per 100 kcal Vitamins Per 100 g Per 100 kcal Corn Syrup Solids, Maltodextrin, Modified Calories 494 100 Vitamin A, IU (mcg RE) 1187.(356) 240.(72) Complex Junior MSD Drink Mix Corn Starch, Canola Oil, Safflower Oil, Protein Equivalent, g 13 2.6 Vitamin D3, IU (mcg) 429.(10.7) 87.(2.2) Coconut Oil, L-Glutamine, Soybean Oil. Fat, g 29 6 Vitamin E, IU (mg -TE) 13.8 (9.2) 2.8 (1.9) A valine-, leucine-, and isoleucine-free powdered medical food for the dietary management α Contains 2% or less of the following: Saturated, g 10.2 2.1 Vitamin K, mcg 62 12.6 Tricalcium Phosphate, L-Lysine Acetate, of maple syrup urine disease (MSUD) in young children. Monounsaturated, g 13.4 2.7 Thiamin, mg 1.1 0.22 L-Alanine, L-Phenylalanine, L-Tyrosine, Polyunsaturated, g 5.0 1.0 Riboflavin, mg 0.81 0.16 Sodium Citrate, Dipotassium Phosphate, DHA, mg 88 17.9 Vitamin B6, mg 0.78 0.16 L-Threonine, L-Histidine, L-Asparagine ARA, mg 88 17.9 Vitamin B12, mcg 2 0.4 Linoleic Acid, mg 3730 755 Niacin, mg 5.8 1.2 Monohydrate, L-Proline, L-Tryptophan, Main Features: Indications: Carbohydrate, g 46 9 Folic Acid, mcg 140 28 Choline Bitartrate, L-Arginine, Ascorbyl Amino Acids, g Pantothenic Acid, mg 3.4 0.69 Palmitate, Mixed Tocopherols, • Valine, leucine, and isoleucine free • Maple syrup urine disease (MSUD) L-Alanine 1.40 0.28 Biotin, mcg 36 7.2 Magnesium Chloride, Rosemary Extract, L-Arginine 0.66 0.13 Vitamin C, mg 78 15.8 L-Serine, Potassium Citrate, Potassium • Amino acid profile specifically L-Asparagine 0.75 0.15 Choline, mg 244 49 Chloride, L-Glutamic Acid, L-Methionine, designed for MSUD; clinically L-Cystine 0.29 0.06 Inositol, mg 148 30 Glycine, Silicon Dioxide, L-Cystine, C. Cohnii Oil,* Glucose Syrup Solids, † For details on administration and household measurements, please L-Glutamic Acid 0.45 0.09 Minerals researched in one study Mono- and Diglycerides, see the end of this section. L-Glutamine 3.71 0.75 Calcium, mg 596 121 M. Alpina • Includes DHA & ARA Glycine 0.35 0.07 Phosphorus, mg 444 90 Oil,** Inositol, Ascorbic Acid, Zinc L-Histidine 0.92 0.19 Magnesium, mg 68 13.8 Sulfate, Taurine, Ferrous Sulfate, • Essential vitamins and minerals to L-Isoleucine Nil Added Nil Added Iron, mg 14.7 3 High Oleic Sunflower Oil, Mannitol, help meet needs of young children L-Leucine Nil Added Nil Added Zinc, mg 29 5.8 L-Carnitine, Vitamin E Acetate, Glycerol, with MSUD Ordering/Reimbursement Information: L-Lysine 1.17 0.24 Manganese, mg 0.44 0.09 Mono- and Distearate, Natural Flavor, L-Methionine 0.38 0.08 Copper, mcg 952 193 Sodium Polyphosphate, Sunflower • Mild neutral flavor Product Reimbursement HCPCS Product Calories L-Phenylalanine 1.14 0.23 Iodine, mcg 96 19.4 Lecithin, Sodium Ascorbate, Vitamin Code Code Code* Packaging Per Can L-Proline 0.76 0.16 Molybdenum, mcg 15.8 3.2 K1, Niacinamide, Vitamin A Palmitate, L-Serine 0.58 0.12 Chromium, mcg 16.2 3.3 • Provides 13 g of protein equivalent 5910 00847-0591-02 B4157 or B4162 4 x 400 g (14.1 oz) 1976 Vitamin D3, D-Calcium Pantothenate, per 100 g of powder L-Threonine 0.94 0.19 Selenium, mcg 44 8.9 D-Biotin, Copper Sulfate, Manganese L-Tryptophan 0.69 0.14 Sodium, mg 303 61 ‡ Sulfate, Thiamin Hydrochloride, • Displacement: 0.7 mL/g WIC : Contact your state WIC For more reimbursement L-Tyrosine 1.14 0.23 Potassium, mg 797 161 Potassium Iodide, Pyridoxine office to determine availability. information see Resources. L-Valine Nil Added Nil Added Chloride, mg 384 78 Hydrochloride, Sodium Selenite, L-Carnitine 0.03 0.01 Riboflavin, Vitamin 12B , Folic Acid, Taurine 0.07 0.01 Chromium Chloride, Sodium Molybdate. Metabolics Metabolics * S9435 = For individuals who submit their bills to insurance on their own. Contains: Coconut Oil, Soybean Oil. † Strauss KA et al. Mol Genet Metab. 2010;99:333-45. * A source of Docosahexaenoic Acid (DHA) ** A source of Arachidonic Acid (ARA) 78 79 Complex Essential MSD Drink Mix

Nutrition Information: Ingredients: Complex Essential MSD Drink Mix Per 10 g Protein Per 10 g Protein Sugar, L- Glutamine, Modified Food Equivalent Equivalent Starch, Dextrin, Canola Oil, Maltodextrin, Nutrients Per 100 g (1 scoop) Nutrients Per 100 g (1 scoop) Safflower Oil, L-Lysine Acetate, A valine-, leucine-, and isoleucine-free powdered medical food for the dietary management Calories 380 152 Vitamin A, IU (mcg RE) 1700 (637) 680 (255) L-Alanine, Natural and Artificial Flavor, of maple syrup urine disease (MSUD) in children and adults, including pregnant women and Protein Equivalent, g 25 10 (25% as beta-carotene) Tricalcium Phosphate, Potassium women of child bearing age. Fat, g 12 5 Vitamin D3, IU (mcg) 600 (15) 240 (6) Citrate, L-Phenylalanine, L-Tyrosine. Saturated, g 2.8 1.1 Vitamin E, IU (mg α-TE) 18.4 (12.3) 7.3 (4.9) Contains 2% or less of the following: Monounsaturated, g 6.3 2.5 Vitamin K, mcg 50 20 L-Threonine, L-Histidine, L-Asparagine Polyunsaturated, g 2.7 1.1 Thiamin, mg 1.5 0.6 Monohydrate, Magnesium Phosphate, Linoleic Acid, mg 1733 693 Riboflavin, mg 1.5 0.6 Sodium Chloride, L-Proline, Dipotassium Main Features: Indications: α-Linolenic Acid, mg 662 265 Vitamin B6, mg 1.3 0.52 Phosphate, L-Tryptophan, L-Arginine, • Maple syrup urine disease (MSUD) Carbohydrate, g 47 19 Vitamin B12, mcg 2 0.8 L-Serine, Coconut Oil, Soybean Oil, • Valine, leucine, and isoleucine free Fiber, g 3.9 1.5 Niacin, mg 12 4.8 L-Glutamic Acid, L-Methionine, Glycine, • Amino acid profile specifically Amino Acids, g Folic Acid, mcg 250 100 L-Cystine, Flaxseed Oil, Titanium Dioxide, designed for MSUD; clinically L-Alanine 2.70 1.08 Pantothenic Acid, mg 5.5 2.2 Choline Bitartrate, Ascorbyl Palmitate, L-Arginine 1.28 0.51 Biotin, mcg 30 12 Mixed Tocopherols, Rosemary Extract, † For details on administration and household measurements, please researched in one study L-Asparagine 1.45 0.58 Vitamin C, mg 60 24 Sodium Citrate, Taurine, Ascorbic Acid, see the end of this section. • Complete with essential vitamins L-Cystine 0.56 0.22 Choline, mg 100 40 L-Carnitine, Ferrous Sulfate, Inositol, L-Glutamic Acid 0.86 0.35 Inositol, mg 40 16 Vitamin E Acetate, Zinc Sulfate, Calcium and minerals L-Glutamine 7.17 2.87 Minerals Phosphate, Niacinamide, Copper Glycine 0.67 0.27 Calcium, mg 886 355 Gluconate, D-Calcium Pantothenate, • Provides 25 g of protein equivalent Ordering/Reimbursement Information: L-Histidine 1.79 0.71 Phosphorus, mg 864 346 Vitamin D3, Vitamin A Palmitate, Vitamin per 100 g powder L-Isoleucine Nil Added Nil Added Magnesium, mg 200 80 Product Reimbursement HCPCS Product Calories K1, Manganese Sulfate, Mono –and Code Code Code* Packaging Per Can L-Leucine Nil Added Nil Added Iron, mg 13 5 Diglycerides, D-Biotin, Beta-carotene, • Vanilla flavor L-Lysine 2.27 0.91 Zinc, mg 12 4.8 Chromium Chloride, Sodium Selenite, 5972 00847-0597-22 B4157 or B4162 4 x 454 g (1 lb) 1725 L-Methionine 0.74 0.30 • Osmolality (at 0.63 kcal/mL): Manganese, mg 1.5 0.6 Pyridoxine Hydrochloride, Riboflavin, L-Phenylalanine 2.20 0.88 Copper, mcg 1000 400 Potassium Iodide, Thiamin Mononitrate, 813 mOsm/kg L-Proline 1.48 0.59 Iodine, mcg 120 48 Folic Acid, Sodium Molybdate, Based on dilution of 40 g powder WIC‡: Contact your state WIC For more reimbursement L-Serine 1.12 0.45 Molybdenum, mcg 75 30 Vitamin B12. (1 scoop) to 7 fl oz (210 mL) water office to determine availability. information see Resources. L-Threonine 1.81 0.72 Chromium, mcg 40 16.1 L-Tryptophan 1.33 0.53 Selenium, mcg 100 40 Contains: Coconut Oil, Soybean Oil. • Displacement: 0.75 mL/g L-Tyrosine 2.20 0.88 Sodium, mg 600 240

Metabolics Metabolics * S9435 = For individuals who submit their bills to insurance on their own. L-Valine Nil Added Nil Added Potassium, mg 1301 520 L-Carnitine 0.06 0.02 Chloride, mg 860 344 Taurine 0.14 0.06 † Strauss KA et al. Mol Genet Metab. 2010;99:333-45. 80 81 Complex MSD Amino Acid Blends

Nutrition Information: Ingredients: Per 10 g Protein Per 10 g L-Glutamine, L-Lysine Acetate, L-Alanine, Complex MSD Amino Acid Blend Equivalent Protein Equivalent L-Phenylalanine, L-Tyrosine, L-Threonine, Nutrients Per 100 g (1 scoop) Amino Acids, g Per 100 g (1 scoop) L-Histidine, L-Asparagine Monohydrate, A valine-, leucine-, and isoleucine-free powdered medical food for the dietary management of Calories 323 40 L-Alanine 8.72 1.08 L-Proline, L-Tryptophan, L-Arginine, maple syrup urine disease (MSUD) in children and adults. Protein Equivalent, g 81 10 L-Arginine 4.13 0.51 L-Serine, L-Glutamic Acid, L-Methionine, Fat, g 0 0 L-Asparagine 4.67 0.58 Glycine, L-Cystine, Taurine, Silicon Carbohydrate, g 0 0 L-Cystine 1.80 0.22 Dioxide, L-Carnitine. L-Glutamic Acid 2.79 0.35 L-Glutamine 23.12 2.87 Note: Must combine with an appropriate Glycine 2.16 0.27 Main Features: Indications: vitamin & mineral supplement when L-Histidine 5.76 0.71 • Maple syrup urine disease (MSUD) taken as the sole source of amino acid • Valine, leucine, and isoleucine free L-Isoleucine Nil Added Nil Added protein. L-Leucine Nil Added Nil Added • Amino acid profile specifically For details on administration and household measurements, please L-Lysine 7.32 0.91 designed for MSUD; clinically see the end of this section. L-Methionine 2.38 0.30 researched in one study† L-Phenylalanine 7.08 0.88 L-Proline 4.76 0.59 • Does not contain fat, carbohydrates, L-Serine 3.60 0.45 vitamins and minerals L-Threonine 5.83 0.72 L-Tryptophan 4.28 0.53 • 40 calories per serving Ordering/Reimbursement Information: L-Tyrosine 7.08 0.88 • Provides 81 g of protein equivalent L-Valine Nil Added Nil Added per 100 g of powder Product Reimbursement HCPCS Product Calories L-Carnitine 0.19 0.02 Code Code Code* Packaging Per Can Taurine 0.46 0.06 • Unflavored 5900 00847-0590-00 B4155 or B9998 4 x 454 g (1 lb) 1466

• Displacement: 0.48 mL/g WIC‡: Contact your state WIC For more reimbursement office to determine availability. information see Resources. Metabolics Metabolics † Strauss KA et al. Mol Genet Metab. 2010;99:333-45. * S9435 = For individuals who submit their bills to insurance on their own.

82 83 MSUD Maxamum

Nutrition Information: Ingredients: ® Per 10 g Per 10 g MSUD Maxamum Nutrients Per 100 g Protein Equivalent Vitamins Per 100 g Protein Equivalent Sugar, L-Lysine L-Glutamate, Corn Syrup Calories 305 76 Vitamin A, IU (mcg RE) 2228 (669) 557 (167) Solids, Calcium Phosphate Dibasic, L-Proline, L-Arginine, Glycine, L-Aspartic Protein Equivalent, g 40 10 Vitamin D , IU (mcg) 332 (8.3) 83 (2.1) A valine-, leucine-, and isoleucine-free powdered medical food for the dietary 3 Acid, L-Threonine, L-Phenylalanine, Fat, g <1 <0.25 management of maple syrup urine disease (MSUD) in older children and adults, including Vitamin E, IU (mg α-TE) 18.3 (12.3) 4.6 (3.1) L-Tyrosine, L-Serine, Artificial Flavor, pregnant women and women of child-bearing age. Carbohydrate, g 34 8.5 Vitamin K, mcg 82 21 L-Histidine, L-Alanine, Citric Acid, Amino Acids, g Thiamin, mg 0.91 0.23 Magnesium Acetate, L-Cystine, L-Alanine 2.4 0.60 Riboflavin, mg 0.97 0.24 L-Tryptophan, Tripotassium Citrate, Choline Bitartrate, L-Methionine, L-Arginine 4.1 1.03 Vitamin B6, mg 1.6 0.4 L-Aspartic Acid 3.4 0.85 Vitamin B , mcg 3.1 0.8 Sodium Chloride, Soy Lecithin, Disodium 12 Hydrogen Phosphate, Dipotassium Main Features: Indications: L-Cystine 1.6 0.40 Niacin, mg 5.7 1.4 Hydrogen Phosphate, L-Glutamine, • Valine, leucine, and isoleucine free • Maple syrup urine disease (MSUD) L-Glutamic Acid 4.6 1.15 Folic Acid, mcg 491 123 Tricalcium Phosphate, L-Glutamic Acid, Glycine 3.7 0.93 Pantothenic Acid, mg 4.9 1.2 • Contains a balanced mixture of all other Taurine, L-Ascorbic Acid, M-Inositol, L-Histidine 2.3 0.58 Biotin, mcg 24.5 6.1 Ferrous Sulfate, L-Carnitine, Zinc Sulfate, essential and non-essential amino acids, L-Isoleucine Nil Added Nil Added Vitamin C, mg 62 16 DL-Alpha Tocopheryl Acetate, carbohydrate, vitamins, minerals and For details on administration and household measurements, please L-Leucine Nil Added Nil Added Choline, mg 430 108 Niacinamide, Manganese Sulfate, Calcium D-Pantothenate, Cupric trace elements see the end of this section. L-Lysine 4.2 1.05 Inositol, mg 80 20 Sulfate, Pyridoxine Hydrochloride, L-Methionine 0.99 0.25 • Age-appropriate DRI formulation Minerals Sodium Selenite, Thiamin Chloride • MSUD Maxamum provides L-Phenylalanine 2.8 0.70 Calcium, mg 1183 296 Hydrochloride, Riboflavin, Vitamin A 40 g of protein equivalent per L-Proline 4.5 1.13 Phosphorus, mg 1137 284 Acetate, Folic Acid, Artificial Colors L-Serine 2.8 0.70 Magnesium, mg 306 77 (Beta-Carotene, Beet Red), Potassium 100 g of powder Ordering/Reimbursement Information: L-Threonine 3.1 0.78 Iron, mg 14.7 3.7 Iodide, Chromium Chloride, Sodium Molybdate, Phylloquinone, D-Biotin, • Available in orange flavor Product Reimbursement HCPCS Product Calories L-Tryptophan 1.3 0.33 Zinc, mg 9 2 Vitamin D , Cyanocobalamin. • Contains <1 g of fat per 10 g PE thereby Code Code Code* Packaging Per Can L-Tyrosine 2.8 0.70 Manganese, mg 1.8 0.5 3 allowing greater flexibility in modifying 117789 49735-0177-89 B4157 or B4162 6 x 454 g (1 lb) 1385 L-Valine Nil Added Nil Added Copper, mcg 820 205 Contains: Soy. energy intake L-Carnitine 0.04 0.01 Iodine, mcg 131 33 Taurine 0.14 0.04 Molybdenum, mcg 37 9 • Osmolality (mOsm/kg): WIC‡: Contact your state WIC For more reimbursement L-Glutamine 0.32 0.08 Chromium, mcg 26 7 Dilution Metabolics Metabolics office to determine availability. information see Resources. Selenium, mcg 58 15 1 to 7 750 Sodium, mg 560 140 1 to 5 1090 Potassium, mg 700 175 • Displacement: 0.6 mL/g Chloride, mg 560 140 84 * S9435 = For individuals who submit their bills to insurance on their own. 85 MSUD Lophlex LQ

Nutrition Information: ® Ingredients: MSUD Lophlex LQ Per Pouch Per 10 g Per Pouch Per 10 g Nutrients (4.2 fl oz) Protein Equivalent Vitamins (4.2 fl oz) Protein Equivalent Water, Apple Juice from Concentrate (34.1%), Grape Juice from Concentrate A valine-, leucine-, and isoleucine-free* ready-to-drink medical food containing amino Calories 120 60 Vitamin A, IU (mcg RE) 949.(285 475.(143) (6.9%), Blackcurrant Juice from Concentrate (2.5%), L-Lysine Acetate, acids, carbohydrates, vitamins, minerals and DHA, based on mixed fruit juices (from Protein Equivalent, g 20 10 Vitamin D3, IU (mcg) 320.(8.0) 160.(4.0) Fat, g 0.44 0.22 L-Proline, Citric Acid, L-Tyrosine, concentrate) with natural flavor; for the dietary management of proven maple syrup Vitamin E, IU (mg α-TE) 4.8 (3.2) 2.4 (1.6) L-Arginine, Glycine, L-Serine, L-Aspartic urine disease (MSUD) in individuals 4 years and older, including pregnant women and DHA, mg 150 75 Vitamin K, mcg 24.9 12.5 Acid, L-Alanine, L-Threonine, Corn Carbohydrate, g 9.3 4.7 Thiamin, mg 0.54 0.27 Syrup Solids, L-Cystine, L-Phenylalanine, women of child-bearing age. Dicalcium Phosphate, L-Histidine, Fiber, g 0.50 0.25 Riboflavin, mg 0.50 0.25 Elderberry Juice from Concentrate Amino Acids, Vitamin B6, mg 0.58 0.29 (0.6%), Maltodextrin, Magnesium L-Alanine 1.40 0.70 Vitamin B , mcg 1.8 0.90 Acetate, N-Acetyl L-Methionine, 12 L-Tryptophan, Choline Bitartrate, Main Features: Indications: L-Arginine 1.90 0.95 Niacin, mg 7.1 3.6 C. Cohnii Oil*, Sugar, Microcrystalline L-Aspartic Acid 1.45 0.73 Folic Acid, mcg 120 60.0 Cellulose, Natural Flavor, Fruit • Maple syrup urine disease (MSUD) • Valine, leucine, and isoleucine free L-Cystine 1.20 0.60 Pantothenic Acid, mg 1.8 0.9 Concentrate (Apple, Blackcurrant, Radish), L-Ascorbic Acid, Taurine, Guar Glycine 1.90 0.95 • Low volume; provides 20 g PE in just 4.2 For details on administration, please see the end of this section. Biotin, mcg 53.4 26.7 Gum, Sunflower Lecithin, Xanthan fl oz (125 mL) L-Histidine 1.00 0.50 Vitamin C, mg 17.8 8.9 Gum, M-Inositol, Potassium Sorbate L-Isoleucine Trace* Trace* (preservative to delay spoilage), • Convenient ready-to-drink Choline, mg 153 76.5 Artificial Sweetener: Sucralose, L-Leucine Trace* Trace* Inositol, mg 40.6 20.3 Ferrous Lactate, Artificial Sweetener: • Based on fruit juices (from concentrate) L-Lysine 1.78 0.89 Mineral Acesulfame Potassium, Sodium Benzoate (preservative to delay spoilage), Zinc with natural flavor - no artificial flavors L-Methionine 0.43 0.22 Calcium, mg 319 160 Sulfate, L-Carnitine, Niacinamide, • Contains a balanced mixture of L-Phenylalanine 1.18 0.59 Phosphorus, mg 244 122 DL-alpha Tocopheryl Acetate, Calcium L-Proline 2.38 1.19 Magnesium, mg 96.3 48.2 D-Pantothenate, Manganese Sulfate, other essential and non-essential amino Ordering/Reimbursement Information: Cupric Sulfate, Thiamin Chloride acids, carbohydrates, vitamins, minerals Product Reimbursement HCPCS Product Calories L-Serine 1.65 0.83 Iron, mg 5.1 2.6 Hydrochloride, Pyridoxine Hydrochloride, Code Code Code* Packaging Per Pouch L-Threonine 1.30 0.65 Zinc, mg 3.8 1.9 Vitamin A Palmitate, Riboflavin, Folic and DHA Acid, Potassium Iodide, Ascorbyl 82112 49735-0167-49 B4157 or B4162 30 X 4.2 fl oz (125 mL) 120 L-Tryptophan 0.53 0.27 Manganese, mg 0.56 0.28 • Provides 150 mg DHA per pouch Palmitate, Mixed Tocopherols, Sodium L-Tyrosine 2.00 1.00 Copper, mcg 563 282 Molybdate, D-Biotin, Sodium Selenite, • Available in mixed berry blast flavor L-Valine Trace* Trace* Iodine, mcg 56.3 28.2 Chromium Chloride, Phylloquinone, WIC‡: Contact your state WIC For more reimbursement Vitamin D , Cyanocobalamin. L-Carnitine 0.01 0.01 Molybdenum, mcg 25.0 12.5 3 • Osmolality (at 0.96 kcal/mL): office to determine availability. information see Resources. 2460 mOsm/kg Taurine 0.06 0.03 Chromium, mcg 10.8 5.4 Contains: 44.1% juice.

Metabolics Metabolics Selenium, mcg 26.8 13.4 * S9435 = For individuals who submit their bills to insurance on their own. *A Source of Docosahexaenoic Acid Sodium, mg <25.0 <12.5 (DHA). Potassium, mg 90 45 *May contain traces of leucine, isoleucine and valine from fruit juice (from concentrate) at a level of <1.5 mg per 100 mL. Chloride, mg <25.0 <12.5 86 87 IVA Early Years Anamix

Nutrition Information: Per 10 g Per 10 g Ingredients: ® IVA Anamix Early Years Nutrients Per 100 g Protein Equivalent Vitamins Per 100 g Protein Equivalent Corn Syrup Solids, Refined Vegetable Calories 473 350 Vitamin A, IU (mcg RE) 1305.(392) 967.(290) Oils (High Oleic Sunflower, Soy, Non-Hydrogenated Coconut, Canola, A leucine-free powdered infant formula with iron, for the dietary management of Protein Equivalent,g 13.5 10.0 Vitamin D3, IU (mcg) 348.(8.7) 258.(6.4) Fat, g 23.0 17.0 Vitamin E, IU (mg α-TE) 10.9 (7.3) 8.1 (5.4) Sunflower), Galacto-oligosaccharides isovaleric acidemia (IVA). Contains DHA and ARA, and a unique blend of prebiotic fiber. Saturated, g 7.5 5.6 Vitamin K, mcg 38.0 28.1 (GOS) Dried Syrup (from milk), L-Lysine Monounsaturated, g 9.4 7.0 Thiamin, mcg 640 474 L-Glutamate, L-Proline, Glycine, L-Arginine, Tricalcium Phosphate, Polyunsaturated, g 5.0 3.7 Riboflavin, mcg 500 370 L-Aspartic Acid, CAEM (an emulsifier), DHA, mg 70.0 51.9 Vitamin B6, mcg 500 370 L-Tyrosine, L-Phenylalanine, L-Alanine, ARA, mg 140 104 Main Features: Indications: Vitamin B12, mcg 1.2 0.89 L-Threonine, L-Histidine, L-Serine, Linoleic Acid,mg 3500 2593 Niacin, mcg 2200 1630 Potassium Chloride, Fructo- Carbohydrate, g 53.0 39.3 • Leucine-free powdered infant • Isovaleric acidemia Folic Acid, mcg 55.0 40.7 oligosaccharides (FOS), Tripotassium Fiber, g 5.3 3.9 Pantothenic Acid, mcg 2800 2074 Citrate, L-Valine, L-Cystine, L-Isoleucine, formula with iron • Other proven disorders of leucine metabolism GOS, g 4.7 3.5 Biotin, mcg 18.2 13.5 Sodium Phosphate Dibasic, Magnesium • Includes DHA & ARA FOS, g 0.55 0.41 Vitamin C, mg 49.0 36.3 Acetate, M. Alpina Oil*, L-Methionine, Amino Acids, g Choline, mg 91.0 67.4 L-Glutamic Acid, L-Tryptophan, Choline • Contains a prebiotic fiber blend L-Alanine 0.74 0.55 Inositol, mg 98.0 72.6 Bitartrate, C. Cohnii Oil**, L-Glutamine, Sodium Chloride, M-Inositol, L-Ascorbic • Unflavored powder For details on administration and household measurements, please L-Arginine 1.21 0.90 Minerals L-Aspartic Acid 1.02 0.76 Calcium, mg 371 275 Acid, Taurine, Ferrous Sulfate, Zinc see the end of this section. Sulfate, L-Carnitine, DL-Alpha Tocopheryl • Contains a balanced mixture of all other L-Cystine 0.44 0.33 Phosphorus, mg 270 200 Acetate, Calcium D-Pantothenate, essential and non-essential amino acids, L-Glutamic Acid 1.35 1 Magnesium, mg 52.5 38.9 Niacinamide, Ascorbyl Palmitate, carbohydrates, fat, vitamins, minerals L-Glutamine 0.11 0.08 Iron, mg 7.3 5.4 Manganese Sulfate, Cupric Sulfate, and trace elements Ordering/Reimbursement Information: Glycine 2.18 1.6 Zinc, mg 5.2 3.9 Thiamine Chloride Hydrochloride, L-Histidine 0.7 0.52 Manganese, mcg 390 289 Pyridoxine Hydrochloride, Riboflavin, • Provides 13.5 g of protein equivalent Product Reimbursement HCPCS Product Calories L-Isoleucine 0.42 0.31 Copper, mcg 390 289 Vitamin A Acetate, DL-alpha Tocopherol, Code Code Code* Packaging Per Can per 100 g of powder L-Leucine Nil Added Nil Added Iodine, mcg 82.5 61.1 Mixed Tocopherols, Potassium Iodide, 90211 49735-0102-11 B4162 6 x 400 g (14 oz) 1892 L-Lysine 1.06 0.79 Molybdenum, mcg 10.9 8.1 Chromium Chloride, Folic Acid, • Osmolality (at 0.7 kcal/mL): L-Methionine 0.3 0.22 Chromium, mcg 12.5 9.3 Phylloquinone, Sodium Selenite, Sodium Molybdate, D-Biotin, Vitamin D , 380 mOsm/kg L-Phenylalanine 0.82 0.61 Selenium, mcg 14.1 10.4 3 Based on dilution of 5 g powder WIC‡: Contact your state WIC For more reimbursement L-Proline 2.19 1.6 Sodium, mg 171 127 Cyanocobalamin. (1 unpacked level scoop) to 1 fl oz office to determine availability. information see Resources. L-Serine 0.68 0.50 Potassium, mg 454 336 Contains: Soy Oil, Coconut Oil, Milk. L-Threonine 0.72 0.53 Chloride, mg 321 238 (30 mL) water L-Tryptophan 0.27 0.20 *A source of Arachidonic Acid (ARA)

Metabolics Metabolics * S9435 = For individuals who submit their bills to insurance on their own. • Displacement: 0.75 mL/g L-Tyrosine 0.8 0.59 **A source of Docosahexaenoic Acid L-Valine 0.48 0.36 (DHA) L-Carnitine 0.01 0.01 Taurine 0.02 0.01 Contains GOS/FOS 0.8 g per 100 mL 88 89 IVA Next Anamix

Nutrition Information: Per 10 g Per 10 g Ingredients: ® Nutrients Per 100 g Protein Equivalent Vitamins Per 100 g Protein Equivalent IVA Anamix Next Calories 377 134.6 Vitamin A, IU (mcg R.E.) 1748.(525) 628.(187.5) Corn Syrup Solids, Refined Vegetable Oils (High Oleic Sunflower, Canola, Medium Protein Equivalent, g 28 10 Vitamin D3, IU (mcg) 1052.(26.3) 379.(9.4) Chain Triglycerides (Palm Kernel and/ A leucine-free powdered medical food for the dietary management of isovaleric Fat, g 12.5 4.5 Vitamin E, IU (mg-α-TE) 15.6.(10.5) 5.6.(3.8) or Coconut Oil), Sunflower), Glycine, Vitamin K, mcg 17.5 6.3 acidemia in individuals over 1 year of age. Saturated, g 2.0 0.71 L-Proline, Calcium Phosphate Dibasic, Thiamin, mg 1.2 0.43 Monounsaturated, g 8.2 2.9 Fructo-oligosaccharides, L-Glutamine, Polyunsaturated, g 1.8 0.64 Riboflavin, mg 0.91 0.33 Inulin, L-Lysine Acetate, Corn Starch, Vitamin B , mg 0.91 0.33 DHA, mg 180 64 6 Microcrystalline Cellulose, L-Arginine, Vitamin B , mcg 1.6 0.57 Carbohydrate, g 41.2 14.7 12 Artificial Flavor, Magnesium L-Aspartate, Fiber, g 11.2 4.0 Niacin, mg 2.5 0.89 Gum Arabic, L-Glutamic Acid, L-Tyrosine, Main Features: Indications: Soluble, g 8.0 2.9 Folic Acid, mcg 263 93.9 L-Phenylalanine, Tripotassium Citrate, Insoluble, g 3.2 1.1 Pantothenic Acid, mg 5.3 1.9 L-Threonine, L-Serine, L-Histidine, • Leucine free • Isovaleric acidemia Amino Acids, g Biotin, mcg 14 5 L-Alanine, L-Valine, Trisodium Citrate, L-Cystine, L-Isoleucine, Maltodextrin, L-Alanine 1.35 0.48 Vitamin C, mg 52.5 18.8 • Contains a balanced mixture of all other Choline Bitartrate, Calcium Chloride, For details on administration and household measurements, please L-Arginine 2.35 0.84 Choline, mg 280 100 essential and non-essential amino acids, L-Methionine, L-Tryptophan, CAEM see the end of this section. L-Aspartic Acid 2.0 0.71 Inositol, mg 70 25 carbohydrate (including a multi-fiber Minerals (an emulsifier),C. Cohnii Oil*, L-Aspartic L-Cystine 0.94 0.34 Acid, Sodium Chloride, L-Ascorbic Acid, blend), fat (including DHA), vitamins, Calcium, mg 1225 437.5 L-Glutamic acid 1.88 0.67 M-Inositol, Soy Fiber, Taurine, Ferrous Phosphorus, mg 801 286.1 minerals, and trace elements L-Glutamine 3.16 1.1 Sulfate, Sunflower Lecithin, L-Carnitine, • Provides 28 g protein equivalent and Glycine 4.6 1.6 Magnesium, mg 140 50 Zinc Sulfate, DL-alpha Tocopheryl Acetate, Iron, mg 12.5 4.5 12.5 g fat per 100 g of powder L-Histidine 1.4 0.50 Calcium D-Pantothenate, Manganese Ordering/Reimbursement Information: L-Isoleucine 0.92 0.33 Zinc, mg 6.1 2.2 Sulfate, Niacinamide, Cupric Sulfate, • Available in Plain L-Leucine Nil Added Nil Added Manganese, mg 1.2 0.4 Thiamine Chloride Hydrochloride, Product Reimbursement HCPCS Product Calories L-Lysine 2.2 0.79 Copper, mcg 700 250 Pyridoxine Hydrochloride, Riboflavin, • Osmolality (at 1.1 kcal/mL): Code Code Code* Packaging Per Can 1220 mOsm/kg L-Methionine 0.64 0.23 Iodine, mcg 174 62.1 Vitamin A Acetate, Folic Acid, Ascorbyl 89471 49735-0194-71 B4157 or 6 x 400 g (14 oz) 1508 L-Phenylalanine 1.7 0.61 Molybdenum, mcg 31.9 11.4 Palmitate, Potassium iodide, Mixed Based on dilution of 36 g powder B4162 Tocopherols, Sodium Molybdate, Sodium L-Proline 4.1 1.5 Chromium, mcg 12.8 4.6 (approx. 4 scoops) to 3.5 fl oz Selenite, Chromium Chloride, DL-alpha L-Serine 1.5 0.52 Selenium, mcg 35.3 12.6 Tocopherol, Vitamin D , Phylloquinone, (100 mL) water WIC‡: Contact your state WIC For more reimbursement Sodium, mg 350 125 3 L-Threonine 1.5 0.54 D-Biotin, Cyanocobalamin. • Displacement: 0.72 mL/g office to determine availability. information see Resources. L-Tryptophan 0.58 0.21 Potassium, mg 556 198.6 Metabolics L-Tyrosine 1.7 0.61 Chloride, mg 508 181.4 Contains: Coconut Oil, Soy. * S9435 = For individuals who submit their bills to insurance on their own. L-Valine 1.0 0.37 *A source of Docosahexaenoic Acid L-Carnitine 0.02 0.01 (DHA) Taurine 0.06 0.02 90 91 XLeu Maxamum

® Nutrition Information: Ingredients: XLeu Maxamum Per 10 g Per 10 g Nutrients Per 100 g Protein Equivalent Vitamins Per 100 g Protein Equivalent Sugar, L-Lysine L-Glutamate, L-Proline, A leucine-free powdered medical food for the dietary management of isovaleric acidemia Calories 305 76 Vitamin A, IU (mcg RE) 2228 (669) 557 (167) Glycine, Corn Syrup Solids, Calcium Phosphate Dibasic, L-Arginine, L-Aspartic and other proven disorders of leucine metabolism in older children and adults, including Protein Equivalent, g 40 10 Vitamin D , IU (mcg) 332 (8.3) 83 (2.1) 3 Acid, L-Phenylalanine, Artificial Flavor, Fat, g <1 pregnant women and women of child-bearing age. <0.25 Vitamin E, IU (mg α-TE) 18.3 (12.3) 4.6 (3.1) L-Tyrosine, L-Alanine, L-Threonine, Citric Carbohydrate, g 34 8.5 Vitamin K, mcg 82 21 Acid, L-Histidine, L-Serine, Magnesium Amino Acids, g Thiamin, mg 0.91 0.23 Acetate, L-Valine, L-Cystine, L-Isoleucine, L-Alanine 2.2 0.55 Riboflavin, mg 0.97 0.24 Tripotassium Citrate, Choline Bitartrate, L-Arginine 3.6 Sodium Chloride, L-Methionine, 0.90 Vitamin B6, mg 1.6 0.4 L-Aspartic Acid 3 0.75 Vitamin B , mcg 3.1 0.8 L-Glutamic Acid, L-Tryptophan, Soy Main Features: Indications: 12 Lecithin, Disodium Hydrogen Phosphate, L-Cystine 1.3 0.33 Niacin, mg 5.7 1.4 Dipotassium Hydrogen Phosphate, • Leucine free • Isovaleric acidemia L-Glutamic Acid 3.9 0.98 Folic Acid, mcg 491 123 L-Glutamine, Tricalcium Phosphate, • Contains a balanced mixture of all other • Other proven disorders of leucine metabolism Glycine 6.3 1.58 Pantothenic Acid, mg 4.9 1.2 Taurine, L-Ascorbic Acid, M-Inositol, essential and non-essential amino acids, L-Histidine 2.1 0.53 Biotin, mcg 24.5 6.1 Ferrous Sulfate, L-Carnitine, Zinc carbohydrate, vitamins, minerals and L-Isoleucine 1.2 0.30 Vitamin C, mg 62 16 Sulfate, DL-Alpha Tocopheryl Acetate, L-Leucine Nil Added Nil Added Choline, mg 430 108 Niacinamide, Manganese Sulfate, trace elements (including iron, selenium Calcium D-Pantothenate, Cupric For details on administration and household measurements, please L-Lysine 3 0.75 Inositol, mg 80 20 and chromium) Sulfate, Pyridoxine Hydrochloride, L-Methionine 0.86 see the end of this section. 0.22 Minerals Sodium Selenite, Thiamin Chloride • Age-appropriate DRI formulation L-Phenylalanine 2.3 0.58 Calcium, mg 1183 296 Hydrochloride, Riboflavin, Vitamin A • XLeu Maxamum provides 40 g of L-Proline 6.3 1.58 Phosphorus, mg 1137 284 Acetate, Folic Acid, Artificial Colors protein equivalent per 100 g of powder L-Serine 1.9 0.48 Magnesium, mg 306 77 (Beta-Carotene, Beet Red), Potassium Ordering/Reimbursement Information: L-Threonine 2.1 0.53 Iron, mg 14.7 3.7 Iodide, Chromium Chloride, Sodium • Available in orange flavor L-Tryptophan 0.79 0.20 Zinc, mg 9 2 Molybdate, Phylloquinone, D-Biotin, Product Reimbursement HCPCS Product Calories Vitamin D , Cyanocobalamin. • Contains <1 g of fat per 10 g PE Code Code Code* Packaging Per Can L-Tyrosine 2.3 0.58 Manganese, mg 1.8 0.5 3 thereby allowing greater flexibility in 117790 49735-0177-90 B4157 or 6 x 454 g (1 lb) 1385 L-Valine 1.4 0.35 Copper, mcg 820 205 Contains: Soy. modifying energy intake B4162 L-Carnitine 0.04 0.01 Iodine, mcg 131 33 • Osmolality (mOsm/kg): Taurine 0.14 0.04 Molybdenum, mcg 37 9 Dilution WIC‡: Contact your state WIC For more reimbursement L-Glutamine 0.32 0.08 Chromium, mcg 26 7 Metabolics Metabolics 1 to 7 800 office to determine availability. information see Resources. Selenium, mcg 58 15 1 to 5 1120 Sodium, mg 560 140 * S9435 = For individuals who submit their bills to insurance on their own. Potassium, mg 700 175 • Displacement: 0.6 mL/g Chloride, mg 560 140 92 93 GA-1 Anamix Early Years

Nutrition Information: Ingredients: Per 10 g Per 10 g ® Corn Syrup Solids, Refined Vegetable Nutrients Per 100 g Protein Equivalent Vitamins Per 100 g Protein Equivalent GA-1 Anamix Early Years Oils (High Oleic Sunflower, Soy, Calories 473 350 Vitamin A, IU (mcg RE) 1305.(392) 967.(290) Non-hydrogenated Coconut, Canola, Protein Equivalent,g 13.5 10.0 Vitamin D3, IU (mcg) 348.(8.7) 258.(6.4) A lysine-free, low tryptophan powdered infant formula with iron, for the Sunflower), Galacto-oligosacharides Fat, g 23.0 17.0 Vitamin E, IU (mg α-TE) 10.9 (7.3) 8.1 (5.4) (GOS) Dried Syrup (from milk), L-Leucine, dietary management of glutaric aciduria type 1 (GA-1). Contains DHA and Saturated, g 7.5 5.6 Vitamin K, mcg 38.0 28.1 L-Glutamic Acid, L-Proline, L-Arginine, ARA, and a unique blend of prebiotic fiber. Monounsaturated, g 9.4 7.0 Thiamin, mcg 640 474 L-Valine, L-Isoleucine, Tricalcium Polyunsaturated, g 5.0 3.7 Riboflavin, mcg 500 370 Phosphate, Glycine, L-Aspartic Acid, DHA, mg 70.0 51.9 Vitamin B6, mcg 500 370 CAEM (an emulsifier), L-Threonine, ARA, mg 140 104 Vitamin B12, mcg 1.2 0.89 L-Tyrosine, L-Phenylalanine, L-Serine, Linoleic Acid,mg 3500 2593 Niacin, mcg 2200 1630 Main Features: Indications: L-Histidine, L-Alanine, Potassium Carbohydrate, g 53.0 39.3 Folic Acid, mcg 55.0 40.7 Chloride, Fructo-oligosaccharides (FOS), Fiber, g 5.3 3.9 Pantothenic Acid, mcg 2800 2074 • Lysine-free, low tryptophan • Glutaric acidemia type I Tripotassium Citrate, L-Cystine, Sodium GOS, g 4.7 3.5 Biotin, mcg 18.2 13.5 powdered infant formula with iron Phosphate Dibasic, Magnesium Acetate, FOS, g 0.55 0.41 Vitamin C, mg 49.0 36.3 Oil*, L-Methionine, Choline • Includes DHA & ARA Amino Acids, g Choline, mg 91.0 67.4 M. Alpina Bitartrate, Oil**, L-Glutamine, L-Alanine 0.68 0.50 Inositol, mg 98.0 72.6 C. Cohnii • Contains a prebiotic fiber blend For details on administration and household measurements, please Sodium Chloride, M-Inositol, L-Ascorbic L-Arginine 1.18 0.87 Minerals Acid, L-Tryptophan, Taurine, Ferrous • Unflavored powder see the end of this section. L-Aspartic Acid 0.98 0.73 Calcium, mg 371 275 Sulfate, Zinc Sulfate, L-Carnitine, L-Cystine 0.45 0.33 Phosphorus, mg 270 200 • Contains a balanced mixture of all DL-alpha Tocopheryl Acetate, Calcium L-Glutamic Acid 1.33 0.99 Magnesium, mg 52.5 38.9 D-Pantothenate, Niacinamide, other essential and non-essential L-Glutamine 0.11 0.08 Iron, mg 7.3 5.4 Ordering/Reimbursement Information: Ascorbyl Palmitate, Manganese Sulfate, amino acids, carbohydrates, fat, Glycine 1.06 0.79 Zinc, mg 5.2 3.9 Cupric Sulfate, Thiamine Chloride Product Reimbursement HCPCS Product Calories L-Histidine 0.68 0.50 Manganese, mcg 390 289 vitamins, minerals and trace elements Hydrochloride, Pyridoxine Hydrochloride, Code Code Code* Packaging Per Can L-Isoleucine 1.06 0.79 Copper, mcg 390 289 Riboflavin, Vitamin A Acetate, DL-alpha • Provides 13.5 g of protein equivalent 90217 49735-0102-17 B4162 6 x 400 g (14 oz) 1892 L-Leucine 1.81 1.3 Iodine, mcg 82.5 61.1 Tocopherol, Mixed Tocopherols, per 100 g of powder L-Lysine Nil Added Nil Added Molybdenum, mcg 10.9 8.1 Potassium Iodide, Chromium Chloride, L-Methionine 0.29 0.21 Chromium, mcg 12.5 9.3 • Osmolality (at 0.7 kcal/mL): ‡ Folic Acid, Phylloquinone, Sodium WIC : Contact your state WIC For more reimbursement L-Phenylalanine 0.8 0.59 Selenium, mcg 14.1 10.4 Selenite, Sodium Molybdate, D-Biotin, 380 mOsm/kg office to determine availability. information see Resources. L-Proline 1.29 0.96 Sodium, mg 171 127 Vitamin D , Cyanocobalamin Based on dilution of 5 g powder L-Serine 0.8 0.59 Potassium, mg 454 336 3 (1 unpacked level scoop) to 1 fl oz L-Threonine 0.89 0.66 Chloride, mg 321 238 Contains: Soy Oil, Coconut Oil, Milk. * S9435 = For individuals who submit their bills to insurance on their own. Metabolics Metabolics (30 mL) water L-Tryptophan 0.09 0.07 L-Tyrosine 0.8 0.59 *A source of Arachidonic Acid (ARA) • Displacement: 0.75 mL/g L-Valine 1.16 0.86 **A source of Docosahexaenoic Acid L-Carnitine 0.01 0.01 (DHA) Taurine 0.02 0.01 94 Contains GOS/FOS 0.8 g per 100 mL 95 GlutarAde Junior GA-1 Drink Mix

Nutrition Information: Ingredients: ™ Nutrients Per 100 g Per 100 kcal Vitamins Per 100 g Per 100 kcal Corn Syrup Solids, Modified Corn Starch, GlutarAde Junior GA-1 Drink Mix Calories 410 100 Vitamin A, IU (mcg RE) 1373.(412 335.(101) Canola Oil, Maltodextrin, Safflower Oil, Protein Equivalent, g 10 2.5 Vitamin D3, IU (mcg) 525.(13.1) 128.(3.2) Natural and Artificial Flavor. Contains A lysine-free and low tryptophan powdered medical food for the dietary management of Fat, g 10 2.5 Vitamin E, IU (mg α-TE) 8.5 (5.7) 2.1 (1.4) 2% or less of the following: L-Glutamine, glutaric aciduria type 1 (GA-1) in young children. Saturated, g 2.2 0.55 Vitamin K, mcg 34 8.3 L-Leucine, Tricalcium Phosphate, Monounsaturated, g 5.2 1.3 Thiamin, mg 0.83 0.2 L-Arginine, L-Valine, Coconut Oil, Polyunsaturated, g 2.6 0.63 Riboflavin, mg 0.69 0.17 Soybean Oil, L-Isoleucine, L-Asparagine Linoleic Acid, mg 1487 363 Vitamin B6, mg 0.66 0.16 Monohydrate, Dipotassium Phosphate, α-Linolenic Acid, mg 815 199 Vitamin B12, mcg 1.4 0.35 Flaxseed Oil, L-Tyrosine, L-Phenylalanine, Carbohydrate, g 69 17 Niacin, mg 6.6 1.6 L-Threonine, L-Proline, Choline Bitartrate, Main Features: Indications: Amino Acids, g Folic Acid, mcg 150 37 Potassium Chloride, L-Ornithine-Alpha • Glutaric acidemia type I L-Alanine 0.48 0.12 Pantothenic Acid, mg 6 1.5 Ketoglutarate, L-Alanine, Magnesium • Lysine free L-Arginine 1.08 0.26 Biotin, mcg 31 7.6 Phosphate, L-Methionine, L-Glutamic L-Asparagine 0.72 0.18 Vitamin C, mg 56 13.6 • Amino acid profile specifically For details on administration and household measurements, please Acid, L-Histidine, Glycine, L-Serine, L-Cystine 0.18 0.04 Choline, mg 213 52 Ascorbyl Palmitate, Sodium Citrate, designed for GA-1; clinically see the end of this section. L-Glutamic Acid 0.36 0.09 Inositol, mg 125 30 researched in one study† Mixed Tocopherols, Rosemary L-Glutamine 1.80 0.44 Minerals Extract, L-Cystine, Sodium Chloride, • Contains arginine and 60 mg of Glycine 0.24 0.06 Calcium, mg 460 112 Inositol, Silicon Dioxide, Ascorbic Acid, L-Histidine 0.36 0.09 Phosphorus, mg 400 97 L-Tryptophan, Taurine, Ferrous Sulfate, tryptophan per 100 g powder L-Isoleucine 0.84 0.21 Magnesium, mg 60 14.6 Calcium Phosphate, L-Carnitine, Zinc L-Leucine 1.38 0.34 Iron, mg 8 2 • Contains a source of Omega-3 Sulfate, Vitamin E Acetate, Niacinamide, L-Lysine Nil Added Nil Added Zinc, mg 7 1.6 fatty acids Ordering/Reimbursement Information: Calcium D-Pantothenate, Mono- and L-Methionine 0.41 0.10 Manganese, mg 0.43 0.1 Diglycerides, Vitamin A Palmitate, • Complete with vitamins and minerals Product Reimbursement HCPCS Product Calories L-Ornithine 0.24 0.06 Copper, mcg 500 122 Vitamin D3, Copper Gluconate, Vitamin Code Code Code* Packaging Per Can L-Phenylalanine 0.64 0.16 Iodine, mcg 90 22 • Plain flavor K1, D-Biotin, Manganese Sulfate, 7510 00847-0751-02 B4157 or B4162 4 x 400 g (14.1 oz) 1640 L-Proline 0.60 0.15 Molybdenum, mcg 18 4.4 • Provides 10 g protein equivalent per L-Serine 0.24 0.06 Chromium, mcg 12 2.9 Sodium Selenite, Thiamin Mononitrate, L-Threonine 0.64 0.16 Selenium, mcg 40 9.7 Pyridoxine Hydrochloride, Riboflavin, 100 g powder Potassium Iodide, Chromium Chloride, ‡ L-Tryptophan 0.06 0.01 Sodium, mg 221 54 WIC : Contact your state WIC For more reimbursement Folic Acid, Vitamin B12, Sodium • Displacement: 0.6 mL/g L-Tyrosine 0.72 0.18 Potassium, mg 589 144 information see . office to determine availability. Resources L-Valine 0.96 0.23 Chloride, mg 332 81 Molybdate. L-Carnitine 0.03 0.01 Contains: Coconut Oil, Soybean Oil.

Metabolics Metabolics * S9435 = For individuals who submit their bills to insurance on their own. Taurine 0.06 0.01 † Strauss KA, et al. Mol Genet Metab. 2011.

96 97 GlutarAde Essential GA-1GlutarAde Drink Mix

Nutrition Information: Ingredients: ™ Per 10 g Protein Equivalent Per 10 g Protein Equivalent Sugar, Modified Corn Starch, Canola Oil, GlutarAde Essential GA-1 Drink Mix Nutrients Per 100 g (1 scoop) Vitamins Per 100 g (1 scoop) Maltodextrin, Safflower Oil, Dextrin, Calories 385 154 Vitamin A, IU (mcg RE) 1700.(637) 680.(255) L-Glutamine, L-Leucine, Natural and A lysine-free and low tryptophan powdered medical food for the dietary management of Protein Equivalent, g 25 10 (25% as beta-carotene) Artificial Flavor, L-Arginine, Dicalcium Fat, g 13 5 glutaric aciduria type 1 (GA-1) in children and adults, including pregnant women and women Vitamin D3, IU (mcg) 600.(15) 240.(6) Phosphate, L-Valine, L-Isoleucine, Saturated, g 3.1 1.2 Vitamin E, IU (mg α-TE) 19.1 (12.8) 7.6 (5.1) of child bearing age. Monounsaturated, g 6.9 2.8 L-Asparagine Monohydrate. Contains Vitamin K, mcg 70 28 2% or less of the following: L-Tyrosine, Polyunsaturated, g 2.9 1.2 Thiamin, mg 1.5 0.6 Linoleic Acid, mg 1936 774 Riboflavin, mg 1.5 0.6 Tricalcium Phosphate, L-Phenylalanine, α-Linolenic Acid, mg 730 292 Vitamin B6, mg 1.4 0.56 L-Threonine, L-Proline, Potassium Carbohydrate, g 45 18 Vitamin B12, mcg 3 1.2 Citrate, L-Ornithine-Alpha Ketoglutarate, Main Features: Indications: Fiber, g 3.9 1.5 Niacin, mg 15.5 6.2 L-Alanine, Coconut Oil, Soybean Oil, Amino Acids, g Folic Acid, mcg 300 120 L-Methionine, Sodium Citrate, Potassium • Glutaric acidemia type I L-Alanine 1.20 0.48 • Lysine free Pantothenic Acid, mg 7.5 3 Chloride, L-Glutamic Acid, L-Histidine, L-Arginine 2.7 1.08 Biotin, mcg 30 12 • Amino acid profile specifically L-Asparagine 1.8 0.72 Choline Bitartrate, Glycine, L-Serine, Vitamin C, mg 60 24 L-Cystine, Flaxseed Oil, Magnesium designed for GA-1; clinically L-Cystine 0.45 0.18 Choline, mg 313 125 researched in one study† For details on administration and household measurements, please L-Glutamic Acid 0.9 0.36 Inositol, mg 50 20 Oxide, Ascorbyl Palmitate, Mixed L-Glutamine 4.51 1.8 Tocopherols, Rosemary Extract, Silicon see the end of this section. Minerals: • Contains arginine and 150 mg of Glycine 0.6 0.24 Calcium, mg 1257 503 Dioxide, L-Tryptophan, Taurine, Sodium tryptophan per 100 g powder L-Histidine 0.9 0.36 Phosphorus, mg 787 315 Chloride, Ascorbic Acid, Inositol, Ferric L-Isoleucine 2.1 0.84 Magnesium, mg 201 80 Orthophosphate, Vitamin E Acetate, • Contains a source of Omega-3 L-Leucine 3.46 1.38 Iron, mg 11.3 4.5 L-Lysine Nil Added Nil Added L-Carnitine, Niacinamide, Calcium fatty acids Ordering/Reimbursement Information: Zinc, mg 10 4 Phosphate, Zinc Oxide, Sucralose, L-Methionine 1.02 0.41 Manganese, mg 1.9 0.76 L-Ornithine 0.6 0.24 D-Calcium Pantothenate, Copper • Provides 25 g protein equivalent per Product Reimbursement HCPCS Product Calories Copper, mcg 1000 400 L-Phenylalanine 1.59 0.64 Gluconate, Vitamin K1, Vitamin D3, 100 g powder Code Code Code* Packaging Per Can Iodine, mcg 115 46 L-Proline 1.5 0.6 Molybdenum, mcg 31 12.4 Manganese Sulfate, Vitamin A Palmitate, • Vanilla flavor 7542 00847-0754-22 B4157 or B4162 4 x 454 g (1 lb) 1748 L-Serine 0.6 0.24 Chromium, mcg 21 8.6 Mono- and Diglycerides, D-Biotin, Beta- L-Threonine 1.59 0.64 Selenium, mcg 63 25 carotene, Pyridoxine Hydrochloride, • Osmolality (at 0.64 kcal/mL): L-Tryptophan 0.15 0.06 Sodium, mg 384 154 Potassium Iodide, Riboflavin, Thiamin L-Tyrosine 1.8 0.72 797 mOsm/kg WIC‡: Contact your state WIC For more reimbursement Potassium, mg 874 350 Mononitrate, Sodium Selenite, Folic Acid, L-Valine 2.4 0.96 Chloride, mg 475 190 Based on dilution of 40 g powder office to determine availability. information see Resources. L-Carnitine 0.04 0.01 Vitamin B12, Chromium Chloride, Sodium (1 scoop) to 7 fl oz (210 mL) water Taurine 0.15 0.06 Molybdate. Metabolics • Displacement: 0.75 mL/g * S9435 = For individuals who submit their bills to insurance on their own. Contains: Coconut Oil, Soybean Oil.

† Strauss KA, et al. Mol Genet Metab. 2011. 98 99 GlutarAde Amino Acid Blend

Nutrition Information: Ingredients: ™ Per 10 g Protein Per 10 g Protein L-Glutamine, L-Leucine, L-Arginine, GlutarAde Amino Acid Blend Equivalent Equivalent L-Valine, L-Isoleucine, L-Asparagine Nutrients Per 100 g (1 scoop) Amino Acids, g Per 100 g (1 scoop) Monohydrate, L-Tyrosine, A lysine-free and low tryptophan powdered medical food for the dietary management of Calories 324 40 L-Alanine 3.86 0.48 L-Phenylalanine, L-Threonine, L-Proline, glutaric aciduria type 1 (GA-1) in children and adults. Protein Equivalent, g 81 10 L-Arginine 8.69 1.07 L-Ornithine-Alpha Ketoglutarate, Fat, g 0 0 L-Asparagine 5.79 0.71 L-Alanine, L-Methionine, L-Glutamic Carbohydrate, g 0 0 L-Cystine 1.45 0.18 Acid, L-Histidine, Glycine, L-Serine, L-Cystine, Taurine, L-Tryptophan, Silicon Sodium, mg 355 44 L-Glutamic Acid 2.90 0.36 Dioxide, L-Carnitine. L-Glutamine 14.48 1.79 Main Features: Indications: Glycine 1.93 0.24 • Lysine free • Glutaric acidemia type I L-Histidine 2.90 0.36 Note: Must combine with an L-Isoleucine 6.76 0.83 appropriate vitamin & mineral • Amino acid profile specifically L-Leucine 11.10 1.37 supplement when taken as the sole designed for GA-1; clinically For details on administration and household measurements, please source of amino acid protein. see the end of this section. L-Lysine Nil Added Nil Added researched in one study† L-Methionine 3.28 0.41 • Contains arginine and 480 mg of L-Ornithine 1.93 0.24 tryptophan per 100 g powder L-Phenylalanine 5.12 0.63 L-Proline 4.83 0.60 • Does not contain fat, carbohydrates, L-Serine 1.93 0.24 vitamins and minerals Ordering/Reimbursement Information: L-Threonine 5.12 0.63 • 40 calories per serving Product Reimbursement HCPCS Product Calories L-Tryptophan 0.48 0.06 • Provides 81 g of protein equivalent Code Code Code* Packaging Per Can L-Tyrosine 5.79 0.71 per 100 g of powder 7500 00847-0750-00 B4155 or B9998 4 x 454 g (1 lb) 1471 L-Valine 7.72 0.95 L-Carnitine 0.20 0.03 • Unflavored Taurine 0.48 0.06 • Displacement: 0.48 mL/g WIC‡: Contact your state WIC For more reimbursement office to determine availability. information see Resources. Metabolics Metabolics

† Strauss KA, et al. Mol Genet Metab. 2011. * S9435 = For individuals who submit their bills to insurance on their own. 100 101 HCU Anamix Early Years

Nutrition Information: Ingredients: Per 10 g Per 10 g ® Corn Syrup Solids, Refined Vegetable HCU Anamix Early Years Nutrients Per 100 g Protein Equivalent Vitamins Per 100 g Protein Equivalent Oils (High Oleic Sunflower, Soy, Calories 473 350 Vitamin A, IU (mcg RE) 1305.(392) 967.(290) Non-Hydrogenated Coconut, Canola, Protein Equivalent,g 13.5 10.0 Vitamin D3, IU (mcg) 348.(8.7) 258.(6.4) Sunflower), Galacto-oligosaccharides A methionine-free powdered infant formula with iron, for the dietary Fat, g 23.0 17.0 (GOS) Dried Syrup (from milk), L-Lysine Vitamin E, IU (mg α-TE) 10.9 (7.3) 8.1 (5.4) L-Glutamate, L-Leucine, L-Proline, management of Vitamin B6 non-responsive homocystinuria or Saturated, g 7.5 5.6 Vitamin K, mcg 38.0 28.1 Tricalcium Phosphate, L-Arginine, hypermethioninemia. Contains DHA and ARA, and a unique blend of Monounsaturated, g 9.4 7.0 Thiamin, mcg 640 474 L-Valine, Glycine, L-Isoleucine, CAEM (an prebiotic fiber. Polyunsaturated, g 5.0 3.7 Riboflavin, mcg 500 370 emulsifier), L-Aspartic Acid, L-Threonine, DHA, mg 70.0 51.9 Vitamin B6, mcg 500 370 L-Serine, L-Phenylalanine, L-Tyrosine, L-Alanine, L-Histidine, Potassium ARA, mg 140 104 Vitamin B12, mcg 1.2 0.89 Linoleic Acid,mg 3500 2593 Niacin, mcg 2200 1630 Chloride, Fructo-oligosaccharides (FOS), Tripotassium Citrate, Sodium Phosphate Carbohydrate, g 53.0 39.3 Folic Acid, mcg 55.0 40.7 Dibasic, L-Cystine, Magnesium Acetate, Main Features: Indications: Fiber, g 5.3 3.9 Pantothenic Acid, mcg 2800 2074 M. Alpina Oil*, L-Tryptophan, Choline GOS, g 4.7 3.5 Biotin, mcg 18.2 13.5 Bitartrate, C. Cohnii Oil**, L-Glutamine, • Methionine-free powdered infant • Vitamin B6 non-responsive homocystinuria FOS, g 0.55 0.41 Vitamin C, mg 49.0 36.3 Sodium Chloride, M-Inositol, L-Ascorbic formula with iron • Hypermethioninemia Amino Acids, g Choline, mg 91.0 67.4 Acid, L-Glutamic Acid, Taurine, Ferrous L-Alanine 0.64 0.47 Sulfate, Zinc Sulfate, L-Carnitine, • Includes DHA & ARA Inositol, mg 98.0 72.6 DL-Alpha Tocopheryl Acetate, Calcium L-Arginine 1.09 0.81 Minerals D-Pantothenate, Niacinamide, • Contains a prebiotic fiber blend L-Aspartic Acid 0.93 0.69 Calcium, mg 371 275 Ascorbyl Palmitate, Manganese Sulfate, • Unflavored powder For details on administration and household measurements, please L-Cystine 0.41 0.30 Phosphorus, mg 270 200 Cupric Sulfate, Thiamine Chloride L-Glutamic Acid 1.22 0.90 Magnesium, mg 52.5 38.9 Hydrochloride, Pyridoxine Hydrochloride, • Contains a balanced mixture of all see the end of this section. L-Glutamine 0.11 0.08 Iron, mg 7.3 5.4 Riboflavin, Vitamin A Acetate, DL-alpha Tocopherol, Mixed Tocopherols, other essential and non-essential Glycine 0.99 0.73 Zinc, mg 5.2 3.9 Potassium Iodide, Chromium Chloride, amino acids, carbohydrates, fat, L-Histidine 0.62 0.46 Manganese, mcg 390 289 Folic Acid, Phylloquinone, Sodium Ordering/Reimbursement Information: L-Isoleucine 0.98 0.73 Copper, mcg 390 289 Selenite, Sodium Molybdate, D-Biotin, vitamins, minerals and trace elements L-Leucine 1.67 1.2 Iodine, mcg 82.5 61.1 Vitamin D , Cyanocobalamin Product Reimbursement HCPCS Product Calories 3 L-Lysine 1.14 0.84 Molybdenum, mcg 10.9 8.1 • Provides 13.5 g of protein equivalent Code Code Code* Packaging Per Can Contains: Soy Oil, Coconut Oil, Milk. L-Methionine Nil Added Nil Added Chromium, mcg 12.5 9.3 per 100 g of powder 90169 49735-0101-69 B4162 6 x 400 g (14 oz) 1892 L-Phenylalanine 0.74 0.55 Selenium, mcg 14.1 10.4 *A source of Arachidonic Acid (ARA) • Osmolality (at 0.7 kcal/mL): L-Proline 1.18 0.87 Sodium, mg 171 127 380 mOsm/kg L-Serine 0.73 0.54 Potassium, mg 454 336 **A source of Docosahexaenoic Acid (DHA) Based on dilution of 5 g powder WIC‡: Contact your state WIC For more reimbursement L-Threonine 0.82 0.61 Chloride, mg 321 238 L-Tryptophan 0.33 0.24 Metabolics Metabolics office to determine availability. information see Resources. Contains GOS/FOS 0.8 g per 100 mL (1 unpacked level scoop) to 1 fl oz L-Tyrosine 0.74 0.55 (30 mL) water L-Valine 1.07 0.79 * S9435 = For individuals who submit their bills to insurance on their own. • Displacement: 0.75 mL/g L-Carnitine 0.01 0.01 Taurine 0.02 0.01 102 103 HCU Anamix Next

Nutrition Information: Per 10 g Ingredients: ® Per 10 g HCU Anamix Next Nutrients Per 100 g Protein Equivalent Vitamins Per 100 g Protein Equivalent Corn Syrup Solids, Refined Vegetable Calories 385 137.5 Vitamin A, IU (mcg RE) 1399.(420) 500.(150) Oils (High Oleic Sunflower, Canola, A methionine-free, powdered medical food for the dietary management of Protein Equivalent, g 28 10 Vitamin D3, IU (mcg) 840.(21) 300.(7.5) Medium Chain Triglycerides (Palm Fat, g 12.5 4.5 Kernel and/or Coconut Oil), Sunflower), homocystinuria in individuals over 1 year of age. Vitamin E, IU (mg α-TE) 12.5 (8.4) 4.5 (3) Saturated, g 2.0 0.71 Vitamin K, mcg 14 5 L-Arginine L-Aspartate, L-Leucine, Monounsaturated, g 8.2 2.9 Thiamin, mg 0.93 0.33 Fructo-oligosaccharides, L-Lysine Polyunsaturated, g 1.8 0.64 Riboflavin, mg 0.73 0.26 Acetate, Inulin, Corn Starch, L-Glutamine, DHA, mg 180 64 Calcium Phosphate Dibasic, L-Proline, Vitamin B6, mg 0.73 0.26 Carbohydrate, g 43.2 15.4 Vitamin B , mcg 1.3 0.5 Microcrystalline Cellulose, L-Valine, 12 Artificial Flavor, Glycine, L-Isoleucine, Fiber, g 11.2 4 Niacin, mg 2.0 0.71 L-Threonine, Gum Arabic, L-Tyrosine, Soluble, g 8 2.9 Folic Acid, mcg 210 75 Main Features: Indications: L-Serine, L-Cystine, L-Phenylalanine, Insoluble, g 3.2 1.1 Pantothenic Acid, mg 4.2 1.5 L-Alanine, Tripotassium Citrate, Trisodium • Methionine free • Homocystinuria Amino Acids, g Biotin, mcg 11.2 4 Citrate, L-Histidine, Calcium Chloride, L-Alanine 1.4 0.5 Vitamin C, mg 42 15 • Contains a balanced mixture of all Magnesium Hydrogen Phosphate, L-Arginine 2.25 0.8 Choline, mg 224 80 Maltodextrin, Choline Bitartrate, CAEM other essential and non-essential For details on administration and household measurements, please see the end of this section. L-Aspartic Acid 2 0.71 Inositol, mg 56 20 (an emulsifier), L-Tryptophan,C. Cohnii amino acids, carbohydrate (including a L-Cystine 1.5 0.54 Minerals Oil*, Magnesium L-Aspartate, Tricalcium multi-fiber blend), fat (including DHA), L-Glutamine 2.6 0.93 Calcium, mg 980 350 Citrate, L-Ascorbic Acid, Soy Fiber, vitamins, minerals, and trace elements Glycine 2.13 0.76 Phosphorus, mg 641 229 M-Inositol, Taurine, Ferrous Sulfate, L-Histidine 1.22 0.44 Magnesium, mg 113 40 Sunflower Lecithin, L-Carnitine, Zinc • Provides 28 g protein equivalent and Ordering/Reimbursement Information: L-Isoleucine 2.1 0.75 Iron, mg 10 3.6 Sulfate, DL-alpha Tocopheryl Acetate, 12.5 g fat per 100 g of powder L-Leucine 3.46 1.24 Zinc, mg 4.9 1.8 Calcium D-Pantothenate, Manganese Product Reimbursement HCPCS Product Calories Sulfate, Niacinamide, Cupric Sulfate, L-Lysine 2.44 0.87 Manganese, mg 0.92 0.33 • Available in Plain Code Code Code* Packaging Per Can Thiamine Chloride Hydrochloride, L-Methionine Nil Added Nil Added Copper, mcg 560 200 • Osmolality (at 1.1 kcal/mL): 89470 49735-0194-70 B4162 6 x 400 g 1540 Pyridoxine Hydrochloride, Riboflavin, L-Phenylalanine 1.5 0.54 Iodine, mcg 139 49.6 1340 mOsm/kg B4157 Vitamin A Acetate, Ascorbyl Palmitate, L-Proline 2.52 0.9 Molybdenum, mcg 25.5 9.1 Folic Acid, Potassium iodide, Mixed Based on dilution of 36 g powder L-Serine 1.57 0.56 Chromium, mcg 10.2 3.6 ‡ Tocopherols, Sodium Molybdate, Sodium (approx 4 scoops) to 3.5 fl oz WIC : Contact your state WIC For more reimbursement L-Threonine 2 0.71 Selenium, mcg 28.2 10.1 Selenite, Chromium Chloride, DL-alpha office to determine availability. information see Resources. L-Tryptophan 0.5 0.18 Sodium, mg 280 100 (100 mL) water Tocopherol, Vitamin D3, Phylloquinone, L-Tyrosine 1.6 0.57 Potassium, mg 445 158.9 D-Biotin, Cyanocobalamin Metabolics • Displacement: 0.72 mL/g * S9435 = For individuals who submit their bills to insurance on their own. L-Valine 2.3 0.82 Chloride, mg 406 145 Contains: Coconut Oil, Soy. L-Carnitine 0.02 0.01 Taurine 0.05 0.02 * A source of Docosahexaenoic Acid (DHA) 104 105 XMet Maxamum

Nutrition Information: Ingredients: XMet Maxamum® Per 10 g Per 10 g Nutrients Per 100 g Protein Equivalent Vitamins Per 100 g Protein Equivalent Sugar, L-Lysine L-Glutamate, Corn Syrup Calories 305 76 Vitamin A, IU (mcg RE) 2228.(669) 557.(167) Solids, L-Leucine, Calcium Phosphate A methionine-free powdered medical food for the dietary management of Vitamin B 6 Protein Equivalent, g 40 10 Vitamin D , IU (mcg) 332.(8.3) 83.(2.1) Dibasic, L-Proline, L-Arginine, L-Valine, non-responsive homocystinuria or hypermethioninemia in older children and adults, 3 Fat, g <1 <0.25 Vitamin E, IU (mg α-TE) 18.3 (12.3) 4.6 (3.1) Glycine, L-Isoleucine, L-Aspartic Acid, L-Threonine, Artificial Flavor, including pregnant women and women of child-bearing age. Carbohydrate, g 34 8.5 Vitamin K, mcg 82 21 L-Phenylalanine, L-Tyrosine, L-Serine, Amino Acids, g Thiamin, mg 0.91 0.23 Citric Acid, Magnesium Acetate, L-Alanine 1.8 0.45 Riboflavin, mg 0.97 0.24 L-Alanine, L-Histidine, Tripotassium L-Arginine 3.2 0.80 Vitamin B6, mg 1.6 0.4 Citrate, L-Cystine, Choline Bitartrate, L-Aspartic Acid 2.7 0.68 L-Tryptophan, Sodium Chloride, Soy Vitamin B12, mcg 3.1 0.8 Main Features: Indications: L-Cystine 1.2 0.30 Niacin, mg 5.7 1.4 Lecithin, Disodium Hydrogen Phosphate, L-Glutamic Acid 3.6 0.90 Folic Acid, mcg 491 123 Dipotassium Hydrogen Phosphate, • Methionine free • Vitamin B non-responsive homocystinuria L-Glutamine, Tricalcium Phosphate, 6 Glycine 2.8 0.70 Pantothenic Acid, mg 4.9 1.2 L-Glutamic Acid, Taurine, • Contains a balanced mixture of all other • Hypermethioninemia L-Histidine 1.8 0.45 Biotin, mcg 24.5 6.1 L-Ascorbic Acid, M-Inositol, Ferrous essential and non-essential amino acids, L-Isoleucine 2.8 0.70 Vitamin C, mg 62 16 Sulfate, L-Carnitine, Zinc Sulfate, carbohydrate, vitamins, minerals and L-Leucine 4.9 1.23 Choline, mg 430 108 DL-Alpha Tocopheryl Acetate, L-Lysine 3.3 0.83 Inositol, mg 80 20 Niacinamide, Manganese Sulfate, trace elements For details on administration and household measurements, please L-Methionine Nil Added Nil Added Minerals Calcium D-Pantothenate, Cupric • Age-appropriate DRI formulation Sulfate, Pyridoxine Hydrochloride, see the end of this section. L-Phenylalanine 2.2 0.55 Calcium, mg 1183 296 Sodium Selenite, Thiamin Chloride • XMet Maxamum provides 40 g of L-Proline 3.4 0.85 Phosphorus, mg 1137 284 protein equivalent per 100 g of powder Hydrochloride, Riboflavin, Vitamin A L-Serine 2.1 0.53 Magnesium, mg 306 77 Acetate, Folic Acid, Artificial Colors • Available in orange flavor Ordering/Reimbursement Information: L-Threonine 2.4 0.60 Iron, mg 14.7 3.7 (Beta-Carotene, Beet Red), Potassium • Contains <1 g of fat per 10 g PE L-Tryptophan 0.95 0.24 Zinc, mg 9 2 Iodide, Chromium Chloride, Sodium Product Reimbursement HCPCS Product Calories L-Tyrosine 2.2 0.55 Manganese, mg 1.8 0.5 Molybdate, Phylloquinone, D-Biotin, thereby allowing greater flexibility in Code Code Code* Packaging Per Can Vitamin D , Cyanocobalamin. L-Valine 3.1 0.78 Copper, mcg 820 205 3 modifying energy intake 117795 49735-0177-95 B4157 or 6 x 454 g (1 lb) 1385 L-Carnitine 0.039 0.01 Iodine, mcg 131 33 Contains: Soy. B4162 • Osmolality (mOsm/kg): Taurine 0.14 0.04 Molybdenum, mcg 37 9 Dilution L-Glutamine 0.32 0.08 Chromium, mcg 26 7 1 to 7 800 WIC‡: Contact your state WIC For more reimbursement Selenium, mcg 58 15 Metabolics 1 to 5 1190 office to determine availability. information see Resources. Sodium, mg 560 140 Potassium, mg 700 175 • Displacement: 0.6 mL/g * S9435 = For individuals who submit their bills to insurance on their own. Chloride, mg 560 140 106 107 HCU Lophlex LQ

Nutrition Information: Ingredients: Per Pouch Per 10 g Per Pouch Per 10 g ® Nutrients (4.2 fl oz) Protein Equivalent Vitamins (4.2 fl oz) Protein Equivalent Water, Apple Juice from Concentrate HCU Lophlex LQ (34.1%), Grape Juice from Concentrate Calories 120 60 Vitamin A, IU (mcg RE) 949.(285) 475.(143) (6.9%), Blackcurrant Juice from Concentrate (2.5%), L-Arginine A methionine-free*, ready-to-drink medical food containing amino acids, carbohydrates, Protein Equivalent, g 20 10 Vitamin D3, IU (mcg) 320.(8.0) 160.(4.0) Fat, g 0.44 0.22 L-Aspartate, L-Leucine, L-Lysine Acetate, vitamins, minerals and DHA, based on mixed fruit juices (from concentrate) with natural Vitamin E, IU (mg α-TE) 4.8 (3.2) 2.4 (1.6) Citric Acid, L-Proline, L-Tyrosine, Glycine, flavor; for the dietary management of proven homocystinuria in individuals 4 years and DHA, mg 150 75 Vitamin K, mcg 24.9 12.5 L-Valine, L-Serine, L-Isoleucine, Corn Carbohydrate, g 9.3 4.7 Thiamin, mg 0.54 0.27 Syrup Solids, Dicalcium Phosphate, older, including pregnant women and women of child-bearing age. L-Alanine, L-Threonine, L-Phenylalanine, Fiber, g 0.50 0.25 Riboflavin, mg 0.50 0.25 L-Cystine, L-Histidine, Elderberry Amino Acids, g Vitamin B6, mg 0.58 0.29 Juice from Concentrate (0.6%), L-Alanine 1.18 0.59 Vitamin B , mcg 1.8 0.90 Maltodextrin, Magnesium L-Aspartate, 12 Magnesium Acetate, L-Tryptophan, L-Arginine 1.50 0.75 Niacin, mg 7.1 3.6 Choline Bitartrate, C. Cohnii Oil*, Sugar, Main Features: Indications: L-Aspartic Acid 1.73 0.87 Folic Acid, mcg 120 60.0 Microcrystalline Cellulose, Natural L-Cystine 0.98 0.49 Pantothenic Acid, mg 1.8 0.9 Flavor, Fruit Concentrate (Apple, • Methionine free • Homocystinuria Blackcurrant, Radish), L-Ascorbic Acid, Glycine 1.60 0.80 Biotin, mcg 53.4 26.7 Taurine, Guar Gum, Sunflower Lecithin, • Low volume; provides 20 g PE in just 4.2 For details on administration, please see the end of this section. L-Histidine 0.85 0.43 Vitamin C, mg 17.8 8.9 Xanthan Gum, M-Inositol, Potassium fl oz (125 mL) L-Isoleucine 1.35 0.68 Sorbate (preservative to delay spoilage), Choline, mg 153 76.5 Artificial Sweetener: Sucralose, Artificial • Convenient ready-to-drink L-Leucine 2.30 1.15 Inositol, mg 40.6 20.3 Sweetener: Acesulfame Potassium, L-Lysine 1.55 0.78 Minerals Ferrous Lactate, Sodium Benzoate • Based on fruit juices (from concentrate) (preservative to delay spoilage), Zinc with natural flavor - no artificial flavors L-Methionine Trace* Trace* Calcium, mg 319 160 Sulfate, L-Carnitine, Niacinamide, Ordering/Reimbursement Information: L-Phenylalanine 1.00 0.50 Phosphorus, mg 244 122 DL-alpha Tocopheryl Acetate, Calcium • Contains a balanced mixture of other L-Proline 2.03 1.02 Magnesium, mg 96.3 48.2 D-Pantothenate, Manganese Sulfate, essential and non-essential amino acids, Product Reimbursement HCPCS Product Calories Cupric Sulfate, Thiamin Chloride Code Code Code* Packaging Per Pouch L-Serine 1.38 0.69 Iron, mg 5.1 2.6 Hydrochloride, Pyridoxine Hydrochloride, carbohydrates, vitamins, minerals and L-Threonine 1.13 0.57 Vitamin A Palmitate, Riboflavin, Folic 82111 49735-0167-48 B4157 or B4162 30 X 4.2 fl oz (125 mL) 120 Zinc, mg 3.8 1.9 DHA L-Tryptophan 0.45 0.23 Manganese, mg 0.56 0.28 Acid, Potassium Iodide, Ascorbyl Palmitate, Mixed Tocopherols, Sodium • Provides 150 mg DHA per pouch L-Tyrosine 1.65 0.83 Copper, mcg 563 282 Molybdate, D-Biotin, Sodium Selenite, WIC‡: Contact your state WIC For more reimbursement L-Valine 1.45 0.73 Iodine, mcg 56.3 28.2 Chromium Chloride, Phylloquinone, • Available in mixed berry blast flavor Vitamin D , Cyanocobalamin. office to determine availability. information see Resources. L-Carnitine 0.01 0.01 Molybdenum, mcg 25.0 12.5 3 • Osmolality (at 0.96 kcal/mL): Contains: 44.1% juice. * S9435 = For individuals who submit their bills to insurance on their own. Taurine 0.06 0.03 Chromium, mcg 10.8 5.4

Metabolics 2460 mOsm/kg Selenium, mcg 26.8 13.4 *A Source of Docosahexaenoic Acid Sodium, mg <25.0 <12.5 (DHA). Potassium, mg 90 45 *May contain traces of methionine from fruit juice (from concentrate) at a level of <2 mg per 100 mL. Chloride, mg <25.0 <12.5 108 109 MMA/PA Anamix Early Years

Nutrition Information: Ingredients: ® Per 10 g Per 10 g MMA/PA Anamix Early Years Nutrients Per 100 g Protein Equivalent Vitamins Per 100 g Protein Equivalent Corn Syrup Solids, Refined Vegetable Calories 473 350 Vitamin A, IU (mcg RE) 1305.(392) 967.(290) Oils (High Oleic Sunflower, Soy, Non-hydrogenated Coconut, Canola, A methionine-, threonine- and valine-free, low in isoleucine powdered infant formula Protein Equivalent,g 13.5 10.0 Vitamin D3, IU (mcg) 348.(8.7) 258.(6.4) with iron, for the dietary management of methylmalonic or propionic acidemia (MMA/ Fat, g 23.0 17.0 Vitamin E, IU (mg α-TE) 10.9 (7.3) 8.1 (5.4) Sunflower), Galacto-oligosaccharides (GOS) Dried Syrup (from milk), L-Lysine PA). Contains DHA and ARA, and a unique blend of prebiotic fiber. Saturated, g 7.5 5.6 Vitamin K, mcg 38.0 28.1 Monounsaturated, g 9.4 7.0 Thiamin, mcg 640 474 L-Glutamate, L-Leucine, L-Alanine, Polyunsaturated, g 5.0 3.7 Riboflavin, mcg 500 370 L-Arginine, L-Aspartic Acid, Tricalcium Phosphate, CAEM (an emulsifier), DHA, mg 70.0 51.9 Vitamin B6, mcg 500 370 L-Histidine, L-Phenylalanine, L-Tyrosine, ARA, mg 140 104 Vitamin B12, mcg 1.2 0.89 Main Features: Indications: Linoleic Acid,mg 3500 2593 Niacin, mcg 2200 1630 L-Serine, Glycine, L-Proline, Potassium Carbohydrate, g 53.0 39.3 Folic Acid, mcg 55.0 40.7 Chloride, Fructo-oligosaccharides • A methionine-, threonine- and valine- • Methylmalonic acidemia Fiber, g 5.3 3.9 Pantothenic Acid, mcg 2800 2074 (FOS), Tripotassium Citrate, L-Cystine, GOS, g 4.7 3.5 Biotin, mcg 18.2 13.5 L-Tryptophan, Sodium Phosphate free, low in isoleucine powdered • Propionic acidemia FOS, g 0.55 0.41 Vitamin C, mg 49.0 36.3 Dibasic, Magnesium Acetate, M. Alpina infant formula with iron Amino Acids, g Choline, mg 91.0 67.4 Oil*, Choline Bitartrate, C. Cohnii Oil**, • Includes DHA & ARA For details on administration and household measurements, please L-Alanine 1.6 1.2 Inositol, mg 98.0 72.6 L-Glutamine, Sodium Chloride, M-Inositol, see the end of this section. L-Arginine 1.4 1.0 Minerals L-Ascorbic Acid, L-Glutamic Acid, • Contains a prebiotic fiber blend L-Aspartic Acid 1.27 0.94 Calcium, mg 371 275 Taurine, Ferrous Sulfate, Zinc Sulfate, • Unflavored powder L-Cystine 0.51 0.38 Phosphorus, mg 270 200 L-Carnitine, DL-alpha Tocopheryl Acetate, L-Glutamic Acid 1.59 1.2 Magnesium, mg 52.5 38.9 Calcium D-Pantothenate, Niacinamide, • Contains a balanced mixture of all L-Glutamine 0.11 0.08 Iron, mg 7.3 5.4 Ascorbyl Palmitate, Manganese Sulfate, other essential and non-essential Glycine 0.64 0.47 Zinc, mg 5.2 3.9 Cupric Sulfate, Thiamine Chloride amino acids, carbohydrates, fat, Ordering/Reimbursement Information: L-Histidine 0.94 0.70 Manganese, mcg 390 289 Hydrochloride, Pyridoxine Hydrochloride, Riboflavin, Vitamin A Acetate, DL-alpha vitamins, minerals and trace elements L-Isoleucine <0.01 <0.01 Copper, mcg 390 289 Product Reimbursement HCPCS Product Calories L-Leucine 2.12 1.6 Iodine, mcg 82.5 61.1 Tocopherol, Mixed Tocopherols, • Provides 13.5 g of protein equivalent Code Code Code* Packaging Per Can L-Lysine 1.43 1.1 Molybdenum, mcg 10.9 8.1 Potassium Iodide, Chromium Chloride, per 100 g of powder 90215 49735-0102-15 B4162 6 x 400 g (14 oz) 1892 L-Methionine Nil Added Nil Added Chromium, mcg 12.5 9.3 Folic Acid, Phylloquinone, Sodium L-Phenylalanine 0.93 0.69 Selenium, mcg 14.1 10.4 Selenite, Sodium Molybdate, D-Biotin,

• Osmolality (at 0.7 kcal/mL): L-Proline 0.64 0.47 Sodium, mg 171 127 Vitamin D3, Cyanocobalamin 380 mOsm/kg WIC‡: Contact your state WIC For more reimbursement L-Serine 0.89 0.66 Potassium, mg 454 336 Contains: Soy Oil, Coconut Oil, Milk. Based on dilution of 5 g powder office to determine availability. information see Resources. L-Threonine Nil Added Nil Added Chloride, mg 321 238 L-Tryptophan 0.41 0.30 *A source of Arachidonic Acid (ARA) Metabolics Metabolics (1 unpacked level scoop) to 1 fl oz L-Tyrosine 0.93 0.69 * S9435 = For individuals who submit their bills to insurance on their own. **A source of Docosahexaenoic Acid (30 mL) water L-Valine Nil Added Nil Added (DHA) • Displacement: 0.75 mL/g L-Carnitine 0.01 0.01 Taurine 0.02 0.01 Contains GOS/FOS 0.8 g per 100 mL 110 111 MMA/PA Anamix Next

Nutrition Information: Ingredients: ® Per 10 g Per 10 g MMA/PA Anamix Next Nutrients Per 100 g Protein Equivalent Vitamins Per 100 g Protein Equivalent Corn Syrup Solids, Refined Vegetable Calories 377 135 Vitamin A, IU (mcg RE) 1748.(525) 628.(189) Oils (High Oleic Sunflower, Canola, A methionine-, threonine-, valine-free, low isoleucine, powdered medical food for the Protein Equivalent, g 28 10 Vitamin D3, IU (mcg) 1052.(26.3) 379.(9.6) Medium Chain Triglycerides (Palm dietary management of methylmalonic acidemia and propionic acidemia in individuals over Fat, g 12.5 4.5 Vitamin E, IU (mg α-TE) 15.6 (10.5) 5.6 (3.8) Kernel and/or Coconut Oil), Sunflower), 1 year of age. Saturated, g 2.0 0.71 Vitamin K, mcg 17.5 6.4 L-Leucine, L-Lysine Acetate, L-Glutamine, Monounsaturated, g 8.2 2.9 Thiamin, mg 1.2 0.4 Fructo-oligosaccharides, L-Alanine, Polyunsaturated, g 1.8 0.64 Riboflavin, mg 0.91 0.32 Inulin, Calcium Phosphate Dibasic, DHA, mg 180 64 L-Arginine, Corn Starch, L-Aspartic Vitamin B6, mg 0.91 0.32 Carbohydrate, g 41.2 14.8 Vitamin B , mcg 1.6 0.56 Acid, Microcrystalline Cellulose, 12 Artificial Flavor, L-Histidine, L-Tyrosine, Fiber, g 11.2 4 Niacin, mg 2.5 0.90 L-Phenylalanine, L-Serine, Gum Arabic, Soluble, g 8 2.9 Folic Acid, mcg 263 94.8 Main Features: Indications: Tripotassium Citrate, Trisodium Citrate, Insoluble, g 3.2 1.1 Pantothenic Acid, mg 5.3 1.92 • Methionine, threonine, valine free • Methylmalonic Acidemia L-Cystine, Glycine, L-Proline, Calcium Amino Acids, g Biotin, mcg 14 5.2 Chloride, Magnesium Hydrogen • Low isoleucine • Propionic Acidemia L-Alanine 3.42 1.22 Vitamin C, mg 52.5 18.8 Phosphate, Maltodextrin, Choline L-Arginine 3.0 1.07 Choline, mg 280 100 • Contains a balanced mixture of all other Bitartrate, L-Tryptophan, Tricalcium L-Aspartic Acid 2.72 0.97 Inositol, mg 70 25.2 Citrate, CAEM (an emulsifier), essential and non-essential amino acids, For details on administration and household measurements, please see the end of this section. L-Cystine 1.56 0.56 Minerals C. Cohnii Oil*, L-Ascorbic Acid, M-Inositol, carbohydrate (including a multi-fiber L-Glutamine 3.61 1.29 Calcium, mg 1225 440 Soy Fiber, Taurine, Ferrous Sulfate, blend), fat (including DHA), vitamins, Glycine 1.37 0.49 Phosphorus, mg 801 288 Sunflower Lecithin, L-Carnitine, Zinc minerals, and trace elements L-Histidine 2.02 0.72 Magnesium, mg 140 50.4 Sulfate, DL-alpha Tocopheryl Acetate, Ordering/Reimbursement Information: L-Isoleucine <0.03 <0.03 Iron, mg 12.5 4.4 Calcium D-Pantothenate, Manganese • Provides 28 g protein equivalent and L-Leucine 4.54 1.62 Zinc, mg 6.1 2.2 Sulfate, Niacinamide, Cupric Sulfate, Thiamine Chloride Hydrochloride, 12.5 g fat per 100 g of powder Product Reimbursement HCPCS Product Calories L-Lysine 3.05 1.09 Manganese, mg 1.2 0.44 Pyridoxine Hydrochloride, Riboflavin, Code Code Code* Packaging Per Can L-Methionine Nil Added Nil Added Copper, mcg 700 252 • Available in Plain Vitamin A Acetate, Folic Acid, Ascorbyl 89472 49735-0194-72 B4162 6 x 400 g 1508 • Osmolality (at 1.1 kcal/mL): L-Phenylalanine 1.99 0.71 Iodine, mcg 174 62.8 Palmitate, Potassium iodide, Mixed B4157 L-Proline 1.37 0.49 Molybdenum, mcg 31.9 11.4 1220 mOsm/kg Tocopherols, Sodium Molybdate, Sodium L-Serine 1.98 0.71 Chromium, mcg 12.8 4.8 Selenite, Chromium Chloride, DL-alpha Based on dilution of 36 g powder ‡ L-Threonine Nil Added Nil Added Selenium, mcg 35.3 12.8 Tocopherol, Vitamin D , Phylloquinone, WIC : Contact your state WIC For more reimbursement 3 (approx 4 scoops) to 3.5 fl oz L-Tryptophan 0.69 0.25 office to determine availability. information see Resources. Sodium, mg 350 126 D-Biotin, Cyanocobalamin L-Tyrosine 1.99 0.71 Potassium, mg 556 200 Metabolics (100 mL) water Contains: Coconut Oil, Soy. • Displacement: 0.72 mL/g * S9435 = For individuals who submit their bills to insurance on their own. L-Valine Nil Added Nil Added Chloride, mg 508 183 L-Carnitine 0.02 0.01 *A source of Docosahexaenoic Acid Taurine 0.06 0.02 (DHA) 112 113 XMTVI Maxamum

® Nutrition Information: Ingredients: XMTVI Maxamum Per 10 g Per 10 g Nutrients Per 100 g Protein Equivalent Vitamins Per 100 g Protein Equivalent Sugar, L-Lysine L-Glutamate, L-Leucine, A methionine-, threonine-, valine-free and isoleucine-low medical food for the dietary Calories 305 76 Vitamin A, IU (mcg RE) 2228 (669) 557 (167) Corn Syrup Solids, L-Alanine, Calcium Protein Equivalent, g 40 10 Vitamin D , IU (mcg) 332 (8.3) 83 (2.1) Phosphate Dibasic, L-Arginine, L-Aspartic management of Vitamin B12 non-responsive methylmalonic acidemia or propionic acidemia 3 in older children and adults, including pregnant women and women of child-bearing age. Fat, g <1 <0.25 Vitamin E, IU (mg α-TE) 18.3 (12.3) 4.6 (3.1) Acid, L-Histidine, L-Phenylalanine, L-Tyrosine, L-Serine, Artificial Flavor, Carbohydrate, g 34 8.5 Vitamin K, mcg 82 21 Citric Acid, Magnesium Acetate, Glycine, Amino Acids, g 0.23 Thiamin, mg 0.91 L-Proline, L-Cystine, L-Tryptophan, L-Alanine 4.6 1.15 Riboflavin, mg 0.97 0.24 Tripotassium Citrate, Choline Bitartrate, L-Arginine 4.1 1.03 Vitamin B , mg 1.6 0.4 Sodium Chloride, Soy Lecithin, Disodium Main Features: Indications: 6 L-Aspartic Acid 3.6 0.90 Hydrogen Phosphate, Dipotassium Vitamin B12, mcg 3.1 0.8 L-Cystine 1.5 0.38 Niacin, mg 5.7 1.4 Hydrogen Phosphate, L-Glutamine, • Methionine, threonine, valine free and • Vitamin B12 non-responsive methylmalonic acidemia isoleucine low L-Glutamic Acid 4.5 1.13 Folic Acid, mcg 491 123 Tricalcium Phosphate, L-Glutamic Acid, • Propionic acidemia Taurine, L-Ascorbic Acid, M-Inositol, Glycine 1.8 0.45 Pantothenic Acid, mg 4.9 1.2 • Contains a balanced mixture of all Ferrous Sulfate, L-Carnitine, Zinc L-Histidine 2.8 0.70 6.1 other essential and non-essential For details on administration and household measurements, please Biotin, mcg 24.5 Sulfate, DL-Alpha Tocopheryl Acetate, L-Isoleucine <0.14 Trace Vitamin C, mg 62 16 amino acids, carbohydrate, vitamins, see the end of this section. Niacinamide, Manganese Sulfate, L-Leucine 6.2 1.55 Choline, mg 430 108 Calcium D-Pantothenate, Cupric minerals and L-Lysine 4.2 1.05 Inositol, mg 80 20 Sulfate, Pyridoxine Hydrochloride, trace elements L-Methionine Nil Added Nil Added Minerals Sodium Selenite, Thiamin Chloride Hydrochloride, Riboflavin, Vitamin A • Age-appropriate DRI formulation L-Phenylalanine 2.7 0.68 Calcium, mg 1183 296 Acetate, Folic Acid, Artificial Colors (Beta L-Proline 1.9 0.48 284 • XMTVI Maxamum provides Phosphorus, mg 1137 Carotene, Beet Red), Potassium Iodide, 40 g of protein equivalent per Ordering/Reimbursement Information: L-Serine 2.6 0.65 Magnesium, mg 306 77 Chromium Chloride, Sodium Molybdate, L-Threonine Nil Added Nil Added Iron, mg 14.7 3.7 Phylloquinone, D-Biotin, Vitamin D , 100 g of powder Product Reimbursement HCPCS Product Calories 3 L-Tryptophan 1.2 0.30 Zinc, mg 9 2 Cyanocobalamin. Code Code Code* Packaging Per Can • Available in orange flavor L-Tyrosine 2.7 0.68 Manganese, mg 1.8 0.5 117779 49735-0177-79 B4162 or 6 x 454 g (1 lb) 1385 Contains: Soy. • Contains <1 g of fat per 10 g PE B4157 L-Valine Nil Added Nil Added Copper, mcg 820 205 thereby allowing greater flexibility in L-Carnitine 0.039 0.01 Iodine, mcg 131 33 Taurine 0.14 0.04 Molybdenum, mcg 37 9 modifying energy intake ‡ WIC : Contact your state WIC For more reimbursement L-Glutamine 0.32 0.08 7 • Osmolality (mOsm/kg): Chromium, mcg 26 office to determine availability. information see Resources. Selenium, mcg 58 15 Metabolics Metabolics Dilution * S9435 = For individuals who submit their bills to insurance on their own. Sodium, mg 560 140 1 to 7 740 Potassium, mg 700 175 1 to 5 1070 Chloride, mg 560 140 114 • Displacement: 0.6 mL/g 115 TYR Anamix Early Years

Nutrition Information: Ingredients: ® Per 10 g Per 10 g TYR Anamix Early Years Nutrients Per 100 g Protein Equivalent Vitamins Per 100 g Protein Equivalent Corn Syrup Solids, Refined Vegetable Calories 473 350 Vitamin A, IU (mcg RE) 1305.(392) 967.(290) Oils (High Oleic Sunflower, Soy, Non-hydrogenated Coconut, Canola, A phenylalanine-, tyrosine-free powdered infant formula with iron, for the dietary Protein Equivalent,g 13.5 10.0 Vitamin D , IU (mcg) 348.(8.7) 258.(6.4) 3 Sunflower), Galacto-oligosaccharides Fat, g 23.0 17.0 management of tyrosinemia (TYR). Contains DHA and ARA, and a unique blend of Vitamin E, IU (mg α-TE) 10.9 (7.3) 8.1 (5.4) (GOS) Dried Syrup (from milk), L-Lysine prebiotic fiber. Saturated, g 7.5 5.6 Vitamin K, mcg 38.0 28.1 L-Glutamate, L-Leucine, L-Proline, Monounsaturated, g 9.4 7.0 Thiamin, mcg 640 474 L-Arginine, L-Valine, Tricalcium Polyunsaturated, g 5.0 3.7 Riboflavin, mcg 500 370 Phosphate, Glycine, L-Isoleucine, DHA, mg 70.0 51.9 Vitamin B6, mcg 500 370 L-Aspartic Acid, CAEM (an emulsifier), Main Features: Indications: ARA, mg 140 104 Vitamin B12, mcg 1.2 0.89 L-Threonine, L-Serine, L-Alanine, Linoleic Acid,mg 3500 2593 Niacin, mcg 2200 1630 L-Histidine, Potassium Chloride, Fructo- • A phenylalanine-, tyrosine-free • Tyrosinemia Carbohydrate, g 53.0 39.3 Folic Acid, mcg 55.0 40.7 oligosaccharides (FOS), Tripotassium powdered infant formula with iron Fiber, g 5.3 3.9 Pantothenic Acid, mcg 2800 2074 Citrate, L-Cystine, Sodium Phosphate Dibasic, L-Tryptophan, Magnesium • Includes DHA & ARA GOS, g 4.7 3.5 Biotin, mcg 18.2 13.5 FOS, g 0.55 0.41 Vitamin C, mg 49.0 36.3 Acetate, M. Alpina Oil*, L-Methionine, Choline Bitartrate, Oil**, • Contains a prebiotic fiber blend For details on administration and household measurements, please Amino Acids, g Choline, mg 91.0 67.4 C. Cohnii L-Glutamine, Sodium Chloride, M-Inositol, see the end of this section. L-Alanine 0.68 0.50 • Unflavored powder Inositol, mg 98.0 72.6 L-Ascorbic Acid, L-Glutamic Acid, • Contains a balanced mixture of all L-Arginine 1.19 0.88 Minerals Taurine, Ferrous Sulfate, Zinc Sulfate, L-Aspartic Acid 1 0.74 Calcium, mg 371 275 L-Carnitine, DL-alpha Tocopheryl Acetate, other essential and non-essential L-Cystine 0.45 0.33 Phosphorus, mg 270 200 Calcium D-Pantothenate, Niacinamide, amino acids, carbohydrates, fat, Ordering/Reimbursement Information: L-Glutamic Acid 1.34 0.99 Magnesium, mg 52.5 38.9 Ascorbyl Palmitate, Manganese Sulfate, vitamins, minerals and trace elements L-Glutamine 0.11 0.08 Iron, mg 7.3 5.4 Cupric Sulfate, Thiamine Chloride • Provides 13.5 g of protein equivalent Product Reimbursement HCPCS Product Calories Glycine 1.07 0.79 Zinc, mg 5.2 3.9 Hydrochloride, Pyridoxine Hydrochloride, Code Code Code* Packaging Per Can L-Histidine 0.68 0.50 Manganese, mcg 390 289 Riboflavin, Vitamin A Acetate, DL-alpha per 100 g of powder Tocopherol, Mixed Tocopherols, 90218 49735-0102-18 B4162 6 x 400 g (14 oz) 1892 L-Isoleucine 1.07 0.79 Copper, mcg 390 289 • Osmolality (at 0.7 kcal/mL): L-Leucine 1.83 1.4 Iodine, mcg 82.5 61.1 Potassium Iodide, Chromium Chloride, L-Lysine 1.24 0.92 Molybdenum, mcg 10.9 8.1 Folic Acid, Phylloquinone, Sodium 380 mOsm/kg Selenite, Sodium Molybdate, D-Biotin, L-Methionine 0.29 0.21 Chromium, mcg 12.5 9.3 ‡ Vitamin D3, Cyanocobalamin Based on dilution of 5 g powder WIC : Contact your state WIC For more reimbursement L-Phenylalanine Nil Added Nil Added Selenium, mcg 14.1 10.4 (1 unpacked level scoop) to 1 fl oz office to determine availability. information see Resources. L-Proline 1.3 0.96 Sodium, mg 171 127 Contains: Soy Oil, Coconut Oil, Milk. (30 mL) water L-Serine 0.8 0.59 Potassium, mg 454 336 L-Threonine 0.89 0.66 *A source of Arachidonic Acid (ARA) • Displacement: 0.75 mL/g * S9435 = For individuals who submit their bills to insurance on their own. Chloride, mg 321 238 L-Tryptophan 0.36 0.27 Metabolics Metabolics **A source of Docosahexaenoic Acid L-Tyrosine Nil Added Nil Added (DHA) Visit NutriciaLearningCenter.com L-Valine 1.17 0.9 Contains GOS/FOS 0.8 g per 100 mL for guidelines for the nutritional L-Carnitine 0.01 0.0 Taurine 0.02 0.0 116 management of TYR. 117 TYR Anamix Next

Nutrition Information: Ingredients: Per 10 g Per 10 g ® Nutrients Per 100 g Protein Equivalent Vitamins Per 100 g Protein Equivalent Corn Syrup Solids, Refined Vegetable Oils TYR Anamix Next Calories 385 138 Vitamin A, IU (mcg RE) 1399.(420) 499.(150) (High Oleic Sunflower, Canola, Medium Protein Equivalent, g 28 10 Vitamin D , IU (mcg) 840.(21) 300.(7.5) Chain Triglycerides (Palm Kernel and/ A phenylalanine- and tyrosine-free, powdered medical food for the dietary management 3 Fat, g 12.5 4.5 Vitamin E, IU (mg α-TE) 12.5 (8.4) 4.5 (3) or Coconut Oil), Sunflower), L-Alanine, Glycine, Fructo-oligosaccharides, of tyrosinemia in individuals over 1 year of age. Saturated, g 2 0.71 Vitamin K, mcg 14 5 L-Glutamine, L-Lysine Acetate, ]Inulin, Monounsaturated, g 8.2 2.9 Thiamin, mg 0.93 0.33 Polyunsaturated, g 1.8 0.64 L-Threonine, Corn Starch, L-Cystine, Riboflavin, mg 0.73 0.26 DHA, mg 180 64.3 L-Proline, Calcium Phosphate Dibasic, VitaminB , mg 0.73 0.26 Carbohydrate, g 43.2 15.4 6 L-Leucine, Microcrystalline Cellulose, Artificial Flavor, L-Valine, Gum Arabic, Fiber, g 11.2 4 Vitamin B12, mcg 1.3 0.46 Main Features: Indications: L-Arginine, L-Isoleucine, L-Serine, Soluble 8 2.9 Niacin, mg 2.0 0.71 • Phenylalanine and tyrosine free • Tyrosinemia Folic Acid, mcg 210 75 Tripotassium Citrate, Trisodium Insoluble 3.2 1.1 Citrate, Calcium Chloride, L-Histidine, Pantothenic Acid, mg 4.2 1.5 • Contains a balanced mixture of all Amino Acids, g Maltodextrin, Choline Bitartrate, other essential and non-essential For details on administration and household measurements, please L-Alanine 4.71 1.7 Biotin, mcg 11.2 4 Magnesium Hydrogen Phosphate, amino acids, carbohydrate (including see the end of this section. L-Arginine 1.67 0.6 Vitamin C, mg 42 15 L-Methionine, L-Tryptophan, Magnesium a multi-fiber blend), fat (including L-Aspartic Acid 0.41 0.15 Choline, mg 224 80 L-Aspartate, CAEM (an emulsifier), DHA), vitamins, minerals and trace L-Cystine 2.71 0.97 Inositol, mg 56 20 C. Cohnii Oil*, Tricalcium Citrate, L-Glutamine 3.36 1.2 Minerals L-Ascorbic Acid, Soy Fiber, M-Inositol, elements Taurine, Ferrous Sulfate, Sunflower Glycine 3.94 1.4 Calcium, mg 980 350 • Provides 28 g protein equivalent and Ordering/Reimbursement Information: L-Histidine 0.79 0.28 Lecithin, L-Carnitine, Zinc Sulfate, Phosphorus, mg 641 229 DL-alpha Tocopheryl Acetate, Calcium L-Isoleucine 1.66 0.59 12.5 g fat per 100 g of powder Product Reimbursement HCPCS Product Calories Magnesium, mg 113 40 D-Pantothenate, Manganese Sulfate, L-Leucine 2.52 0.9 • Available in a plain flavor Code Code Code* Packaging Per Can Iron, mg 10 4 Niacinamide, Cupric Sulfate, Thiamin L-Lysine 2.24 0.8 89479 49735-0194-79 B4162 6 x 400 g (14 oz) 1540 Zinc, mg 4.9 1.8 Chloride Hydrochloride, Pyridoxine • Osmolality (at 1.1 kcal/mL): L-Methionine 0.56 0.2 B4157 Manganese, mg 0.92 0.33 Hydrochloride, Riboflavin, Vitamin A 1220 mOsm/kg L-Phenylalanine Nil Added Nil Added Copper, mcg 560 200 Acetate, Ascorbyl Palmitate, Folic Acid, Based on dilution of 36 g powder L-Proline 2.71 0.97 Potassium Iodide, Mixed Tocopherols, Iodine, mcg 139 50 WIC‡: Contact your state WIC For more reimbursement L-Serine 1.59 0.57 Sodium Molybdate, Sodium Selenite, (approx 4 scoops) to 3.5 fl oz Molybdenum, mcg 25.5 9.1 (100 mL) water office to determine availability. information see Resources. L-Threonine 2.94 1.1 Chromium Chloride, DL-alpha L-Tryptophan 0.56 0.2 Chromium, mcg 10.2 3.6 Tocopherol, Vitamin D3, Phylloquinone, • Displacement: 0.72 mL/g Selenium, mcg 28.2 10.1 D-Biotin, Cyanocobalamin

Metabolics Metabolics L-Tyrosine Nil Added Nil Added * S9435 = For individuals who submit their bills to insurance on their own. L-Valine 1.96 0.7 Sodium, mg 280 100 Contains: Coconut Oil, Soy. L-Carnitine 0.02 0.01 Potassium, mg 445 159 Taurine 0.05 0.02 Chloride, mg 406 145 *A source of Docosahexaenoic Acid (DHA) 118 119 TYR Lophlex LQ

Amino Acids, g Vitamin B6, mg 0.58 0.29 Elderberry Juice from Concentrate L-Alanine 1.13 0.57 Vitamin B , mcg 1.8 0.90 (0.6%), Maltodextrin, Magnesium 12 Acetate, Sugar, N-Acetyl L-Methionine, L-Arginine 1.68 0.84 Main Features: Indications: Niacin, mg 7.1 3.6 L-Tryptophan, Choline Bitartrate, C. L-Aspartic Acid 1.63 0.82 Folic Acid, mcg 120 60.0 Cohnii Oil*, Microcrystalline Cellulose, • Tyrosine and phenylalanine free • Tyrosinemia L-Cystine 0.83 0.42 Pantothenic Acid, mg 1.8 0.9 Natural Flavor, Taurine, Fruit Concentrate Glycine 1.63 0.82 Biotin, mcg 53.4 26.7 (Apple, Blackcurrant, Radish), L-Ascorbic • Low volume; provides 20 g PE in just Acid, Guar Gum, Sunflower Lecithin, L-Histidine 0.95 0.48 4.2 fl oz (125 mL) Vitamin C, mg 17.8 8.9 Xanthan Gum, M-Inositol, Potassium L-Isoleucine 1.55 0.78 Choline, mg 153 76.5 For details on administration, please see the end of this section. Sorbate (preservative to delay spoilage), • Convenient ready-to-drink L-Leucine 2.50 1.25 Inositol, mg 40.6 20.3 Artificial Sweetener: Sucralose, • Based on fruit juices (from concentrate) L-Lysine 1.75 0.88 Minerals Ferrous Lactate, Artificial Sweetener: Acesulfame Potassium, L-Carnitine, L-Methionine 0.40 0.20 with natural flavor - no artificial flavors Calcium, mg 319 160 Sodium Benzoate (preservative to delay • Contains a balanced mixture of L-Phenylalanine Trace* Trace* Phosphorus, mg 244 122 spoilage), Zinc Sulfate, Niacinamide, essential and non-essential amino acids, Ordering/Reimbursement Information: L-Proline 2.03 1.02 Magnesium, mg 96.3 48.2 DL-alpha Tocopheryl Acetate, Calcium L-Serine 1.30 0.65 Iron, mg 5.1 2.6 D-Pantothenate, Manganese Sulfate, carbohydrates, vitamins, minerals and Product Reimbursement HCPCS Product Calories Cupric Sulfate, Thiamin Chloride L-Threonine 1.25 0.63 DHA Code Code Code* Packaging Per Pouch Zinc, mg 3.8 1.9 Hydrochloride, Pyridoxine Hydrochloride, L-Tryptophan 0.50 0.25 Manganese, mg 0.56 0.28 82113 49735-0167-50 B4157 or B4162 30 X 4.2 fl oz (125 mL) 120 Vitamin A Palmitate, Riboflavin, Folic • Contains 150 mg of DHA per pouch L-Tyrosine Trace* Trace* Copper, mcg 563 282 Acid, Potassium Iodide, Ascorbyl • Available in mixed berry blast flavor L-Valine 1.65 0.83 Iodine, mcg 56.3 28.2 Palmitate, Mixed Tocopherols, Sodium Molybdate, D-Biotin, Sodium Selenite, ‡ L-Carnitine 0.02 0.01 • Osmolality (at 0.96 kcal/mL): WIC : Contact your state WIC For more reimbursement Molybdenum, mcg 25.0 12.5 Chromium Chloride, Phylloquinone, office to determine availability. information see Resources. Taurine 0.14 0.07 Chromium, mcg 10.8 5.4 Vitamin D , Cyanocobalamin. 2460 mOsm/kg 3

Metabolics Selenium, mcg 26.8 13.4 * S9435 = For individuals who submit their bills to insurance on their own. *A Source of Docosahexaenoic Acid Sodium, mg <25.0 <12.5 (DHA). Potassium, mg 90 45 Chloride, mg <25.0 <12.5 Contains: 44.1% juice. 120 *May contain traces of tyrosine and phenylalanine from fruit juice (from concentrate). 121 TYR Lophlex GMP MIX-IN

Nutrition Information: Ingredients: Per 10 g Protein ® Per 100 g Equivalent Whey Protein Concentrate* (From Milk), TYR Lophlex GMP MIX-IN Nutrients Powder (1 pouch) L-Leucine, Glycine, L-Lysine Acetate, Calories 324 40 L-Histidine, L-Arginine, L-Valine. Contains A powdered medical food, low in phenylalanine and tyrosine, containing a mix Protein Equivalent, g 80 10 2% or less of the following: L-Tryptophan, L-Cystine, L-Methionine, Corn Syrup Solids, of glycomacropeptide (GMP), essential and non-essential amino acids with small Fat, g <0.1 <0.1 Silicon Dioxide, Taurine, L-Carnitine. amounts of carbohydrates and some minerals. For the dietary management of Carbohydrate, g <1 <0.5 proven Tyrosinemia (TYR) in individuals over 1 year of age. Amino Acids, g Contains: Milk. L-Alanine 3.2 0.40 *Contains 18 mg of Phenylalanine and L-Arginine 3.9 0.48 3.5 mg Tyrosine per 10 g Protein Equivalent L-Aspartic Acid 4.7 0.59 L-Cystine 1.2 0.15 Note: Must be combined with an appropriate Main Features: Indications: vitamin & mineral supplement when taken as L-Glutamic Acid 10.8 1.35 • Tyrosinemia a sole source of TYR Medical Food. • A flexible source of GMP-based L-Glutamine protein to help patients adhere Glycine 7.5 0.94 to their nutritional goals For details on administration, please see the end of this section. L-Histidine 3.9 0.49 • The highest amount of protein per L-Isoleucine 5.8 0.72 100 g powder of any TYR product L-Leucine 11.6 1.45 L-Lysine 6.2 0.78 • Mix into TYR formula to increase L-Methionine 1.9 0.23 protein from a GMP-based source L-Phenylalanine, mg 143 18 • Mixes easily with flavored drinks Ordering/Reimbursement Information: L-Proline 6.3 0.79 and foods for variety Product Reimbursement HCPCS Product Calories L-Serine 4.1 0.51 • Provides only 40 kcal per pouch Code Code Code* Packaging Per Pouch L-Threonine 9.0 1.13 135757 49735-0157-57 B4155 20x12.5g (0.44 oz) 40 L-Tryptophan 1.9 0.24 • Less than 0.5 g sugar per pouch L-Tyrosine, mg 28 3.5 • No fat, no artificial flavors L-Valine 7.0 0.88 WIC‡: Contact your state WIC For more reimbursement L-Carnitine 0.08 0.01 office to determine availability. information see Resources. Taurine 0.24 0.030

Metabolics Minerals and Trace Elements *S9435 = For individuals who submit their bills to insurance on their own. Calcium, mg 62.1 7.8 Phosphorus, mg 320 40.0 Potassium, mg 960 120 122 Sodium, mg 1125 141 123 UCD Anamix Junior

UCD Anamix® Junior Nutrition Information: Ingredients: Per 10 g Protein Per 10 g Protein A powdered medical food containing essential amino acids (with Unflavored: Corn Syrup Solids, Refined Nutrients Per 100 g Equivalent Vitamins Per 100 g Equivalent Vegetable Oils (High Oleic Safflower, increased levels of branched chain amino acids (BCAAs) and tryptophan), Calories 385 321 Vitamin A, IU (mcg RE) 3197.(960) 2664.(800) Canola, Fractionated Coconut), Sugar, carbohydrates, fat, vitamins and minerals; for the dietary management Protein Equivalent, g 12.0 10.0 Vitamin D3, IU (mcg) 920.(23.0) 767.(19.2) L-Leucine, Sodium Phosphate Dibasic, of urea cycle disorders (UCD); hyperammonemia, hyperornithinemia, Fat, g 17.0 14.2 Vitamin E, IU (mg α-TE) 27.5 (18.5) 22.9 (15.4) Tripotassium Citrate, Tricalcium Citrate, Tricalcium Phosphate, L-Lysine homocitrullinemia (HHH) syndrome; and gyrate atrophy in patients over Carbohydrate, g 46.0 38.3 Vitamin K, mcg 92.2 76.8 Hydrochloride, L-Valine, L-Isoleucine, Amino Acids, g the age of 1. Thiamin, mg 1.9 1.6 Magnesium Hydrogen Phosphate, L-Alanine Nil Added Nil Added Riboflavin, mg 1.5 1.3 L-Threonine, Choline Bitartrate, Main Features: Indications: L-Arginine Nil Added Nil Added Vitamin B6, mg 1.5 1.3 L-Tyrosine, Potassium Chloride, Citric L-Asparagine Nil Added Nil Added Vitamin B12, mcg 3.8 3.2 Acid Ester of Mono- And Diglycerides (An • Contains essential amino acids to • Urea Cycle Disorders (UCD) L-Aspartic Acid Nil Added Nil Added Niacin, mg 9.6 8.0 Emulsifier), L-Phenylalanine, L-Histidine, L-Tryptophan, L-Cystine, L-Methionine, help maintain a positive nitrogen L-Citrulline Nil Added Nil Added Folic Acid, mcg 461 384 • Hyperammonemia, Hyperornithinemia, Homocitrullinemia L-Ascorbic Acid, M-Inositol, Ferrous L-Cystine 0.43 0.36 balance in UCD (HHH) syndrome Pantothenic Acid, mg 6.1 5.1 Sulfate, Taurine, Zinc Sulfate, DL-alpha • Amino acid profile rich in BCAAs for L-Glutamic Acid Nil Added Nil Added Biotin, mcg 34.6 28.8 Tocopheryl Acetate, Manganese Sulfate, • Gyrate atrophy L-Glutamine Nil Added Nil Added efficient and optimal protein synthesis Vitamin C, mg 120 100 Niacinamide, L-Carnitine, Calcium-D- For details on administration and household measurements, please Glycine Nil Added Nil Added Choline, mg 480 400 Pantothenate, Cupric Sulfate, Thiamin for children with UCD Chloride Hydrochloride, Pyridoxine see the end of this section. L-Histidine 0.51 0.43 Inositol, mg 108 90 • Sufficient non-protein calories to L-Isoleucine 2.26 1.88 Minerals Hydrochloride, Riboflavin, Vitamin A Acetate, Folic Acid, Potassium Iodide, prevent protein being broken down for L-Leucine 3.82 3.18 Calcium, mg 1555 1296 Visit NutriciaLearningCenter.com for a practical guide for Sodium Selenite, Chromium Chloride, L-Lysine 1.92 1.60 energy and toxic metabolites the use of UCD Anamix Junior. Phosphorus, mg 1555 1296 Sodium Molybdate, PhylloquiNil Added, being produced L-Methionine 0.40 0.33 Magnesium, mg 246 205 D-Biotin, Vitamin D3, Cyanocobalamin. L-Phenylalanine 0.67 0.56 Iron, mg 26.9 22.4 • Essential micronutrients such as Vanilla flavored version also contains: L-Proline Nil Added Nil Added calcium, vitamin D, iron and zinc Ordering/Reimbursement Information: Zinc, mg 21.1 17.6 Artificial flavor. L-Serine Nil Added Nil Added Manganese, mg 3.6 3.0 supplied to compensate for low intake Contains: Coconut Oil. Product Reimbursement HCPCS Product Calories L-Threonine 1.59 1.33 Copper, mcg 1900 1583 from natural food Code Code Code* Packaging Per Can L-Tryptophan 0.48 0.40 Iodine, mcg 276 230 • Available in unflavored and vanilla flavor 59292 - Unflavored 49735-0192-92 B4157 or B4162 6 X 400 g (14 oz) 1540 L-Tyrosine 1.23 1.03 Molybdenum, mcg 63.4 52.8 • Osmolality (mOsm/kg): 59293 - Vanilla 49735-0192-93 B4157 or B4162 6 X 400 g (14 oz) 1540 L-Valine 2.29 1.91 Chromium, mcg 41.3 34.4 Metabolics Metabolics Dilution L-Carnitine 0.009 0.008 Selenium, mcg 76.8 64.0 1 to 3.5 1040 WIC‡: Contact your state WIC For more reimbursement Taurine 0.069 0.058 Sodium, mg 1114 928 office to determine availability. information see Resources. Potassium, mg 1613 1344 • Displacement: 0.70 mL/g 124 Chloride, mg 864 720 125 * S9435 = For individuals who submit their bills to insurance on their own. SOD

SOD Anamix® Early Years XMet, XCys Maxamaid®

A methionine- and cystine-free powdered infant formula for the dietary management of A methionine- and cystine-free powdered medical food for the dietary management of conditions requiring conditions requiring restriction of methionine and cystine (e.g. sulphite oxidase deficiency). restriction of methionine and cystine (e.g. sulphite oxidase deficiency) in individuals over 1 year of age.

Main Features: • Methionine and cystine free Indications: Main Features: Indications: • Contains a balanced mixture of all other • Sulphite oxidase deficiency • Methionine-, cystine-free powdered • Sulphite oxidase deficiency essential and non-essential amino acids, carbohydrate, vitamins, minerals and • Other conditions requiring restriction of methionine and infant formula with iron • Other conditions requiring restriction of methionine trace elements cystine • Includes DHA & ARA and cystine • XMet, XCys Maxamaid provides 25 g of • Contains a prebiotic fiber blend For details on administration and household measurements, please For details on administration and household measurements, please protein equivalent per 100 g of powder • Unflavored powder see pages 124-136. see the end of this section. • Available as unflavored powder • Contains a balanced mixture of all other • Contains <1 g of fat per 10 g PE thereby essential and non-essential amino acids, Additional Information: allowing greater flexibility in modifying carbohydrates, fat, vitamins, minerals Additional Information: energy intake Please note that this product is available by special order only. and trace elements Please note that this product is available by special order only. • Osmolality (mOsm/kg): For detailed information on XMet, XCys Maxamaid, please • Provides 13.5 g of protein equivalent For detailed information on SOD Anamix Early Years, please contact our Nutrition Services Department. per 100 g of powder contact our Nutrition Services Department. Dilution 1 to 5 690 • Osmolality (at 0.7 kcal/mL): 1 to 7 490 Ordering/Reimbursement Information: 380 mOsm/kg Ordering/Reimbursement Information: Based on dilution of 5 g powder Product Reimbursement HCPCS Product Calories (1 unpacked level scoop) to 1 fl oz Product Reimbursement HCPCS Product Calories Code Code Code Packaging Per Can Code Code Code* Packaging Per Can (30 mL) water 116194 (Special Order) 49735-0114-57 B4162 3 x 500 g (17.6 oz) 1545 90212 (Special Order) 49735-0102-12 B4162 3 x 400 g (14 oz) 1892

Metabolics Metabolics • Displacement: 0.75 mL/g

* S9435 = For individuals who submit their bills to insurance on their own. 126 127 Metabolic Household Measurements Household Measurements Measure g Product g Protein Measure g Product g Protein Measure g Product g Protein Measure g Product g Protein Equivalent Equivalent Equivalent Equivalent PKU Periflex Early Years HCU Anamix Next PhenylAde 40 Drink Mix Complex Essential MSD Drink Mix Enclosed scoop 5.0 0.68 Enclosed scoop 9.0 2.5 1 pouch 25 10 Enclosed scoop 40 10 1 Tbsp 9.0 1.2 1 Tbsp 8.3 2.3 PhenylAde 60 Drink Mix 1 Tbsp 9.8 2.4 1 cup 144 19.4 1 cup 133 37.2 Enclosed scoop or 1 pouch 16.7 10 1 cup 160 40 MSUD Anamix Early Years IVA, MMA/PA Anamix Next 1 Tbsp 9.8 5.9 Complex MSD Amino Acid Blend Enclosed scoop 5.0 0.68 Enclosed scoop 9.0 2.5 1 cup 148 90 Enclosed scoop 12.4 10 1 Tbsp 9.0 1.2 1 Tbsp 8.8 2.5 PhenylAde MTE Amino Acid Blend 1 Tbsp 8.2 6.6 1 cup 140 19.4 1 cup 140 39.2 Enclosed scoop or 1 pouch 12.8 10 1 cup 124 100 GA-1, TYR, MMA/PA, HCU, IVA Anamix Early Years UCD Anamix Junior Unflavored 1 Tbsp 8.5 6.6 Maxamum Range (Flavored & Unflavored) Enclosed scoop 5.0 0.68 Enclosed scoop 5.0 0.60 1 cup 116 90 1 Tbsp 9 3.6 1 Tbsp 8.8 1.2 1 Tbsp 9.0 1.1 PhenylAde Amino Acid Blend 1 cup 145 58 1 cup 140 18.9 1 cup 143 17.2 Enclosed scoop or 1 pouch 12.4 10 TYR Lophlex GMP MIX-IN PKU Periflex Junior Plus (Plain) UCD Anamix Junior Vanilla 1 Tbsp 8.5 6.9 1 pouch 12.5 10 Enclosed scoop 9.0 2.5 Enclosed scoop 5.0 0.60 1 cup 116 94 1 Tbsp 8.7 2.4 1 Tbsp 8.7 1.0 GlutarAde Junior GA-1 Drink Mix 1 cup 139 38.9 1 cup 139 16.7 Enclosed scoop 9.8 0.98 PKU Periflex Junior Plus (Flavors) Periflex Advance 1 Tbsp 9.3 0.93 Enclosed scoop 9.0 2.5 1 Tbsp 9.4 3.3 1 cup 146 14.6 1 Tbsp 8.3 2.3 1 cup 150 53 GlutarAde Essential GA-1 Drink Mix 1 cup 132 37 PhenylAde Essential Drink Mix Enclosed scoop 40 10 TYR Anamix Next Enclosed scoop or 1 pouch 40 10 1 Tbsp 10 2.5 Enclosed scoop 9.0 2.5 1 Tbsp 10 2.5 1 cup 160 40 1 Tbsp 8.6 2.4 1 cup 160 40 GlutarAde GA-1 Amino Acid Blend 1 cup 137 38.4 PhenylAde GMP Drink Mix Enclosed scoop 12.3 10 Enclosed scoop or 1 pouch 33.3 10 1 Tbsp 8.5 6.9 1 Tbsp 6.6 2 1 cup 116 94 1 cup 128 38.4 Complex Junior MSD Drink Mix Household measures are based on standard US, dry household measures.* PhenylAde GMP Mix-In Enclosed scoop 8.5 1.1 All measures are level and unpacked. These values are approximations Household measures are based on standard US, dry household measures.* 1 pouch 12.5 10 1 Tbsp 8.4 1.1 only and have been provided for convenience: results can vary significantly All measures are level and unpacked. These values are approximations 1 cup 118 15.2 based on the individual, d evice, and method. Nutricia North America only and have been provided for convenience: results can vary significantly recommends using a gram scale for greatest accuracy. The scoops Nutricia based on the individual, device, and method. Nutricia North America Metabolics Metabolics provides are validated for use with Nutricia formulas, so are preferable to recommends using a gram scale for greatest accuracy. The scoops Nutricia household measures. provides are validated for use with Nutricia formulas, so are preferable to household measures. *US measures: 1 cup = 240 mL; ½ cup = 120 mL; ⅓ cup = 80 mL; ¼ cup = 60 mL; 1 Tbsp = 15 mL; 1 tsp = 5 mL; ½ tsp = 2.5 mL *US measures: 1 cup = 240 mL; ½ cup = 120 mL; ⅓ cup = 80 mL; 128 ¼ cup = 60 mL; 1 Tbsp = 15 mL; 1 tsp = 5 mL; ½ tsp = 2.5 mL 129 Metabolic Mixing Instructions PKU Periflex® & Anamix® Early Years PKU Periflex® Junior Plus & Anamix® Next Powdered infant formulas with iron containing DHA and ARA, and a unique blend of prebiotic fiber. Metabolic formulas for the dietary management of metabolic disorders in individuals over one year of age. Amount of Amount of Approx. Approx. Administration: Water Powder Protein Equiv. Final Volume • Must be consumed in conjunction with a whole protein 5 fl oz (150 mL) 5 scoops (25 g) 3.4 g 5.6 fl oz (168 mL) Administration: source (e.g. breast milk or standard infant formula) in Dilution Amount of Water Final 8 fl oz (240 mL) 8 scoops (40 g) 5.4 g 9 fl oz (270 mL) • PKU Periflex Junior Plus & Anamix Next must be (kcal/mL) Powder Added Volume quantities prescribed by a dietitian or physician to meet 12 fl oz (360 mL) 12 scoops (60 g) 8.1 g 13.5 fl oz (405 mL) consumed in conjunction with whole protein and 0.6 18 g (approx 2 scoops) 3.5 fl oz (100 mL) 3.9 fl oz (114 mL) the general nutrition requirements to ensure normal 0.9 27 g (approx 3 scoops) 3.5 fl oz (100 mL) 4.0 fl oz (120 mL) *Boiling is the preferred method of sterilizing water, bottles, other nutrients in quantities prescribed by a dietitian growth and development. 1.1 36 g (approx 4 scoops) 3.5 fl oz (100 mL) 4.3 fl oz (126 mL) nipples and mixing utensils. or physician to supply the fluid and general nutrition • Formula dosage and dilution will vary depending on requirements. the age, body weight, medical condition and protein Important Preparation Information: Freshly prepared • Intended for individuals over 1 year of age. Freshly prepared formula is best. Refrigerate pre- tolerance of the infant. formula is best. Formula prepared in advance must be cooled mixed formula immediately and use within 24 hours rapidly, stored in the refrigerator and used within 24 hours of • Intake of Periflex Junior Plus or Anamix Next is Mixing Instructions: preparation. Formula remaining in the bottle after one hour dependent on the age, body weight and medical of mixing. Shake immediately before use. Tube feeding of feeding should be discarded. Powdered infant formula is condition of the individual. Periflex Junior Plus or hang time not to exceed 4 hours. • To make a standard dilution (21 kcal/fl oz), add 1 not sterile. Anamix Next should be divided into equal portions Precautions: unpacked level scoop (5 g) of powder to each fluid and distributed throughout the day. ounce of water. Only use the scoop provided. For Precautions: • Must only be consumed by individuals with proven inborn errors of metabolism. Not intended for infants. accuracy formula should be weighed on a gram scale. • Do not boil formula or use a microwave to prepare or Mixing Instructions: Not intended as sole source of nutrition. Not for • PKU Periflex/Anamix Early Years may be mixed with warm formula. • Add the desired volume of water to a shaker or parenteral use. warm or cool sterile water. Do not boil formula or use a • Must be given under strict medical supervision other container with a tight fitting lid. Then add the microwave to prepare or warm formula. to infants with a proven metabolic disorder. Not prescribed amount of powder and shake until fully Storage: blended together. Best served chilled. To make formula: intended as the sole source of nutrition. Not for • Store unopened can at room temperature. Do not 1) Wash hands thoroughly and clean the preparation area. parenteral use. • Standard solution is 3.5 fl oz (100 mL) water plus 36 g store at extreme temperatures. Once opened, 2) Pour the required amount of cooled, boiled water into Storage: powder (approx. 4 scoops) made up to a final volume reseal can and store in a cool, dry place (not the sterilized feeding bottle*. of approx. 4.3 fl oz (126 mL). refrigerator) and use within 14 days of opening. • Store unopened cans at room temperature. Avoid Metabolics Metabolics 3) Add the prescribed number of leveled scoops of extreme temperatures. Once opened, reseal can formula to the water in the sterilized feeding bottle. and store in a cool, dry place. Use within two weeks. 4) Place cap on the bottle and shake until the powder 130 dissolves. Shake immediately BEFORE USE. 131 Metabolic Mixing Instructions Periflex® Advance and Periflex® LQ Maxamum® Age-specific medical foods for the dietary management of phenylketonuria (PKU) in children and adults, Age-specific medical foods for the dietary management of metabolic disorders in older children and adults, including pregnant women and women of child-bearing age. including pregnant women and women of child-bearing age. Administration: Dilution Guidelines for Maxamum Administration: Dilution Guidelines for Periflex Advance Dilution Amount of Powder Volume of Water Approx. PE • Must be consumed in conjunction with whole protein, • Use only under the direction of a physician or Volume of Water Prescribed Amount Approx. Protein Equiv. 1 to 7 35 g 245 mL (approx. 8 fl oz) 14 g fluid and other nutrients in quantities prescribed by dietitian. Periflex must be consumed in conjunction 2 to 3 fl oz (60-90 mL) 16 g 6 g 1 to 7 1 sachet (50 g) 350 mL (approx. 12 fl oz) 20 g with whole protein, fluid and other nutrients in 4 to 5 fl oz (120-150 mL) 32 g 11 g a physician or dietitian to meet protein and general 1 to 5 35 g 175 mL (approx. 6 fl oz) 14 g quantities prescribed by a physician or dietitian 8 to 10 fl oz (240-300 mL) 64 g 22 g nutrition requirements. 1 to 5 1 sachet (50 g) 250 mL (approx. 8 fl oz) 20 g to meet phenylalanine and general nutritional 16 to 20 fl oz (480-600 mL) 128 g 45 g • Recommended intake will vary depending on the age, requirements. body weight and medical condition of the individual. • It is recommended that Maxamum be prepared at a 1 Intake is adjusted to maintain normal growth and to 7 dilution initially and advanced to a 1 to 5 dilution. • Periflex Advance is intended for older children and • Once prepared, store formula in a refrigerator for no development. For accuracy, weigh on a gram scale. These dilutions are guidelines only. May be given in adults, including pregnant women and women of longer than 24 hours from the time of preparation. The daily intake should be divided into equal portions a more concentrated form (e.g. drink or paste). If so, child-bearing age. Shake or stir prepared formula immediately before use. and distributed throughout the day. water or diluted drinks should be offered at the same • Intake of Periflex is dependent on the age, Periflex LQ • Intended for older children and adults, including time. bodyweight and medical condition of the individual. Shake well before use. Best served chilled. pregnant women and women of child-bearing age. Precautions: Periflex should be divided into equal portions and • Must only be given under strict medical supervision distributed throughout the day. Precautions: Mixing Instructions: • Must only be consumed by individuals with proven • Best served with meals. to patients with proven phenylketonuria. Not Mixing Instructions: phenylketonuria. Not intended for infants. Not intended • Make a paste of the prescribed amount of powder intended for children under one year of age. Not Periflex Advance as the sole source of nutrition. Not for parenteral use. with a small volume of chilled water. Gradually add suitable as the sole source of nutrition. Not for • Add the desired volume of water to a shaker or sufficient water to make the desired volume. parenteral use. other container with a tight fitting lid. Then add the Storage: • For best taste, consume immediately. Larger volumes Storage: prescribed amount of powder and shake until fully • Store unopened can at room temperature. Do not of prepared formula should be refrigerated and • Store unopened cans/sachets at room temperature. blended together. Dilution guidelines are shown in store at extreme temperatures. Once opened, reseal used within 24 hours of preparation. Shake or stir Do not store at extreme temperatures. Once opened, the tables below. Periflex is best served chilled. can and store in a cool, dry place (not in refrigerator) prepared formula immediately before use. reseal can and store in a cool, dry place (not in • Periflex tastes great when blended with 1 fl oz and use within one month of opening. refrigerator) and use within one month. Metabolics (30 mL) of water for every 5 to 6 grams of powder. • Store Periflex LQ in a cool dry place away from sunlight. Dilutions are guidelines only. Periflex Advance may be given in a more concentrated form (e.g., a paste or concentrated drink) by using less 132 water. If consumed in a concentrated form, water or other drinks should also be consumed at the same time. 133 Metabolic Mixing Instructions PhenylAde® Phlexy-10® Formulas for the dietary management of phenylketonuria (PKU) in children and adults, including pregnant women An interchangeable system of phenylalanine-free medical foods for the dietary management of phenylketonuria and woman of child-bearing age. (PKU) in individuals over 1 year of age, including pregnant women and women of child-bearing age. Administration: • PhenylAde GMP Mix-In Administration: Mixing Instructions: • Use only under the direction of a physician or dietitian. Add contents of 1 pouch or scoop (12.5 g) to approx. 6-8 fl oz (180- • Phlexy-10 Drink Mix: Mix the contents of one PhenylAde must be consumed in conjunction with whole 240 mL) of cold water or a flavored (protein-free) beverage. • The Phlexy-10 products must be consumed in packet with a small amount of water, stirring into a protein, fluid and other nutrients in quantities prescribed • PhenylAde40 Drink Mix conjunction with whole protein, fluid and other thick paste. Continue stirring while adding water by a physician or dietitian to meet the phenylalanine and Add contents of 1 pouch (25 g) to approx. nutrients in quantities prescribed by a physician to make up a final volume of approximately 100 general nutrient requirements. 4-6 fl oz (120-180 mL) of cold water. or dietitian to meet phenylalanine and general nutrition requirements. Fat, carbohydrate and mL (~3.5 fl oz). Once prepared, store formula in a Mixing Instructions: • PhenylAde60 Drink Mix low protein sources may be used to supply refrigerator for no longer than 24 hours from the 1. Add prescribed amount of PhenylAde Powder to cold water, Add contents of 1 scoop or pouch (16.7 g) to approx 3 fl oz (90 mL) needed calories and to fulfill essential fatty acid time of preparation. Shake or stir prepared formula shake or stir until completely mixed together. of cold water. requirements. immediately before use. 2. Dilution may be adjusted to taste. See recommended dilution • PhenylAde Amino Acid Blends • Intake of the Phlexy-10 products will vary • Phlexy-10 Tablets: Should be taken with water or below for a guideline. Simply add one serving to single portion of low protein food, depending on the age, body weight and medical a non-phenylalanine-containing beverage. beverage or other PKU formula. The appropriate serving size must 3. Once prepared, store formula in a refrigerator for no more condition of the individual. Intake is adjusted to be established and consumed to meet prescribed amino acid protein Precautions: than 24 hours from the time of preparation. Shake or stir maintain normal growth and development. One requirements. Not intended to be added to plain water. prepared formula immediately before use. packet of the drink mix or 10 tablets provide 10 g • Must only be given under strict medical supervision of amino acids, equivalent to 8.33 g of protein. to patients with proven phenylketonuria. Not 4. Do not heat, bake or add to food or beverages above 130°F. Precautions: • Must be consumed by individuals with proven phenylketonuria. Not • The Phlexy-10 products do not contain vitamins, intended for use as the sole source of nutrition. Not Recommended Dilution: intended for infants. Not intended as the sole source of nutrition. minerals, or trace elements. It is essential that intended for infants under 1 year of age. Not for Not for parenteral use. these nutrients are supplied separately. parenteral use. • PhenylAde Essential Drink Mix Phlexy-Vits (see separate product listing) are Add contents of 1 scoop or pouch (40 g) to approx. Storage: available to be used as a supplement to the Storage: 7 fl oz (210 mL) of cold water. • Store unopened can at room temperature. Do not store at extreme Phlexy-10 System. One 7 g packet supplies the • Phlexy-10 Drink Mix and Tablets should be stored • PhenylAde GMP Drink Mix temperatures. Once opened, reseal can and store in a cool, dry majority of vitamins and minerals needed to meet in a cool, dry place. Once Phlexy-10 Tablets are Add contents of 1 scoop or pouch (33.3 g) to approx. 5-6 fl oz place (not in refrigerator) and use within one month of opening the RDA of individuals 11 years and older.

Metabolics Metabolics opened, reseal can and store in a cool, dry place for all PhenylAde products except PhenylAde GMP Drink Mix and (150-180 mL) of cold water. (not in refrigerator) and use within one month. PhenylAde GMP READY. PhenylAde GMP Drink Mix must be used • PhenylAde GMP READY within 2 weeks after being opened. PhenylAde GMP READY must Shake well before use. Best served chilled or at room 134 be used immediately or refrigerated promptly once opened. Use 135 temperature. within 24 hours. Metabolic Mixing Instructions PhenylAde® PheBLOC™ LNAA and Lanaflex® Lophlex® Powder and Lophlex® LQ Phenylalanine-free powdered medical foods containing the remaining essential amino acids (large neutral amino acids A low volume, amino acid-based medical food for the dietary management of metabolic disorders in children and and lysine). For the dietary management of phenylketonuria (PKU) in individuals over 12 years of age. adults, including pregnant women and women of child-bearing age.

Administration: PhenylAde PheBLOC LNAA Administration: while adding water to make up a final volume of approximately 70 mL (2.5 fl oz). This would be a • Use only under the direction of a physician or • Add contents of 1 pouch (3 g) to ~1/2 cup of any • Use only under the direction of a physician 1 to 5 dilution. Lophlex may be taken in a more dietitian. The product must be consumed in semi-solid food (i.e., applesauce, mashed potatoes, or dietitian. Lophlex and Lophlex LQ must be concentrated form, either as a drink or paste. If so, conjunction with whole protein, fluid and other pudding, sorbet, etc). consumed in conjunction with whole protein, water or diluted drinks should be offered at the nutrients in quantities prescribed by a physician Precautions: fluid and other nutrients in quantities prescribed same time. Lophlex is best prepared with chilled or dietitian to meet phenylalanine and general by a physician or dietitian to meet amino • Must only be consumed by individuals with proven water and after preparation. Larger volumes of nutrition requirements. acid and general nutrition requirements. Fat, phenylketonuria. Suitable for individuals over 12 prepared formula should be refrigerated and carbohydrate and low protein sources may be • Intake of the product is dependent on the age, years of age. Not suitable as the sole source of used within 24 hours of preparation. Shake or stir used to supply needed calories and to fulfill bodyweight and medical condition of the individual. nutrition. Not for parenteral use. prepared formula immediately before use. essential fatty acid requirements. Mixing Instructions: • Not for use when planning or • Intake of Lophlex and Lophlex LQ will vary Precautions: during pregnancy. Lanaflex depending on the age, body weight and medical • Must only be given under strict medical supervision • Seek medical advice before using with condition of the individual. Intake is adjusted to in individuals with a proven metabolic disorder. • It is recommended that the product is prepared serotonergic medications or any kind of maintain normal growth and development. Lophlex Not intended as a sole source of nutrition. Not for at an approximate 1 to 4 dilution. In a screw-top psychotropic medications. container, add 60 mL (2 fl oz) of chilled water. and Lophlex LQ should be divided into equal parenteral use. portions and distributed throughout the day. Add the contents of one sachet and shake well Storage: Storage: until dissolved. The product is best consumed • Store unopened sachets in a cool, dry place. Preparation (Lophlex LQ) immediately. • Store in a cool, dry place, below 86oF (not in the • If taken in a more concentrated form, water • Lophlex LQ is ready-to-drink and tastes best when refrigerator) and away from sunlight. or diluted drinks should be consumed served chilled. Shake vigorously before use. Visit NutriciaLearningCenter.com for guidelines Recommended Dilution immediately afterwards. on the usage of PhenylAde PheBloc LNAA, and • TYR Lophlex GMP Mix-In

Metabolics Metabolics • Opened sachets should be stored in an airtight guidelines for the usage of Lanaflex in the dietary Mixing Instructions (Lophlex): Add contents of 1 pouch (12.5 g) to approx. 6-8 fl oz container and used within 24 hours. management of PKU • Mix the contents of one sachet with a small (180-240 mL) of refrigerated flavored drink. amount of water to make a paste. Continue stirring 136 137 Metabolic Mixing Instructions Complex MSD: Junior, Essential and Amino Acid Blend GlutarAde™ GA-1: Junior, Essential and Amino Acid Blend Medical foods for the dietary management of Maple Syrup Urine Disease (MSUD) in children and adults, Medical foods for the dietary management of Glutaric Aciduria Type 1 (GA-1) in children and adults, including including pregnant women and woman of child-bearing age. pregnant women and woman of child-bearing age. Dilution of approx. 30 calories per 1 fl oz (30 mL) Dilution of approx. 30 calories per 1 fl oz (30 mL) Administration: Administration: To Make Approx. Water Amount of Powder To Make Approx. Water Amount of Powder • Use only under the direction of a physician or • Use only under the direction of a physician or dietitian. 2 fl oz (60 mL) 2 fl oz (60 mL) 14.7 g 2 fl oz (60 mL) 2 fl oz (60 mL) 12.2 g dietitian. Complex must be consumed in conjunction GlutarAde must be consumed in conjunction with whole 4 fl oz (120 mL) 4 fl oz (120 mL) 29.4 g 4 fl oz (120 mL) 4 fl oz (120 mL) 24.3 g with whole protein, fluid and other nutrients in protein, fluid and other nutrients in quantities prescribed 6 fl oz (180 mL) 6 fl oz (180 mL) 44.1 g 6 fl oz (180 mL) 6 fl oz (180 mL) 36.5 g 8 fl oz (240 mL) 8 fl oz (240 mL) 58.8 g quantities prescribed by a physician or dietitian 8 fl oz (240 mL) 8 fl oz (240 mL) 48.6 g by a physician or dietitian to meet the lysine and general to meet the branched-chain and general nutrient nutrient requirements. • GlutarAde GA-1 Essential Drink Mix requirements. • Complex Essential MSD Drink Mix Mixing Instructions: Add contents of 1 scoop (40g) to approx. 7 fl oz (210 mL) Add contents of 1 scoop (40 g) to approx. 7 fl oz (210 mL) Mixing Instructions: • Add prescribed amount of GlutarAde Powder to cold water, of cold water. of cold water. • Add prescribed amount of Complex Powder to cold shake or stir until completely mixed together. • GlutarAde GA-1 Amino Acid Blend water, shake or stir until completely mixed together. • Complex MSD Amino Acid Blend • Dilution may be adjusted to taste. See recommended Simply add one serving to single portion of low protein • Dilution may be adjusted to taste. See recommended Simply add one serving to single portion of low dilution below for a guideline. food, beverage or other GA-1 formula. dilution below for a guideline. protein food, beverage or other MSUD formula. The • Once prepared, store formula in a refrigerator for no more The appropriate serving size must be established and • Once prepared, store formula in a refrigerator for no appropriate serving size must be established and than 24 hours from the time of preparation. Shake or stir consumed to meet prescribed amino acid protein more than 24 hours from the time of preparation. Shake consumed to meet prescribed amino acid protein prepared formula immediately before use. requirements. Not intended to be added requirements. Not intended to be added to plain water. or stir prepared formula immediately before use. • Do not heat, bake or add to food or beverages above 130°F. to plain water. • Do not heat, bake or add to food or beverages above Precautions: 130°F. • Must be consumed by individuals with proven maple Recommended Dilution Precautions: • Must be consumed by individuals with proven glutaric syrup urine disease. Not intended for infants. Not • GlutarAde Junior GA-1 Drink Mix Recommended Dilution: aciduria type 1. Not intended for infants. Not intended intended as the sole source of nutrition. Not for • Complex Junior MSD Drink Mix as the sole source of nutrition. Not for parenteral use. parenteral use. Dilution of approx. 20 calories per 1 fl oz (30 mL) Dilution of approx. 20 calories per 1 fl oz (30 mL) To Make Approx. Water Amount of Powder To Make Approx. Water Amount of Powder Storage: Storage: 2 fl oz (60 mL) 2 fl oz (60 mL) 9.8 g 2 fl oz (60 mL) 2 fl oz (60 mL) 8.5 g • Store unopened can at room temperature. Do not store • Store unopened can at room temperature. Do not store 4 fl oz (120 mL) 4 fl oz (120 mL) 19.5 g Metabolics Metabolics 4 fl oz (120 mL) 4 fl oz (120 mL) 17 g at extreme temperatures. Once opened, reseal can and at extreme temperatures. Once opened, reseal can and 6 fl oz (180 mL) 6 fl oz (180 mL) 29 g 6 fl oz (180 mL) 6 fl oz (180 mL) 26 g store in a cool, dry place (not in refrigerator) and use store in a cool, dry place (not in refrigerator) and use 8 fl oz (240 mL) 8 fl oz (240 mL) 39 g 8 fl oz (240 mL) 8 fl oz (240 mL) 34 g within one month of opening. within one month of opening.

138 1 level, unpacked scoop provides approx. 8.5 g of powder. For better accuracy use a gram scale. 1 level, unpacked scoop provides approx. 9.8 g of powder. For better accuracy use a gram scale. 139 UCD Anamix® Junior Medical food for the dietary management of urea cycle disorders in individuals over 1 year of age. Administration: • It is recommended that UCD Anamix Junior be prepared at a 1 to 3.5 dilution. This dilution • Must be consumed in conjunction with whole is a guideline only. An alternative dilution may protein, fluid and other nutrients in quantities be recommended by a clinician or dietitian (e.g. prescribed by a physician or dietitian to meet drink or paste). If given in a more concentrated protein and general nutrition requirements. form, water or diluted drinks should be offered • Recommended intake of UCD Anamix Junior at the same time. is dependent on age, body weight and medical • Once prepared, store formula in a refrigerator condition of the individual. Intake is adjusted for no longer than 24 hours from the time of to maintain normal growth and development. preparation. Shake or stir prepared formula For accuracy, weigh on a gram scale. The daily immediately before use. intake should be divided into equal portions and distributed throughout the day. Dilution Guidelines for UCD Anamix Junior • UCD Anamix Junior is intended for patients over Dilution Amount of Powder Volume of Water Approx. PE 1 year of age, including pregnant women and 1 to 3.5 50 g (10 scoops) 175 mL (approx. 6 fl oz) 6.0 g women of child-bearing age. Precautions: Mixing Instructions: • Must only be given under strict medical • Best served chilled with meals. supervision to patients with proven urea cycle LOW PROTEIN disorders. Not intended for children under one • Pour the required volume of cold water into a year of age. Not suitable as the sole source of glass or cup. FOODS nutrition. Not for parenteral use. • Add the prescribed amount of UCD Anamix Junior powder to the water and stir. For accuracy it is Storage: recommended to use a gram scale.

Metabolics • Store unopened cans in a cool, dry place. Do not store at extreme temperatures. Once opened, Visit NutriciaLearningCenter.com for a practical guide for Find more information and resources on these and all of our the use of UCD Anamix Junior. reseal can and store in a cool, dry place (not in refrigerator) and use within one month. products at www.NutriciaLearningCenter.com 140 141141 Loprofin, Milupa and Low Protein Foods A line of low protein foods including cake mix, pasta, rice, cereals, drink mix, crackers and more

Main Features: Indications: • A wide range of products to add variety to a • Dietary management of inherited metabolic disorders protein-restricted diet • Renal or liver failure requiring a low protein diet • Other medical conditions where a low protein diet is indicated Protein Foods Low For more information, visit www.MedicalFood.com

142 Nathan Diagnosed with KETOGENIC intractable epilepsy DIET

TM Find more information and resources on these and all of our products at www.NutriciaLearningCenter.com 143 144 KETOGENIC DIET • Fat • • • • • • • • • • • Main

150 mOsm/kg energy requirements Displacement Energy distribution: Osmolality (at1kcal/mL) Standard dilution=1kcal/mL Nutrient denseforchildrenwithlow 0 gof No artificialsweeteners Appropriate forenteral ororal feedings Unflavored DHA andARA 3:1 ratio Nutritionally complete Carbohydrate Protein (LCT 100%) Fea trans tures: fat

medical food. where theketogenic dietisindicated;designedforindividualsover 1year ofage.A for thedietarymanagementofrefractory epilepsyandotherepilepsy-relatedconditions A nutritionallycomplete,ketogenic formulaina3:1ratio (fat:carbohydrate +protein) KetoCal 87.1% 1 mL/g 4.1% 8.8%

® 3:1Powder For detailsonadministration, please seetheendofthissection. • • • Indications: 16672 Code Product Ordering/Reimbursement Information:

• • • is indicated,including: Other epilepsy-relatedconditionswheretheketogenic diet Refractory orintractable epilepsy office todetermine availability. WIC SuperRefractory Status Epilepticus(SRSE) Dravet syndrome Pyruvatedehydrogenase deficiency(PDH) Glucosetransporter type-1deficiencysyndrome (GLUT1) ‡ : Contactyour stateWIC 49735-0166-72 Reimbursement B4154 Code Packaging HCPCS information see information For morereimbursement 6 x300g(11oz) Product Resources 2097 Per Calories Can .

Nutrition Information: Inositol, mg Choline, mg Vitamin C,mg Biotin, mcg Pantothenic Acid,mg Folic Acid,mcg Niacin, mg Vitamin B Vitamin B Riboflavin, mg Thiamin, mg Vitamin K,mcg Vitamin E,IU(mg Vitamin D, IU(mcg) Vitamin A,IU(mcgRE) Vitamins ARA,mg DHA,mg a LinoleicAcid,mg Polyunsaturated, g Monounsaturated, g Saturated, g Fat, g Carbohydrate, g Protein, g Calories Nutrients *Standard Dilution =30kcal/fl oz - Linoleic Acid,mg 12 6 , mg , mcg a - 508(12.7) TE) Per 100g 1748(525) 12700 1270 182 153 180 140 116 116 699 100 11.8 (7.9) 84.0 13.9 55.9 16.6 22.3 25.5 67.7 15.3 0.70 1.4 2.8 4.9 0.70 0.89 7.2 Per 100kcal* 250(75.1) 1817 21.9 25.8 12.0 20.0 72.7 (1.8) 16.6 16.6 0.10 0.20 1.7 (1.1) 2.0 0.40 0.70 0.10 0.13 8.0 2.4 3.2 3.6 9.7 1.0 2.2 Taurine, mg L-Carnitine, mg Chloride, mg(mEq) Potassium, mg(mEq) Sodium, mg(mEq) Selenium, mcg Chromium, mcg Molybdenum, mcg Iodine, mcg Copper, mcg Manganese, mg Zinc, mg Iron, mg Magnesium, mg Phosphorus, mg Calcium, mg Minerals 287(12.5)41.0(1.8) Per 100g 440 (12.6)62.9(1.8) 900 (23.1) 124 760 510 760 48.0 48.0 18.9 26.2 31.3 10.9 95.0 0.57 7.4 Per 100kcal* 129(3.3) 109 109 17.7 13.6 73.0 4.6 4.6 2.7 3.7 4.5 0.08 1.1 1.6

(DHA) **A SourceofDocosahexaenoic Acid *A SourceofArachidonic Acid(ARA) Milk,SoyContains: Cyanocobalamin. Sodium Selenite,D-Biotin,VitaminD Sodium Molybdate,Phylloquinone, Tocopherols, ChromiumChloride, Folic Acid,Ascorbyl Palmitate, Mixed Vitamin AAcetate,Potassium Iodide, Pyridoxine Hydrochloride, Riboflavin, Thiamin ChlorideHydrochloride, Cupric Sulfate,Manganese Soy Lecithin, CalciumD-Pantothenate, alpha Tocopheryl Acetate,Niacinamide, Mono andDiglycerides, ZincSulfate,DL- L-Ascorbic Acid,L-Carnitine, Taurine, Sodium Chloride,Ascorbate, Ferrous Sulfate,SodiumCaseinate(Milk), C. Cohnii Bitartrate, L-Cystine, Dibasic, MagnesiumAcetate,Choline Potassium Chloride,CalciumPhosphate Tripotassium Citrate, CornSyrupSolids, Lactose (Milk),Tricalcium Phosphate, Sunflower), MilkProtein (Casein, Whey), Refined Vegetable Oils(Palm, Soy, Ingredients: Oil**, L-Tryptophan, M-Inositol, M. Alpina Oil*,

3 ,

145 KetoCal 3:1 Powder 3:1 KetoCal 146 KETOGENIC DIET • • • • • • • • • • • • • Main

Nu Fat Carbohydrate energy requirements 0 gof Osmolality (at1kcal/mL): 170mOsm/kg Displacement Energy distribution: Standard dilution=1kcal/mL Nutrient denseforchildrenwithlow Contains sucralose Contains amulti-fiberblend Appropriate forenteral ororal feedings Vanilla flavor DHA andARA 4:1 ratio Fiber Protein tritionally complete (LCT 100%) Fea trans tures: fat

over 1 year of age. A medicalfood. over 1year ofage. related conditionswheretheketogenic diet isindicated;designedforindividuals protein) forthedietarymanagementofrefractory epilepsyandotherepilepsy- A nutritionallycomplete,ketogenic formulaina4:1ratio (fat:carbohydrate + KetoCal 88.3% 3.5% 1 mL/g 1.5% 8.2%

® 4:1Powder For detailsonadministration, please seetheendofthissection. • • • Indications: 101777 Code Product Ordering/Reimbursement Information:

office todetermine availability. WIC • • • is indicated,including: Other epilepsy-relatedconditionswheretheketogenic diet Refractory orintractable epilepsy SuperRefractory Status Epilepticus(SRSE) Dravet syndrome Pyruvatedehydrogenase deficiency(PDH) Glucosetransporter type-1deficiencysyndrome (GLUT1) ‡ : Contactyour stateWIC 49735-0166-70 Reimbursement B4154 Code Packaging HCPCS information see information For morereimbursement 6 x300g(11oz) Product Resources 2115 Per Calories Can .

Nutrition Information: Inositol, mg Choline, mg Vitamin C,mg Biotin, mg Pantothenic Acid,mg Folic Acid,mcg Niacin, mg Vitamin B Vitamin B Riboflavin, mg Thiamin, mg Vitamin K,mcg Vitamin E,IU(mg Vitamin D Vitamin A,IU(mcgRE) Vitamins ARA,mg DHA,mg a LinoleicAcid,mg Polyunsaturated, g Monounsaturated, g Saturated, g Fat, g Insoluble,g Soluble,g Fiber, g Carbohydrate, g Protein, g Calories Nutri -Linolenic Acid,mg ents

12 6 3 , mg , IU(mcg) , mcg a -TE) 420(10.5) 1232(370) Per 100g 16200 1700 320 116 120 110 705 100 15.9 (10.7) 19.1 40.0 31.0 19.7 22.8 26.7 69.2 14.4 0.70 0.0191 2.9 4.9 1.3 0.70 0.89 2.1 3.2 5.3 8.2 Per 100kcal 2300 175(52.5) 241 59.6 (1.5) 45.5 16.5 17.0 15.6 0.10 2.3 (1.5) 2.7 5.7 0.0027 0.41 0.70 0.18 0.10 0.13 4.4 2.8 3.2 3.8 9.8 0.30 0.45 0.75 1.2 2.0 Fiber isnotincludedincalculatingtheketogenic ratio * StandardDilution=1kcal/mL Taurine, mg L-Carnitine, mg Chloride, mg Potassium, mg Sodium, mg Selenium, mcg Chromium, mcg Molybdenum, mcg Iodine, mcg Copper, mcg Manganese, mg Zinc, mg Iron, mg Magnesium, mg Phosphorus, mg Calcium, mg Minerals Per 100g 726 771 481 500 420 700 33.0 45.0 27.0 14.8 28.7 86.2 96.3 0.83 3.6 7.0 Per 100kcal 103 109 68.2 12.2 71.0 13.7 59.6 99.3 4.7 6.4 3.8 2.1 4.1 0.12 0.51 0.99

§ Containssucralose 14.2 mg/100mL (DHA) ** AsourceofDocosahexaenoic Acid * AsourceofArachidonic Acid(ARA) Milk,SoyContains: Cyanocobalamin. Phylloquinone, D-Biotin,Cholecalciferol, Chromium Chloride,SodiumSelenite, Potassium Iodide,Sodium Molybdate, Mixed Tocopherols, Folic Acid, Vitamin AAcetate,Ascorbyl Palmitate, Pyridoxine Hydrochloride, Riboflavin, Thiamine ChlorideHydrochloride, Manganese Sulfate,Cupric Niacinamide, CalciumD-Pantothenate, Inositol, L-Histidine, ZincSulfate, Tocopheryl Acetate,Sucralose§,M- Sulfate, Mono-andDiglycerides, DL-alpha L-Tyrosine, L-Carnitine, Taurine, Ferrous Maltodextrin, L-Ascorbic Acid,L-Valine, Sodium Ascorbate,L-Phenylalanine, Oxide, SodiumCaseinate(Milk), Oil**, Soy Fiber, L-Tryptophan, Magnesium Choline Flavor (Vanilla), L-Arginine, L-Cystine, High Amylose CornStarch,Artificial Chloride, CalciumPhosphateTribasic, Citrate, Potassium Chloride,Sodium Dioxide, GumArabic, Tripotassium Fructooligosaccharide (FOS),Silicon Calcium Carbonate,CornSyrupSolids, Cellulose, ChicoryRootInulin, Lecithin, Whey (Milk),Microcrystalline Oleic Sunflower),Casein(Milk),Soy Refined Vegetable Oils(Palm, Soy, High Ingredients: Bitartrate , M. Alpina Oil*, C. Cohnii

147 KetoCal 4:1 Powder 4:1 KetoCal 148 KETOGENIC DIET • • • • • • • • • • • • • • Main Density Fat Fiber Carbohydrate Unflavored Vanilla low energyrequirements Free water Energy distribution: Osmolality (at1.5kcal/mL): Nutrient denseforchildrenwith 0 gof Contains sucralose Contains amulti-fiberblend Appropriate forenteral ororal feedings Convenient ready-to-feedliquid Great vanillaflavor orunflavored DHA andARA 4:1 ratio Nutritionally complete Protein (LCT 100%) Fea trans tures: fat

280 mOsm/kg 260 mOsm/kg individuals over 1year ofage.Amedicalfood. other epilepsy-relatedconditionswheretheketogenic dietisindicated;designedfor (fat:carbohydrate +protein)forthedietarymanagementofrefractory epilepsyand A nutritionallycomplete,ready-to-feedketogenic formulain a4:1ratio KetoCal 88.7% 1.5% 3.1% 1.01g/mL 77.8% 8.2%

® 4:1LQ For detailsonadministration, please seetheendofthissection. • • • Indications: 80183 –Unflavored 80180 –Vanilla Code Product Ordering/Reimbursement Information:

office todetermine availability. WIC • • • is indicated,including: Other epilepsy-relatedconditionswheretheketogenic diet Refractory orintractable epilepsy SuperRefractory Status Epilepticus(SRSE) Dravet syndrome Pyruvatedehydrogenase deficiency(PDH) Glucosetransporter type-1deficiencysyndrome (GLUT1) ‡ : Contactyour stateWIC 49735-0130-54 49735-0187-96 Code Reimbursement B4154 B4154 Code HCPCS information see information For morereimbursement (8 floz) 27 x237mL (8 floz) 27 x237mL Packaging Product Resources 356 356 Per DrinkBox Calories

Taurine, mg L-Carnitine, mg Chloride, mg Potassium, mg Sodium, mg Selenium, mcg Chromium, mcg Molybdenum, mcg Iodine, mcg Copper, mcg Manganese, mg Zinc, mg Iron, mg Magnesium, mg Phosphorus, mg Calcium, mg Minerals, Trace Elements Fiber isnotincluded incalculatingtheketogenic ratio. (mEq) (mEq) (mEq) 103(4.5) 155(4.4) 165 (4.2) 120 100 mL 18.5 22.7 88.4 88.4 Per 6.3 8.3 7.1 3.2 6.2 0.18 1.2 1.5 Per DrinkBox 244(10.6) 391(10.02) (237 mL) 367 (10.4) 284 210 210 14.9 19.7 16.8 14.7 43.8 53.8 7.6 0.43 2.8 3.6

(DHA) ** ASource of Docosahexaenoic Acid * ASourceofArachidonic Acid(ARA) Choline Chloride. Sweetener: Sucralose, SodiumHydroxide, Artificial Flavor, Sugar, Artificial Vanilla Unflavored Milk,SoyContains: Vitamin D Molybdate, Phylloquinone, D-Biotin, Chloride, SodiumSelenite, Folic Acid,Potassium Iodide,Chromium Tocopherols, DL-Alpha Tocopherol, Riboflavin, VitaminAAcetate,Mixed Hydrochloride, Pyridoxine Hydrochloride, Cupric Sulfate,ThiaminChloride Manganese Sulfate,Ascorbyl Palmitate, Niacinamide, CalciumD-Pantothenate, Tocopheryl Acetate,Soy Lecithin, L-Tryptophan, ZincSulfate,DL-Alpha Lactate, L-Carnitine, Taurine, M-Inositol, Propylene Glycol Alginate,Ferrous L-Cystine, CalciumPhosphateDibasic, Sodium Hydroxide, CholineChloride, and Diglycerides, Trisodium Citrate, Calcium PhosphateMonobasic,Mono Fructooligosaccharide ,L-Ascorbic Acid, C. Cohnii Magnesium Acetate,Potassium Chloride, Arabic, CalciumChloride,M.AlpinaOil*, CITREM, DipotassiumPhosphate,Gum (Milk), Soy Fiber, CornStarch,Inulin, (Milk), Whey ProteinConcentrate Sunflower, Soy, Palm), SodiumCaseinate Water, Refined Vegetable Oil(HighOleic Ingredients: flavored version alsocontains: Oil**, MicrocrystallineCellulose, 3 , Cyanocobalamin. version alsocontains:Sugar.

149 KetoCal 4:1 LQ 4:1 KetoCal 150 KETOGENIC DIET • • Fat • • • • • • • • • • • • • Main

MCT energy requirements Osmolality (at1.5kcal/mL): 347mOsm/kg Density Free water Energy distribution: Nutrient denseforchildrenwithlow 0 gof Contains sucralose Contains amulti-fiberblend Appropriate forenteral ororal feedings Convenient ready-to-feedliquid Great vanillaflavor DHA 2.5:1 ratio Nutritionally complete Fiber Carbohydrate Protein (MCT 25%LCT 75%) Fea trans tures: fat age. Amedicalfood. conditions wheretheketogenic dietisindicated.Designedforindividuals over 8years of + protein)forthedietarymanagementofrefractory epilepsyandotherepilepsy-related A nutritionallycomplete,ready-to-feedketogenic formulain a2.5:1ratio (fat:carbohydrate KetoCal

84% 1.02 g/mL 76.7% 1.5% 2.9% 12% ® 2.5:1LQ For detailsonadministration, please seetheendofthissection. • • • Indications: 137246 Code Product Ordering/Reimbursement Information: For morereimbursementinformation see

• • • is indicated,including: Other epilepsy-relatedconditionswheretheketogenic diet Refractory orintractable epilepsy SuperRefractory Status Epilepticus(SRSE) Dravet syndrome Pyruvatedehydrogenase deficiency(PDH) Glucosetransporter type-1deficiencysyndrome (GLUT1) 49735-01-7246 Code Reimbursement B4154 Code HCPCS 27 x237mL(8floz) Packaging Product Resources . 363 Per DrinkBox Calories

12 6 1 3 , mg , mcg , IU(mcg) , mcg a

-TE) 300(90.0) 100 mL 153 92.0 (2.3) 52.0 29.0 58.2 14.3 Per 0.15 7.8 5.9 (4.0) 9.4 4.1 0.62 1.8 0.30 0.15 0.15 1.5 8.0 3.6 0.0 4.8 0.56 0.56 1.1 2.2 4.5 4.1 Per DrinkBox 710(213) 218(5.5) (237 mL) 123 138 363 18.5 14.0 (9.5) 22.3 68.7 19.0 11.4 33.9 10.7 0.36 9.7 1.5 4.3 0.71 0.36 0.36 3.6 8.5 0.0 1.3 1.3 2.6 5.2 9.7

Taurine, mg L-Carnitine, mg Chloride, mg Potassium, mg Sodium, mg Selenium, mcg Chromium, mcg Molybdenum, mcg Iodine, mcg Copper, mcg Manganese, mcg Zinc, mg Iron, mg Magnesium, mg Phosphorus, mg Calcium, mg Minerals, Trace Elements Fiber isnotincluded incalculatingtheketogenic ratio. 100 mL 100 207 250 117 120 180 115 19.0 26.0 90.0 Per 6.3 8.3 5.4 2.5 5.5 0.91 1.4

Per DrinkBox (237 mL (237 14.9 20.0 12.8 13.0 45.0 61.6 491 593 277 284 427 213 273 5.9 2.2 3.3 )

**Contains Sucralose 43mg/237mL (DHA) * ASourceofDocosahexaenoic Acid Milk,SoyContains: Cholecalciferol, Cyanocobalamin. Phylloquinone, SodiumSelenite,D-Biotin, Sodium Molybdate,ChromiumChloride, Tocopherols, Folic Acid, Potassium Iodide, Riboflavin, Ascorbyl Palmitate, Mixed Hydrochloride, VitaminAPalmitate, Thiamin Mononitrate, Pyridoxine Manganese Sulfate,Copper Niacinamide, CalciumD-Pantothenate, Alpha Tocopheryl Acetate,ZincSulfate, Taurine, M-Inositol,L-Tryptophan, DL- Alginate, L-Carnitine, Ferrous Lactate, Tribasic, Sucralose**, Propylene Glycol L-Ascorbic Acid,CalciumPhosphate Chloride, SodiumHydroxide, L-Cystine, Maltodextrin, Trisodium Citrate, Choline Chloride, Mono-AndDiglycerides, Phosphate Monobasic,Potassium Oligosaccharides (FOS),Calcium Microcrystalline Cellulose,Fructo- Dipotassium Phosphate, Gum Arabic, MagnesiumAcetate, Inulin, CITREM,ArtificialFlavors, Citrate, CornStarch,CalciumChloride, (Milk), Sugar, Soy Fiber, Tripotassium (Milk), Whey ProteinConcentrate Coconut Oil),Soy Oil),SodiumCaseinate Triglycerides (Palm Kernel And/Or Oleic SunflowerOil,MediumChain Water, Refined Vegetable Oil(High Ingredients: C. Cohnii Oil*,

151 KetoCal 2.5:1 LQ 2.5:1 KetoCal 152 KETOGENIC DIET • • • • • • Directions forUse: 4. 3. 2. 1. KetoCal 3:1and4:1Powders For oral andtubefeeding: Directions forPreparation: KetoCal

24 hours. promptly at 39°F/4°C orlessand use within formula is open,useimmediately orrefrigerate 39°F-77°F (4°C-25°C). between eachfeed. lukewarm water(approximately 98°For37°C) should bediscarded. for upto4hours,atwhichtimeunusedformula formula canbesafelykept atroomtemperature Tube feedinghangtime consumed immediately. refrigerator andkept nolongerthan24hours. the water. be atatemperature of113-122°F(45-50°C). completely dissolve thepowder, thewatermust into acontainerthatsealswithscrewtoplid.To Once powderedformula ispreparedorliquid Do notfreeze.Recommended storage between Do notboilormicrowave formula. It isrecommendedthatthetubeflushed with For oral feeding,bestserved chilledand Once prepared,theformulashouldbestoredina Cover andshake untilthepowderhasdissolved. Add theprescribedamountofKetoCal to Measure therequiredamountofwarmwater ® Family : Nutriciarecommends

place (notinarefrigerator). Usewithin one month. opened, replacecontainer lidandstoreinacool,dry KetoCal 3:1and4:1Powders: from sunlight. Store unopenedproductinacool,dryplace andaway Storage: A gram scaleisnecessaryforproperpreparation offormula. Dilution tobedeterminedby medicalprescription: KetoCal 3:1Powder A gram scaleisnecessaryforproperpreparation offormula. Dilution tobedeterminedby medicalprescription: KetoCal 4:1Powder Mixing Guide: 45 kcal/fl oz 30 kcal/fl oz 24 kcal/fl oz 20 kcal/fl oz Dilution 45 kcal/fl oz 30 kcal/fl oz 24 kcal/fl oz 20 kcal/fl oz Dilution Powder Powder 21.4 g 14.3 g 11.4 g 21.3 g 14.2 g 11.4 g 9.5 g 9.5 g Water Water 79 mL 86 mL 89 mL 90 mL 79 mL 86 mL 88 mL 91 mL Final Volume Final Volume 100 mL 100 mL 100 mL 100 mL 100 mL 100 mL 100 mL 100 mL Once canhasbeen 240 mOsm/kg 150 mOsm/kg 120 mOsm/kg 100 mOsm/kg 260 mOsm/kg 170 mOsm/kg 130 mOsm/kg 110 mOsm/kg Osmolality Osmolality

• • • Caution: *Number ofcansandcasespermonthareroundedupto thenearestwholenumber Volume andOrderingGuide

Calories/day

condition ofthepatient. USE ONLY UNDERSTRICTMEDICALSUPERVISION.NOT FORPARENTERAL USE. Intake andmedical tobedeterminedbyweight, amedical professionalandisdependentonthe age,body Monitor forhypoglycemia, renalstones,hyperlipidemia, andadequategrowth. 2000 1900 1800 1700 1600 1500 1400 1300 1200 1100 1000 900 800 700 600 Calories/mo 60000 57000 54000 51000 48000 45000 42000 39000 36000 33000 30000 27000 24000 21000 18000 Cans/mo 29 28 26 25 23 22 20 19 18 16 15 13 12 10 9 KetoCal 4:1 Powder Cases/mo 5 5 5 5 4 4 4 4 3 3 3 3 2 2 2 Cans/mo 29 28 26 25 23 22 21 19 18 16 15 13 12 11 9 KetoCal 3:1 Powder Cases/mo . 5 5 5 5 4 4 4 4 3 3 3 3 2 2 2 Drink Boxes/mo (Vanilla orUnflavored) 169 161 152 144 135 127 118 110 102

93 85 76 68 59 51 KetoCal 4:1LQ Cases/mo 7 6 6 6 5 5 5 5 4 4 4 3 3 3 2 Drink Boxes/mo 165 157 149 140 132 124 116 107 99 91 83 74 66 58 50 2.5:1 LQ KetoCal Cases/mo

7 6 6 6 5 5 5 4 4 4 4 3 3 3 2 153 Use for Directions KetoCal 154 KETOGENIC DIET SPECIALIZED Meredith ADULT NUTRITION Long-term care resident

Find more information and resources on these and all of our products at www.NutriciaLearningCenter.com 155 Pro-Stat Sugar Free

® Nutrition Information: Administration: Precautions: Pro-Stat Sugar Free Nutrients Per 30 mL (2 Tbsp) • Use under medical supervision • Do not add Pro-Stat to an Calories 100 • Healthcare professionals should open or closed system of the A sugar free, ready-to-drink liquid protein medical food providing 15 g Protein, g 15 tube feeding formula or into a determine intake based on of enzyme-hydrolyzed complete protein and 100 calories per 1 fl oz. Fat, g 0 container of enteral feeding. Carbohydrate, g 10 condition, estimated need, and dietary intake. • Do not add medications to Minerals Pro-Stat, even after dilution. Main Features: Indications: Sodium, mg 50 • Administer orally or through Potassium, mg 20 feeding tube. • Not suitable for use as a sole • Clinically supported in 3 published trials • Increased protein needs in low volume related to: Phosphorus, mg 50 source of nutrition. • Managing wounds (1 study)1 • Stage 1 & 2 pressure injuries Suggested Intake: • Not for parenteral use. • Improving anabolism (1 study)2 • Wounds (diabetic, venous, surgical, burns) • 1-3 servings per day. 3 • Preserving lean body mass (1 study) • Unintentional muscle loss One serving = 1 fl oz. Storage: • Nutrient dense, providing 15 g of • Protein-energy malnutrition Ingredients: protein and 100 calories per 1 fl oz • Low serum proteins Oral Feeding: • Store at room temperature. • Hydrolyzed for rapid absorption & Hydrolyzed Collagen Protein, Water, • Sarcopenia Glycerin, Malic Acid. Contains 2% or less: • Shake well before opening. Can • Refrigeration causes gelling. efficient utilization4 • Dialysis Potassium Sorbate (to help protect flavor), be taken straight or mixed with • Discard 3 months after opening. • High in conditionally indispensable Sodium Benzoate (to help protect flavor), • Fluid restricted • Record date opened on bottom amino acids needed for recovery5-10 Sucralose, L-Tryptophan, Phosphoric Acid. any hot or cold beverages or food. of container. • Sugar free Ordering/Reimbursement Information: Wild Cherry Punch flavored version of this product contains 2% or less of each of Tube Feeding: • Do not use if seal is broken. • Nectar-like viscosity Product Reimbursement HCPCS Product Calories the following: Natural flavors. • Lactose free, gluten free, and soy free Code Code Code Packaging Per Unit 1. Flush feeding tube with Citrus Splash, Grape and Vanilla flavored 30-60 mL of water. • Kosher 10064 – Wild Cherry Punch (bottle) 26974-0410-07 B4155 6 x 30 fl oz 3000 versions of this product contains 2% or • Available in 4 great tasting flavors 30064 - Citrus Splash (bottle) 26974-0300-64 B4155 6 x 30 fl oz 3000 less of each of the following: Natural and 2. Pour 30 mL of Pro-Stat in a • Available in bottles (6/case) or unit 40064 - Vanilla (bottle) 26974-0410-40 B4155 6 x 30 fl oz 3000 Artificial flavors. 4-6 fl oz container. doses (96/case) 50064 - Grape (bottle) 26974-0410-50 B4155 6 x 30 fl oz 3000 3. Add 30-60 mL water and mix well • For oral and enteral use 10464-U - Wild Cherry Punch (unit) 26974-0410-17 B4155 96 x 1 fl oz 100 with disposable spoon or tongue 30464-U - Citrus Splash (unit) 26974-0304-64 B4155 96 x 1 fl oz 100 blade. Specialized Adult Nutrition 40464-U - Vanilla (unit) 26974-0410-42 B4155 96 x 1 fl oz 100 1. Lee SK, et al. Adv Skin Wound Care. 2006;19:92-6. 2. Sundell MB, et al. J Ren Nutr. 2009;19:412-21. 50464-U - Grape (unit) 26974-0410-52 B4155 96 x 1 fl oz 100 4. Administer Pro-Stat via syringe. 3. Hays NP, et al. J Am Diet Assoc. 2009;109:1082-7. 5. Flush with 30-60 mL of water. 4. Koopman R, et al. Am J Clin Nutr. 2009;90:106-15. 5. Morris Jr, SM. Annu Rev Nutr. 1992;12:81-101. 6. Curis E, et al. Amino Acids. 2005;29:177-205. 156 7. Wu G, et al. Biochem J. 1992;281:45-8. 8. Schwedhelm E, et al. Br J Clin Pharmacol. 2008;65:51-9. 9. Waugh WH, et al. Am J Hematol. 1999;61:216. 157 10. Waugh WH, et al. J Natl Med Assoc. 2001;93:363-71. Pro-Stat Sugar Free AWC

Pro-Stat® Sugar Free AWC (Advanced Wound Care) Nutrition Information: Administration: Precautions: Nutrients Per 30 mL (2 Tbsp) • Use under medical supervision • Do not add Pro-Stat to an Calories 100 A sugar free ready-to-drink liquid protein medical food providing 17 g of enzyme- • Healthcare professionals should open or closed system of the Protein, g 17 tube feeding formula or into a hydrolyzed complete protein and 100 calories per 1 fl oz, with added arginine, citrulline, determine intake based on L-Arginine, g 3.3 container of enteral feeding. cystine, vitamin C and zinc. Fat, g 0 condition, estimated need, and • Do not add medications to Carbohydrate, g 7 dietary intake. Pro-Stat, even after dilution. Vitamins • Administer orally or through Vitamin C, mg 175 feeding tube. • Not suitable for use as a sole Main Features: Indications: Minerals source of nutrition. • Clinically supported in 1 published trial to • Increased protein needs in low volume related to: Zinc, mg 10 Suggested Intake: • Not for parenteral use. promote pressure injury and • Stage 3 & 4 pressure injuries Sodium, mg 50 • 1-3 servings per day. 1 Potassium, mg 20 wound healing • Multiple pressure injuries One serving = 1 fl oz. Storage: (Advanced Wound Care) Phosphorus, mg 50 • Nutrient dense, providing 17 g of protein • Hard to heal wounds (diabetic, venous, surgical, burns) Oral Feeding: • Store at room temperature. and 100 calories per 1 fl oz • Unintentional muscle loss • Shake well before opening. Can be • Refrigeration causes gelling. • Contains added arginine, citrulline, • Protein-energy malnutrition Ingredients: taken straight or mixed with any cystine, vitamin C and zinc • Low serum proteins • Discard 3 months after opening. Hydrolyzed Collagen Protein, Water, hot or cold beverages or food. • Hydrolyzed for rapid absorption & • Sarcopenia Glycerin, Malic Acid, L-Arginine. Contains • Record date opened on bottom 2 of container. efficient utilization 2% or less: L-Citrulline, L-Tryptophan, Tube Feeding: L-Cysteine HCL, Phosphoric Acid, Ascorbic • Do not use if seal is broken. • High in conditionally indispensable amino Ordering/Reimbursement Information: Acid, Potassium Sorbate (to help protect 1. Flush feeding tube with 30-60 mL acids needed for recovery3-8 flavor), Sodium Benzoate (to help protect Product Reimbursement HCPCS Product Calories flavor), Sucralose, Zinc Sulfate. of water. • Sugar free Code Code Code Packaging Per Unit Wild Cherry Punch flavored version of this 2. Pour 30 mL of Pro-Stat in a 4-6 40130 - Wild Cherry Punch (bottle) 26974-0410-30 B4155 4 x 30 fl oz 3000 • Honey-like viscosity product contains 2% or less of each of the fl oz container. 40230 - Citrus Splash (bottle) 26974-0410-23 B4155 4 x 30 fl oz 3000 following: Natural flavors. • Lactose free, gluten free, and soy free 3. Add 30-60 mL water and mix 40130-U - Wild Cherry Punch (unit) 26974-0410-31 B4155 96 x 1 fl oz 100 Citrus Splash flavored version of this • Kosher 40230-U - Citrus Splash (unit) 26974-0410-24 B4155 96 x 1 fl oz 100 product contains 2% or less of each of the well with disposable spoon or following: Natural and Artificial Flavors. tongue blade.

Specialized Adult Nutrition • Available in 2 great tasting flavors 1. Lee SK, et al. Adv Skin Wound Care. 2006;19:92-6. 2. Koopman R, et al. Am J Clin 4. Administer Pro-Stat via syringe. • Available in bottles (4/case) or unit doses Nutr. 2009;90:106-15. 3. Morris Jr, SM. Annu Rev Nutr. 1992;12:81-101. 4. Curis (96/case) E, et al. Amino Acids. 2005;29:177-205. 5. Wu G, et al. Biochem J. 1992;281:45-8. 5. Flush with 30-60 mL of water. 6. Schwedhelm E, et al. Br J Clin Pharmacol. 2008;65:51-9. 7. Waugh WH, et al. Am J 158 • For oral and enteral use Hematol. 1999;61:216. 8. Waugh WH, et al. J Natl Med Assoc. 2001;93:363-71. 159 Pro-Stat MAX

Nutrition Information: Administration: Precautions: ® Nutrients Per 30 mL (2 Tbsp) • Use under medical supervision. • Do not add Pro-Stat to an Pro-Stat MAX Calories 80 • Healthcare professionals should open or closed system of the Protein, g 11 determine intake based on tube feeding formula or into a A sugar free, hydrolyzed whey-based liquid protein medical food. 100% PDCAAS* protein Fat, g 0 condition, estimated need, and container of enteral feeding. and 80 calories in 1 fl oz. Carbohydrate, g 9 dietary intake. • Do not add medications to Minerals Pro-Stat, even after dilution. Sodium, mg 35 • Administer orally or through • Not suitable for use as a sole Main Features: Indications: Potassium, mg 40 feeding tube. Phosphorus, mg 60 source of nutrition. • Nutrient dense, providing 11 g of • Increased protein needs in low volume related to: Suggested Intake: Calcium, mg 60 • Not for parenteral use. 100%-PDCAAS* protein and 80 calories in • Pressure injuries • 1-3 servings per day. 1 One serving = 1 fl oz. 1 fl oz to help meet high protein demands • Wounds (diabetic, venous, surgical, burns) Storage: • Whey-based hydrolyzed complete protein • Critical illness** Oral Feeding: • Store at room temperature. • Unintentional muscle loss Ingredients: • Optimal amount of all 9 indispensable • Shake well before opening. Can • Refrigeration causes gelling. • Protein-energy malnutrition be taken straight or mixed with amino acids needed for efficient Water, Glycerin, Hydrolyzed Whey • Discard 3 months after opening. protein synthesis2-6 • Low serum proteins Protein, Hydrolyzed Collagen Protein, any hot or cold beverages or food. • Sarcopenia Malic Acid. Contains 2% or less: • Record date opened on bottom • Sugar free Natural & Artificial Flavor, Phosphoric Tube Feeding: • Pre- and post-surgery Acid, L-Phenylalanine, L-Histidine, of container. • Nectar-like viscosity • Cancer L-Tryptophan, L-Methionine, Sucralose, 1. Flush feeding tube with 30-60 mL • Do not use if seal is broken. • Gluten free and soy free Potassium Sorbate (to help protect of water. flavor), Sodium Benzoate (to help • Available in great tasting grape flavor Ordering/Reimbursement Information: protect flavor). 2. Shake well before opening unit dose or bottle. • Available in bottles (4/case) and unit doses Product Reimbursement HCPCS Product Calories Contains: Milk (Whey). (96/case) Code Code Code Packaging Per Unit 3. Pour 30 mL of Pro-Stat MAX • For oral and enteral use 90001 - Grape (bottle) 26974-0900-01 B4155 4 x 30 fl oz 2400 in a 4-6 fl oz container. 90001-U - Grape (unit) 26974-1900-01 B4155 96 x 1 fl oz 80 4. Add 30-60 mL water and mix well

* PDCAAS = Protein Digestibility Corrected Amino Acid Score. with disposable spoon or tongue

Specialized Adult Nutrition The Food and Agriculture Organization (FAO), World Health Organization (WHO), Institute of Medicine (IOM) and Food and Drug Administration (FDA) use blade. this method for evaluating protein quality. PDCAAS rates the utilization and digestibility of protein. ** ICU stay for >2-3 days. 5. Administer Pro-Stat MAX via 1. McClave SA, et al. JPEN J Parenter Enteral Nutr. 2016;40:159-211. 2. Pennings B, et al. Am J Clin Nutr. 2011;93:997-1005. 3. Katsanos CS, et al. Nutr syringe. Res. 2008;28:651-8. 4. FAO/WHO Expert Consultation. FAO Food and Nutrition Paper. 1991;51. 5. Institute of Medicine of the National Academies. Dietary 160 Reference Intakes for Energy, Carbohydrates, Fiber, Fat, Fatty Acids, Cholesterol, Protein, and Amino Acids (Macronutrients). 2005: 737. 6. Castellanos VH, et 6. Flush with 30-60 mL of water. 161 al. Nutr in Clin Prac. 2006;21:485-504. Pro-Stat Renal Care

Nutrition Information: Administration: Precautions: ® Nutrients Per 30 mL (2 Tbsp) • Use under medical supervision. • Do not add Pro-Stat to an Pro-Stat Renal Care Calories 100 • Healthcare professionals should open or closed system of the Protein, g 15 A sugar free, ready-to-drink liquid protein medical food providing 15 g of enzyme-hydrolyzed determine intake based on tube feeding formula or into a Fat, g 0 container of enteral feeding. complete protein, 3 g of prebiotic fiber and 100 calories per 1 fl oz. Carbohydrate, g 6 condition, estimated need, and • Do not add medications to Fiber, g 3 dietary intake. Pro-Stat, even after dilution. Minerals • Administer orally or through Sodium, mg 50 feeding tube. • Not suitable for use as a sole Main Features: Indications: Potassium, mg 20 source of nutrition. Suggested Intake: • Clinically supported in 1 published trial • Increased protein needs in low volume related to: Phosphorus, mg 50 • Not for parenteral use. • 1-3 servings per day. to improve protein anabolism in • Pressure injuries One serving = 1 fl oz. hemodialysis patients1 • Wounds (diabetic, venous, surgical, burns) Storage: • 15 g of complete protein and 3 g • Dialysis Oral Feeding: • Store at room temperature. of prebiotic soluble fiber in 1 fl oz Ingredients: • Fluid restricted • Shake well before opening. Can • Refrigeration causes gelling. • Hydrolyzed for rapid absorption and Hydrolyzed Collagen Protein, Water, • Unintentional muscle loss be taken straight or mixed with • Discard 3 months after opening. efficient utilization2 Glycerin, Polydextrose, Fructo- • Protein-energy malnutrition Oligosaccharides (FOS), Malic Acid. any hot or cold beverages or food. • Record date opened on bottom • High in conditionally indispensable amino Contains 2% or Less: Natural & Artificial 3-8 • Low serum proteins of container. acids needed for recovery Flavor, Phosphoric Acid, L-Tryptophan, Tube Feeding: • Low volume making it appropriate for • Sarcopenia Sucralose, Potassium Sorbate (to help • Do not use if seal is broken. • Inadequate protein & fiber intake protect flavor), Sodium Benzoate (to help 1. Flush feeding tube with 30-60 mL fluid restricted protect flavor). • Sugar free of water. 2. Pour 30 mL of Pro-Stat in a 4-6 fl • Honey-like viscosity Ordering/Reimbursement Information: • Lactose free, gluten free, and soy free oz container. • Kosher Product Reimbursement HCPCS Product Calories 3. Add 30-60 mL water and mix well Code Code Code Packaging Per Unit • Available in great tasting tangerine flavor with disposable spoon or tongue 60064 - Tangerine (bottle) 26974-0410-64 B4155 6 x 30 fl oz 3000 • Available in bottles (6/case) or unit doses blade. 60064-U - Tangerine (unit) 26974-0420-65 B4155 96 x 1 fl oz 100 Specialized Adult Nutrition (96/case) 4. Administer Pro-Stat via syringe. • For oral and enteral use 5. Flush with 30-60 mL of water. 1. Sundell MB, et al. J Ren Nutr. 2009;19:412-21. 2. Koopman R, et al. Am J Clin Nutr. 2009;90:106-15. 3. Morris Jr, SM. Annu Rev Nutr. 1992;12:81-101. 162 4. Curis E, et al. Amino Acids. 2005;29:177-205. 5. Wu G, et al. Biochem J. 1992;281:45-8. 6. Schwedhelm E, et al. Br J Clin Pharmacol. 2008;65:51-9. 7. Waugh 163 WH, et al. Am J Hematol. 1999;61:216. 8. Waugh WH, et al. J Natl Med Assoc. 2001;93:363-71. UTI-Stat

Nutrition Information: Administration: Storage: UTI-Stat® Nutrients Per 30 mL (2 Tbsp) • Use under medical supervision. • Store at room temperature. Calories 70* • Refrigeration not necessary. Sugar, g 1 • Administer orally or through A ready-to-drink medical food providing cranberry concentrate with added ingredients Minerals feeding tube. • Discard 3 months after opening. (vitamin C, D-Mannose, fructo-oligosaccharides (FOS), and bromelain) in a 1 fl oz serving Sodium, mg 15 Suggested Intake: • Record date opened on bottom for urinary tract health. Potassium, mg 20 • 1-2 servings per day as needed. of container. Phosphorus, mg 0 One serving = 1 fl oz. • Do not use if seal is broken. Proprietary Blend (Cranberry Concentrate, Ascorbic Acid, D-Mannose, FOS, Bromelain) Oral Feeding: Main Features: Indications: • Clinically supported in 2 published trials to • Management of urinary tract infections *Glycerin provides a source of calories • Can be taken straight or mixed 1-2 with any hot or cold beverage. manage urinary tract infections • Urinary tract health • Low in sugar (1 g sugar per serving) Ingredients: Tube Feeding: • Thin viscosity Water, Glycerin, Proprietary Blend 1. Flush feeding tube with 30-60 • Lactose free, gluten free, and soy free Ordering/Reimbursement Information: (Cranberry Concentrate, Ascorbic Acid, mL water. D-Mannose, Fructo-oligosaccharides (FOS), 2. Measure and administer dose • Kosher Product Reimbursement HCPCS Product Calories Bromelain). Contains 2 % or less: Sucralose, Code Code Code Packaging Per Unit Acesulfame Potassium, Potassium Sorbate prescribed. • Available in cranberry flavor (to preserve flavor), Sodium Benzoate (to 60001 - Cranberry (bottle) 26974-0410-66 B4104 4 x 30 fl oz 2100 3. Administer UTI-Stat straight • Available in bottles (4/case) or unit doses preserve flavor), Phosphoric Acid. into the enteral tube. (96/case) 60001-U - Cranberry (unit) 26974-0410-67 B4104 96 x 1 fl oz 70 4. Flush the tube with additional • For oral and enteral use 30 mL water. Precautions: • Not a substitute for antibiotics. • This product is not intended for use as an analgesic.

Specialized Adult Nutrition • Not suitable for use as a sole 1. Posthauer ME. The Director. 2008;16:13-6. 2. Efros M, et al. Urology. 2010;76:841-5. source of nutrition. • Not for parenteral use. 164 165 Diff-Stat Suggested Use: ** For residents/patients with a Supplement Facts: CVC, open Diff-Stat capsule • Take two capsules or one powder Serving Size: 2 Capsules or powder pack in a separate ® Diff-Stat Amount Per Serving Per 2 Capsules pack once daily. room. Probiotics could be Calories 5 Directions for Oral aerosolized or inadvertently A dietary supplement providing a blend of probiotics and a prebiotic that Carbohydrate, g <1 introduced into a CVC line when Dietary Fiber, g <1 Consumption: helps to promote intestinal health.† the pack is opened, presenting a Proprietary blend, mg 942 FOS (Fructo-oligosaccharides) • Consume 2 capsules with fluid contamination risk. Saccharomyces boulardii, or break open capsule and Bacillus coagulans Precautions: Main Features: Helps Promote: consume.* • Dissolve powder pack in 30 mL • Two probiotics and a prebiotic working to • Intestinal health† Supplement Facts: • Diff-Stat should be taken 1 hour promote intestinal health† juice and consume.* before or 2 hours after antifungal Serving Size: 1 Powder Pack (2 g) medication. • Saccharomyces boulardii Amount Per Serving Per Powder Pack Directions for Diff-Stat Through • Do not use in case of Calories 5 a Feeding Tube:** • Bacillus coagulans Ordering/Reimbursement Information: yeast allergy. • Fructo-oligosaccharides Carbohydrate, g 1 Product Reimbursement Product Calories Sugar, g 0.5 1. Flush the tube with 30-60 ml Storage: • Available in a convenient capsule or an Code Code Packaging Per Unit Proprietary blend, mg 942 water. unflavored powder pack 80001 - Capsules (plain) 26974-0410-80 6 x 60 capsules 150 FOS (Fructo-oligosaccharides) 2. Mix contents of Diff-Stat with • Do not expose to excessive heat. 80001-U - Powder pack (Unflavored) 26974-0410-81 96 x 2 g 5 Saccharomyces boulardii, • No refrigeration required Bacillus coagulans 30 mL water and administer • Do not refrigerate. immediately straight into the • For oral and enteral use • Store in a cool, dry place. enteral tube.* Processing may impact level of colony 3. Flush the tube with additional † These statements have not been evaluated by the Food and Drug Administration. forming units (CFU). 30-60 mL water. This product is not intended to diagnose, treat, cure or prevent any disease. Other Ingredients: * Diff-Stat is only viable for Capsules: Gelatin, Natural & Artificial 30 minutes in solution. Flavors. Contains 2% or less: Rice Bran † These statements have not been evaluated Extract, Rice Concentrate, Sucralose, by the Food and Drug Administration. This Silicon Dioxide, and Ethyl Maltol.

Specialized Adult Nutrition product is not intended to diagnose, treat, Contains: Milk. cure or prevent any disease. Powder Packs: Microcrystalline cellulose, Maltodextrin, Silicon Dioxide. 166 167 Fiber-Stat

Nutrition Information: Administration: Precautions: ® Nutrients Per 30 mL (2 Tbsp) • Use under medical supervision. • Do not consume more than Fiber-Stat Calories 50* • Healthcare professionals should 60 mL (4 Tbsp) per day Carbohydrate, g 25 without healthcare provider A low volume liquid prebiotic fiber medical food for the dietary management of bowel Sugar, g 2.5 determine intake based on recommendation. regularity in persons unable to meet fiber requirements through a normal diet. Dietary Fiber, g 15 condition, estimated need, and Soluble Fiber, g 15 dietary intake. • Do not use if intestinal Minerals obstruction, fecal impaction or Sodium, mg 15 • Administer orally or through Potassium, mg 52 feeding tube. known allergy to any component Phosphorus, mg 25 is present. Indications: Suggested Intake: Main Features: * Glycerin & polydextrose provide a source • Not suitable for use as a sole • 15 grams of soluble prebiotic fiber • Constipation of calories • 1-2 servings per day. source of nutrition. One serving = 1 fl oz. (60% DV) in 1 fl oz • Bowel regularity • Not for parenteral use. • Contains fructo-oligosaccharides (FOS) • Low fiber intake Oral Feeding: and polydextrose to support Storage: • Colon health Ingredients: • Can be taken straight or mixed total gut health1- 2 Water, Polydextrose, Glycerin, Prune Juice with any hot or cold beverage. • Store and serve at room • Ready to drink or mix with any food or Concentrate, Fructo-oligosaccharides temperature. beverage (FOS). Contains 2% or less: Sodium Tube Feeding: Benzoate (to help protect flavor), • Refrigeration not necessary. • Thin viscosity Ordering/Reimbursement Information: 1. Flush feeding tube with Potassium Sorbate (to help protect flavor), • Discard 3 months after opening. Phosphoric Acid. 30-60 mL water. • Lactose free, soy free and gluten free Product Reimbursement HCPCS Product Calories Record date opened on bottom • Kosher Code Code Code Packaging Per Unit 2. Measure and administer the of container. 70001 - Prune (bottle) 26974-0410-70 B4104 6 x 30 fl oz 1500 prescribed dose via syringe. • Available in bottles (6/case) or unit doses 70400-U - Prune (unit) 26974-0410-71 B4104 96 x 1 fl oz 50 • Do not use if seal is broken. (96/case) 3. Administer Fiber-Stat straight into the enteral tube. • For oral and enteral use 4. Flush the tube with additional 30-60 mL water. Specialized Adult Nutrition

1. Losada MA, et al. Nutr Res. 2002;22:71-84. 2. Jie Z, et al. Am J Clin Nutr. 2000;72:1503-9.

168 169 Specialized Adult Nutrition

170 OTHER Habul NUTRITIONALS Diagnosed with MSUD

TM Find more information and resources on these and all of our products at www.NutriciaLearningCenter.com 171 172 OTHER NUTRITIONALS • Dilution • • • • • • Main Features: Displacement: 0.83mL/g 1.0 kcal/mL: 0.73 kcal/mL: Osmolality (mOsm/kg): minerals includingtrace elements Contains completeprofileofvitaminsand casein 61%,whey 39% Provides protein asacasein/whey mix; Contains DHAandARA 84% offatasMCT 26% ofcaloriesfromfat

289 mOsm/kg 235 mOsm/kg A medicalfoodforindividualsover 1year ofage. A milkprotein-basedpowder, lowinfat,andhighmediumchaintriglycerides(MCT). Monogen

® • • • • Indications: 106033 Code Product Ordering/Reimbursement Information: Chylothorax high MCTdietisindicated Other lipidandlymphaticdisorderswherealowfat, Hyperlipoproteinemia type1 Long chainfattyacidoxidation disorders office todetermine availability. WIC ‡ : Contactyour stateWIC 49735-0160-33 Code Reimbursement B4150 orB4158 Code HCPCS information see information For morereimbursement 6 x400g(14.1oz) Packaging Product Resources

1776 Per Can Calories .

Nutrition Information: Folic Acid,mcg Niacin, mg Vitamin B Vitamin B Riboflavin, mg Thiamin, mg Vitamin K,mcg Vitamin E,IU(mg Vitamin D Vitamin A,IU(mcgRE) Vitamins Carbohydrate, g DHA,mg ARA,mg Linoleicacid,g LCT, g MCT, g Fat, g Protein Equivalent,g C Nutrients Contains: CoconutOil, Milk,WalnutContains: Oil ** ASourceof Docosahexaenoic Acid(DHA) * ASourceof Arachidonic Acid(ARA) D-Biotin, SodiumSelenite, VitaminD Acid, Potassium Iodide,ChromiumChloride,Sodium Molybdate,Phylloquinone, Hydrochloride, ThiaminChloride Hydrochloride, VitaminAAcetate,Riboflavin, Folic Tocopherol, CalciumD-Pantothenate, CopperSulfate, ManganeseSulfate,Pyridoxine Sulfate, ZincL-Carnitine, DL-Alpha Tocopheryl Acetate,Niacinamide, DL-Alpha L-Ascorbic Acid,L-Tryptophan, SodiumL-Ascorbate, Taurine, L-Isoleucine, Ferrous Hydrogen Phosphate,CholineChloride, Syrup Solids,SodiumCitrate, Potassium Chloride,CalciumCarbonate,Magnesium Oil [MediumChainTriglycerides]), Whey (Milk),CaseinateWalnut Oil,Corn Maltodextrin, SkimMilk,Refined Vegetable Oil(Modified Palm KernelAnd/Or Coconut Ingredients: alories, kcal

a -Linolenic acid,g

6 12 3 , mg , IU(mcg) , mcg a -TE) 428(10.7) 61.0 Per 100g 974(293) 444 16.1 69.1 60.0 60.0 10.8 12.9 12.9 6.6 (4.4) 0.17 0.60 2.7 0.44 0.41 0.90 2.10 0.40 219(66.0) Per 100kcal 100 13.7 96.4 (2.4) 15.6 13.5 13.5 3.6 2.4 2.9 2.9 1.5 (0.99) 0.04 0.14 0.14 0.61 0.10 0.09 0.2 0.5 0.09 3

. M.Alpina Chloride, mg Potassium, mg Sodium, mg Selenium, mcg Chromium, mcg Molybdenum, mcg Iodine, mcg Copper, mcg Manganese, mg Zinc, mg Iron, mg Magnesium, mg Phosphorous, mg Calcium, mg Minerals Inositol, mg Choline, mg Vitamin C,mg Biotin, mcg Pantothenic Acid,mg Nutrients Oil*, C. Cohnii Oil**, L-Valine, Inositol, Per 100g 284 372 192 324 193 321 10.4 18.8 72.3 39.6 79.8 90.9 54.1 12.8 9.5 0.32 4.1 6.0 2.0 Per 100kcal 64.0 83.8 43.2 73.0 43.5 72.3 16.3 16.3 18.0 20.5 12.2 2.3 4.2 8.9 2.9 2.1 2.1 0.07 0.92 1.4 0.45 firmly after use. use contentswithinone month.Replacelid Store inacool,dryplace. Onceopened, Storage: • accuracy, useascale. Unpacked, level scoopsareapproximations only. For (1.0 kcal/mL) 30 kcal/30 mL (0.73 kcal/mL) 22 kcal/30 mL Concentration desired concentrations: The followingareguidelinesforachieving 3. 2. 1. For oral ortubefeeding: • • • Important Information: Administration:

Add theprescribedamountofMonogen Measure therequiredamountof Cover andshakeCover untilthepowderhas immediately beforeuse. preparation. Shake orstirMonogen longer than24hoursfromthetimeof be storedinarefrigerator andkept no Once prepared,theformulashould dissolved. can measures5.6g(level andunpacked). to thewater. Thescoopprovided inthe warmed waterintoacontainer. feeding inindividualsover 1year ofage. Not forparenteral use.For oral andtube acids isrecommended. Regular monitoringofessentialfatty the patient. andmedicalconditionof body weight professional andisdependentontheage, Intake isdeterminedby amedical

(4 Scoops) 22.8 g (3 Scoops) 16.8 g Powder (2 1/2 fl oz) 80 mL (3 floz) 90 mL Water

100 mL 100 mL Final Volume

173 Monogen 174 OTHER NUTRITIONALS • • • • • • • • • Main Features: Can beusedinbeverages andfoods For oral orenteral use Density: 0.97g/mL Osmolality: 10mOsm/kg High energy:4.5kcal/mL Unflavored 50% MCT/ 50%water Emulsified forbettermixing Simplifies additionofMCTs tothediet A medicalfood. acid oxidation disordersorconditionsmanagedby theketogenic diet. where MCTs arepartofdietarymanagement,suchaslong-chainfatty A highenergymedium-chaintriglyceride (MCT)emulsionforconditions Liquigen • Indications: 71957-3 (Unit) 71957 (Case) Code Product Ordering/Reimbursement Information: • • • • • Conditions whereMCTs arepartofdietarymanagement,suchas: office todetermine availability. WIC Short bowelsyndrome Fat malabsorption Chylothorax Long-chain fattyacidoxidation disorders Conditions managedby theketogenic diet ® ‡ : Contactyour stateWIC 49735-0195-73 49735-0119-57 Code Reimbursement B4155 B4155 Code HCPCS 4 x250mL(8.5floz) 3 x4250mL(8.5floz) Packaging Product information see information For morereimbursement Resources 1125 1125 Per Bottle Calories .

Nutrition Information: Diglycerides (Emulsifier),CitricAcid. and Diglycerides (Emulsifier),Monoand Oil)), Water, CitricAcidEstersofMono Triglycerides (Palm Kernel and/or Coconut Refined Vegetable Oil(MediumChain Ingredients: * Listedasfattyacids Chloride, mg Potassium, mg Sodium, mg Calcium, mg Minerals *LCT, g *MCT, g Saturated, g Fat, g Carbohydrate, g Protein, g Calories Nutrients Per 100mL None None 45.4 47.1 50 450 0.10 0.10 5.0 0.30 1.39 Per 250mL None None 114 118 125 1125 12.5 0.25 0.25 0.75 3.49 • • • Precautions: • • Administration: Not forparenteral use. source ofnutrition. Not suitableforuseasasole Use undermedicalsupervision. Shake wellbeforeuse. 5 years ofage. dilution forchildrenunder Liquigen may require condition ofthepatient. bodyweight andmedical and isdependentontheage, healthcare professionalonly should bedeterminedby a The quantityofLiquigen

• • • Storage: after use. Always replacethebottlelid 14 days. refrigerator andusewithin Once opened,storeina between 39°-77°F (4°-25°C). Recommended storage

175 Liquigen 176 OTHER NUTRITIONALS • • • • • • • • • Main Features: Displacement Fat Energy distribution: PRSL oflessthan0.017mOsm/g 0.93 mOsmwhenaddedtoasolution 1 gram contributesapproximately 310 mOsm/kg (1:3dilution): Osmolality Milk proteinfree or texture offoodsandbeverages Super solublewithoutalteringthetaste of fatandcarbohydrate Unique dualenergysource provides 25kcal Calorically dense–1scoop(5g) Carbohydrate (MCT 35%/LCT 65% A powderedmedicalfoodproviding fatandcarbohydrate forincreasedcaloricintake. Duocal )

0.77 mL/g 59% 41%

• • • • • • • Indications: 118262 Code Product Ordering/Reimbursement Information: Suitable fororal andtubefeedings Catabolic states(e.g. burns, trauma, post-operative stress) Modular diets Malabsorptive states Disorders ofproteinandaminoacidmetabolism Protein restricteddiets is indicated Conditions whereahighenergy, lowfluid,electrolytesdiet office todetermine availability. WIC ‡ : Contactyour stateWIC 49735-0182-62 Code Reimbursement B4155 Code HCPCS 6 x400g(14.1oz) Packaging Product information see information For morereimbursement Resources 1968 Per Can Calories

Nutrition Information: Phosphorus, mg Calcium, mg Chloride, mg Potassium, mg Sodium, mg Minerals Carbohydrate, g Polyunsaturated, g Monounsaturated, g Saturated, g Fat, g Protein, g Calories Nutrients of Monoglycerides. Diglycerides, DiacetylTartaric AcidEsters Coconut Oil,Palm Kernel Oil),Monoand Chain Triglycerides (Fractionated Vegetable Oils(Corn, Coconut),Medium Hydrolyzed Cornstarch,BlendofRefined Ingredients: Per 100g ≤ ≤ 492 ≤ ≤ 20.0 20.0 ≤ None 72.7 13.8 22.3 5.0 5.0 5.0 4.8 2.5

Per 10gsachet ≤ ≤ ≤ ≤ ≤ None 49.2 0.50 0.50 2.0 0.50 2.0 0.48 0.25 7.3 1.4 2.2 • • Precautions: For accuracy useagram scale. measurements areapproximations only. are level andunpacked. Household measurements. Allmeasurements Based onstandardUSdry, household 1 sachet 1 cup 1/2 cup 1/4 cup 1 Tbsp 1 tsp 1 Scoop Measure (all measuresareunpacked) Household Measures: source ofnutrition. Not suitableforuseasasole Not forparenteral use. 117 g Weight 10 g 56 g 30 g 8.5 g 2.6 g 5 g

Calories 576 276 148 49 42 13 25 • Storage: 3. 2. 1. For oral ortubefeeding: • Administration: one month. refrigerator) and usewithin in acool,dryplace(not in replace containerlid andstore temperature. Once opened, Store unopenedcanatroom serving. or stirimmediatelybefore and usedwithin24hours.Shake Duocal shouldberefrigerated powder hasdissolved. and stircontinuouslyuntilthe volume ofliquid and/or softfood of Duocal. the patient. weight andmedicalconditionof dependent ontheage,body medical is professionaland Intake tobedeterminedby a Unused foodsmixed with Add powdertotherequired Measure theprescribedamount

177 Duocal 178 OTHER NUTRITIONALS • • • • • • • • Main Features: Displacement: 0.57mL/g PRSL <0.01mOsm/10g 1.04 mOsmwhenaddedtoasolution 1 gram contributesapproximately 104 mOsm/kg Osmolality (10%solution): of foodsanddrinks Neutral taste–doesnotalterthe Completely solubleinfoodsanddrinks Easily digestiblemaltodextrin approximately 5g(~20kcal) Calorically dense–1scoopprovides A powdered,concentrated sourceofenergy(maltodextrin). Amedicalfood. Polycal

™

• • Indications: 89461 Code Product Ordering/Reimbursement Information: inborn errorsofmetabolism. For thedietarymanagementof energyneedsinpatientswith indicated. Conditions whereahighenergy, lowfluidormodulardietis office todetermine availability. WIC ‡ : Contactyour stateWIC 49735-0111-52 Code Reimbursement B4155 Code HCPCS information see information For morereimbursement 12 x400g(14.1oz) Packaging Product Resources 1536 Per Can Calories .

Nutrition Information: Potassium, mg Sodium, mg Minerals Fat, g Protein, g Carbohydrate, g Calories 384 Nutrients Maltodextrin. Ingredients: Per 100g 96.0 2.0 0 0 0

• • Precautions: For accuracy useagram scale. measurements areapproximations only. are level andunpacked. Household measurements. Allmeasurements Based onstandardUSdry, household 1 cup ½ cup ⅓ ¼ cup 1 Tbsp 1 tsp ½ tsp 1 Scoop Measure (all measuresareunpacked) Household Measures: cup source ofnutrition. Not suitableforuseasasole Not forparenteral use. Weight 116 g 38.7 g 58.0 g 29.0 g 7.3 g 2.4 g 1.2 g 5 g 148 kcal 445 kcal 223 kcal 111 kcal ~20 kcal Calories

27.8 kcal 9.3 kcal 4.6 kcal use contentswithin 1 month. dry place(notinrefrigerator) and container lidandstoreinacool, dry place.Onceopened,replace Store unopenedcaninacool, Storage: • • • the patient. weight andmedicalconditionof dependent ontheage,body a medicalprofessionalandis Intake tobedeterminedby Administration: refrigerator forupto24hours. foods shouldbestoredina Unfinished beverages and stirring intohotorcoldfood. to formasmoothpastebefore powder withsufficientliquid Foods: For bestresults,mix into thebeverage andstirwell. required amountofpowder Beverages: Sprinklethe

179 Polycal 180 OTHER NUTRITIONALS • • • • • Main Features: Approximate displacement:0.75mL/g Osmolality (5%w/v): of proteinequivalentper5gpowder Complete AminoAcidMixprovides 4g enteral formulas Supplements increasedproteinneedsin non-essential aminoacids Balanced mixtureofessentialand 360 mOsm/kg A medicalfood. An unflavored, powderedmixture ofessentialandnon-essentialaminoacids. Complete AminoAcidMix

• • • • • • Indications: 553341 Code Product Ordering/Reimbursement Information: Short bowelsyndromefollowingintestinal resection Chronic intestinaldiseases(e.g. Crohn’s disease) Feeding difficultiesinpost-operative conditions Intractable malabsorption protein orotherwholeprotein) eosinphilic esophagitisorindividualswith intolerance to milk whereanoligoallergenicdietisindicated(e.g. Conditions suitable oramodularapproachisrequired Conditions inwhichanutritionallycompletefeedisnot office todetermine availability. WIC ‡ : Contactyour stateWIC 49735-0133-41 Reimbursement B4155 Code Packaging HCPCS information see information For morereimbursement 6 x200g(7oz) Product Resources 656 Per Calories Can

Nutrition Information: L-Glutamine L-Valine L-Tyrosine L-Tryptophan L-Threonine L-Serine L-Proline L-Phenylalanine L-Methionine L-Lysine L-Leucine L-Isoleucine L-Histidine Glycine L-Glutamic Acid L-Cystine L-Aspartic Acid L-Arginine L-Alanine Amino Acids,g Carbohydrate, g Fat, g Protein Equivalent,g Calories 328 Nutrients 10.22 0.7 6.5 4.3 2.0 5.0 4.29 7.0 4.5 1.65 7.55 5.95 4.38 6.0 8.17 2.4 6.39 7.66 3.5 None None Per 100g 82 L-Glutamic Acid. L-Tryptophan, L-Methionine, L-Glutamine, L-Tyrosine, L-Alanine, L-Cystine, L-Phenylalanine, L-Histidine, L-Serine, Acid, Glycine, L-Isoleucine, L-Threonine, L-Arginine, L-Proline, L-Valine, L-Aspartic L-Lysine L-Glutamate, L-Leucine, Ingredients: • • • Precautions: a gram scale. are approximations only. For accuracy use and unpacked. Householdmeasurements measurements. Allmeasurementsarelevel Based onstandardUSdry, household 1 Tbsp 1/2 Tbsp 1 tsp 1/2 tsp Measure (all measuresareunpacked) Household Measures: source ofnutrition. Not suitable foruseasasole Not forparenteral use. of thepatient. weight andmedical condition dependent ontheage, body by adoctorordietitianandis Dosage shouldbedetermined Weight 9.5 g 4.4 g 2.9 g 1.4 g Protein Equivalent

7.79 3.61 2.38 1.15 • • Storage: • • • Administration: immediately beforeserving. time ofpreparation. Stir orshake longer than24hoursfromthe refrigerator andmustnot bekept feed shouldbestoredina reconstituted,theprepared Once after use. Always replacecontainerlid opened, usewithinonemonth. Store inacool,dryplace.Once applesauce. to soft,moistfoods,suchas Acid Mixmay alsobeadded other formulas.CompleteAmino mixed withwater, fruitjuicesor Best served as achilleddrink, that readsingrams. should beweighedonascale Complete AminoAcidMix 5 gpowderto100mLofwater). concentration is5%w/v (e.g. The recommendedfeeding

181 Complete Amino Acid Mix Acid Amino Complete 182 OTHER NUTRITIONALS • • • • Main Features: Approximate displacement:0.75mL/g Osmolality (5%w/v): 360mOsm/kg 5 gofpowder 4 gofproteinequivalentper Essential AminoAcidMixprovides amino acids Balanced mixtureofessential histidine. Amedicalfood. An unflavored, powderedmixture ofessentialaminoacids,includingcystineand Essential AminoAcidMix

• • Indications: 553342 Code Product Ordering/Reimbursement Information: suitable oramodularapproachisrequired Conditions inwhichanutritionallycompletefeedisnot Dietary managementofureacycle disorders office todetermine availability. WIC ‡ : Contactyour stateWIC 49735-0133-42 Reimbursement B4155 Code Packaging HCPCS information see information For morereimbursement 6 x200g(7oz) Product Resources 632 Per Calories Can

Nutrition Information: L-Valine 14.56 L-Tyrosine 9.46 L-Tryptophan 2.36 L-Threonine 11.35 L-Phenylalanine 5.67 L-Methionine 3.78 L-Lysine 13.25 L-Leucine 16.17 L-Isoleucine 10.4 L-Histidine 3.78 L-Cystine 3.78 Amino Acids,g Carbohydrate, g Fat, g Protein Equivalent,g Calories 316 Nutrients L-Methionine, L-Tryptophan. L-Phenylalanine, L-Histidine, L-Cystine, L-Threonine, L-Isoleucine, L-Tyrosine, L-Lysine Acetate,L-Leucine, L-Valine, Ingredients: None None Per 100g 79 • • • Precautions: a gram scale. are approximations only. For accuracy use and unpacked. Householdmeasurements measurements. Allmeasurementsarelevel Based onstandardUSdry, household 1 Tbsp 1/2 Tbsp 1 tsp 1/2 tsp Measure (all measuresareunpacked) Household Measures:

source ofnutrition. Not suitableforuseasasole Not forparenteral use. of thepatient. weight andmedicalcondition dependent ontheage,body by adoctoror dietitianandis Dosage shouldbedetermined Weight 9.0 g 4.1 g 2.7 g 1.4 g Protein Equivalent

7.11 3.24 2.13 1.11

• • Storage: • • • Administration:

before serving. Stir orshake immediately from thetimeofpreparation. be kept longerthan24hours in arefrigerator andmustnot prepared feedshouldbestored after use. Always replacecontainerlid opened, usewithinonemonth. as applesauce. added tosoft,moistfoods,such other formulas.Itmay alsobe mixed withwater, fruitjuicesor that readsingrams. should beweighedonascale of water). 5 gpowderto100mL concentration is5%w/v (e.g. Once reconstituted,the Store inacool,dryplace.Once Best served as achilleddrink, Essential AminoAcidMix The recommendedfeeding

183 Essential Amino Acid Mix Acid Amino Essential 184 OTHER NUTRITIONALS • Main Features: and older meet theDRIofindividuals11years of vitaminsandminerals needed to One 7gpacket suppliesthemajority (11 years and older)andadults. designed tohelpmeetthemicronutrientrequirementsofolderchildren A concentrated, powderedvitamin,mineral andtrace elementpreparation Phlexy-Vits • Indications: 10685 Code Product Ordering/Reimbursement Information: Restrictive therapeutic diets 49735-0106-85 Reimbursement A9270 Code Packaging HCPCS 30 x7g Product 0.2 Calories Per Packet

Nutrition Information: Chloride, mg Potassium, mg Sodium, mg Selenium, mcg Chromium, mcg Molybdenum, mcg Iodine, mcg Copper, mcg Manganese, mg Zinc, mg Iron, mg Magnesium, mg Phosphorus, mg Calcium, mg Minerals Vitamin C,mg Biotin, mcg Pantothenic Acid,mg Folic Acid,mcg Niacin, mg Vitamin B Vitamin B Riboflavin, mg Thiamin, mg Vitamin K,mcg Vitamin E,IU(mg Vitamin D, IU(mcg) Vitamin A,IU(mcgRE) Vitamins Carbohydrate, g Fat, g Protein, g Calories 0.20 Nutrients 12 6 , mg , mcg a -TE) 400(10) 1505 1000 150 300 775 150 700 2664(800) Per 7gpacket 75.0 30.0 70.0 11.1 15.1 50.0 20.0 70.0 5.0 1.5 5.0 1.4 1.2 1.6 <0.35 <1.4 0.02 8.8 None 13.5 (9) 0.04 • • Precautions: a gram scale. are approximations only. For accuracy use and unpacked. Householdmeasurements measurements. Allmeasurementsarelevel Based onstandardUSdry, household 1 Tbsp 1 tsp 1/2 tsp 1/4 tsp Measure Weight (all measuresareunpacked) Household Measures: Vitamin D Phylloquinone, Selenite, D-Biotin,ChromiumChloride, Iodide, SodiumMolybdate, A Acetate,Folic Acid,Potassium Mononitrate, Riboflavin, Vitamin Pyridoxine Hydrochloride, Thiamin D-Pantothenate, Manganese Sulfate, Acetate, CopperGluconate,Calcium Nicotinamide, DL-Alpha Tocopheryl Ferrous Sulfate,Dextrose, ZincSulfate, Acetate, GuarGum,L-Ascorbic Acid, Calcium PhosphateDibasic,Magnesium Ingredients: source ofnutrition. Not suitable foruseasasole Not forparenteral use. 3 , Cyanocobalamin.

1.4 g 0.7 g

10 g

3 g • Storage: • • Administration: and usedwithin2-3days. be storedinanairtightcontainer Once opened,asachetshould Store inacool,dryplace. to taste. water usedwillvaryaccording amount oflemonade/juiceand volume of150 mL(5floz).The (approx. 1.5floz).Dilutetoafinal amount oflemonadeorfruitjuice to asmoothpastewithsmall Mix onesachetofPhlexy-Vits volume ofapproximately 100 mL. remaining watertogive afinal mix toasmoothpaste.Addthe Add asmallamountofwaterand

185 Phlexy-Vits 186 OTHER NUTRITIONALS • • • • • Main Features: directed by physician ordietitian. DIRECTIONS FOR USE: cool dryplace. cover withresealablelidandstoreina heat, humidityandlight.Afteropening, temperature (68º-77ºF). Protectfrom STORAGE: to hotfoodorbeverages above 130°F. WARNING: parenteral use. is nutritionallyincomplete.Notfor medical supervision.Thisproduct IMPORTANT: of metabolism. dietary managementofinbornerrors Amino Acidmedicalfoodsforthe Keep atcontrolledroom Do notheat,bake oradd For useunder Useas L-VALINE L-TYROSINE L-LEUCINE L-ISOLEUCINE GLYCINE L-ARGININE Product Ordering/Reimbursement Information: water soluble.L-Tyrosine isnotwatersoluble. Note: • Suggested mixingdilution: Pure aminoacidpowder. Single AminoAcids provides 10mg ofaminoacids. into 100mLofsterilizedwater. Every 1mLofsolution A 10%solutioncanbemadeby mixing1gofaminoacids This method will work forallaminoacidsthatare work This methodwill 0170V 0160T 0150L 0140I 0130G 0120A Code Product Reimbursement 00847-0017-00 00847-0016-00 00847-0015-00 00847-0014-00 00847-0013-00 00847-0012-00 B4155 B4155 B4155 B4155 B4155 B4155 Code HCPCS 1 -50gbottle 1 -50gbottle 1 -50gbottle 1 -50gbottle 1 -50gbottle 1 -50gbottle Description Package

187 Single Amino Acids Amino Single 188 OTHER NUTRITIONALS Nutricia Navigator Team RESOURCES

TM Find more information and resources on these and all of our products at www.NutriciaLearningCenter.com 189 190 RESOURCES • • • • • resources andclinicaltoolsincludes: specialized nutritionalrequirements. Thissetofvaluable Nutricia Learning Centerisaunique educationtoolforcliniciansmanagingpatientswith Please visitourwebsiteforfreeelectronic copiesoftheseandtheotherflowchartsweoffer. manage patients. used asdecision-makingguidestohelpcliniciansdeterminethebestway tonutritionally specialized medicalnutritionmay behelpful.They areevidence-based toolsthatmay be These five flowchartsillustrate optionsforthenutritionmanagementofconditionswhere are available freeatwww.NutriciaLearningCenter.com The followingpagesincludepocket-sized versions flowchartsthat oftreatment Case Studies Reference Library Patient EducationTools Healthcare Professional Tools Recorded Webinars (CE eligible) Other ReimbursementInformation: Reimbursement Codes: WIC: Reimbursement United States, pleasecontactusat(800) 365-7354orvisitourwebsites: United States, For additionalinformationonreimbursement ofNutriciaNorthAmerica’s productsinthe codes arenotNDCnumbers. to USdatawarehousesbasedonthe formatestablishedby thedatawarehouses.These Reimbursement codeslistedinthis Guidehave beensubmittedby NutriciaNorth America http://www.fns.usda.gov/wic office todeterminethe availability ofaspecificWICeligibleNutriciaproductin yourarea– ContactyourEach WICStateagencydeterminesitsownallowedformulalist. StateWIC may Stateagencysochooses. the foodlistif beincludedonaStateagency’sauthorized WIC eligible Supplemental NutritionProgram forWomen, InfantsandChildren. ‡ WIC isaregisteredservicemarkoftheU.S. DepartmentofAgricultureforUSDA’s Special www.Nutricia-NA.com means that aproduct hasbeenapproved meansthat Federal atthe level and,therefore, . www.Neocate.com MedicalFood.com/Reimbursement

191 Resources 192 RESOURCES • • • • • • • b food, beverages, anddrinkingwater a Individual patientrecommendationsmay vary. percentage ofindividualscovered by thisintake. prevent beingabletospecifywithconfidencethe group, butlackofdataoruncertaintyinthe believed tocover needsofallindividualsinthe The AIforotherlifestageandgendergroupsis breastfed infants,theAIismeanintake. percent) individualsinagroup.For healthy set tomeettheneedsofalmostall(9798 used asgoalsforindividualintake. RDAs are by anasterisk(*).RDAs andAIsmay bothbe Adequate Intakes (AIs)inordinarytypefollowed Dietary Allowances(RDAs) in see www.nap.edu) presentRecommended * Note: National Academies(1997-2005) Food andNutritionBoard, InstituteofMedicine, Nutrients, VitaminsandElements Adequate Intakes, Total Water, Macro- Recommended DietaryAllowancesand § Resources To Basedongproteinperkgbodyweight:

Dietary ReferenceIntakes (DRIs):

1.5 g/kg/day forinfants7-12months 1.52 g/kg/day forinfants 0-6months 1.1 g/kg/day forpregnant(usingpre- 0.80 g/kg/day foradults 0.85 g/kg/day for14-18y 0.95 g/kg/day for4-13y 1.1 g/kg/day for1-3 pregnancy weight) andlactating women tal Water includesallwatercontainedin Thetables(taken fromtheDRIreports, bold type and 31-50 y 19-30 y 14-18 y Lactation 31-50 y 19-30 y 14-18 y Pregnancy >70 y 51-70 y 31-50 y 19-30 y 14-18 y 9-13 y Females > 70y 51-70 y 31-50 y 19-30 y 14-18 y 9-13 y Males 4-8 y 1-3 y Children 7-12 mo 0-6 mo Infants Group Life Stage Macronutrients Total Water (L/d) 3.8* 3.8* 3.8* 3.0* 3.0* 3.0* 2.7* 2.7* 2.7* 2.7* 2.3* 2.1* 3.7* 3.7* 3.7* 3.7* 3.3* 2.4* 1.7* 1.3* 0.8* 0.7* a

Carbohydrate 210 210 210 175 175 175 130 130 130 130 130 130 130 130 130 130 130 130 130 130 95* 60* (g/d)

29* 29* 29* 28* 28* 21* 21* 25* 26* 26* Total Fiber ND ND (g/d) 28* 25* 30* 30* 38* 38* ND 38* 31* 25* ND 19* ND ND ND ND ND ND ND ND ND ND ND ND ND ND ND ND ND ND 30* 31* (g/d) Fat 13* 13* 13* 13* 13* 13* 11* 11* 12* 12* 11* 10* Linoleic Acid 14* 14* 17* 4.6* 17* 16* 12* 10* (g/d) 7* 4.4* a -Linolenic Acid (g/d) 1.2* 0.7* 0.5* 1.3* 1.3* 1.3* 1.4* 1.4* 1.4* 1.1* 1.1* 1.1* 1.1* 1.1* 1.0* 1.6* 1.6* 1.6* 1.6* 1.6* 0.9* 0.5*

Protein

71 71 71 71 71 71 46 46 46 46 46 34 56 56 56 56 52 34 19 13 11.0+ (g/d) 9.1* Resources Vitamins 31-50 y 19-30 y 14-18 y Lactation 31-50 y 19-30 y 14-18 y Pregnancy > 70y 51-70 y 31-50 y 19-30 y 14-18 y 9-13 y Females > 70y 51-70 y 31-50 y 19-30 y 14-18 y 9-13 y Males 4-8 y 1-3 y Children 7-12 mo 0-6 mo Infants Group Life Stage Vitamin A (mcg/d) 1,300 1,300 1,200 500* 400* 770 770 750 700 700 700 700 700 600 900 900 900 900 900 600 400 300

VitaminD (mcg/d)

10* 10*

15 15 15 15 15 15 20 15 15 15 15 15 20 15 15 15 15 15 15 15

VitaminE (mg/d)

5* 4* 19 19 19 15 15 15 15 15 15 15 15 11 15 15 15 15 15 11 7 6

120* 120* 120* 120* VitaminK 90* 90* 75* 90* 90* 75* 90* 90* 90* 90* 75* 60* 60* 55* 30* 75* (mcg/d) 2.5* 2.0* ThiaminRiboflavin Niacin

0.3* 0.2* (mg/d) 1.4 1.4 1.1 1.2 1.4 1.4 1.4 1.4 1.1 1.1 1.1 1.0 0.9 1.2 1.2 1.2 1.2 0.9 0.6 0.5 0.4* 0.3* 1.6 1.6 1.4 1.4 1.1 1.1 1.1 1.0 0.9 1.3 1.3 1.3 1.3 0.9 0.6 0.5 1.6 1.4 1.1 1.3 (mg/d) (mg/d 17 17 18 18 14 14 14 14 12 16 16 16 16 12 17 18 14 16 4* 2* 8 6

)

1.0 (mg/d) Vitamin B 2.0 2.0 2.0 1.9 1.9 1.9 1.5 1.3 1.3 1.2 1.7 1.7 1.3 1.3 1.3 1.0 0.6 0.5 0.3* 0.1* 1.5 6

(mcg/d) Folate 500 500 500 600 600 600 400 400 400 400 400 400 400 400 400 300 200 150 300 400 80* 65* VitaminB (mcg/d) 2.8 2.8 2.8 2.6 2.6 2.6 2.4 2.4 2.4 2.4 2.4 2.4 2.4 2.4 1.8 1.2 0.9 0.5* 0.4* 2.4 2.4 1.8

7* 7* 6* 6* 5* 5* 5* 4* 3* 2* 7* 6* 5* 5* 5* 5* 5* 5* 5* Pantothenic Acid (mg/d) 4* 1.8* 1.7* (mcg/d) Biotin 35* 35* 30* 30* 30* 25* 30* 20* 12* 20* 35* 30* 30* 30* 30* 30* 30* 30* 25* 8* 6* 5* 375* 250* 200* 550* 550* 550* 550* 550* 450* 450* 425* 400* 550* 375* 150* 125* 550* 450* 425* 425* 425* 550* Choline (mg/d)

Vitamin C 50* 40*

120 115 80 (mg/d)

120 90 45 25 45 90 85 85 75 75 75 75 65 90 90 75 15 193 Resources 194 RESOURCES 31-50 y 19-30 y 14-18 y Lactation 31-50 y 19-30 y 14-18 y Pregnancy > 70y 51-70 y 31-50 y 19-30 y 14-18 y 9-13 y Females > 70y 51-70 y 31-50 y 19-30 y 14-18 y 9-13 y Males 4-8 y 1-3 y Children 7-12 mo 0-6 mo Infants Group Life Stage Minerals

Calcium

260* 200* (mg/d)

1,000 1,000 1,000 1,300 1,000 1,000 1,300 1,200 1,200 1,000 1,300 1,300 1,200 1,000 1,000 1,000 1,300 1,300 1,000 700

Phosphorus

275* 100*

(mg/d) 1,250 1,250 1,250 1,250 1,250 1,250 700 700 700 500 460 700 700 700 700 700 700 700 700 700 Magnesium 360 350 320 320 320 310 400 360 240 310 320 360 420 400 420 420 240 410 75* 30* 130 80 (mg/d)

0.01* Fluoride 0.5* 0.7* (mg/d) 4* 4* 4* 4* 3* 2* 3* 3* 3* 3* 3* 3* 3* 3* 3* 3* 3* 2* 1* Selenium (mcg/d) 20* 15*

70 70 70 60 60 60 55 55 55 55 55 40 55 55 55 55 55 40 30 20

Chromium 0.2* 45* 45* 44* 30* 30* 29* 20* 20* 25* 25* 24* 21* 30* 30* 35* 35* 35* 25* 15* 11* 5.5

(mcg/d)

1,300 290 1,300 290 1,300 1,000 1,000 1,000 (mcg/d) Copper 220* 130* 200* 900 150 900 150 900 900 890 700 120 900 150 900 150 900 150 900 150 890 700 120 440 340 (mcg/d) Iodine 110* 150 18 150 18 150 15 150 11 290 10 220 27 220 27 220 27 90 10 90 7 (mg/d) 11 Iron 0.27* 8 8 8 8 8 8 8 8 9 9 2.6* 2.6* 2.6* 2.0+* 2.0+* 2.0+* 1.8* 1.8* 1.8* 1.8* 1.6* 1.6* 2.3* 2.3* 2.3* 2.3* 2.2* 1.9* 1.5* 1.2* Manganese (mg/d) 0.6* 0.003* Molybdenum (mcg/d) 50 12 50 12 50 13 50 11 50 11 50 12 45 8 45 8 45 8 45 8 43 9 34 8 45 11 45 11 45 11 45 11 43 11 34 8 22 5 17 3 3* 2* (mg/d) Zinc 2* 3 * Approximately 10%ofthetotalcancomefromlonger-chainn-3orn-6fattyacids. Protein Carbohydrate n-3 polyunsaturated fattyacids*( n-6 polyunsaturated fattyacids*(linoleicacid) Fat Macronutrient Acceptable MacronutrientDistributionRanges a -linolenic acid)

Children, 1-3y 0.6 -1.2 45-65 30-40 5-20 5 -10

Range (percent ofenergy) (percent Range Children, 4-18y 0.6 -1.2 45-65 10-30 25-35 5-10

0.6-1.2 45-65 10-35 20-35 5-10 Adults 195 Resources 196

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Specializ ed AdultNutrition • • www.SpecializedAdultNutrition.com • www.MyKetoCal.com/KetocalConnect • • • www.MyKetoCal.com • • • • • www.MedicalFood.com/Connect Features tips,recipesandinformation onhowtoorderourproducts. Nutrition products. Educates cliniciansandconsumersaboutNutricia’s SpecializedAdult • • • • the ketogenic diet.Dedicatedpillarsofsupporttoprovide informationaboutKetoCal’s: Provides patientsandcaregivers theresourcesthey needtosuccessfullystartandstay on Helpful recipesandtoolsforfamiliesdietitiansusingtheketogenic diet. “Find aKetogenic Center”tool. Locate aketogenic dietcenterinNorthAmericaby usingthe Provides valuableresourcesandeducationalmaterialsforcliniciansfamilies. And more! Support resourcestohelpanswerquestionsandfamiliesmeettheirdietgoals. Easy tounderstanddisorderandproductspecificeducation. One-on-one supporttoobtainformulacoverage atnocosttofamilies. life whilemanagingametabolicdisorder. Nutricia Connectprovides theresourcesyour patientsneedtohelpthemlive theirbest Tasty ketogenic recipes Caregiver support Coverage andcost assistance Trusted products

197 Resources 198 RESOURCES NOTES ......

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