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What is CSID_booklet_HCP_17JUN2019ƒ.qxp_Layout 1 6/17/19 9:33 AM Page 1 Sucrose Intolerance Sucrose Intolerance Due to Congenital Sucrase- Isomaltase Deficiency (CSID) Is More Common Than You Think! What is CSID_booklet_HCP_17JUN2019ƒ.qxp_Layout 1 6/17/19 9:33 AM Page 2 Introduction What is CSID? For many patients with chronic gastrointestinal symptoms, properly CSID, sometimes referred to as Genetic Sucrase- diagnosing Congenital Sucrase-Isomaltase Deficiency (CSID) is a Isomaltase Deficiency (GSID), is a rare disease that difficult journey. With varied health issues and complicated symptoms, it affects a person’s ability to digest sucrose (a type SuCroSe may take months or years to get a correct diagnosis. The time before the of sugar) due to absent or low levels of the diges - In FooD: deficiency is diagnosed can become a very frustrating experience. However, tive enzyme sucrase- isomaltase. Sucrose (sugar) exists in nearly once a patient receives a CSID diagnosis, a positive breakthrough occurs. everything we Finally, proper care and management are within reach. Sucrase-isomaltase is instrumental in the digestion eat. In fact, of sugar and starch. Sucrase-isomaltase is the list of foods may surprise The information in this booklet is designed to be educational. We encourage produced in the small intestine and helps break you. Here are you to share it with your healthcare provider. ONLY a doctor can properly down sucrose into glucose and fructose, which are just a few... diagnose you. used by the body as fuel. It is also one of several Flavored Yogurt enzymes that helps digest starches. Instant oatmeal Salad Dressing Failure to absorb dietary sucrose and starch may energy Drinks Contents impact the absorption of other nutrients and Granola Dried Fruit disrupt the regulation of gastrointestinal function. n What is CSID? Many Frozen Meals Unabsorbed carbohydrates can inhibit gastric n What are the symptoms of CSID? Snack Bars emptying, accelerate small-intestinal transit, and Bottled Sauces n How is CSID diagnosed? contribute to malabsorption of starch, fat, and Coconut Water n Tests that aid in the diagnosis other nutrients.* Cereal n What are treatment options? Some Breads Apples Mangos Fructose oranges Grapefruits Glucose Carrots Sucrose Potatoes Bananas Cantaloupe Pineapples Gluten-Free Bread Brown rice Sucrase Enzyme *Treem WR. Congenital CSID Patients with Congenital Sucrase- sucrase-isomaltase DISEASE deficiency. J Pediatr Isomaltase Deficiency may be at risk Gastroenterol Nutr. INFO.COM for chronic malnutrition. 1995;21(1):1-14. 2 CSIDDiseaseInfo.com 3 What is CSID_booklet_HCP_17JUN2019ƒ.qxp_Layout 1 6/17/19 9:33 AM Page 4 What are the symptoms of CSID? MoSt CoMMon Symptoms can range from mild to severe chronic, In adults, symptoms persist but may appear to be SYMPtoMS: watery, acidic diarrhea to gas, bloating, less severe than those experienced by children. diarrhea, nausea, and abdominal pain. Infants may not CSID is not a disease that a patient can “outgrow. ” onlIne gas, show symptoms of CSID until they begin to eat In some adults, symptoms may include an reSourCeS: bloating, sucrose- and starch-containing foods such as increase in bowel frequency, nausea, abdominal For the Patient sucroseintolerance.com and juices, solid foods, and medications sweetened with distention, and flatulence, although episodic watery abdominal sucrose. Watery diarrhea, failure to thrive, diaper diarrhea upon ingestion of high levels of sucrose pain sucroseintolerance.com/ rash, irritability, and acidic stools are the most may occur. In some patients, diarrhea may doctor-directory/ common symptoms in infants and toddlers.* Other alternate with constipation, leading to a symptoms include abdominal distention, gassiness, misdiagnosis of irritable bowel syndrome (IBS). For the Doctor colic, excoriated buttocks, indigestion, and vomiting. Call: 1-800-705-1962 to speak with a Normal Intestine CSID Intestine CSID Support Specialist. *Treem WR. Clinical aspects and treatment of A small number of patients may require congenital sucrase- hospitalization for diarrhea and dehydration, isomaltase deficiency. Fructose J Pediatr Gastroenterol malnutrition, muscle wasting, and weakness. Sucrose Sucrose Nutr. 2012;55(suppl 2):S7-13. Glucose Patients with confirmed CSID commonly report †Karakoyun M, Kilicoglu E, being examined for toddler’s diarrhea/irritable SMALL Sahan YO, Baran M, Unal F, INTESTINE Water 9.3% Aydogdu S. Our cases with bowel syndrome (IBS)-D, celiac disease, cystic sucrase isomaltase Sucrase † enzyme deficiency. J Gastrointest fibrosis, and food allergies. Bacteria Irritation, Dig Sys. 2015;5:6. increased motility LARGE Fermentation INTESTINE CSID may be more common Most common Gasses, organic than you think Normal acids, other osmotically Sucrase deficiency may symptoms stool active molecules be more common than CSID sufferers’ most you think. In a recent common symptoms are... retrospective analysis of 27,875 small intestinal As with pediatric patients, the symptoms in adults biopsy samples taken 56% 33% during an endoscopy vary. With the introduction of dietary sucrose, and sent to a specialty some patients may experience several severe effects laboratory for a four- panel disaccharidase from this disease, while others may experience only enzyme activity mild symptoms. assessment , 9.3% of the samples showed a sucrase deficiency. * Diarrhea ‡ Stomach pain ‡ *Nichols BL Jr, Adams B, Roach ‡ Data on file Download the App! Learn more about CSID with CSID CM, Ma CX, Baker SS. Fre - DISEASE our Augmented Reality app; search “ CSID AR .” quency of sucrase INFO.COM deficiency in mucosal biopsies. J Pediatr Gastroenterol Nutr. 2012;55(suppl 2):S28-30. 4 CSIDDiseaseInfo.com 5 What is CSID_booklet_HCP_17JUN2019ƒ.qxp_Layout 1 6/17/19 9:33 AM Page 6 FULL PRESCRIBING INFORMATION How is CSID diagnosed? Tests that aid in the diagnosis Additional Important Safety Who MIGht Diagnosing CSID can be difficult ® hAve CSID? Information for Sucraid (sacrosidase) because the symptoms are similar to Oral Solution Infants, endoscopy DID You KnoW? Sucraid ® other gastrointestinal disorders. n Sucraid ® does not break down some sugars that come children, n The disaccharidase assay directly measures In some is ONLY from the digestion of starch. You may need to restrict the and Your healthcare provider can choose enzyme activity levels in biopsy samples societies, like Greenland amount of starch in your diet. Your doctor will tell you if you available at adults from several testing methods to obtained from the small intestine during an upper should restrict starch in your diet. can suffer eskimos and US Bioservices from CSID. help diagnose you. Here is a list of GI endoscopy . some Alaskan n Tell your doctor if you have diabetes, as your blood Specialty natives, glucose levels may change if you begin taking Sucraid ®. Pharmacy. tests and resources for your doctor n For more information about disaccharidase a low- Your doctor will tell you if your diet or diabetes medicines assay testing, call 1-800-705-1962. carbohydrate, need to be changed. For the Doctor to consider. high-protein, high-fat n Some patients treated with Sucraid ® may have worse The physician must diet may mask abdominal pain, vomiting, nausea, or diarrhea. Constipation, complete the Sucraid ® Sucrose Breath test CSID difficulty sleeping, headache, nervousness, and dehydration Prescription Form symptoms. have also occurred in patients treated with Sucraid ®. Check and fax it to n The test is noninvasive, short in duration, and with your doctor if you notice these or other side effects. 1-866-850-9155. can be administered by patients at home. n Sucraid ® has not been tested to see if it works in patients rm with secondary (acquired) sucrase deficiency. est Fo n Patients with CSID may experience symptoms ONLY T due to consumption of table sugar during the test. n NEVER HEAT SUCRAID ® OR PUT IT IN WARM OR HOT YOUR BEVERAGES OR INFANT FORMULA. Do not mix Sucraid ® with n For more information, call 1-800-705-1962. fruit juice or take it with fruit juice. Take Sucraid ® as prescribed *DOCTOR by your doctor. Normally, half of the dose of Sucraid ® is taken OR CSID DISEASE before a meal or snack and the other half is taken during the HEALTHCARE 4-4-4 At-home Food INFO.COM meal or snack. Sucraid ® should be refrigerated at 36°F-46°F (2°C-8°C) and PROVIDER Because CSID n should be protected from heat and light. CAN Challenge symptoms are Step 1: Stir 4 tablespoons of ordinary table similar to other ORDER n gastrointestinal You are encouraged to report negative side effects sugar into a 4-ounce glass of water. Mix until disorders, of prescription drugs to the FDA. Visit A TEST www.FDA.gov/medwatch or call 1-800-FDA-1088. sugar is completely dissolved. patients are often n Step 2: Drink it on an empty stomach. misdiagnosed or delayed in ® n Step 3: See if symptoms such as bloating, gas, reaching mith and diarrhea occur during the next 4-8 hours; a diagnosis. Dr. S this suggests Sucrose Intolerance is possible. (sacrosidase) Oral Solution n NOTE: This test is not appropriate for infants, young children, geriatric patients, and those with Please see additional co-morbid conditions like diabetes. Patients may Important Safety Call: 1-800-705-1962 Information on page have severe symptoms if they are very sensitive S1 and enclosed to sugar. A physician should be consulted before Email: [email protected] Full Prescribing this test is taken. Information. 6 CSIDDiseaseInfo.com 7 Sucraid.com S2 What is CSID_booklet_HCP_17JUN2019ƒ.qxp_Layout 1 6/17/19 9:33 AM Page 9 What are treatment options? Sucraid ® (sacrosidase) Oral Solution is the only FDA-approved enzyme replacement therapy indicated for the treatment of genetically determined sucrase deficiency. Ask your doctor if Sucraid ® is right for you. Clinical Trials Confirm Efficacy for Use of Sucraid ® in Both Children SuCrAID ® and Adults the goal with Sucraid ® therapy is to eat as normal and O as healthy TM P a diet as E Sucrose Intolerance N possible.
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  • Sucrase-Isomaltase Deficiency Causing Persistent Bloating and Diarrhea in an Adult Female

    Sucrase-Isomaltase Deficiency Causing Persistent Bloating and Diarrhea in an Adult Female

    Open Access Case Report DOI: 10.7759/cureus.14349 Sucrase-Isomaltase Deficiency Causing Persistent Bloating and Diarrhea in an Adult Female Varsha Chiruvella 1 , Ayesha Cheema 1 , Hafiz Muhammad Sharjeel Arshad 2 , Jacqueline T. Chan 3 , John Erikson L. Yap 2 1. Internal Medicine, Medical College of Georgia at Augusta University, Augusta, USA 2. Gastroenterology and Hepatology, Medical College of Georgia at Augusta University, Augusta, USA 3. Pediatric Endocrinology, Medical College of Georgia at Augusta University, Augusta, USA Corresponding author: Varsha Chiruvella, [email protected] Abstract Congenital sucrase isomaltase deficiency (CSID) is an autosomal recessive disorder which leads to chronic intestinal malabsorption of nutrients from ingested starch and sucrose. Symptoms usually present after consumption of fruits, juices, grains, and starches, leading to failure to thrive and malnutrition. Diagnosis is suspected on detailed patient history and confirmed by a disaccharidase assay using small intestinal biopsies or sucrose hydrogen breath test. Treatment of CSID consists of limiting sucrose in diet and replacement therapy with sacrosidase. Due to its nonspecific symptoms, CSID may be undiagnosed in many patients for several years. We present a case of a 50-year-old woman with persistent symptoms of bloating in spite of extensive evaluation and treatment. Categories: Endocrinology/Diabetes/Metabolism, Gastroenterology Keywords: sucrase-isomaltase, starch intolerance, disaccharidase assay, ibs, hydrogen breath test Introduction Congenital sucrase isomaltase deficiency (CSID), also known as genetic sucrase deficiency, is a multifaceted intestinal malabsorption disorder with an autosomal recessive mutation in the sucrase-isomaltase (SI) gene on chromosome 3 (3q25-q26). Sucrase-isomaltase is a type II membrane enzyme complex and member of the disaccharidase family required for the breakdown of α-glycosidic linkages in sucrose and maltose.
  • Intestinal Sucrase Deficiency Presenting As Sucrose Intolerance in Adult Life

    Intestinal Sucrase Deficiency Presenting As Sucrose Intolerance in Adult Life

    BRnUTE 20 November 1965 MEDICAL JOURNAL 1223 Br Med J: first published as 10.1136/bmj.2.5472.1223 on 20 November 1965. Downloaded from Intestinal Sucrase Deficiency Presenting as Sucrose Intolerance in Adult Life G. NEALE,* M.B., M.R.C.P.; M. CLARKt M.B., M.R.C.P.; B. LEVIN,4 M.D., PH.D., F.C.PATH. Brit. med. J., 1965, 2, 1223-1225 Diarrhoea due to failure of the small intestine to hydrolyse studies of the jejunal mucosa. He was discharged from hospital a certain dietary disaccharides is now a well-recognized congenital week after starting a sucrose-free and restricted starch diet and has disorder of infants and children. It was first suggested for since remained well. lactose (Durand, 1958) and later for sucrose (Weijers et al., 1960) and for isomaltose in association with sucrose (Auricchio Methods et al., 1962). It has been confirmed for lactose and for sucrose intolerance by quantitative estimation of enzyme activities in Initially the patient was on a normal ward diet estimated to the intestinal mucosa (Auricchio et al., 1963b; Dahlqvist et al., contain 50-80 g. of sucrose a day. After 10 days sucrose was 1963 ; Burgess et al., 1964; Levin, 1964). In all cases present- eliminated from the diet, and after a further three days starch ing in childhood there is a history of diarrhoea when food intake was restricted to 150 g. a day. Carbohydrate tolerance yielding the relevant disaccharide is introduced into the diet. was determined after an overnight fast by estimating blood Symptoms decrease with age, so that older children may be glucose levels, using a specific glucose oxidase method, after asymptomatic (Burgess et al., 1964) and are able to tolerate ingestion of 50 g.
  • SI Gene Sucrase-Isomaltase

    SI Gene Sucrase-Isomaltase

    SI gene sucrase-isomaltase Normal Function The SI gene provides instructions for producing the enzyme sucrase-isomaltase. This enzyme is found in the intestinal tract, where it is involved in breaking down the sugars sucrose (a sugar found in fruits, and also known as table sugar) and maltose (the sugar found in grains). Sucrose and maltose are called disaccharides because they are each made up of two simple sugar molecules. Disaccharides must be broken down into simple sugar molecules to be digested properly. The sucrase-isomaltase enzyme is found on the surface of the intestinal epithelial cells, which are cells that line the walls of the intestine. These cells have fingerlike projections called microvilli that absorb nutrients from food as it passes through the intestine. Based on their appearance, groups of these microvilli are known collectively as the brush border. The role of the sucrase-isomaltase enzyme is to break down sucrose and maltose into simple sugars so that they can be absorbed by microvilli into intestinal epithelial cells. Health Conditions Related to Genetic Changes Congenital sucrase-isomaltase deficiency At least 10 mutations in the SI gene have been found to cause congenital sucrase- isomaltase deficiency. These mutations disrupt the folding and processing of the sucrose-isomaltase enzyme, transportation of the enzyme within the intestinal epithelial cells, the orientation of the enzyme to the cell surface, or its normal functioning. An impairment in any of these cell processes results in a sucrase-isomaltase enzyme that cannot effectively break down sucrose, maltose, or other compounds made from these sugar molecules (carbohydrates).
  • Congenital Sucrase-Isomaltase Deficiency

    Congenital Sucrase-Isomaltase Deficiency

    Congenital sucrase-isomaltase deficiency Description Congenital sucrase-isomaltase deficiency is a disorder that affects a person's ability to digest certain sugars. People with this condition cannot break down the sugars sucrose and maltose. Sucrose (a sugar found in fruits, and also known as table sugar) and maltose (the sugar found in grains) are called disaccharides because they are made of two simple sugars. Disaccharides are broken down into simple sugars during digestion. Sucrose is broken down into glucose and another simple sugar called fructose, and maltose is broken down into two glucose molecules. People with congenital sucrase- isomaltase deficiency cannot break down the sugars sucrose and maltose, and other compounds made from these sugar molecules (carbohydrates). Congenital sucrase-isomaltase deficiency usually becomes apparent after an infant is weaned and starts to consume fruits, juices, and grains. After ingestion of sucrose or maltose, an affected child will typically experience stomach cramps, bloating, excess gas production, and diarrhea. These digestive problems can lead to failure to gain weight and grow at the expected rate (failure to thrive) and malnutrition. Most affected children are better able to tolerate sucrose and maltose as they get older. Frequency The prevalence of congenital sucrase-isomaltase deficiency is estimated to be 1 in 5, 000 people of European descent. This condition is much more prevalent in the native populations of Greenland, Alaska, and Canada, where as many as 1 in 20 people may be affected. Causes Mutations in the SI gene cause congenital sucrase-isomaltase deficiency. The SI gene provides instructions for producing the enzyme sucrase-isomaltase.
  • Digestion of Carbohydrates

    Digestion of Carbohydrates

    Digestion of carbohydrates Alongside fat and protein, carbohydrates are one of the three macronutrients in our diet with their main function being to provide energy to the body. They occur in many different forms, like sugars and dietary fibre, and in many different foods, such as whole grains, fruit and vegetables. Digesting or metabolizing carbohydrates breaks foods down into sugars, which are also called saccharides. These molecules begin digesting in the mouth and continue through the body to be used for anything from normal cell functioning to cell growth and repair. It is also directly used as energy source in muscle, brain and other cells. Dietary carbohydrate principally consists of …. Polysaccharides: - Starch, glycogen and cellulose Disaccharides: - Sucrose and maltose Monosaccharide:-Glucose and fructose Out of these three types of carbohydrates, monosaccharide does not need digestion. Digestion of carbohydrates During digestion, carbohydrates that consist of more than one sugar get broken down into their monosaccharides by digestive enzymes, and then get directly absorbed causing a glycaemic response. Some of the carbohydrates cannot be broken down and they get either fermented by our gut bacteria or they transit through the gut without being changed. Digestive enzymes and their source Mouth: - Salivary amylase (α-amylase, Ptyalin) Stomach: - No enzymes Pancreas: - Pancreatic α-amylase Intestine: - Dextrinase, Maltase, Isomaltase, Sucrase, Lactase Digestion in mouth Digestion of carbohydrates starts at the mouth. In mouth,
  • Disaccharidase Deficiency and Malabsorption of Carbohydrates

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  • Chrebp-Knockout Mice Show Sucrose Intolerance and Fructose Malabsorption

    Chrebp-Knockout Mice Show Sucrose Intolerance and Fructose Malabsorption

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  • Chrebp-Knockout Mice Show Sucrose Intolerance and Fructose Malabsorption

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  • Genetic Sucrase-Isomaltase Deficiency (GSID), Visit ® a Two-Phase (Dose Response Preceded by a Breath Hydrogen Phase) Double-Blind, Dehydration (1)

    Genetic Sucrase-Isomaltase Deficiency (GSID), Visit ® a Two-Phase (Dose Response Preceded by a Breath Hydrogen Phase) Double-Blind, Dehydration (1)

    Sucraid Patient Sales Aid (DM)_109-ƒx-R3.qxp_Layout 1 12/18/15 11:15 AM Page 1 disease therapy Prescribing Information Patient Package Insert Sucraid® (sacrosidase) Oral Solution: GENERAL INFORMATION FOR PATIENTS Figure 1. Measure dose with measuring scoop. Although Sucraid provides replacement therapy for the deficient sucrase, it does not ® DESCRIPTION provide specific replacement therapy for the deficient isomaltase. Therefore, Sucraid (sacrosidase) Oral Solution Sucraid® (sacrosidase) Oral Solution is an enzyme replacement therapy for the restricting starch in the diet may still be necessary to reduce symptoms as much as 1. treatment of genetically determined sucrase deficiency, which is part of congenital possible. The need for dietary starch restriction for patients using Sucraid should be Please read this leaflet carefully before you take Sucraid® sucrase-isomaltase deficiency (CSID). evaluated in each patient. (sacrosidase) Oral Solution or give Sucraid to a child. Please CHEMISTRY It may sometimes be clinically inappropriate, difficult, or inconvenient to perform a do not throw away this leaflet. You may need to read it again Genetic Sucrase-Isomaltase Sucraid is a pale yellow to colorless, clear solution with a pleasant sweet taste. Each small bowel biopsy or breath hydrogen test to make a definitive diagnosis of CSID. If at a later date. This leaflet does not contain all the information milliliter (mL) of Sucraid contains 8,500 International Units (I.U.) of the enzyme the diagnosis is in doubt, it may be warranted to conduct a short therapeutic trial (e.g., sacrosidase, the active ingredient. The chemical name of this enzyme is one week) with Sucraid to assess response in a patient suspected of sucrase deficiency.
  • Lactose Intolerance and Health: Evidence Report/Technology Assessment, No

    Lactose Intolerance and Health: Evidence Report/Technology Assessment, No

    Evidence Report/Technology Assessment Number 192 Lactose Intolerance and Health Prepared for: Agency for Healthcare Research and Quality U.S. Department of Health and Human Services 540 Gaither Road Rockville, MD 20850 www.ahrq.gov Contract No. HHSA 290-2007-10064-I Prepared by: Minnesota Evidence-based Practice Center, Minneapolis, MN Investigators Timothy J. Wilt, M.D., M.P.H. Aasma Shaukat, M.D., M.P.H. Tatyana Shamliyan, M.D., M.S. Brent C. Taylor, Ph.D., M.P.H. Roderick MacDonald, M.S. James Tacklind, B.S. Indulis Rutks, B.S. Sarah Jane Schwarzenberg, M.D. Robert L. Kane, M.D. Michael Levitt, M.D. AHRQ Publication No. 10-E004 February 2010 This report is based on research conducted by the Minnesota Evidence-based Practice Center (EPC) under contract to the Agency for Healthcare Research and Quality (AHRQ), Rockville, MD (Contract No. HHSA 290-2007-10064-I). The findings and conclusions in this document are those of the authors, who are responsible for its content, and do not necessarily represent the views of AHRQ. No statement in this report should be construed as an official position of AHRQ or of the U.S. Department of Health and Human Services. The information in this report is intended to help clinicians, employers, policymakers, and others make informed decisions about the provision of health care services. This report is intended as a reference and not as a substitute for clinical judgment. This report may be used, in whole or in part, as the basis for the development of clinical practice guidelines and other quality enhancement tools, or as a basis for reimbursement and coverage policies.
  • Sucraid Review

    Sucraid Review

    TAB B United States CONSUMER PRODUCT SAFETY COMMISSION Washington, D.C. 20207 APR I 1998 MEMORAh?)UM TO: Mary Ann Danello, Ph.D., Associate Executive Director, Directorate for Epidemiology and Health Sciences THROUGH: Marilyn L. Wind, Ph.D., Director, Division of Health Sciences,w&; Directorate for Epidemiology and Health Sciences FROM: Jacqueline N. Ferrante, Ph.D., Pharmacologist, Division of Health Sciences, JF Directorate for Epidemiology and Health Sciences SUBJECT: Sucraid Review 1 Introduction Orphan Medical petitioned the Commission to exempt SucraidrM, an oral solution of the enzyme sacrosidase, from special packaging requirements for oral prescription drugs under the Poison Prevention Packaging Act (PPPA). This memorandum reviews scientific information related to Sucraidr”. Product Description and Use SucraidTM is the brand name for sacrosidase, a yeast-derived form of the sucrase enzyme. This enzyme is obtained from baker’s yeast which is also known as Saccharomyces cerevisiae. SucraidTM contains 1.5 milligrams (8,500 International Units) per milliliter (mg/ml) of the enzyme in a 5050 solution of glycerol and water at an unbuffered slightly acidic pH of 4.6. SucraidTM is the only available treatment for congenital sucrase-isomaltase deficiency (CSID). It is replacement therapy for sucrase, not isomaltase. The petitioner argues that the confectionery and baking industry has used this enzyme extensively and that under 21 CFR 170.30 it is a “Generally Recognized as Safe” (GRAS) food material because of its long history of safe use in humans. It is used as a flavoring agent and adjuvant at a level not to exceed five percent in food. The recommended dose of SucraidTM for patients with CSID is one ml per meal or snack for patients weighing up to 15 kilograms (kg) (33 pounds) and 2 ml for patients over 15 kg.
  • CSID) Symptoms, Diagnosis, and Treatment

    CSID) Symptoms, Diagnosis, and Treatment

    CONGENITAL SUCRASE-ISOMALTASE DEFICIENCY (CSID) Symptoms, Diagnosis, and Treatment FOR ADULT GASTROENTEROLOGISTS PMS 294 2020 QOL Medical, LLC 1 Financial Disclosures o [Disclose financial relationships with manufacturers and medical organizations here (e.g., QOL Medical, LLC); if none, list “None.”] PMS 294 2020 QOL Medical, LLC 2 WHAT IS CSID? PMS 294 2020 QOL Medical, LLC 3 CSID: Congenital Sucrase-Isomaltase Deficiency Sucrase-Isomaltase o An enzyme that digests the majority of dietary carbohydrates o Table sugar (sucrose) and many starches (e.g., potatoes, bread) o Expressed in the microvilli of the brush border membrane o Releases glucose and fructose from sucrose (sugar) so they can be absorbed into the bloodstream PMS 294 2020 QOL Medical, LLC 4 Congenital Sucrase-Isomaltase Deficiency The first report of an autosomal recessive Congenital Sucrase-Isomaltase Deficiency (CSID) was published in 1960. Diarrhoea Caused by Deficiency of Sugar-Splitting Enzymes Weijers HA, Van De Kamer JH, Mossel DAA, Dicke WK. Diarrhoea Caused by Deficiency of Sugar-Splitting Enzymes. Lancet. 1960;276(7145):296-7. PMS 294 2020 QOL Medical, LLC 5 Sucrase-Isomaltase Substrates Sucrose Isomaltose Glucose + Fructose Glucose + Glucose (a-1,2 glycosidic bond) (a-1,6 glycosidic bond) PMS 294 2020 QOL Medical, LLC 6 How Sucrase Works to Hydrolyze Sucrose 1. Enzyme Substrate binds to disaccharide (Sucrose) substrate Enzyme H20 (Sucrase) 4. Active site available for another substrate 3. Products are released and absorbed 2. Substrate is converted to monosaccharides Glucose Fructose PMS 294 2020 QOL Medical, LLC 7 CSID Carb Maldigestion Pathophysiology NORMAL CSID PMS 294 2020 QOL Medical, LLC 8 SI Gene o Encodes a heterodimer with 2 active sites – sucrase and isomaltase • Located on chromosome 31 • Very large – approximately 100 kilobases1 • 48 exons encoding 1827 amino acids1 o 2146 rare variants with 880 SI rare pathogenic variants (SI-RPVs)2 • Sucrase-Isomaltase protein transported and anchored to apical membrane of enterocytes to digest disaccharides in intestinal lumen3 1.