Jaundice is clinically apparent when important. The clinician needs to educate hemolytic hyperbilirubinemia with normal hypotonia, , high-pitched cry, for α-1 Antitrypsin deficiency, Alagille there is a yellowish discoloration of the the family on symptoms of dehydration histology); Dubin-Johnson syndrome hyperpyrexia, seizures, paresis of gaze, syndrome, FIC1 deficiency, BSEP skin, sclera, and mucous membranes and secondary to inadequate breastmilk intake (autosomal recessive disorder that causes oculogyric crisis, and death. Milder forms of deficiency, and MDR3 deficiency. Further is evident when the total bilirubin level and poor feeding. When jaundice persists an increase of conjugated bilirubin without bilirubin encephalopathy include cognitive imaging options include cholangiogram, rises above 4-5 mg/dl in infants and 3 beyond two weeks after birth, cholestasis elevation of liver enzymes); Biliary atresia dysfunction and learning disabilities. CT, MRI, ERCP, and/or liver biopsies. mg/dl in older children. It is important or conjugated hyperbilirubinemia must be (improper opening of bile ducts which leads Initial diagnostic evaluation for Treatment options for moderate to severe to identify the cause of excess bilirubin considered in the differential diagnoses. to bile accumulation); Alpha-1 antitrypsin unconjugated hyperbilirubinemia jaundice consist of: alteration of breast- in order to initiate appropriate treatment. Neonatal cholestasis is defined as a direct deficiency (genetic disorder that causes may include: complete blood count, feeding with formula feeding; Phototherapy Elevated bilirubin levels should always be bilirubin level of ≥ 2 mg/dl and ≥ 20% defective production of A1AT that protects reticulocytes, direct and indirect Coombs to change the shape and structure of the fractionated into unconjugated (indirect) or Youhanna Al-Tawil , MD of the total bilirubin level. Persistent the lungs and liver from damage); Hemolytic test, haptoglobin, and Hb electrophoresis. bilirubin molecules in such a way that conjugated (direct) bilirubin and classified Misty Murphy FNP-C jaundice associated with acholic stools diseases (hereditary spherocytosis or G6PD Initial laboratory testing for conjugated they can be excreted in the urine and stool; as pre-hepatic, hepatic, or post-hepatic. and dark urine may suggest biliary atresia. deficiency); Hemoglobinopathies (Sickle hyperbilirubinemia consists of: CBC, Intravenous immunoglobulin if caused by Pre-hepatic jaundice arises when the bilirubin excretion may be directly related to Such findings must undergo immediate cell anemia, Alpha/Beta Thalasemias); liver enzymes, albumin, total protein, ABO or Rh isoimmunisation; Exchange excess levels overwhelm the hepatocyte’s breast-feeding, inborn errors of metabolism, diagnostic evaluation since the Kasai Congenial Hypothyroidism; Cystic fibrosis coagulation factors, cholesterol, and transfusions for severe jaundice that doesn’t ability to conjugate bilirubin. Hepatic hormones, drugs, prematurity, hepatic portoenterostomy surgical procedure for (results in abnormal bile production); ammonia levels. Additional diagnostic respond to other treatments; Interruption jaundice presents when there is failure of hypoperfusion, cholestatic syndromes, or biliary atresia requires diagnosis at an early Infections (Hepatitis, EBV, CMV, sepsis); tests include: TSH, Free T4, hepatitis and of the enterohepatic circulation with bile metabolism or excretion. Post-hepatic biliary tree obstructions. Sepsis, intrauterine age. Hepatotoxins (APAP, antibiotics, EBV serologies, serum alpha-1 antitrypsin medications such as Agar, Cholestyramine, jaundice occurs when there is interruption infection, and congenital cirrhosis may also Differential diagnoses include: Breastfed anticonvulsants); or Vascular causes levels, immunoglobulins, serum amino activated charcoal, calcium phosphate; of bile drainage into the biliary system. be the culprit. induced (insufficient production or intake (Budd Chiari syndrome or Veno-occlusive acids, and an EKG. Imaging studies or Surgery if caused by biliary atresia or Jaundice can occur in all age groups Physiologic jaundice manifests after the of breast milk); physiologic (mild jaundice disease). directed toward diagnosis may begin choledochal cysts. but is more prevalent in neonates and first 24 hours of life. This is commonly due to the immaturity of the liver); Gilbert Complications of hyperbilirubinemia with an abdominal ultrasound to measure Investigation of jaundice and timely commonly results from the accumulation of breastfeeding induced and is associated Syndrome (a common benign genetic liver causes concern since unconjugated bilirubin hepatic size and/or consistency, and to treatment of the underlining pathology is unconjugated bilirubin. Neonatal jaundice with suboptimal milk and caloric intake. condition in which the liver doesn’t properly is neurotoxic and may lead to acute detect abnormal echotexture as well as crucial to patient outcomes. Age, medical appears from either increased bilirubin Jaundice manifesting before the first 24 process bilirubin and leads to isolated bilirubin encephalopathy, death, or lifelong identifying masses, cysts, abscesses, and history, and physical examination are key production or decreased secretion. Increased hours is always considered pathologic until unconjugated hyperbilirubinemia); Crigler- neurologic sequelae. Serum unconjugated biliary tree abnormalities. New testing factors for accurate diagnosis. The providers production may be caused from fetal- proven otherwise. Breast milk jaundice Najjar syndrome (genetic disorder affecting bilirubin levels greater than 30 mg/dl have known as the Jaundice Chip Resequencing at GI for Kids would be pleased to evaluate maternal blood group incompatibilities, manifests after the first 4-7 days after birth metabolism of bilirubin); Rotor’s syndrome been known to cause kernicterus. Clinical Array is available for inherited intrahepatic and help manage patients presenting with hemorrhage, polycythemia, red blood cell and there is a peak in levels around 14 days. (a benign, genetic liver disorder characterized findings of kernicterus may consist of cholestasis of unknown origin. This test jaundice symptoms or assist with referral to abnormalities, or labor induction. Decreased In the clinic setting, identifying BMJ is by chronic, predominantly conjugated, non- a sluggish Moro reflex, opisthotonos, looks at several genes that are responsible the appropriate medical team. Jaundice in Breast Fed Babies JAUNDICE AT A GLANCE Jaundice is more common in babies who fectively established. are breastfed than babies who are formula • Work with a lactation consultant to make sure the baby is Two types of jaundice may occur in newborns who are • Bleeding underneath the scalp (cephalohematoma) fed. Breastfeeding jaundice usually oc- latching on the breast well. breastfed. Both types are most often harmless. caused by a difficult delivery curs when a newborn does not get a good • It is perferable to breastfed, but if the serum bilirubin is • Breastfeeding jaundice is seen in breastfed babies • Higher levels of red blood cells, which is more common in start on breastfeeding from either improper eleveated and if the child is not intaking the appropriate volume during the first week of life. It is more likely to occur small-for-gestational-age babies and some twins latch, inability to feed well, or when the then we will need to hold breastfeeding for 2-3 days and supple- when babies do not nurse well or the mother’s milk is • Infection child is supplemented with formula which ment with formula or supplement every other feed with formula slow to come in. • Lack (deficiency) of certain important proteins, called can interfere with breastfeeding. Adequate to try to adequatley hydrate the child. If this decreases the se- • Breast milk jaundice may appear in some healthy, enzymes amounts of breast milk will increase the rum bilirubin sufficiently we can try to restart breastfeeding. breastfed babies after day 7 of life. It is likely to peak Things that make it harder for the baby’s body to remove baby’s bowel movements, which will then Ashley Treadway, • If supplementation is needed to increase the baby’s intake, during weeks 2 and 3 but may last at low levels for a bilirubin may also lead to more severe jaundice, including: MS, RDN, LDN help excrete the buildup of bilirubin. we encourage the mother to continue to pump during this time month or more. The problem may be due to how • Certain medicines If the bilirubin levels are less than 20mg/ to not interrupt the production of her milk. substances in the breast milk affect the breakdown of • Infections present at birth, such as rubella, syphilis, dl the following treatments are recommended: • Closely monitor baby’s weight gain. • Initiate the breastfeeding relationship as soon as possible The baby should recover fully with the right monitoring and bilirubin in the liver. Breast milk jaundice is different and others after birth. treatment. Although jaundice cannot be prevented, there are than breastfeeding jaundice. • Diseases that affect the liver or biliary tract, such as • Increase feedings to 8-12 times a day. ways to avoid it becoming serious and reaching levels that re- Severe newborn jaundice may occur if the baby has a cystic fibrosis or hepatitis • Feed whenever the baby is alert, sucking on the hands, and quire additional interventions, such as phototherapy and in se- condition that increases the number of red blood cells that • Low oxygen level (hypoxia) smacking the lips. This is one way babies let you know they vere cases, a blood transfusion. need to be replaced in the body, such as: • Infections (sepsis) are hungry. Do not try to put the baby on a “schedule,” feed References: www.americanpregnancy.com, www.kidshealth. • Abnormal blood cell shapes • Many different genetic or inherited disorders the baby frequently until the breastfeeding relationship is ef- org, www.nlm.nih.gov • Blood type mismatch between the mother and baby Our behavior health clinicians can help your pediatric patients with: . GI-related disorders including recurrent , food allergies, celiac disease, irritable bowel syndrome & inflammatory bowel disease, & encopresis . Other disorders including adjustment to illness, family dysfunction, sleep difficulties, , depression, generalized anxiety, GI for Kids, PLLC panic attacks, eating disorders, non-compliance to medical regimens, and more… Visit our website for more details: www.GIforKids.com Call for an appointment today!

865.546.3998 Fall/Winter 2015 www.giforkids.com Volume 6 Issue 8 Friends and Colleagues, As the seasons are changing and we are approaching winter, we at GI for Kids hope for a great holiday season for you and your family. Our focus for this issue is jaundice in infants and children. Hyperbilirubinemia resulting in jaundice is one of the most common problems encountered in BEE FIT 4 KIDS term newborns. Few will have serious underlying pathology. However, hyperbilirubinemia in the A one-on-one pediatric weight management program newborn period can be associated with severe illnesses such as hemolytic disease, anatomic abnormalities of the liver, metabolic administered by a multi-disciplinary team. and endocrine disorders and infections. The management goals at The format allows Registered Dietitians to identify GI for Kids are to exclude pathologic causes of hyperbilirubinemia Supportnutrition and fitness Group trouble areas immediately. Meeting This and initiate treatment to prevent bilirubin neurotoxicity. Please helps the staff“Back make appropriate to School” changes so weight contact us with any questions or referrals and visit our website at management success is maximized. www.giforkids.com for more information on all of the pediatric If your child diagnosed with food allergies or celiac disease attends school, services our practice provides. Visit our website, www.BeeFit4Kids.com, for more details, or call 865.546.3998. Now acceptingthis support insurance! group meeting is a must!

Learn about your child’s legal rights by experts from Support and Training for Exceptional Parents (STEP).

Followed by FACET Chapter updates and openPRSRT forum. STD U.S. POSTAGE PERMIT NO. 433 When: KNOXVILLE, TN July 28, 2014 @ 6:30 PM Celiac Disease, or gluten intolerance, is a genetic autoimmune disease estimated to affect 1 of every 133 people in the U.S. Research indicates approximately 2.5 million people in the U. S. have celiac disease Where: with approximately 80,000 diagnosed. Celi-Act is sponsored by GI For Kids, PLLC, East Tennessee Children’s Hospital located at East Tennessee Children’s Hospital, and open to anyone with celiac disease or Meschendorf Conference Room AB their family members.