Jaundice in Children

Jaundice in Children Causes of Prolonged Jaundice Unconjugated Conjugated (more than 25 micro mol/L) Neonatal hepatitis Intrahepatic Biliary Hypoplasia Congenital infection Inborn errors of metabolism Breastmilk jaundice Alpha-1 antitrypsin deficiency Infection esp. UTI Tyrosinemia (type 1) Haemolytic anaemia e.g. G6PD deficiency Hypothyroidism Biliary atresia Errors of bile acid synthesis Choledochal cyst Progressive familial intrahepatic cholestasis Alagille syndrome High GIT obstruction Cystic fibrosis Crigler Najjar syndrome Intestinal failure associated liver disease Associated with long term parental nutrition Causes of Jaundice Unconjugated Conjugated Mixed Physiological jaundice of newborn Low RBC lifespan and immature liver Haemolytic anaemia G6DP deficiency Hereditary spherocytosis Dubin Johnson syndrome Blood transfusions Defects MP2 Rh -ve mum and Rh + baby Decreased bilirubin movements into bile caliculi Antibodies cross the placenta MP3 upregulation More common in subsequent pregnancies Increased bilirubin movement into bloodstream Immune mediate destruction of the baby’s RBCs Hepatitis Biliary atresia Management: give mum anti-D antibodies Progressive obliteration Breast milk jaundice Obstructive jaundice Metabolites of progesterone -> inhibit UDP Gallstone Increased enterohepatic circulation Pancreatic or a biliary tumor Delayed gut motility and meconium passage Gilbert syndrome Decreased UDP Criger Najjar syndrome No UDP Breast Milk Jaundice vs Breastfeeding Jaundice Breastfeeding Jaundice Breast Milk Jaundice Aetiology and pathophysiology -> increase betaglucuronidase concentration in breast milk -> increase deconjugation and resorption of the bilirubin -> persistence of physiologic jaundice with unconjugated hyperbilirubinemia (summary: metabolites of testosterone that inhibit UDP) Aetiology and pathophysiology -> Insufficient milk production or poor feeding -> Insufficient breastfeeding -> lack of calories and inadequate quantities of bowel movements -> unable to remove bilirubin from the body -> increased enterohepatic circulation -> increased reabsorption of bilirubin from the intestines -> unconjugated hyperbilirubinemia ?-Glucuronidase is found in breast milk and the intestinal brush border. Deconjugation of bilirubin by bacterial ?glucuronidase can lead to pigment stone formation. Clinical features -> onset within 1 week Treatment -> increase breastfeeding sessions and rehydration Clinical features -> onset within 2 weeks after birth -> lasts for 4 to 13 weeks Usually, bilirubin levels peak within 2 weeks after birth, are elevated for 4–10 days, then return to normal levels at 3–12 weeks. Treatment -> continue breast feeding and supplementation with formula feeds and consider phototherapy if needed DO NOT STOP BREASTFEEDING Investigations Depends on timeframe Transcutaneous bilirubinometer check If within 24 hours – pathological Total serum bilirubin FBE Coombs test – autoimmune haemolytic anaemia 24 hours to 14 days -> normal Only if there are red flags E.g. dehydration -> check Na+ and BSL Over two weeks – prolonged Total serum bilirubin FBE TFTs Group and direct antibody test (DAT) LFTs if conjugated is more than 10% Kramer’s rule Describes the relationship between serum bilirubin levels and progression of skin discolouration Limitations: Not reliable on dark skin infant Subjective and prone to inter-observer variability Cannot quantify bilirubin -> only estimate ranges Not reliable in early onset Limited sensitivity, specificity, and accuracy