Positive Coombs Negative Coombs Gilbert syndrome Alagille syndrome Hepatitis A, B, C, Progressive familial Total parenteral Idiopathic neonatal Autoimmune test test Physiologic Nonsyndromic D, E, G intrahepatic nutrition hepatitis chronic hepatitisABO and Rh RBC enzyme jaundice of the paucity of Cytomegalovirus cholestasis Acetaminophen Familial benign Sclerosingincompatibility defect (G6PD newborn intrahepatic bile Herpes simplex   Wilson disease Ethanol recurrent cholangitis Autoimmune, deficiency) Breast milk ducts 1, 2, 6 ˜1-Antitrypsin Salicylates cholestasis Graft versus host systemic lupus Hemoglobinopathy jaundice Biliary atresia Epstein-Barr virus deficiency Cholestasis with disease
Figure 130-1 Differential diagnosis of jaundice in childhood. G6PD, Glucose-6-phosphate dehydrogenase; RBC, red blood cell.
AST, ALT Hepatocellular injury
Very long chain fatty acids Zellweger syndrome, peroxisomal disorders
Abdominal ultrasound Choledochal cyst, gallstones, mass lesion, Caroli disease
Percutaneous liver biopsy Biliary atresia, idiopathic giant cell hepatitis, α1-antitrypsin deficiency
ALT, Alanine aminotransferase; AST, aspartate aminotransferase; CF, cystic fibrosis; GGT, γ-glutamyltransferase; RBC, red blood cell.
Laboratory studies No diagnosis Specific diagnosis Abdominal ultrasound Biliary cyst or obstruction No biliary lesions seen Hepatobiliary scan Excretion into bowel Liver biopsy Surgery Evidence of biliary atresia No evidence of biliary atresia Specific therapy (if available), or Supportive care No excretion
Figure 130-2 Flow chart for evaluation of neonatal cholestasis.
is removed and replaced with a roux-en-Y loop of jejunum.This operation must be performed before 3 months of age tohave the best chance of success. Even so, the success rate is low; many children require liver transplantation. Some metabolic causes of neonatal cholestasis are treatable by dietarymanipulation (galactosemia) or medication (tyrosinemia);all affected patients require supportive care. This includesfat-soluble vitamin supplements (vitamins A, D, E, and K)and formula containing medium-chain triglycerides, whichcan be absorbed without bile salt–induced micelles. Choleretic agents, such as ursodeoxycholic acid, may improve bileflow in some conditions.
SEE CHAPTER 113.
Fulminant liver failure is defined as severe liver disease with onset of hepatic encephalopathy within 8 weeks afterinitial symptoms, in the absence of chronic liver disease.Etiology includes viral hepatitis, metabolic disorders, autoimmune hepatitis, ischemia, neoplastic disease, and toxins(Table 130-2).
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