Sections View Full Chapter Figures Tables Videos Full Chapter Figures Tables Videos Supplementary Content +++ Key Features ++ Type 1: rapid severe unconjugated hyperbilirubinemia (> 30–40 mg/dL) with neurologic consequences (kernicterus) Type 2 Milder than type 1 Rarely associated with neurologic complications +++ Clinical Findings ++ Type 1 Consanguinity is often present Neurologic signs may not develop until adolescence or early adulthood, at which time deterioration may occur suddenly Type 2 Hyperbilirubinemia is less severe Bile is pigmented and contains small amounts of bilirubin monoglucuronide and diglucuronide +++ Diagnosis ++ Type 1 May be useful to obtain a duodenal bile specimen; findings include Being colorless Containing a predominance of unconjugated bilirubin, small amounts of monoconjugates, and only traces of unconjugated bilirubin Liver biopsy findings and liver function tests are consistently normal Type 2 Liver biopsy findings and liver function tests are consistently normal +++ Treatment ++ Type 1 Exchange transfusions are required followed by phototherapy A combination of aggressive phototherapy and cholestyramine may keep bilirubin levels below 25 mg/dL Orlistat therapy may decrease bilirubin in a subset of patients Liver transplantation is curative and may prevent kernicterus if performed early Auxiliary orthotopic transplantation also relieves the jaundice while the patient retains native liver Type 2 Phenobarbital (4 mg/kg/d in infants) lowers serum bilirubin levels An increased proportion of monoconjugated and diconjugated bilirubin in the bile follows phenobarbital treatment GET ACCESS TO THIS RESOURCE Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Username? Forgot Password? Sign in via OpenAthens Sign in via Shibboleth Get Free Access Through Your Institution Contact your institution's library to ask if they subscribe to McGraw-Hill Medical Products. Access My Subscription GET ACCESS TO THIS RESOURCE Subscription Options Pay Per View Timed Access to all of AccessPediatrics 24 Hour $34.95 (USD) Buy Now 48 Hour $54.95 (USD) Buy Now Best Value AccessPediatrics Full Site: One-Year Individual Subscription $595 USD Buy Now View All Subscription Options