Rare, autosomal recessive disease
Characterized by significant unconjugated hyperbilirubinemia
History / PE:
Type 1 Severe jaundice, neurologic impairment (kernicterus)
Indirect bilirubin levels typically 20-25mg/dL, can rise as high as 50mg/dL
IV phenobarbital fails to reduce bilirubin levels
Type 2 Milder disorder, no kernicterus, or neuro impairment
Indirect bilirublin levels typically < 20mg/dL
Treatment:
Phototherapy, plasmapherisis (short term)
Liver transplant (type 2 long term cure)
