Liver Disease and Transplant Hepatology
Scope
Neonatal cholestasis, autoimmune hepatitis, Wilson disease, metabolic liver disease, portal hypertension, and post-transplant care.
Concepts
- Cholestasis: direct hyperbilirubinemia requires prompt evaluation; differential includes biliary atresia, metabolic disease, infection, and Alagille syndrome.
- Autoimmune hepatitis: elevated transaminases, hypergammaglobulinemia, autoantibodies (ANA, anti-smooth muscle); treat with corticosteroids and azathioprine.
- Wilson disease: low ceruloplasmin, elevated urinary copper, Kayser-Fleischer rings; treat with chelation.
- Portal hypertension: splenomegaly, varices, thrombocytopenia; manage variceal bleeding with octreotide and endoscopic therapy.
- Transplant: monitor for rejection (fever, transaminitis, cholestasis), infections, and drug toxicity (tacrolimus).
Traps
Candidates forget to screen for Wilson disease in adolescents with unexplained hepatitis. Another trap: attributing coagulopathy solely to vitamin K deficiency when synthetic failure from liver disease is present. Always check INR and factor V.
Worked example
A 15-year-old with jaundice, tremor, and elevated transaminases: check ceruloplasmin, urinary copper, and slit-lamp exam; low ceruloplasmin and elevated urinary copper support Wilson disease.
Rule of thumb
Any adolescent with unexplained hepatitis gets Wilson screening. Direct hyperbilirubinemia in infancy is never normal.