A case of severe benign intrahepatic cholestasis treated with liver transplantation.
A male patient with benign recurrent cholestasis since age 2.5 yr developed unremitting cholestasis with incapacitating pruritus and hepatic fibrosis by age 21. He was tried on numerous medical therapies for pruritus with transient or no relief. He responded only temporarily to biweekly plasmapheresis, which was carried out for 4 yr. He underwent orthotopic liver transplantation at age 25 with immediate resolution of his pruritus. At age 30 he is a happy, asymptomatic, fully employed professional.
Mezey
Esteban
E
Department of Medicine, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA.
Burns
Clinton
C
Burdick
James F
JF
Braine
Hayden G
HG
D000328:Adult; D001707:Biopsy, Needle; D002779:Cholestasis / Q000473:pathology* / Q000601:surgery*; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D008111:Liver Function Tests; D016031:Liver Transplantation / Q000379:methods*; D008297:Male; D012008:Recurrence; D012720:Severity of Illness Index; D016896:Treatment Outcome