Drug-associated acute-onset vanishing bile duct and Stevens-Johnson syndromes in a child.
Acute vanishing bile duct syndrome is a rare but established cause of progressive cholestasis in adults, is most often drug or toxin related, and is of unknown pathogenesis. It has not been reported previously in children. Stevens-Johnson syndrome is a well-recognized immune complex-mediated hypersensitivity reaction that affects all age groups, is drug or infection induced, and has classic systemic, mucosal, and dermatologic manifestations. A previously healthy child who developed acute, severe, rapidly progressive vanishing bile duct syndrome shortly after Stevens-Johnson syndrome is described; this was temporally associated with ibuprofen use. Despite therapy with ursodeoxycholic acid, prednisone, and then tacrolimus, her cholestatic disease was unrelenting, with cirrhosis shown by biopsy 6 months after presentation. This case documents acute drug-related vanishing bile duct syndrome in the pediatric age group and suggests shared immune mechanisms in the pathogenesis of both Stevens-Johnson syndrome and vanishing bile duct syndrome.
Srivastava
M
M
Combined Program in Gastroenterology, Department of Medicine, Children's Hospital, Boston, Massachusetts, USA.
Perez-Atayde
A
A
Jonas
M M
MM
D000208:Acute Disease; D000328:Adult; D001649:Bile Duct Diseases / Q000139:chemically induced* / Q000150:complications / Q000188:drug therapy; D001706:Biopsy; D002648:Child; D002779:Cholestasis / Q000139:chemically induced* / Q000150:complications / Q000188:drug therapy; D005260:Female; D006801:Humans; D007052:Ibuprofen / Q000009:adverse effects*; D008099:Liver / Q000473:pathology; D008103:Liver Cirrhosis / Q000209:etiology* / Q000473:pathology; D011241:Prednisone / Q000627:therapeutic use; D013262:Stevens-Johnson Syndrome / Q000139:chemically induced* / Q000150:complications; D016559:Tacrolimus / Q000627:therapeutic use; D013997:Time Factors; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
D014580:Ursodeoxycholic Acid; D011241:Prednisone; D007052:Ibuprofen; D016559:Tacrolimus