Fulminant hepatic failure: Wilson's disease or autoimmune hepatitis? Implications for transplantation.
Fulminant hepatic failure (FHF) accounts for 10-15% of pediatric liver transplants in the USA annually. Because the onset of FHF may be the first presentation of Wilson's disease (WD) and autoimmune hepatitis (AIH) in previously asymptomatic adolescents, determination of the etiology of FHF is critical as treatment and prognosis differ between these two entities. Patients with AIH may be salvaged by medical treatment. On the contrary, liver transplantation is currently the only life saving therapeutic option available for patients with WD who present with fulminant liver failure. To establish the diagnosis of WD and AIH in the setting of FHF remains challenging for diagnosticians and decisions regarding liver transplantation may be necessary before a diagnosis is firmly established. We report a previously asymptomatic patient who presented with FHF and clinical and laboratory features suggestive of both WD and AIH and who underwent successful therapeutic liver transplantation before the diagnosis of WD could be confirmed.
Santos
R G
RG
Department of Transplant Surgery, Children's Hospital of Pittsburgh, Pittsburgh, PA 15213, USA.
Alissa
F
F
Reyes
J
J
Teot
L
L
Ameen
N
N
D000293:Adolescent; D005260:Female; D019693:Hepatitis, Autoimmune / Q000150:complications* / Q000175:diagnosis; D006527:Hepatolenticular Degeneration / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D006801:Humans; D008099:Liver / Q000473:pathology; D017114:Liver Failure, Acute / Q000209:etiology* / Q000601:surgery; D016031:Liver Transplantation