title: Fulminant hepatic failure and acute intravascular haemolysis as presenting manifestations of Wilson's disease in young children.
abstract: The cases of three young children (mean age 5.8 years) in whom fulminant hepatic failure and acute intravascular haemolysis were the presenting manifestations of Wilson's disease are reported. Although diagnosis was made ante-mortem and chelation therapy instituted, the course was relentlessly fatal in all three cases. This presentation of Wilson's disease at such a young age is noteworthy.
authors.affiliation: Department of Pediatrics, Postgraduate Institute of Medical Education and Research, India.
authors.forename: B N
authors.initials: BN
authors.lastname: Walia
authors.forename: S
authors.initials: S
authors.lastname: Singh
authors.forename: R K
authors.initials: RK
authors.lastname: Marwaha
authors.forename: S R
authors.initials: SR
authors.lastname: Bhusnurmath
authors.forename: J B
authors.initials: JB
authors.lastname: Dilawari
mesh_terms: D002648:Child; D002675:Child, Preschool; D005260:Female; D006461:Hemolysis; D006501:Hepatic Encephalopathy / Q000097:blood / Q000209:etiology*; D006521:Hepatitis, Chronic / Q000150:complications; D006527:Hepatolenticular Degeneration / Q000097:blood / Q000150:complications*; D006801:Humans; D008103:Liver Cirrhosis / Q000150:complications; D008297:Male