title: D-penicillamine and plasmapheresis in acute liver failure secondary to Wilson's disease.
abstract: We report a case of a 19-year-old woman with acute liver failure, Coombś negative hemolytic anemia, and renal failure as initial manifestations of Wilsoń disease with recovery following medical treatment. The clinical picture and low serum transaminase and alkaline phosphatase levels gave us a clue to suspect Wilsoń disease and to initiate plasmapheresis and D-penicillamine soon after admission. The serum and urinary copper levels were elevated with low serum ceruloplasmin. We proceeded to ambulatory follow-up with medical treatment with D-penicillamine. A few months later, during the course of a laparoscopic cholecystectomy because of symptomatic gallstone disease, a liver biopsy sample was obtained that showed histological liver fibrosis and strongly elevated levels of liver tissue copper.
authors.affiliation: Gastroenterology and Intestive Care Units. Belltvige Hospital. Barcelona. Spain. evarodfa@hotmail.com
authors.forename: E
authors.initials: E
authors.lastname: Rodríguez Fariña
authors.forename: G
authors.initials: G
authors.lastname: Tremosa Llurba
authors.forename: X
authors.initials: X
authors.lastname: Xiol Quingles
authors.forename: A
authors.initials: A
authors.lastname: Lores Obradors
authors.forename: J
authors.initials: J
authors.lastname: Castellote Alonso
authors.forename: J B
authors.initials: JB
authors.lastname: Gornals Soler
authors.forename: C
authors.initials: C
authors.lastname: López Núñez
mesh_terms: D000328:Adult; D000743:Anemia, Hemolytic / Q000150:complications; D001706:Biopsy; D002570:Ceruloplasmin / Q000032:analysis; D002614:Chelating Agents / Q000627:therapeutic use*; D003300:Copper / Q000097:blood / Q000652:urine; D005260:Female; D005500:Follow-Up Studies; D006527:Hepatolenticular Degeneration / Q000097:blood / Q000150:complications* / Q000175:diagnosis / Q000473:pathology / Q000652:urine; D006801:Humans; D008099:Liver / Q000473:pathology; D017114:Liver Failure, Acute / Q000188:drug therapy / Q000209:etiology* / Q000628:therapy*; D010396:Penicillamine / Q000627:therapeutic use*; D010956:Plasmapheresis; D013997:Time Factors
chemical_list: D002614:Chelating Agents; D003300:Copper; D002570:Ceruloplasmin; D010396:Penicillamine