title: Blood exchange and transfusion therapy for acute cholestasis in protoporphyria.
abstract: Acute cholestasis is a rare complication of EPP with a high mortality rate despite extensive treatment with corticosteroids, cholestyramine, and antioxidants. A single survivor, reported in the literature, was treated with blood exchange transfusions. We treated two EPP patients with blood exchange and additional blood transfusions which resulted in full clinical and biochemical recovery from the cholestasis and accompanying hepatitis. Recurrences of the cholestasis and hepatitis could repeatedly be corrected by additional blood transfusions.
authors.affiliation: Department of Gastroenterology, University Hospital, Utrecht, The Netherlands.
authors.forename: H J
authors.initials: HJ
authors.lastname: van Wijk
authors.forename: J
authors.initials: J
authors.lastname: van Hattum
authors.forename: H
authors.initials: H
authors.lastname: Baart de la Faille
authors.forename: J W
authors.initials: JW
authors.lastname: van den Berg
authors.forename: A
authors.initials: A
authors.lastname: Edixhoven-Bosdijk
authors.forename: J H
authors.initials: JH
authors.lastname: Wilson
mesh_terms: D000208:Acute Disease; D000328:Adult; D001803:Blood Transfusion; D002779:Cholestasis / Q000209:etiology / Q000628:therapy*; D005078:Exchange Transfusion, Whole Blood; D005260:Female; D006801:Humans; D008107:Liver Diseases / Q000150:complications*; D008297:Male; D011164:Porphyrias / Q000150:complications*