title: A second attack of cholestasis associated with erythropoietic protoporphyria was successfully treated by plasma exchange and blood transfusion.
abstract: Erythropoietic protoporphyria (EPP) is a rare hereditary disease that can sometimes cause acute liver failure based on cholestasis. Acute liver failure is a fatal complication and is associated with EPP in 1-4 % of patients. Although it is extremely difficult to recover from acute liver failure, we experienced an important case of EPP where the patient recovered from the first attack of cholestasis with antibiotic treatment. The patient recovered from a second attack of cholestasis with blood infusion and plasma transfusion. This case suggests that the supply of heme by blood transfusion and the elimination of excess protoporphyrin production by plasma exchange may be a useful treatment for patients with acute cholestasis associated with EPP.
authors.affiliation: Department of Gastroenterology and Metabolism, Hiroshima University Hospital, Hiroshima, Japan, hyohji@hiroshima-u.ac.jp.
authors.forename: Yohji
authors.initials: Y
authors.lastname: Honda
authors.forename: Yoshiiku
authors.initials: Y
authors.lastname: Kawakami
authors.forename: Hiromi
authors.initials: H
authors.lastname: Kan
authors.forename: Hatsue
authors.initials: H
authors.lastname: Fujino
authors.forename: Takayuki
authors.initials: T
authors.lastname: Fukuhara
authors.forename: Noriaki
authors.initials: N
authors.lastname: Naeshiro
authors.forename: Daisuke
authors.initials: D
authors.lastname: Miyaki
authors.forename: Tomokazu
authors.initials: T
authors.lastname: Kawaoka
authors.forename: Akira
authors.initials: A
authors.lastname: Hiramatsu
authors.forename: Masataka
authors.initials: M
authors.lastname: Tsuge
authors.forename: Michio
authors.initials: M
authors.lastname: Imamura
authors.forename: Hideyuki
authors.initials: H
authors.lastname: Hyogo
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Aikata
authors.forename: Kazuaki
authors.initials: K
authors.lastname: Chayama
mesh_terms: D000328:Adult; D002779:Cholestasis / Q000209:etiology* / Q000628:therapy*; D017707:Erythrocyte Transfusion; D006801:Humans; D008297:Male; D010951:Plasma Exchange; D046351:Protoporphyria, Erythropoietic / Q000150:complications*; D012008:Recurrence; D012074:Remission Induction