TITLE

Erythropoietic protoporphyria with fatal liver failure

AUTHOR(S)
Ishibashi, Akiko; Ogata, Riko; Sakisaka, Shotaro; Kumashiro, Ryukichi; Koga, Yuriko; Mitsuyama, Keiichi; Kuromatsu, Ryoko; Uchimura, Yasuyo; Ijyuin, Hiroyasu; Tanaka, Kumi; Iwao, Tadashi; Ishii, Kunihide; Sata, Michio; Inoue, Yoshiko; Kin, Yasuko; Oizumi, Kotaro; Nishida, Hidemi; Imaizumi, Tsutomu; Tanikawa, Kyuichi; Ishibashi, A
PUB. DATE
June 1999
SOURCE
Journal of Gastroenterology;1999, Vol. 34 Issue 3, p405
SOURCE TYPE
Academic Journal
DOC. TYPE
journal article
ABSTRACT
A 33-year-old woman with a history of photosensitivity, persistent abdominal pain, and liver dysfunction was admitted to our department because of abdominal pain and progression of liver dysfunction. On admission, levels of protoporphyrin and coproporphyrin within erythrocytes were markedly increased. Autofluorescent erythrocytes were also detected, leading to a diagnosis of erythropoietic protoporphyria. A liver biopsy specimen revealed cirrhosis with dark brown granules filling hepatocytes, bile canaliculi, and bile ductules. Transfusion of washed erythrocytes, hemodialysis, and administration of cholestyramine and beta-carotene transiently improved levels of porphyrins and liver function. The patient died of rupture of esophageal varices followed by multiple organ failure. However, the treatments were believed to have extended survival.
ACCESSION #
4690213

 

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