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- Title
Erythropoietic protoporphyria with fatal liver failure.
- Authors
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
- 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.
- Subjects
LIVER failure; ERYTHROPOIETIC failure; ESOPHAGEAL varices; TREATMENT of cirrhosis of the liver; AUTOPSY; COMBINATION drug therapy; HEMODIALYSIS; CIRRHOSIS of the liver; LIVER function tests; MULTIPLE organ failure; NEEDLE biopsy; ORGAN rupture; TREATMENT effectiveness; DISEASE progression; HEPATIC porphyria; DISEASE complications
- Publication
Journal of Gastroenterology, 1999, Vol 34, Issue 3, p405
- ISSN
0944-1174
- Publication type
journal article
- DOI
10.1007/s005350050284