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Accession number;03A0059376
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| Title;A Case of Erythropoietic Protoporphyria with Severe Cholestasis. |
| Author;
YAMAMOTO TAKESHI
(Nippon Med. Sch.)
NAGATO TAKAMICHI
(Nippon Med. Sch.)
SHIMIZU SHUJI
(Nippon Med. Sch.)
ARAMAKI TAKUMI
(Nippon Med. Sch.)
YOKOYAMA MUNEHIRO
(Nippon Med. Sch.)
NAITO ZEN'YA
(Nippon Med. Sch.)
ASANO ARISA
(Nippon Med. Sch.)
WATANABE ATSUSHI
(Nippon Med. Sch.)
SHIMADA TAKASHI
(Nippon Med. Sch.)
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Journal Title;Japanese Pharmacology & Therapeutics
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Journal Code:Z0947A
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ISSN:0386-3603
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VOL.30;NO.Suppl.2;PAGE.S.385-S.391(2002)
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| Figure&Table&Reference;FIG.4, TBL.3, REF.33 |
| Pub. Country;Japan |
| Language;Japanese |
| Abstract;A 24-year-old man was admitted to our hospital for an evaluation and treatment of severe jaundice (total bilirubin 15.5 mg/dL) with hepatosplenomegaly in August, 1999. Labolatory tests on admission demonstrated cholestatic liver dysfunction and mild anemia with microcytosis. Hepatitis B surface antigen, antibody to hepatitis C virus and anti-nuclear antibody were negative. Upper gastrointestinal endoscopy detected mild esophageal varices. Wedged hepatic venous pressure was 11 mmHg on catheterization, which suggested pre-sinusoidal portal hypertension. Liver biopsy disclosed a fibrous expansion of portal tracts, porto-portal bridging fibrosis and severe cholestasis. We tentatively diagnosed congenital hepatic fibrosis. Jaundice was gradually disappeared after treatment with prednisolone and ursodeoxycholic acid. In August, 2001, familial clustering of photosensitivity was documented and this patient had also presented it from infancy. Porphyrin study revealed markedly elevated erythrocyte and fecal protoporphyrin levels, leading to a diagnosis of erythropoietic protoporphyria. Treatment with beta carotene and cholestyramine were started. Follow-up liver biopsy performed in April, 2002 showed mild progression of hepatic fibrosis. Minority of erythropoietic protoporphyria complicates hepatic involvement, usually showing cholestasis and fibrosis. We reported a rare case of erythropietic protporphyria with severe cholestasis and intermittent jaundice. (author abst.) |
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