原発性肝癌を併発した晩発性皮膚ポルフィリン症の１症例

Material type: 記事

Author: 松永高志ほか

Publisher: The Japan Society of Hepatology

Publication date: 1990

Material Format: Digital

Journal name: 肝臓 31 11

Publication Page: p.1353-1358

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Summary, etc.：: 晩発性皮膚ポルフィリン症(PCT)の経過中に肝細胞癌を発症した1症例を報告する.症例は56歳,男性.49歳時に皮膚の黄染,掻痒感,食欲不振にて入院した.30年来の飲酒歴,肝障害,皮膚生検所見ならびに尿中ウロポルフィリン・コプロポルフィリンの増加からPCTと診断された.肝庇護剤・デフェロキサミンの投与...

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Digital

Material Type: 記事
Title: 原発性肝癌を併発した晩発性皮膚ポルフィリン症の１症例
Author Heading: 松永高志
道免和文
岩切龍一
石橋大海
長野政則
溝口幹朗
岩田康
Publication Date: 1990
Publication Date (W3CDTF): 1990
Alternative Title: A case report
Periodical title: 肝臓
No. or year of volume/issue: 31 11
Volume: 31
Issue: 11
Pages: 1353-1358
Publication date of volume/issue (W3CDTF): 1990
ISSN (Periodical Title): 04514203
ISSN-L (Periodical Title): 04514203
Publication (Periodical Title): The Japan Society of Hepatology
Text Language Code: ja
Subject Heading: 晩発性皮膚ポルフィリン症
肝癌
ヘモクロマトーシス
Target Audience: 一般
DOI: 10.2957/kanzo.31.1353
https://doi.org/10.2957/kanzo.31.1353
Related Material (URI): http://www.jstage.jst.go.jp/article/kanzo1960/31/11/31_11_1353/_pdf
https://search.jamas.or.jp/link/ui/1991159484
Data Provider (Database): 国立情報学研究所 : CiNii Research
https://cir.nii.ac.jp/
Original Data Provider (Database): Japan Link Center
https://japanlinkcenter.org/top
Crossref
https://www.crossref.org
CiNii Articles
NAID: 130000878470

Digital

Summary, etc.: We report a case of porphyria cutanea tarda (PCT), who developed hepatocellular carcinoma seven years after the onset of PCT. A 49-year-old male was admitted to our hospital in 1982 because of itching and jaundice. Diagnosis of PCT was made on the base of long-term alcoholic ingestion, liver dysfunction, histological findings of skin biopsy sample and marked urinary excretion of uroporphyrin and coproporphyrin. Deferoxamine was effective but itching and jaundice again appeared seven years later and he was readmitted. He was markedly jaundiced and died soon after the admission of hepatocellular carcinoma which was confirmed at autopsy. Deposition of fat and iron were demonstrated in the non-carcinomatous tissues. The pathogenic mechanism of the occurrence of hepatocellular carcinoma in PCT is still a problem to be solved.
晩発性皮膚ポルフィリン症(PCT)の経過中に肝細胞癌を発症した1症例を報告する.症例は56歳,男性.49歳時に皮膚の黄染,掻痒感,食欲不振にて入院した.30年来の飲酒歴,肝障害,皮膚生検所見ならびに尿中ウロポルフィリン・コプロポルフィリンの増加からPCTと診断された.肝庇護剤・デフェロキサミンの投与により軽快し,以後愁訴なく経過していたが,7年後の56歳時皮膚の黄染,掻痒感が再発し再入院となった.α-fetoprotein, PIVKA IIは高値を呈し,US,CT所見にて肝両葉に多数の結節像を認めた.部検にて肝臓はびまん性に大小の結節で占められていた.組織学的にはEdmondson III型の肝細胞癌であり,非癌部の肝細胞には脂肪と鉄の沈着が認められた.肝細胞癌と基礎疾患としてのPCTの肝病変との関連について文献的考察を加え報告する.
DOI: 10.2957/kanzo.31.1353
Access Restrictions: インターネット公開
Data Provider (Database): 科学技術振興機構 : J-STAGE
http://www.jstage.jst.go.jp

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