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姐妹中的“红细胞生成性”原卟啉症与肝硬化

'Erythropoietic' protoporphyria and cirrhosis in sisters.

作者信息

Thompson R P, Molland E A, Nicholson D C, Gray C H

出版信息

Gut. 1973 Dec;14(12):934-8. doi: 10.1136/gut.14.12.934.

DOI:10.1136/gut.14.12.934
PMID:4785283
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1412859/
Abstract

The clinical and histopathological features of two sisters with erythropoietic protoporphyria and liver disease are described. They had unusually severe photosensitivity with blistering, and both died at the age of 31 from cirrhosis. The association of erythropoietic protoporphyria with several hepatic abnormalities is discussed.

摘要

描述了两名患有红细胞生成性原卟啉症和肝病的姐妹的临床和组织病理学特征。她们有异常严重的光敏性并伴有水疱形成，两人均在31岁时死于肝硬化。讨论了红细胞生成性原卟啉症与几种肝脏异常的关联。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3385/1412859/43589db93bb5/gut00637-0026-c.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3385/1412859/e7dcc1c50e95/gut00637-0024-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3385/1412859/c476f8cd9a90/gut00637-0026-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3385/1412859/2cb679583b97/gut00637-0026-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3385/1412859/43589db93bb5/gut00637-0026-c.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3385/1412859/e7dcc1c50e95/gut00637-0024-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3385/1412859/c476f8cd9a90/gut00637-0026-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3385/1412859/2cb679583b97/gut00637-0026-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3385/1412859/43589db93bb5/gut00637-0026-c.jpg

相似文献

1
'Erythropoietic' protoporphyria and cirrhosis in sisters.姐妹中的“红细胞生成性”原卟啉症与肝硬化
Gut. 1973 Dec;14(12):934-8. doi: 10.1136/gut.14.12.934.
2
Erythropoietic protoporphyria: two deaths from hepatic cirrhosis.
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3
Erythropoietic protoporphyria: a family study.红细胞生成性原卟啉症：一项家族研究。
Br Med J. 1967 Mar 18;1(5541):659-63. doi: 10.1136/bmj.1.5541.659.
4
Erythropoietic protoporphyria with hepatic cirrhosis.伴有肝硬化的红细胞生成性原卟啉症
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5
Erythropoietic protoporphyria showing solar purpura.表现为日光性紫癜的红细胞生成性原卟啉病。
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Isotopic studies of the erythropoietic and hepatic components of congenital porphyria and 'erythropoietic' protoporphyria.先天性卟啉症和“红细胞生成性”原卟啉症的红细胞生成及肝脏成分的同位素研究。
Clin Sci. 1973 Feb;44(2):135-50. doi: 10.1042/cs0440135.
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8
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9
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Liver disease in erythropoietic protoporphyria: insights and implications for management.红细胞生成性原卟啉症中的肝脏疾病：见解与管理意义
Postgrad Med J. 2007 Dec;83(986):739-48. doi: 10.1136/gut.2006.097576.
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Liver disease in erythropoietic protoporphyria: insights and implications for management.

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Blood exchange and transfusion therapy for acute cholestasis in protoporphyria.
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Am J Med. 1963 Oct;35:450-4. doi: 10.1016/0002-9343(63)90144-x.
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Disturbance of porphyrin metabolism caused by griseofulvin in mice.灰黄霉素所致小鼠卟啉代谢紊乱
Br J Dermatol. 1963 Mar;75:91-104. doi: 10.1111/j.1365-2133.1963.tb13945.x.
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Increase in activity of alpha-aminolevulinic acid synthetase in liver mitochondria induced by feeding of 3,5-dicarbethoxy-1,4-dihydrocollidine.喂食3,5 - 二乙氧羰基 - 1,4 - 二氢可力丁诱导肝脏线粒体中α - 氨基乙酰丙酸合成酶活性增加。
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Faecal porphyrin excretion in normal subjects and in patients with the "malabsorption syndrome".正常受试者及“吸收不良综合征”患者的粪便卟啉排泄情况
Clin Sci. 1962 Jun;22:447-57.
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Erythropoietic protoporphyria. A new porphyria syndrome with solar urticaria due to protoporphyrinaemia.红细胞生成性原卟啉病。一种因原卟啉血症导致伴有日光性荨麻疹的新卟啉病综合征。
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[Light hypersensitivity and porphyrinemia].[光过敏与卟啉血症]
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