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阵发性夜间血红蛋白尿症和布加综合征。

Paroxysmal nocturnal haemoglobinuria and Budd-Chiari syndrome.

作者信息

Wyatt H A, Mowat A P, Layton M

机构信息

Department of Child Health, King's College Hospital, Denmark Hill, London.

出版信息

Arch Dis Child. 1995 Mar;72(3):241-2. doi: 10.1136/adc.72.3.241.

DOI:10.1136/adc.72.3.241
PMID:7741575
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1511044/
Abstract

An 11 year old boy developed pancytopenia, haemolysis, and Budd-Chiari syndrome. The venous thrombosis extended to involve other intra-abdominal vessels before paroxysmal nocturnal haemoglobinuria was recognised as the underlying haematological abnormality. Earlier diagnosis would have made curative bone marrow transplantation a possibility.

摘要

一名11岁男孩出现了全血细胞减少、溶血和布加综合征。在阵发性夜间血红蛋白尿被确认为潜在的血液学异常之前,静脉血栓形成已扩展至累及其他腹内血管。早期诊断本可使治愈性骨髓移植成为可能。

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Paroxysmal nocturnal haemoglobinuria and Budd-Chiari syndrome.阵发性夜间血红蛋白尿症和布加综合征。
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引用本文的文献

1
Paroxysmal nocturnal hemoglobinuria in childhood and adolescence--a retrospective analysis of 18 cases.儿童及青少年阵发性夜间血红蛋白尿——18例回顾性分析
Indian J Pediatr. 2008 Jun;75(6):575-8. doi: 10.1007/s12098-008-0111-9. Epub 2008 Aug 31.
2
Paroxysmal nocturnal hemoglobinuria in children.儿童阵发性夜间血红蛋白尿症
Paediatr Drugs. 2007;9(1):11-6. doi: 10.2165/00148581-200709010-00002.

本文引用的文献

1
Deficiency of the GPI anchor caused by a somatic mutation of the PIG-A gene in paroxysmal nocturnal hemoglobinuria.阵发性夜间血红蛋白尿中因PIG-A基因体细胞突变导致的糖基磷脂酰肌醇(GPI)锚缺陷。
Cell. 1993 May 21;73(4):703-11. doi: 10.1016/0092-8674(93)90250-t.
2
Phosphatidylinositol-linked proteins and paroxysmal nocturnal hemoglobinuria.磷脂酰肌醇连接蛋白与阵发性夜间血红蛋白尿症
Blood. 1990 Apr 15;75(8):1595-601.
3
Marrow transplantation for paroxysmal nocturnal hemoglobinuria.阵发性夜间血红蛋白尿的骨髓移植
Am J Hematol. 1992 Apr;39(4):283-8. doi: 10.1002/ajh.2830390409.