Budd-Chiari Syndrome Associated with Porto-Mesenteric Venous Thrombosis: an Unusual Revealing Mode of Paroxystic Nocturnal Hemoglobinuria

被引：0

作者：

Khedher, S. ^{[1
]}

Said, Y. ^{[1
]}

Foudhaili, N. ^{[1
]}

Ben Ismail, K. ^{[1
]}

Salem, M. ^{[1
]}

机构：

[1] Hop Charles Nicolle, Unite Soins Intensifs, Serv Gastroenterol, Tunis, Tunisia

来源：

MEDECINE INTENSIVE REANIMATION | 2019年 / 28卷 / 06期

关键词：

Budd-Chiari syndrome; Paroxysmal nocturnal hemoglobinuria; Diagnosis; Treatment; LIVER-TRANSPLANTATION; VEIN-THROMBOSIS; THROMBOLYSIS;

D O I：

10.3166/rea-2019-0108

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Budd-Chiari syndrome (BCS) is a vascular disease of the liver, mainly caused by hepatic venous thrombosis. Paroxysmal nocturnal hemoglobinuria (PNH), acquired clonal hematopoietic stem cell disorder, is one of the prothrombotic conditions predisposing to the development of primary BCS. BCS-PNH combination is rare, often reported as isolated clinical cases or small series, and has prognostic and therapeutic specificities. We report the case of a 27-year-old girl with PNH revealed as part of the etiological assessment of a BCS, associated with portal venous thrombosis, mesenteric thrombosis, and pulmonary embolism.

引用

页码：451 / 455

页数：5

共 19 条

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