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镰状细胞病;概述

Sickle cell disease; a general overview.

作者信息

Schnog J B, Duits A J, Muskiet F A J, ten Cate H, Rojer R A, Brandjes D P M

机构信息

Department of Internal Medicine, St Elisabeth Hospital, Curaçao, the Netherlands Antilles.

出版信息

Neth J Med. 2004 Nov;62(10):364-74.

PMID:15683091
Abstract

Sickle cell disease (SCD) is a heterogeneous disorder, with clinical manifestations including chronic haemolysis, an increased susceptibility to infections and vaso-occlusive complications often requiring medical care. Patients with SCD can develop specific and sometimes life-threatening complications, as well as extensive organ damage reducing both their quality of life and their life expectancy. Proven effective treatment options for sickle cell patients are limited to hydroxyurea, blood transfusions and bone marrow transplantation. With the increasing prevalence of SCD in the Netherlands, a fundamental understanding of its pathophysiology and clinical syndromes is of importance for local medical practitioners.

摘要

镰状细胞病(SCD)是一种异质性疾病,其临床表现包括慢性溶血、易感染以及血管阻塞性并发症,这些并发症常需要医疗护理。SCD患者可能会出现特定的、有时甚至危及生命的并发症,以及广泛的器官损伤,从而降低他们的生活质量和预期寿命。镰状细胞病患者经证实有效的治疗选择仅限于羟基脲、输血和骨髓移植。随着SCD在荷兰的患病率不断上升,深入了解其病理生理学和临床综合征对当地医生而言至关重要。

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