Quéré G, Tempescul A, Couturaud F, Paleiron N, Leroyer C, De Saint-Martin L
Institut de cancérologie et d'hématologie, hôpital Morvan, CHRU de Brest, France.
Rev Pneumol Clin. 2011 Dec;67(6):335-41. doi: 10.1016/j.pneumo.2011.07.001. Epub 2011 Oct 13.
Sickle cell disease is a common but often poorly understood by chest physicians. The acute chest syndrome represents its main respiratory complication.
Sickle cell disease is an autosomal recessive disorder inducing, in certain circumstances, sickling of red cells. Natives from western or central Africa and from the Caribbean islands are mainly affected. Acute chest syndrome is defined by the association of chest pain or fever and recent radiographic infiltrates, in patients suffering from sickle cell disease. Determination of etiology, infection, fat embolism or hypoventilation, is difficult, as a self-perpetuating vicious circle is ongoing. Support, largely undervalued, is based on etiological treatment and measures to avoid worsening linked to complications, especially microcirculatory disease.
Acute chest syndrome is a severe respiratory complication of sickle cell disease. Therapeutic measures are simple but undervalued.
镰状细胞病很常见，但胸部科医生对此往往了解不足。急性胸部综合征是其主要的呼吸并发症。
镰状细胞病是一种常染色体隐性疾病，在某些情况下会导致红细胞镰变。主要受影响的是来自西非或中非以及加勒比群岛的本地人。急性胸部综合征的定义是，患有镰状细胞病的患者出现胸痛或发热，并伴有近期影像学上的浸润。病因（感染、脂肪栓塞或通气不足）的确定很困难，因为存在一个自我持续的恶性循环。在很大程度上被低估的支持措施基于病因治疗以及避免与并发症（尤其是微循环疾病）相关的病情恶化的措施。
急性胸部综合征是镰状细胞病的一种严重呼吸并发症。治疗措施简单但被低估。
