Siddiqui A K, Ahmed S
Division of Pulmonary and Critical Care Medicine, Department of Medicine, Long Island Jewish Medical Center, New Hyde Park, USA.
Postgrad Med J. 2003 Jul;79(933):384-90. doi: 10.1136/pmj.79.933.384.
Pulmonary complications account for significant morbidity and mortality in patients with sickle cell disease. Clinical lung involvement manifests in two major forms: the acute chest syndrome and sickle cell chronic lung disease. Acute chest syndrome is characterised by fever, chest pain, and appearance of a new infiltrate on chest radiograph. Sickle cell chronic lung disease, on the other hand, manifests as radiographic interstitial abnormalities, impaired pulmonary function, and, in its most severe form, by the evidence of pulmonary hypertension. Progress has been made in understanding the pathophysiology and management of these complications. In this review the current knowledge of the mechanism, diagnosis, and treatment of pulmonary complications of sickle cell disease are discussed.
肺部并发症在镰状细胞病患者中导致了显著的发病率和死亡率。临床肺部受累主要表现为两种形式：急性胸综合征和镰状细胞慢性肺病。急性胸综合征的特征为发热、胸痛以及胸部X光片上出现新的浸润影。另一方面，镰状细胞慢性肺病表现为影像学上的间质异常、肺功能受损，最严重的形式则有肺动脉高压的证据。在理解这些并发症的病理生理学和管理方面已取得进展。在本综述中，将讨论镰状细胞病肺部并发症的机制、诊断和治疗的当前知识。
