State University of New York/Downstate Medical Center, Kings County Hospital Center, 450 Clarkson Ave., Brooklyn, NY 11203, USA.
Blood. 2011 May 19;117(20):5297-305. doi: 10.1182/blood-2010-11-261834. Epub 2011 Mar 15.
Acute chest syndrome describes new respiratory symptoms and findings, often severe and progressive, in a child with sickle cell disease and a new pulmonary infiltrate. It may be community-acquired or arise in children hospitalized for pain or other complications. Recognized etiologies include infection, most commonly with atypical bacteria, and pulmonary fat embolism (PFE); the cause is often obscure and may be multifactorial. Initiation of therapy should be based on clinical findings. Management includes macrolide antibiotics, supplemental oxygen, modest hydration and often simple transfusion. Partial exchange transfusion should be reserved for children with only mild anemia (Hb > 9 g/dL) but deteriorating respiratory status. Therapy with corticosteroids may be of value; safety, efficacy and optimal dosing strategy need prospective appraisal in a clinical trial. On recovery, treatment with hydroxyurea should be discussed to reduce the likelihood of recurrent episodes.
急性胸部综合征是指镰状细胞病患儿出现新的呼吸症状和体征，常伴有新的肺部浸润，且通常较为严重和进行性加重。它可发生于社区获得性感染，也可发生于因疼痛或其他并发症住院的患儿。其公认的病因包括感染，最常见的是感染非典型细菌，以及肺脂肪栓塞（PFE）；其病因通常不明确，可能是多因素的。治疗的启动应基于临床发现。治疗包括大环内酯类抗生素、补充氧气、适度补液，通常还需要输血。仅轻度贫血（Hb > 9 g/dL）但呼吸状态恶化的患儿可考虑部分换血疗法。皮质类固醇治疗可能有价值；需要前瞻性临床试验评估其安全性、疗效和最佳剂量策略。在康复后，应讨论使用羟基脲治疗，以降低复发的可能性。
