Causes and outcomes of the acute chest syndrome in sickle cell disease.
Causes and outcomes of the acute chest syndrome in sickle cell disease.
复制标题
DOI：
10.1056/nejm200006223422502
复制
发表时间：
2000-06-22
影响因子：
158.5
通讯作者：
Manci, EA
中科院分区：
文献类型：
--
作者：
Vichinsky, EP;Neumayr, LD;Manci, EA
背景：急性胸综合征是镰状细胞病患者死亡的主要原因。由于其原因在很大程度上是未知的，治疗是支持性的。使用改进的诊断技术进行的初步研究表明，感染和脂肪栓塞在该综合征患者中未被充分诊断。方法：在一项30个中心的研究中，我们分析了538例镰状细胞病患者的671次急性胸综合征发作，以确定病因、结局和对治疗的反应。我们评估了一种治疗方案，包括匹配输血、支气管扩张剂和支气管镜检查。血液和呼吸道分泌物样本送到中心实验室进行抗体检测、培养、DNA检测和组织病理学分析。结果：近一半的患者最初是由于其他原因入院的，主要是疼痛。急性胸综合征确诊时，患者出现缺氧、血红蛋白值下降、进行性多叶肺炎。平均住院时间为10.5天。13%的患者需要机械通气，3%的患者死亡。20岁或以上的患者病程比年轻患者更严重。11%的患者出现神经系统疾病，其中46%的患者出现呼吸衰竭。用表型匹配的输血治疗改善了氧合，异体免疫率为1%。五分之一接受支气管扩张剂治疗的患者有临床改善。81%需要机械通气的患者恢复了健康。急性胸综合征的特定病因在38%的发作中被确定，70%的发作有完整的数据。具体病因包括肺脂肪栓塞和27种不同的感染性病原体。18例患者死亡，最常见的死亡原因是肺栓塞和传染性支气管肺炎。感染是造成56%死亡的一个因素。结论：在镰状细胞病患者中，急性胸综合征常由脂肪栓塞和感染引起，尤其是社区获得性肺炎。在老年患者和有神经系统症状的患者中，该综合征常发展为呼吸衰竭。输血和支气管扩张剂治疗可改善氧合，积极治疗可使大多数呼吸衰竭患者康复。[J] .中华医学杂志，2000;32(2):555 - 565。(C)2000年，马萨诸塞州医学协会。
Background: The acute chest syndrome is the leading cause of death among patients with sickle cell disease. Since its cause is largely unknown, therapy is supportive. Pilot studies with improved diagnostic techniques suggest that infection and fat embolism are underdiagnosed in patients with the syndrome.Methods: In a 30-center study, we analyzed 671 episodes of the acute chest syndrome in 538 patients with sickle cell disease to determine the cause, outcome, and response to therapy. We evaluated a treatment protocol that included matched transfusions, bronchodilators, and bronchoscopy. Samples of blood and respiratory tract secretions were sent to central laboratories for antibody testing, culture, DNA testing, and histopathological analyses.Results: Nearly half the patients were initially admitted for another reason, mainly pain. When the acute chest syndrome was diagnosed, patients had hypoxia, decreasing hemoglobin values, and progressive multilobar pneumonia. The mean length of hospitalization was 10.5 days. Thirteen percent of patients required mechanical ventilation, and 3 percent died. Patients who were 20 or more years of age had a more severe course than those who were younger. Neurologic events occurred in 11 percent of patients, among whom 46 percent had respiratory failure. Treatment with phenotypically matched transfusions improved oxygenation, with a 1 percent rate of alloimmunization. One fifth of the patients who were treated with bronchodilators had clinical improvement. Eighty-one percent of patients who required mechanical ventilation recovered. A specific cause of the acute chest syndrome was identified in 38 percent of all episodes and 70 percent of episodes with complete data. Among the specific causes were pulmonary fat embolism and 27 different infectious pathogens. Eighteen patients died, and the most common causes of death were pulmonary emboli and infectious bronchopneumonia. Infection was a contributing factor in 56 percent of the deaths.Conclusions: Among patients with sickle cell disease, the acute chest syndrome is commonly precipitated by fat embolism and infection, especially community-acquired pneumonia. Among older patients and those with neurologic symptoms, the syndrome often progresses to respiratory failure. Treatment with transfusions and bronchodilators improves oxygenation, and with aggressive treatment, most patients who have respiratory failure recover. (N Engl J Med 2000;342:1855-65.) (C)2000, Massachusetts Medical Society.