Department of Surgery, Primary Children's Medical Center, Salt Lake City, UT, USA.
J Pediatr Surg. 2012 May;47(5):E21-3. doi: 10.1016/j.jpedsurg.2011.12.029.
A 9-year-old boy presented with pneumonia, bilateral pulmonary lesions, and fulminant respiratory failure requiring support with extracorporeal membrane oxygenation (ECMO). Open lung biopsy and subsequent bronchoscopy identified Nocardia cyriacigeorgica and Burkholderia cepacia pneumonia. Chronic granulomatous disease (CGD) was diagnosed by an abnormal neutrophil oxidative burst assay. An aggressive diagnostic and therapeutic strategy, which included ECMO, allowed for patient survival and return to baseline function. No ECMO survivors with CGD have previously been reported. It is now recognized that several forms of CGD exist, and some forms may be compatible with long-term survival. Therefore, the diagnosis of CGD should not necessarily be considered a contraindication to ECMO. This is the first known survivor of CGD-related acute respiratory failure supported by ECMO.
一位 9 岁男孩因肺炎、双侧肺部病变和暴发性呼吸衰竭需要体外膜氧合（ECMO）支持而就诊。开胸肺活检和随后的支气管镜检查发现了触须棒状杆菌和洋葱伯克霍尔德菌肺炎。中性粒细胞氧化爆发试验异常诊断为慢性肉芽肿病（CGD）。通过积极的诊断和治疗策略，包括 ECMO，患者得以存活并恢复到基线功能。以前没有 CGD 患者 ECMO 存活的报道。现在已经认识到，CGD 存在几种形式，有些形式可能与长期生存兼容。因此，CGD 的诊断不应被视为 ECMO 的禁忌证。这是首例已知的 CGD 相关急性呼吸衰竭患者通过 ECMO 支持而存活的病例。
