Objective:

Extracorporeal Membrane Oxygenation (ECMO) may serve as a life-saving rescue therapy in critically ill children with respiratory failure. While survival rates of ECMO in children with secondary immunodeficiency is considered relatively poor, survival rates in children with primary immunodeficiencies (PID) has yet to be thoroughly investigated.

Design:

Retrospective analysis of prospectively collected data from children (29 days–18 years old). PID patients were identified by using International Classification of Diseases (ICD) codes.

Setting:

Data were retrieved from Extracorporeal Life Support Organization Registry (1989–2018).

Interventions:

ECMO for a pulmonary support indication. The survival-to-discharge rate was calculated and factors influencing outcomes were compared between survivors and non-survivors.

Measurements and main results:

A total of 73 eligible ECMO runs were included. The survival-to-discharge rate in pediatric PID patients was 45.2%. No differences were noted in survival based on type of immunodeficiency (p = 0.42) or decade of support (p = 0.98). There was no difference in the rate of pre-ECMO infection in survivors versus non-survivors (p = 0.69). The survival-to-discharge rate in patients with a culture positive infection during the ECMO run was 45.0% versus 45.3% in those with no infection (p = 0.98). In multivariate analysis, only cardiac complications (OR 5.09, 95% CI: 1.15–22.53), pulmonary complications (OR: 13.00, 95% CI: 1.20–141.25), and neurologic complications (OR: 9.86, 95% CI: 1.64–59.21) were independently associated with increased mortality.

Conclusion:

Children with a PID who require extracorporeal life support due to respiratory failure have a reasonable chance of survival and should be considered candidates for ECMO. The presence of a pre-ECMO infection should not be considered an ECMO contraindication.

中文翻译：

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原发性免疫缺陷儿童呼吸衰竭体外生命支持的结果

客观的：

体外膜肺氧合 (ECMO) 可作为呼吸衰竭危重患儿的挽救生命的抢救疗法。虽然继发性免疫缺陷儿童的 ECMO 存活率被认为相对较差，但原发性免疫缺陷 (PID) 儿童的存活率尚未得到彻底调查。

设计：

对儿童（29 天至 18 岁）前瞻性收集数据的回顾性分析。PID 患者是通过使用国际疾病分类 (ICD) 代码来识别的。

环境：

从体外生命支持组织登记处（1989-2018）检索数据。

干预措施：

ECMO 用于肺部支持指示。计算出院生存率，并比较幸存者和非幸存者之间影响结果的因素。

测量和主要结果：

总共包括 73 次符合条件的 ECMO 运行。儿科 PID 患者的出院生存率为 45.2%。根据免疫缺陷类型 (p = 0.42) 或支持十年 (p = 0.98)，存活率没有差异。幸存者与非幸存者的 ECMO 前感染率没有差异 (p = 0.69)。在 ECMO 运行期间培养阳性感染患者的出院存活率为 45.0%，而没有感染的患者为 45.3% (p = 0.98)。在多变量分析中，只有心脏并发症 (OR 5.09, 95% CI: 1.15–22.53)、肺部并发症 (OR: 13.00, 95% CI: 1.20–141.25) 和神经系统并发症 (OR: 9.86, 95% CI: 1.64– 59.21) 与死亡率增加独立相关。

结论：

由于呼吸衰竭需要体外生命支持的 PID 儿童有合理的生存机会，应被视为 ECMO 的候选者。存在 ECMO 前感染不应被视为 ECMO 禁忌症。