Background Home mechanical ventilation (HMV) is the treatment for chronic hypercapnic alveolar hypoventilation. The proportion and evolution of paediatric invasive (IMV) and non-invasive (NIV) HMV across the world is unknown, as well as the disorders and age of children using HMV. Methods Search of Medline/PubMed for publications of paediatric surveys on HMV from 2000 to 2023. Results Data from 32 international reports, representing 8815 children (59% boys) using HMV, were analysed. A substantial number of children had neuromuscular disorders (NMD; 37%), followed by cardiorespiratory (Cardio-Resp; 16%), central nervous system (CNS; 16%), upper airway (UA; 13%), other disorders (Others; 10%), central hypoventilation (4%), thoracic (3%) and genetic/congenital disorders (Gen/Cong; 1%). Mean age±SD (range) at HMV initiation was 6.7±3.7 (0.5–14.7) years. Age distribution was bimodal, with two peaks around 1–2 and 14–15 years. The number and proportion of children using NIV was significantly greater than that of children using IMV (n=6362 vs 2453, p=0.03; 72% vs 28%, p=0.048), with wide variations among countries, studies and disorders. NIV was used preferentially in the preponderance of children affected by UA, Gen/Cong, Thoracic, NMD and Cardio-Resp disorders. Children with NMD still receiving primary invasive HMV were mainly type I spinal muscular atrophy (SMA). Mean age±SD at initiation of IMV and NIV was 3.3±3.3 and 8.2±4.4 years (p<0.01), respectively. The rate of children receiving additional daytime HMV was higher with IMV as compared with NIV (69% vs 10%, p<0.001). The evolution of paediatric HMV over the last two decades consists of a growing number of children using HMV, in parallel to an increasing use of NIV in recent years (2020–2023). There is no clear trend in the profile of children over time (age at HMV). However, an increasing number of patients requiring HMV were observed in the Gen/Cong, CNS and Others groups. Finally, the estimated prevalence of paediatric HMV was calculated at 7.4/100 000 children. Conclusions Patients with NMD represent the largest group of children using HMV. NIV is increasingly favoured in recent years, but IMV is still a prevalent intervention in young children, particularly in countries indicating less experience with NIV.

中文翻译：

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有创与无创儿科家用机械通气：过去 24 年国际发展回顾

背景 家庭机械通气 (HMV) 是治疗慢性高碳酸血症肺泡通气不足的方法。全世界儿科侵入性 (IMV) 和非侵入性 (NIV) HMV 的比例和演变以及使用 HMV 的儿童的疾病和年龄尚不清楚。方法 在 Medline/PubMed 中检索 2000 年至 2023 年有关 HMV 的儿科调查出版物。 结果 对来自 32 份国际报告的数据进行了分析，这些报告代表了 8815 名使用 HMV 的儿童（59% 是男孩）。大量儿童患有神经肌肉疾病 (NMD; 37%)，其次是心肺疾病 (Cardio-Resp; 16%)、中枢神经系统疾病 (CNS; 16%)、上呼吸道疾病 (UA; 13%)、其他疾病 (Others) ; 10%）、中枢性通气不足（4%）、胸部疾病（3%）和遗传/先天性疾病（Gen/Cong；1%）。 HMV 开始时的平均年龄±SD（范围）为 6.7±3.7 (0.5–14.7) 岁。年龄分布呈双峰分布，在 1-2 岁和 14-15 岁之间有两个峰值。使用 NIV 的儿童数量和比例显着高于使用 IMV 的儿童（n=6362 vs 2453，p=0.03；72% vs 28%，p=0.048），不同国家、研究和疾病之间存在很大差异。 NIV 优先用于大多数患有 UA、Gen/Cong、胸科、NMD 和心脏呼吸疾病的儿童。仍接受原发性侵入性 HMV 的 NMD 儿童主要为 I 型脊髓性肌萎缩症 (SMA)。 IMV 和 NIV 开始时的平均年龄±SD 分别为 3.3±3.3 和 8.2±4.4 岁 (p<0.01)。与 NIV 相比，IMV 儿童接受额外日间 HMV 的比例较高（69% vs 10%，p<0.001）。过去二十年儿科 HMV 的发展包括越来越多的儿童使用 HMV，同时近年来（2020-2023 年）NIV 的使用也不断增加。随着时间的推移（HMV 年龄），儿童的情况没有明显的趋势。然而，在 Gen/Cong、CNS 和其他组中观察到需要 HMV 的患者数量不断增加。最后，儿科 HMV 的估计患病率为 7.4/100 000 名儿童。结论 NMD 患者是使用 HMV 的最大儿童群体。近年来，NIV 越来越受到青睐，但 IMV 仍然是幼儿的普遍干预措施，特别是在 NIV 经验较少的国家。