Abstract：
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.
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.
摘要：
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。儿科侵入性（IMV）和非侵入性（NIV）HMV在世界范围内的比例和演变是未知的，以及使用HMV的儿童的疾病和年龄。
方法：搜索Medline/PubMed，查找2000年至2023年有关HMV的儿科调查出版物。
结果：来自32份国际报告的数据，代表使用HMV的8815名儿童（59％的男孩），进行了分析。大量儿童患有神经肌肉疾病（NMD；37％），其次是心肺（Cardio-Resp;16%），中枢神经系统（CNS；16%），上气道（UA；13%），其他疾病（其他；10%），中央通气不足（4%），胸部（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。年龄分布是双峰的,在1-2年和14-15年左右有两个高峰。使用NIV的儿童数量和比例显着大于使用IMV的儿童（n=6362vs2453，p=0.03；72％vs28％，p=0.048),各国之间差异很大，研究和障碍。NIV优先用于大多数受UA影响的儿童，Gen/Cong,胸科,NMD和心血管疾病。仍接受原发性侵袭性HMV的NMD儿童主要是I型脊髓性肌萎缩症（SMA）。IMV和NIV开始时的平均年龄±SD为3.3±3.3和8.2±4.4岁（p<0.01），分别。与NIV相比，IMV的儿童接受额外日间HMV的比率更高（69％vs10％，p<0.001)。在过去的二十年中，儿科HMV的演变包括越来越多的儿童使用HMV，与近年来（2020-2023年）使用NIV的增加同时。随着时间的推移（HMV的年龄），儿童的概况没有明显的趋势。然而,在Gen/Cong中观察到越来越多的需要HMV的患者，CNS和其他组。最后,儿童HMV的估计患病率为7.4/100,000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，但是IMV仍然是幼儿普遍的干预措施，特别是在NIV经验较少的国家。
方法：搜索Medline/PubMed，查找2000年至2023年有关HMV的儿科调查出版物。
结果：来自32份国际报告的数据，代表使用HMV的8815名儿童（59％的男孩），进行了分析。大量儿童患有神经肌肉疾病（NMD；37％），其次是心肺（Cardio-Resp;16%），中枢神经系统（CNS；16%），上气道（UA；13%），其他疾病（其他；10%），中央通气不足（4%），胸部（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。年龄分布是双峰的,在1-2年和14-15年左右有两个高峰。使用NIV的儿童数量和比例显着大于使用IMV的儿童（n=6362vs2453，p=0.03；72％vs28％，p=0.048),各国之间差异很大，研究和障碍。NIV优先用于大多数受UA影响的儿童，Gen/Cong,胸科,NMD和心血管疾病。仍接受原发性侵袭性HMV的NMD儿童主要是I型脊髓性肌萎缩症（SMA）。IMV和NIV开始时的平均年龄±SD为3.3±3.3和8.2±4.4岁（p<0.01），分别。与NIV相比，IMV的儿童接受额外日间HMV的比率更高（69％vs10％，p<0.001)。在过去的二十年中，儿科HMV的演变包括越来越多的儿童使用HMV，与近年来（2020-2023年）使用NIV的增加同时。随着时间的推移（HMV的年龄），儿童的概况没有明显的趋势。然而,在Gen/Cong中观察到越来越多的需要HMV的患者，CNS和其他组。最后,儿童HMV的估计患病率为7.4/100,000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，但是IMV仍然是幼儿普遍的干预措施，特别是在NIV经验较少的国家。
