目的:先天性中枢性通气不足综合征(CCHS)是一种罕见的疾病,易导致儿童因通气驱动异常而导致呼吸衰竭。据报道,通气不足和呼吸支持需求存在差异。我们旨在确定与CCHS患儿气管造口术和住院的常见病因相关的临床变量。
方法:从儿童住院数据库中获取了2006年至2019年间CCHS住院儿童(<21岁)的出院记录。总结了CCHS住院的主要诊断类别。采用多变量logistic回归模型探讨气管切开相关危险因素。
结果:在2404例CCHS住院患者中,133例(5.5%)发生气管切开术,1230(51.2%)已建立气管造口术,和1041(43.3%)没有气管造口术。与没有气管造口术的儿童相比,那些气管造口术的人更年轻,有早熟史,先天性心脏病,喉,声门,声门下狭窄(LGSS),先天性气道异常,神经肌肉无力,胃食管反流病.无气管造口术的儿童死亡率高于有气管造口术的儿童(2.19%vs.0.66%)。多变量调整分析表明,气管造口术与婴儿期(0-1年)有关,神经肌肉无力,先天性心脏病.最常见的诊断类别包括(1)呼吸系统疾病(30.23%),(2)伤害和中毒(9.35%),(3)神经系统和感觉器官疾病(6.71%)。
结论:接受气管切开术的CCHS患儿更有可能更年轻,并且有LGSS,神经肌肉无力和先天性心脏病。临床医生应该意识到这些风险因素代表更严重的CCHS,早期表现需要气管造口术。非气管造口术组的死亡率较高,这凸显了在照顾CCHS患儿时考虑气管造口术的必要性。
OBJECTIVE: Congenital central hypoventilation syndrome (CCHS) is a rare disease predisposing children to respiratory failure due to abnormal ventilatory drive. Variability in hypoventilation and respiratory support need have been reported. We aim to identify clinical variables associated with incident tracheostomy and common etiologies of hospitalization among children with CCHS.
METHODS: Hospital discharge records were obtained for children (<21 years) with CCHS hospitalized between 2006 and 2019 from the Kid\'s Inpatient Database. Primary diagnostic categories for hospitalizations with CCHS were summarized. Multivariable logistic regression models were used to explore risk factors associated with incident tracheostomy.
RESULTS: Among 2404 hospitalizations with CCHS, 133 (5.5%) had incident tracheostomy, 1230 (51.2%) had established tracheostomy, and 1041 (43.3%) had no tracheostomy. Compared with children without tracheostomy, those with incident tracheostomy were younger, had a history of prematurity, congenital heart disease, laryngeal, glottic, and subglottic stenosis (LGSS), congenital airway anomalies, neuromuscular weakness, gastroesophageal reflux disease. Children without tracheostomy had higher mortality than those with tracheostomy status (2.19% vs. 0.66%). Multivariable-adjusted analyses showed that incident tracheostomy was associated with infancy (0-1 years), neuromuscular weakness, and congenital heart disease. Most common diagnostic categories include (1) diseases of the respiratory system (30.23%), (2) injury and poisoning (9.35%), and (3) diseases of the nervous system and sense organs (6.71%).
CONCLUSIONS: Children with CCHS who received incident tracheostomy are more likely to be younger and with LGSS, neuromuscular weakness and congenital heart disease. Clinicians should be aware of these risk factors representing more severe CCHS with earlier manifestation needing tracheostomy. Higher mortality among nontracheostomy group highlights the need for considering tracheostomy in caring for children with CCHS.