肌强直性营养不良 1 型机械通气的效果和影响：一项前瞻性队列研究。

Effect and impact of mechanical ventilation in myotonic dystrophy type 1: a prospective cohort study.

机构信息

CIC 1429, INSERM, AP-HP, Hôpital Raymond Poincaré, Garches, France.

Association Française contre les Myopathies-Téléthon, Évry, France.

出版信息

Thorax. 2018 Nov;73(11):1075-1078. doi: 10.1136/thoraxjnl-2017-210610. Epub 2018 Mar 23.

DOI:10.1136/thoraxjnl-2017-210610

PMID:29572271

Abstract

Few studies have assessed the impact of home ventilation in patients with myotonic dystrophy type 1 (DM1) and no specific recommendations are available. We assessed the survival associated with category of home ventilation adherence of patients with DM1 followed up at a home ventilation unit using a Cox proportional hazards model. 218 patients were included; those who refused or delayed their acceptance of non-invasive ventilation were at higher risk for severe events (invasive ventilation or death) (P=0.03). Risk of death was associated with orthopnoea (HR 2.37; 95% CI 1.17 to 4.80; P<0.02) and adherence category (100 to 90% vs >75%: HR 3.26; 95% CI 1.32 to 8.04; P<0.03). Failure to use home ventilation as prescribed may be associated with increased mortality in patients with DM1.

摘要

很少有研究评估家庭通气对 1 型肌强直性营养不良（DM1）患者的影响，也没有具体的建议。我们使用 Cox 比例风险模型评估了在家通气单位接受随访的 DM1 患者的家庭通气依从性类别与生存率的关系。共纳入 218 例患者；那些拒绝或延迟接受无创通气的患者发生严重事件（有创通气或死亡）的风险更高（P=0.03）。呼吸困难与死亡风险相关（HR 2.37；95%CI 1.17 至 4.80；P<0.02），且与依从性类别相关（100%至 90%比>75%：HR 3.26；95%CI 1.32 至 8.04；P<0.03）。未按规定使用家庭通气可能与 DM1 患者的死亡率增加有关。