医学
杜氏肌营养不良
膈式呼吸
睡眠呼吸障碍
肌营养不良
呼吸
意义(存在)
睡眠呼吸暂停综合征
睡眠(系统调用)
物理医学与康复
物理疗法
心脏病学
多导睡眠图
内科学
呼吸暂停
麻醉
病理
阻塞性睡眠呼吸暂停
心理治疗师
替代医学
心理学
计算机科学
操作系统
作者
Federica Trucco,Matthew Davies,Alberto Zambon,Deborah Ridout,François Abel,Francesco Muntoni
出处
期刊:Thorax
[BMJ]
日期:2024-05-09
卷期号:: thorax-220729
标识
DOI:10.1136/thorax-2023-220729
摘要
Background Diaphragmatic sleep disordered breathing (dSDB) has been recently identified as sleep dysfunction secondary to diaphragmatic weakness in Duchenne muscular dystrophy (DMD). However, scoring criteria for the identification of dSDB are missing. This study aimed to define and validate dSDB scoring criteria and to evaluate whether dSDB severity correlates with respiratory progression in DMD. Methods Scoring criteria for diaphragmatic apnoea (dA) and hypopnoeas (dH) have been defined by the authors considering the pattern observed on cardiorespiratory polygraphy (CR) and the dSDB pathophysiology. 10 sleep professionals (physiologists, consultants) blinded to each other were involved in a two-round Delphi survey to rate each item of the proposed dSDB criteria (Likert scale 1–5) and to recognise dSDB among other SDB. The scorers’ accuracy was tested against the authors’ panel. Finally, CR previously conducted in DMD in clinical setting were rescored and diaphragmatic Apnoea–Hypopnoea Index (dAHI) was derived. Pulmonary function (forced vital capacity per cent of predicted, FVC%pred), overnight oxygen saturation (SpO2) and transcutaneous carbon dioxide (tcCO2) were correlated with dAHI. Results After the second round of Delphi, raters deemed each item of dA and dH criteria as relevant as 4 or 5. The agreement with the panel in recognising dSDB was 81%, kappa 0.71, sensitivity 77% and specificity 85%. 32 CRs from DMD patients were reviewed. dSDB was previously scored as obstructive. The dAHI negatively correlated with FVC%pred (r=−0.4; p<0.05). The total number of dA correlated with mean overnight tcCO2 (r 0.4; p<0.05). Conclusions dSDB is a newly defined sleep disorder that correlates with DMD progression. A prospective study to evaluate dSDB as a respiratory measure for DMD in clinical and research settings is planned.
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