Definition of diaphragmatic sleep disordered breathing and clinical meaning in Duchenne muscular dystrophy

IF 9 1区医学 Q1 RESPIRATORY SYSTEM

Thorax Pub Date : 2024-05-09 DOI:10.1136/thorax-2023-220729

Federica Trucco, Matthew Davies, Alberto Andrea Zambon, Deborah Ridout, Francois Abel, Francesco Muntoni

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Abstract

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. All data relevant to the study are included in the article or uploaded as online supplemental information.

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杜氏肌肉萎缩症中横膈膜睡眠呼吸紊乱的定义和临床意义

背景横膈膜睡眠呼吸障碍（dSDB）最近被确定为继发于杜氏肌营养不良症（DMD）患者横膈膜无力的睡眠功能障碍。然而，目前尚缺乏识别 dSDB 的评分标准。本研究旨在定义和验证 dSDB 的评分标准，并评估 dSDB 的严重程度是否与 DMD 的呼吸进展相关。方法作者根据心肺复律仪（CR）观察到的模式和 dSDB 的病理生理学，定义了膈肌呼吸暂停（dA）和低通气（dH）的评分标准。10 名睡眠专业人员（生理学家、顾问）在相互盲视的情况下参与了两轮德尔菲调查，对建议的 dSDB 标准（李克特量表 1-5）的每个项目进行评分，并在其他 SDB 中识别出 dSDB。评分者的准确性由作者小组进行测试。最后，对以前在临床环境中对 DMD 进行的 CR 进行了重新评估，并得出了膈肌呼吸暂停-低通气指数（dAHI）。肺功能（预测肺活量的百分比，FVC%pred）、夜间血氧饱和度（SpO2）和经皮二氧化碳（tcCO2）与 dAHI 相关。结果在第二轮德尔菲法之后，评分者认为 dA 和 dH 标准的每项相关性均为 4 或 5。专家组在识别 dSDB 方面的一致性为 81%，kappa 为 0.71，敏感性为 77%，特异性为 85%。对 32 例 DMD 患者的 CR 进行了复查。dAHI 与 FVC%pred 呈负相关（r=-0.4；p<0.05）。dA 总数与平均夜间 tcCO2 相关（r=0.4；p<0.05）。结论 dSDB 是一种新定义的睡眠障碍，与 DMD 的进展相关。我们计划开展一项前瞻性研究，以评估 dSDB 作为 DMD 在临床和研究环境中的呼吸测量指标。与该研究相关的所有数据均包含在文章中或作为在线补充信息上传。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Thorax 医学-呼吸系统

CiteScore

16.10

自引率

2.00%

发文量

197

审稿时长

1 months

期刊介绍： Thorax stands as one of the premier respiratory medicine journals globally, featuring clinical and experimental research articles spanning respiratory medicine, pediatrics, immunology, pharmacology, pathology, and surgery. The journal's mission is to publish noteworthy advancements in scientific understanding that are poised to influence clinical practice significantly. This encompasses articles delving into basic and translational mechanisms applicable to clinical material, covering areas such as cell and molecular biology, genetics, epidemiology, and immunology.

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