Dubowitz Neuromuscular Centre, UCL Great Ormond Street Institute of Child Health and Great Ormond Street Hospital, London, UK
Paediatric Respiratory Department, Royal Brompton Hospital, Guy's and St Thomas' Trust, London, UK.
Thorax. 2024 Jun 14;79(7):652-661. doi: 10.1136/thorax-2023-220729.
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.
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.
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).
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.
膈肌睡眠呼吸障碍（dSDB）最近被认为是杜氏肌营养不良症（DMD）中膈肌无力导致的睡眠功能障碍。然而，目前还缺乏识别 dSDB 的评分标准。本研究旨在定义和验证 dSDB 评分标准，并评估 dSDB 的严重程度是否与 DMD 的呼吸进展相关。
作者根据心肺多导睡眠图（CR）上观察到的模式和 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 名睡眠专家（生理学家、顾问）相互之间不知情，参与了两轮 Delphi 调查，对拟议的 dSDB 标准的每一项进行评分（Likert 量表 1-5），并识别其他睡眠呼吸暂停中的 dSDB。评分者的准确性与作者小组进行了测试。最后，对临床环境中先前在 DMD 中进行的 CR 进行重新评分，并得出膈肌呼吸暂停-低通气指数（dAHI）。肺功能（用力肺活量占预计值的百分比，FVC%pred）、夜间血氧饱和度（SpO2）和经皮二氧化碳（tcCO2）与 dAHI 相关。
经过第二轮 Delphi 调查，评分者认为 dA 和 dH 标准的每一项都与 4 或 5 相关。识别 dSDB 的小组的一致性为 81%，kappa 值为 0.71，灵敏度为 77%，特异性为 85%。对 32 例 DMD 患者的 CR 进行了回顾。dSDB 先前被评为阻塞性。dAHI 与 FVC%pred 呈负相关（r=-0.4；p<0.05）。dA 的总数与夜间平均 tcCO2 相关（r 0.4；p<0.05）。
dSDB 是一种新定义的睡眠障碍，与 DMD 进展相关。计划进行一项前瞻性研究，以评估 dSDB 作为 DMD 临床和研究环境中的呼吸测量指标。
