Prader-Willi综合征睡眠呼吸障碍的患病率。

IF 2.1 4区医学 Q3 RESPIRATORY SYSTEM Canadian respiratory journal Pub Date : 2023-10-26 eCollection Date: 2023-01-01 DOI:10.1155/2023/9992668

Ahmed Abushahin, Amal Al-Naimi, Mutasim Abu-Hasan, Rania Arar, M Lina Hayati, Antonisamy Belavendra, Ibrahim A Janahi

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引用次数: 0

摘要

引言：睡眠呼吸障碍（SDB）在Prader-Willi综合征（PWS）患者中很常见。然而，不同研究的SDB患病率差异很大。早期识别SDB及其发病因素至关重要，尤其是在考虑生长激素（GH）治疗时。目的：本研究旨在描述Prader-Willi综合征（PWS）患者睡眠呼吸障碍（SDB）的患病率和表型，并确定年龄、性别、症状、生长激素治疗和体重指数对SDB严重程度的影响。方法：本研究是对所有在Sidra Medicine睡眠实验室接受诊断性夜间多导睡眠图（PSG）的遗传确诊的Prader-Willi综合征患者的回顾性图表回顾。收集入选患者的临床和PSG数据。结果：我们确定了20名PWS患者（9名男性，11名女性），他们在中位年龄（IQR）为5.83（2.7-12）岁时进行了夜间睡眠多导睡眠图（PSG）检查。中位呼吸暂停低通气指数（AHI）为8.55（IQR 5.8-16.9）次/小时。REM-AHI的中位数为27.8（IQR 15-50.6）次/小时。阻塞性呼吸暂停低通气指数（OAHI）中位数为7.29（IQR 1.8-13.5）次/小时。中位中枢性呼吸暂停低通气指数（CAHI）为1.77（IQR 0.6-4.1）次/小时。19名患者（95%）通过基于AHI≥1.5事件/小时的多导睡眠图（PSG）显示SDB。9名患者（45%）被诊断为阻塞性睡眠呼吸暂停（OSA）。3名患者（15%）被诊断为中枢性睡眠呼吸暂停（CSA）。7名患者（35%）被诊断为混合性睡眠呼吸暂停。AHI与年龄、性别、BMI、症状或GH治疗之间没有相关性。然而，REM-AHI与BMI显著相关（P=0.031）。结论：本研究表明，在我们的PWS患者中，SDB的患病率很高。阻塞性睡眠呼吸暂停是主要表型。BMI是高REM-AHI的唯一预测因素。有必要对大型队列进行进一步研究，以确定PWS中的SDB并设计适当的治疗方法。

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Prevalence of Sleep-Disordered Breathing in Prader-Willi Syndrome.

Introduction: Sleep-disordered breathing (SDB) is common in patients with Prader-Willi Syndrome (PWS). However, the prevalence of SDB varies widely between studies. Early identification of SDB and factors contributing to its incidence is essential, particularly when considering growth hormone (GH) therapy.

Objectives: The aims of the study were to describe the prevalence and phenotypes of sleep-disordered breathing (SDB) in patients with Prader-Willi syndrome (PWS) and to determine the effects of age, gender, symptoms, GH therapy and body mass index on SDB severity.

Methods: This study was a retrospective chart review of all patients with genetically confirmed Prader-Willi syndrome who underwent diagnostic overnight polysomnography (PSG) in the sleep laboratory at Sidra Medicine. Clinical and PSG data of enrolled patients were collected.

Results: We identified 20 patients (nine males, eleven females) with PWS who had overnight sleep polysomnography (PSG) at a median age (IQR) of 5.83 (2.7-12) years. The median apnea-hypopnea index (AHI) was 8.55 (IQR 5.8-16.9) events/hour. The median REM-AHI was 27.8 (IQR 15-50.6) events/hour. The median obstructive apnea-hypopnea index (OAHI) was 7.29 (IQR 1.8-13.5) events/hour. The median central apnea-hypopnea index (CAHI) was 1.77 (IQR 0.6-4.1) events/hour. Nineteen patients (95%) demonstrated SDB by polysomnography (PSG) based on AHI ≥1.5 events/hour. Nine patients (45%) were diagnosed with obstructive sleep apnea (OSA). Three patients (15%) were diagnosed with central sleep apnea (CSA). Seven patients (35%) were diagnosed with mixed sleep apnea. No correlations were observed between AHI and age, gender, BMI, symptoms, or GH therapy. However, REM-AHI was significantly correlated with BMI (P=0.031).

Conclusion: This study shows a high prevalence of SDB among our patients with PWS. Obstructive sleep apnea was the predominant phenotype. BMI was the only predictor for high REM-AHI. Further studies of large cohorts are warranted to define SDB in PWS and design the appropriate treatment.

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来源期刊

Canadian respiratory journal 医学-呼吸系统

CiteScore

4.20

自引率

0.00%

发文量

审稿时长

6-12 weeks

期刊介绍： Canadian Respiratory Journal is a peer-reviewed, Open Access journal that aims to provide a multidisciplinary forum for research in all areas of respiratory medicine. The journal publishes original research articles, review articles, and clinical studies related to asthma, allergy, COPD, non-invasive ventilation, therapeutic intervention, lung cancer, airway and lung infections, as well as any other respiratory diseases.