دورية أكاديمية
Pulmonary functions and sleep-related breathing disorders in lipid storage disease
| العنوان: | Pulmonary functions and sleep-related breathing disorders in lipid storage disease |
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| المؤلفون: | Bingöl, Züleyha, Tekce, Hacer Durmuş, Sağcan, Gülseren, Serdaroğlu, Piraye, Kıyan, Esen |
| المساهمون: | Biruni Üniversitesi, orcid:0000-0001-8202-5313 |
| بيانات النشر: | Springer Nature Switzerland |
| المجموعة: | Biruni University Institutional Repository (DSpace@Biruni) |
| مصطلحات موضوعية: | Lipid Storage Disease, Neuromuscular Disease, Obstructive Sleep Apnea, Pulmonary Functions |
| الوصف: | Purpose Pulmonary function abnormalities and sleep-related breathing disorders (SRBD) are frequent in subjects with several neuromuscular diseases but there is no data about lipid storage diseases (LSD). Therefore, we aimed to evaluate pulmonary functions and SRBD in adults with LSD. Methods Pulmonary functions (forced expiratory volume (FEV1), forced vital capacity (FVC), supine FVC, upright-supine FVC% change, maximal inspiratory pressure (MIP), maximal expiratory pressure (MEP), peak cough flow (PCF)), arterial blood gases, and polysomnographic data of all subjects were evaluated. Results Twenty-five subjects with LSD were evaluated [17 males, 8 females; age 34.915 years; BMI 26.5 +/- 3.4 kg/m(2)]. MIP was -72.2 +/- 32.7cmH(2)O (< -80 cmH(2)O in 13 subjects), MEP was 80.9 +/- 39.1 cmH(2)O (< 80 cmH(2)O in 9 subjects, < 40 cmH(2)O in 6 subjects), and PCF was 441.3 +/- 190.9 L/min (< 360 L/min in 11 subjects). FVC was 87.8% +/- 25.7 and 6 subjects had FVC < 80%. Seven subjects had diaphragm dysfunction (four upright-supine FVC% 15, three dyspnea in supine position with paradoxical abdominal respiration). Five subjects had hypoxemia (PaO2 < 80 mmHg) and 8 subjects had hypercapnia (PaCO2 > 45 mmHg). REM sleep had decreased in all subjects (10.2% +/- 6.1). Obstructive sleep apnea (OSA) was found in 80% of the subjects (n = 20; 9 mild, 9 moderate, 2 severe). For subjects with OSA, apnea-hypopnea index (AHI) was 20.8 +/- 15.9/h, oxygen desaturation index (ODI) was 11.9 +/- 15.4/h, AHI(REM) was 30.6 +/- 19.7/h, AHI(NREM) was 19.7 +/- 16.6/h, ODIREM was 27.2 +/- 26.1/h, and ODINREM was 11.4 +/- 15/h. Five subjects (20%) diagnosed as REM-related OSA. Nocturnal mean SpO(2) was 94.9% +/- 1.7, lowest SpO(2) was 73.3% +/- 13.9, and time spent with SpO(2) < 90% was 2.4% +/- 7.2. Conclusion In subjects with LSD, pulmonary function impairment, daytime hypercapnia and hypoxemia, and OSA, especially REM-related OSA, are frequent. Therefore, pulmonary functions and polysomnography should be performed ... |
| نوع الوثيقة: | article in journal/newspaper |
| وصف الملف: | application/pdf |
| اللغة: | English |
| تدمد: | 1520-9512 1522-1709 |
| العلاقة: | Sleep and Breathing; Makale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanı; https://doi.org/10.1007/s11325-018-1647-1Test; https://hdl.handle.net/20.500.12445/640Test; 22; 1137; 1142 |
| DOI: | 10.1007/s11325-018-1647-1 |
| الإتاحة: | https://doi.org/20.500.12445/640Test https://doi.org/10.1007/s11325-018-1647-1Test https://hdl.handle.net/20.500.12445/640Test |
| حقوق: | info:eu-repo/semantics/closedAccess |
| رقم الانضمام: | edsbas.A313F36D |
| قاعدة البيانات: | BASE |
| تدمد: | 15209512 15221709 |
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| DOI: | 10.1007/s11325-018-1647-1 |