Pulmonary functions and sleep-related breathing disorders in lipid storage disease

Sleep & Breathing = Schlaf & Atmung
Züleyha BingölEsen Kıyan

Abstract

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. 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. Twenty-five subjects with LSD were evaluated [17 males, 8 females; age 34.9 ± 15 years; BMI 26.5 ± 3.4 kg/m2]. MIP was - 72.2 ± 32.7 cmH2O (< - 80 cmH2O in 13 subjects), MEP was 80.9 ± 39.1 cmH2O (< 80 cmH2O in 9 subjects, < 40 cmH2O 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 ...Continue Reading

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Citations

May 20, 2020·Frontiers in Bioengineering and Biotechnology·Kai MiDesi Shang

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