Züleyha Bingöl1, Hacer Durmuş Tekce2, Gülseren Sağcan3, Piraye Serdaroğlu2, Esen Kıyan4,5. 1. Istanbul Faculty of Medicine, Department of Pulmonary Disease, Istanbul University, Istanbul, Turkey. 2. Istanbul Faculty of Medicine, Department of Neurology, Istanbul University, Istanbul, Turkey. 3. Department of Pulmonary Disease, Biruni University Hospital, Istanbul, Turkey. 4. Istanbul Faculty of Medicine, Department of Pulmonary Disease, Istanbul University, Istanbul, Turkey. kiyanesen@gmail.com. 5. Istanbul Tip Fakultesi, Gogus Hastaliklari Anabilim Dali, Istanbul Universitesi, Capa, 34360, Istanbul, Turkey. kiyanesen@gmail.com.
Abstract
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 34.9 ± 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 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, AHIREM was 30.6 ± 19.7/h, AHINREM 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 SpO2 was 94.9% ± 1.7, lowest SpO2 was 73.3% ± 13.9, and time spent with SpO2 < 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 routinely.
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 34.9 ± 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 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, AHIREM was 30.6 ± 19.7/h, AHINREM 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 SpO2 was 94.9% ± 1.7, lowest SpO2 was 73.3% ± 13.9, and time spent with SpO2 < 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 routinely.
Authors: R B Bell; A K Brownell; C R Roe; A G Engel; S I Goodman; F E Frerman; D W Seccombe; F F Snyder Journal: Neurology Date: 1990-11 Impact factor: 9.910
Authors: Aya Ohkuma; Satoru Noguchi; Hideo Sugie; May Christine V Malicdan; Tokiko Fukuda; Kunio Shimazu; Luis Carlos López; Michio Hirano; Yukiko K Hayashi; Ikuya Nonaka; Ichizo Nishino Journal: Muscle Nerve Date: 2009-03 Impact factor: 3.217