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Pulmonary function in patients with hereditary motor and sensory neuropathy: A comparison of patients with and without spinal deformity
Institution:1. Department of Rehabilitation and Sports Medicine, Second Faculty of Medicine, Charles University and University Hospital Motol, Prague, Czech Republic;2. Department of Pneumology, Thomayer Hospital, First Faculty of Medicine, Charles University,Prague, Czech Republic;3. Department of Neurology, Second Faculty of Medicine, Charles University and University Hospital Motol, Prague, Czech Republic;4. School of Aging Studies, University of South Florida, Tampa, FL, USA;5. International Clinical Research Center, St. Anne’s University Hospital Brno, Brno, Czech Republic;1. Otorhinolaryngologist, Outpatient Clinic for Mouth-Breathers, Federal University of Minas Gerais, Belo Horizonte, Minas Gerais, Brazil;2. Associate professor, Graduate Program in Orthodontics, Pontifical Catholic University of Minas Gerais, Belo Horizonte, Minas Gerais, Brazil;3. Orthodontist, Outpatient Clinic for Mouth-Breathers, Federal University of Minas Gerais, Belo Horizonte, Minas Gerais, Brazil;4. Graduate student, Graduate Program in Orthodontics, Pontifical Catholic University of Minas Gerais, Belo Horizonte, Minas Gerais, Brazil;5. Associate professor of otorhinolaryngology and chief, Outpatient Clinic for Mouth-Breathers, Federal University of Minas Gerais, Belo Horizonte, Minas Gerais, Brazil;6. Associate professor of pediatrics and chief, Division of Immunology, School of Medicine, Federal University of Minas Gerais, Belo Horizonte, Minas Gerais, Brazil;1. Department of Biochemistry, Institute of Science, Banaras Hindu University, Varanasi, India;2. Department of Bioscience and Biotechnology, Banasthali University, Banasthali, India;3. Department of Chemistry, Institute of Science, Banaras Hindu University, Varanasi, India;4. Department of Medicine, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India;1. Department of Rehabilitation and Sports Medicine, Second Faculty of Medicine, Charles University and University Hospital Motol, Prague, Czech Republic;2. Department of Internal Medicine, Second Faculty of Medicine, Charles University and University Hospital Motol, Prague, Czech Republic;3. Department of Statistics and Probability, Faculty of Informatics and Statistics, University of Economics, Prague, Czech Republic;4. School of Aging Studies, University of South Florida, Tampa, FL, USA;5. International Clinical Research Center, St. Anne''s University Hospital, Brno, Czech Republic;6. Chirurgie Generale Viscerale et Digestive, Centre Hospitalier Émile Roux, France;1. Private practice, Colorado Springs, Colo;2. Arthur A. Dugoni School of Dentistry, University of the Pacific, San Francisco, Calif;1. NYU Honors Program in Aesthetics, Department of Cariology and Comprehensive Care, New York City, 345 E 24th street, NY 10010, USA;2. Private Practice, 10 Norton street, Irvine, CA 92612, USA
Abstract:We assessed pulmonary function in hereditary motor and sensory neuropathy. Fourteen neuropathy patients without spinal deformity (group 1), 14 with spinal deformity (group 2), and 16 individuals with idiopathic spinal deformity (group 3) matched to group 2 for age, height and Cobb angle, were included. Hereditary motor and sensory neuropathy severity was measured with Charcot–Marie–Tooth Neuropathy Score. All participants exhibited mild decrease in maximal inspiratory pressure at the mouth. One-way analysis of variance yielded significant main effects for lung volumes – slow vital capacity, forced expiratory volume in 1 s, and total lung capacity (p’s < .01), attributable to greater volumes in group 1 compared to groups with spinal deformity – and transfer factor for carbon monoxide (p = .013), reflecting differences between groups 1 vs. 2. Slow vital capacity and total lung capacity correlated with maximal inspiratory pressure at the mouth in group 2, whereas slow vital capacity correlated with muscle work in group 3 (p’s < .05). Decreased lung volume may be due to impaired respiratory muscle strength in hereditary motor and sensory neuropathy with spinal deformity and due to spinal deformity in idiopathic patients.
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