Fragility fractures and bone mineral density in male patients affected by type 1 and type 2 myotonic dystrophy |
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| Affiliation: | 1. Endocrinology and Diabetology Service, IRCCS Istituto Ortopedico Galeazzi, Via Riccardo Galeazzi 4, Milan 20161, Italy;2. Neurorehabilitation Unit, University of Milan, The NEMO (NEuroMuscular Omniservice) Clinical Center, Milan 20162, Italy;3. Department of Biomedical Sciences for Health, University of Milan, Milan 20133, Italy;4. Diagnostic and Interventional Radiology Unit, IRCCS Istituto Ortopedico Galeazzi, Milan 20161, Italy;5. Neurology Unit, IRCCS Policlinico San Donato, San Donato Milanese, Milan 20097, Italy;6. Department of Biomedical, Surgical and Odontoiatric Sciences, University of Milan, Milan 20122, Italy;1. Department of Paediatric Neurology and Neurophysiology, Children''s University Hospital, Temple Street, Dublin, Ireland;2. Department of Clinical Genetics, Children''s University Hospital, Temple Street, Dublin, Ireland;1. IRCCS Istituto Ortopedico Galeazzi, via Riccardo Galeazzi 4, 20161 Milan, Italy;2. Dipartimento di Scienze Biomediche per la Salute, Università degli Studi di Milano, via Carlo Pascal, 36, 20133, Milan, Italy;3. Scuola di Specializzazione in Radiodiagnostica, Università degli Studi di Milano, Via Festa del Perdono, 7, 20122 Milan, Italy;4. Radiologia Diagnostica e Interventistica Generale, Area Diagnostica per Immagini, Dipartimento Diagnostica per Immagini, Radioterapia Oncologica ed Ematologia, IRCCS Fondazione Policlinico Universitario A. Gemelli, Roma, Italia;5. Istituto di Radiologia, Università Cattolica del Sacro Cuore, Roma, Italia;1. Cancer Center, Tongji Hospital of Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei 430030, PR China;2. Department of Pain Management, Wuhan Pu''ai Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430034, PR China;3. Department of Anesthesiology and Pain Medicine, Tongji Hospital of Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei 430030, PR China;4. Department of Anaesthesiology, The University of Hong Kong, Queen Mary Hospital, Hong Kong, PR China |
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| Abstract: | Myotonic dystrophy is a multisystemic disorder affecting skeletal muscle. Male patients have an increased risk of fractures and develop a number of endocrine/metabolic impairments known to adversely affect bone health. The aim of this study was primarily to determine the occurrence of fragility fractures and the bone mineralization status (lumbar spine, hip and total body by dual X-ray absorptiometry) in 36 male patients affected with type 1 myotonic dystrophy and 13 male patients affected with type 2 myotonic dystrophy. Fragility fractures occurred in 15 type 1 and 7 type 2 myotonic dystrophy in non-classical osteoporotic sites, such as metatarses. Hip osteopenia was the most frequent finding, particularly in type 2 (n = 6) than type 1 myotonic dystrophy patients (n = 1), while osteoporosis was rare. Patients with type 1 myotonic dystrophy presented higher total body bone mass density than patients with type 2 myotonic dystrophy and healthy controls and lumbar spine was associated positively with the severity of the disease. Gonadic failure, with low testosterone and reduced INSL3 levels, visceral adiposity and insulin resistance correlated with reduced body mass index in both type 1 and type 2 myotonic dystrophic patients. The independent determinant of fragility fractures were low total body mass index, low blood testosterone and low global muscle mass. |
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