Forty-five years of Duchenne muscular dystrophy in The Netherlands
| Autor: | Jan J.G.M. Verschuuren, B.H.A. Wokke, M. E. Van Der Tol, I.J.M. de Groot, Peter J. Wijkstra, Chiara S. M. Straathof, Erik H. Niks, Marianne Zijnen, M.E.B. Rijlaarsdam, Nicole A. M. Cobben, J.C. van den Bergen, A. M. C. Horemans, Johanna M. Fock, Elizabeth Vroom, H.B. Ginjaar, I.F.M. de Coo, R.F. Pangalila, A. J. van Essen, Mike J. Kampelmacher |
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| Jazyk: | angličtina |
| Rok vydání: | 2014 |
| Předmět: |
Mechanical ventilation
Duchenne muscular dystrophy Pediatrics medicine.medical_specialty Survival business.industry medicine.medical_treatment Cardiomyopathy Natural history Disease Mechanical home ventilation medicine.disease Disorders of movement Donders Center for Medical Neuroscience [Radboudumc 3] Neurology Cohort Physical therapy Medicine Population study Corticosteroids Neurology (clinical) business Historical Cohort |
| Zdroj: | Journal of Neuromuscular Diseases, 1(1), 99-109 Journal of Neuromuscular Diseases, 1, pp. 99-109 Journal of Neuromuscular Diseases, 1, 99-109 |
| ISSN: | 2214-3602 2214-3599 |
| Popis: | Background: Duchenne muscular dystrophy (DMD) is a progressive muscle disease. No curative therapy is currently available, but in recent decades standards of care have improved. These improvements include the use of corticosteroids and mechanical ventilation. Objective: To present a detailed population based report of the DMD disease course in The Netherlands (1980–2006) and evaluate the effect of changes in care by comparing it with an historical Dutch DMD cohort (1961–1974). Methods: Information about DMD patients was gathered through the Dutch Dystrophinopathy Database using a standardized questionnaire and information from treating physicians. Results: The study population involved 336DMDpatients (70% of the estimated prevalence), of whom 285 were still alive. Mean age at disease milestones was: diagnosis 4.3 years, wheelchair dependence 9.7 years, scoliosis surgery 14 years, cardiomyopathy (fractional shortening |
| Databáze: | OpenAIRE |
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