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Permanent URL to this publication: http://dx.doi.org/10.5167/uzh-10604

Kohler, M; Clarenbach, C F; Bahler, C; Brack, T; Russi, E W; Bloch, K E (2009). Disability and Survival in Duchenne Muscular Dystrophy. Journal of Neurology, Neurosurgery, and Psychiatry, 80(3):32-325.

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Abstract

BACKGROUND: Duchenne muscular dystrophy (DMD) leads to progressive impairment of muscle function, respiratory failure and premature death. Longitudinal data on the course of physical disability and respiratory function are sparse. OBJECTIVES: To prospectively assess physical impairment and disability, respiratory function and survival in DMD patients over several years in order to describe the course of the disease with current care. METHODS: In 43 patients with DMD, aged 5-35 years, yearly assessments of physical disability by the Duchenne muscular dystrophy physical Impairment and Dependence on care (DID) score ranging from 9 (no disability) to 80 (complete dependence), and forced vital capacity (FVC) were obtained over a mean +/-SD time interval of 5.4 +/-2.1 years. RESULTS: DID scores were correlated with age according to a hyperbolic function (f=85.3*age/(10.05+age), R=0.62, P<0.0001). FVC declined exponentially with age (f=139.1*exp(-0.08*age)), R=0.52, P<0.0001. Mean +/-SD age at which patients lost their ambulation was 9.4 +/-2.4 years and they became dependent on an electro-wheelchair at 14.6 +/-4.0 years. The age at beginning of assisted ventilation was 19.8 +/-3.9 years, Three patients deceased during the observation period. The estimated probability of survival to age 30 years was 85%, median survival was 35 years. CONCLUSIONS: Our detailed observations of the progression of physical disability, dependence on care and respiratory impairment in DMD patients from childhood to adult life is valuable for predicting the clinical course with current medical care. Compared to historical data, survival has considerably improved.

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Clinic for Pneumology
04 Faculty of Medicine > Center for Integrative Human Physiology
DDC:570 Life sciences; biology
610 Medicine & health
Language:English
Date:19 March 2009
Deposited On:19 Jan 2009 10:35
Last Modified:27 Nov 2013 21:33
Publisher:BMJ Publishing Group
ISSN:0022-3050
Publisher DOI:10.1136/jnnp.2007.141721
PubMed ID:18713792
Citations:Web of Science®. Times Cited: 40
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Scopus®. Citation Count: 47

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