UZH-Logo

Maintenance Infos

Disability and Survival in Duchenne Muscular Dystrophy


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.

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.

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.

Citations

69 citations in Web of Science®
78 citations in Scopus®
Google Scholar™

Altmetrics

Downloads

114 downloads since deposited on 19 Jan 2009
7 downloads since 12 months
Detailed statistics

Additional indexing

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
Dewey Decimal Classification:570 Life sciences; biology
610 Medicine & health
Language:English
Date:19 March 2009
Deposited On:19 Jan 2009 10:35
Last Modified:05 Apr 2016 12:50
Publisher:BMJ Publishing Group
ISSN:0022-3050
Publisher DOI:https://doi.org/10.1136/jnnp.2007.141721
PubMed ID:18713792

Download

[img]
Preview
Content: Accepted Version
Filetype: PDF
Size: 1MB
View at publisher

TrendTerms

TrendTerms displays relevant terms of the abstract of this publication and related documents on a map. The terms and their relations were extracted from ZORA using word statistics. Their timelines are taken from ZORA as well. The bubble size of a term is proportional to the number of documents where the term occurs. Red, orange, yellow and green colors are used for terms that occur in the current document; red indicates high interlinkedness of a term with other terms, orange, yellow and green decreasing interlinkedness. Blue is used for terms that have a relation with the terms in this document, but occur in other documents.
You can navigate and zoom the map. Mouse-hovering a term displays its timeline, clicking it yields the associated documents.

Author Collaborations