Article ; Online: Respiratory comorbidities and treatments in Duchenne muscular dystrophy: impact on life expectancy and causes of death.
2024
Abstract: Background: Duchenne muscular dystrophy (DMD) is a neuromuscular disorder with progressive decline of pulmonary function increasing the risk of early mortality. The aim of this study was to explore the respiratory-related comorbidities, and the effect ... ...
Abstract | Background: Duchenne muscular dystrophy (DMD) is a neuromuscular disorder with progressive decline of pulmonary function increasing the risk of early mortality. The aim of this study was to explore the respiratory-related comorbidities, and the effect of these comorbidities and treatments on life expectancy and causes of death. Methods: All male patients living in Sweden with DMD, born and deceased 1970-2019, were included. Data regarding causes of death were collected from the Cause of Death Registry and cross-checked with the medical records along with diagnostics and relevant clinical features. Results: Hundred and twenty nine patients were included with a median lifespan of 24.3 years. Acute respiratory failure accounted for 63.3% of respiratory-related causes of death. 70.1% suffered at least one pneumonia, with first episode at a median age of 17.8 years. Hypoventilation was found in 73.0% with onset at 18.1 years. 60.5% had their first pneumonia before established hypoventilation. Age at onset of hypoventilation showed a strong correlation with age at first pneumonia. First pneumonia and scoliosis non-treated with scoliosis surgery increased the risk of dying of respiratory-related causes. In 10% of the patients, first pneumonia resulted in acute tracheostomy or early death. Patients treated with assisted ventilation had higher life expectancy compared to untreated patients. Conclusions: Our results highlight the importance of identifying subclinical hypoventilation in a timely manner and the importance of an active treatment regime upon clinical signs of pneumonia. |
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Language | English |
Publishing date | 2024-04-17 |
Publishing country | Germany |
Document type | Journal Article |
ZDB-ID | 187050-6 |
ISSN | 1432-1459 ; 0340-5354 ; 0012-1037 ; 0939-1517 ; 1619-800X |
ISSN (online) | 1432-1459 |
ISSN | 0340-5354 ; 0012-1037 ; 0939-1517 ; 1619-800X |
DOI | 10.1007/s00415-024-12372-7 |
Database | MEDical Literature Analysis and Retrieval System OnLINE |
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