Respiratory comorbidities and treatments in Duchenne muscular dystrophy : impact on life expectancy and causes of death
© 2024. The Author(s)..
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.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2024 |
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| Erschienen: |
2024 |
| Enthalten in: |
Zur Gesamtaufnahme - year:2024 |
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| Enthalten in: |
Journal of neurology - (2024) vom: 17. Apr. |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Wahlgren, Lisa [VerfasserIn] |
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| Links: |
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| Themen: |
Cause of death |
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| Anmerkungen: |
Date Revised 17.04.2024 published: Print-Electronic Citation Status Publisher |
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| doi: |
10.1007/s00415-024-12372-7 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM371194156 |
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| 100 | 1 | |a Wahlgren, Lisa |e verfasserin |4 aut | |
| 245 | 1 | 0 | |a Respiratory comorbidities and treatments in Duchenne muscular dystrophy |b impact on life expectancy and causes of death |
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| 500 | |a Date Revised 17.04.2024 | ||
| 500 | |a published: Print-Electronic | ||
| 500 | |a Citation Status Publisher | ||
| 520 | |a © 2024. The Author(s). | ||
| 520 | |a 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 | ||
| 520 | |a 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 | ||
| 520 | |a 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 | ||
| 520 | |a 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 | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Cause of death | |
| 650 | 4 | |a Duchenne muscular dystrophy | |
| 650 | 4 | |a Pulmonary | |
| 650 | 4 | |a Risk factors | |
| 650 | 4 | |a Survival | |
| 700 | 1 | |a Kroksmark, Anna-Karin |e verfasserin |4 aut | |
| 700 | 1 | |a Lindblad, Anders |e verfasserin |4 aut | |
| 700 | 1 | |a Tulinius, Mar |e verfasserin |4 aut | |
| 700 | 1 | |a Sofou, Kalliopi |e verfasserin |4 aut | |
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