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Cardiomyopathy and changes of skeletal muscles in cystic fibrosis
V Herout1, D Benesová, V Vávrová
1Faculty of Medicine, Charles University, Hradec Králové, Czechoslovakia.
Insights
Cardiomyopathy (CMP) is a serious concern in children with cystic fibrosis (CF). Early suspicion of CMP in CF patients with heart failure is crucial for timely diagnosis and intervention.
Area of Science:
- Pediatrics
- Cardiology
- Genetics
Background:
- Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs.
- Cardiomyopathy (CMP) is a known but under-recognized complication in CF.
- The interplay between CF and cardiac muscle dysfunction requires further investigation.
Purpose of the Study:
- To investigate the incidence and outcomes of cardiomyopathy in pediatric cystic fibrosis patients.
- To highlight the association between cardiomyopathy and sudden death in CF.
- To report the concurrent presentation of skeletal and cardiac muscle abnormalities in CF.
Main Methods:
- Retrospective analysis of pediatric patients diagnosed with cystic fibrosis.
- Review of clinical records for diagnosis of cardiomyopathy and heart failure.
- Correlation of cardiomyopathy with mortality and age at death.
Main Results:
- Cardiomyopathy was identified in 26 children with cystic fibrosis.
- A high mortality rate (92%) was observed in these patients, with most deaths occurring before age 3.
- The study identified a novel combination of skeletal and cardiac muscle changes in CF.
Conclusions:
- Cardiomyopathy should be suspected in young children with CF presenting with heart failure.
- Cardiomyopathy is a significant cause of mortality in pediatric CF, particularly in early childhood.
- This study is the first to report combined skeletal and cardiac muscle pathology in CF.
Abstract:
Cardiomyopathy (CMP) was found in 26 children with cystic fibrosis (CF), 24 of them died, the majority of them during the first 3 years of life. Only 4 of them were older than 10 years. 2 children are living. CMP must be suspected in young children with CF and early heart failure. When CMP is the cause of sudden death, CF has to be suspected. The combination of changes in skeletal and cardiac muscles in CF is reported here for the first time.