Efficacy of cibenzoline for hypertrophic obstructive cardiomyopathy in paediatric patients with RAS/MAPK pathway syndromes.
Kota WatanabeYuji MaruoAtsuhito TakedaPublished in: Cardiology in the young (2022)
RASopathies - caused by mutations in the RAS/MAPK signaling pathway - are frequently associated with cardiac diseases, such as hypertrophic obstructive cardiomyopathy. Although cibenzoline is useful for adult hypertrophic obstructive cardiomyopathy patients, little is known about its effect in children. Here, we report two paediatric cases of hypertrophic obstructive cardiomyopathy associated with RASopathies where the condition was improved by cibenzoline.
Keyphrases
- signaling pathway
- heart failure
- pi k akt
- end stage renal disease
- intensive care unit
- emergency department
- ejection fraction
- oxidative stress
- newly diagnosed
- epithelial mesenchymal transition
- young adults
- peritoneal dialysis
- left ventricular
- prognostic factors
- induced apoptosis
- patient reported outcomes
- atrial fibrillation
- endoplasmic reticulum stress