Publications by authors named "K Wahbi"
Background: Although genetic variants in are the most frequent cause of pediatric genetic dilated cardiomyopathy (DCM), there are no studies available describing this entity. We sought to describe clinical features, analyze variant location, and explore predictors of bad prognosis in pediatric -related DCM.
Methods And Results: We evaluated clinical records from 44 patients (24 men; median age at diagnosis, 0.
Mexiletine (NaMuscla™) is indicated for the symptomatic treatment of myotonia in adults with non-dystrophic myotonia. A cardiac assessment is required as mexiletine may have a pro-arrhythmic effect. Long-term safety data supporting the use of mexiletine in patients with non-dystrophic myotonia combined with the extensive clinical experience of an expert group resulted in creation of an algorithm for cardiac monitoring of patients treated with mexiletine.
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- Duchenne muscular dystrophy (DMD) leads to severe muscle degeneration and increased risk of heart problems; the review aimed to identify factors predicting cardiac disease in DMD patients.
- The systematic review examined literature from 2000 to 2022, analyzing 33 studies involving over 9,200 DMD patients and assessing the strength of the evidence using the GRADE framework.
- Key findings indicated that certain cardiac medications and DMD gene mutations are linked to better heart function, while glucocorticoids improve left ventricular performance, with varying evidence quality regarding their effectiveness.
Background: Although the optimal approach is debated, systematic genetic screening for hypertrophic cardiomyopathy (HCM) is recommended.
Aims: The performance of this approach was tested in GEREMY, a HCM prospective observational French register.
Methods: Screening was based on a 12-gene panel, including the Fabry disease (GLA) and the transthyretin (TTR) genes.