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Efficacy of cibenzoline for hypertrophic obstructive cardiomyopathy in paediatric patients with RAS/MAPK pathway syndromes

Published online by Cambridge University Press:  12 October 2022

Kota Watanabe Open the ORCID record for Kota Watanabe [Opens in a new window] ,
Yuji Maruo  and
Atsuhito Takeda Open the ORCID record for Atsuhito Takeda [Opens in a new window]
Kota Watanabe
Affiliation:
Pediatrics, Kitami Red Cross Hospital, North 6-East 2, Kitami, Hokkaido, Japan
Yuji Maruo
Affiliation:
Pediatrics, Kitami Red Cross Hospital, North 6-East 2, Kitami, Hokkaido, Japan
Atsuhito Takeda*
Affiliation:
Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Japan
*
Author for correspondence: Atsuhito Takeda, Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Japan. E-mail: a-takeda@med.hokudai.ac.jp
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Abstract

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.

Keywords

Hypertrophic obstructive cardiomyopathyRASopathiescibenzoline
Type
Brief Report
Information
Cardiology in the Young , Volume 33 , Issue 6 , June 2023 , pp. 1007 - 1009
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Copyright
© The Author(s), 2022. Published by Cambridge University Press

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