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儿科限制型心肌病-诊断与挑战:两例报告。

Paediatric Restrictive Cardiomyopathy - Diagnosis and Challenges: A report of two cases.

机构信息

Sultan Qaboos University, Muscat, Oman.

Child Health Department, Sultan Qaboos University Hospital.

出版信息

Sultan Qaboos Univ Med J. 2024 May;24(2):283-287. doi: 10.18295/squmj.9.2023.055. Epub 2024 May 27.

Abstract

Restrictive cardiomyopathy is one of the rarest forms of cardiomyopathies in paediatric patients characterised by impaired myocardial relaxation or compliance with restricted ventricular filling, leading to a reduced diastolic volume with a preserved systolic function. We report 2 cases-a 5-year-old boy who presented with abdominal distension and palpitation with family history of similar complaints but no definite genetic diagnosis as yet and a 5-year-old girl who presented with chronic cough and shortness of breath. Both cases were diagnosed in a tertiary care hospital in Muscat, Oman, in 2019 and are managed supportively with regular outpatient follow-up. This is the first series of reported cases of paediatric restrictive cardiomyopathy from Oman.

摘要

限制型心肌病是儿科患者中最罕见的心肌病形式之一,其特征为心肌松弛或顺应性受损,心室充盈受限,导致舒张期容积减少而收缩功能保持。我们报告了 2 例病例,一例为 5 岁男孩,表现为腹胀和心悸,有类似症状的家族史,但尚未明确遗传诊断,另一例为 5 岁女孩,表现为慢性咳嗽和呼吸急促。这两例病例均于 2019 年在阿曼的一家三级医疗机构确诊,目前采用支持性治疗并定期进行门诊随访。这是来自阿曼的首例儿科限制型心肌病系列报告。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/17b0/11139361/3b27cc354e1f/squmj2405-283-287f1.jpg

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