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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.

DOI:10.18295/squmj.9.2023.055
PMID:38828243
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11139361/
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
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/17b0/11139361/3b27cc354e1f/squmj2405-283-287f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/17b0/11139361/3b27cc354e1f/squmj2405-283-287f1.jpg

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本文引用的文献

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Case report: Restrictive cardiomyopathy presenting with complete thromboembolism occlusion of the terminal part of the abdominal aorta in a preadolescent Saudi girl.病例报告:一名青春期前沙特女孩患限制性心肌病,出现腹主动脉末端完全血栓栓塞性闭塞。
Front Pediatr. 2022 Jul 19;10:944627. doi: 10.3389/fped.2022.944627. eCollection 2022.
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Overview of Restrictive Cardiomyopathies.限制型心肌病概述。
Methodist Debakey Cardiovasc J. 2022 Mar 14;18(2):4-16. doi: 10.14797/mdcvj.1078. eCollection 2022.
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Pediatric Restrictive Cardiomyopathies.
小儿限制性心肌病
Front Pediatr. 2022 Jan 25;9:745365. doi: 10.3389/fped.2021.745365. eCollection 2021.
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Restrictive cardiomyopathy: Importance of early diagnosis.限制性心肌病:早期诊断的重要性。
An Pediatr (Engl Ed). 2021 Nov;95(5):368-370. doi: 10.1016/j.anpede.2020.11.007. Epub 2021 Oct 26.
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Cardiomyopathy in Children: Classification and Diagnosis: A Scientific Statement From the American Heart Association.儿童心肌病:分类与诊断——美国心脏协会科学声明
Circulation. 2019 Jul 2;140(1):e9-e68. doi: 10.1161/CIR.0000000000000682. Epub 2019 May 28.
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Contemporary Outcomes of Pediatric Restrictive Cardiomyopathy: A Single-Center Experience.儿童限制性心肌病的当代结局:单中心经验
Pediatr Cardiol. 2019 Apr;40(4):694-704. doi: 10.1007/s00246-018-2043-0. Epub 2018 Dec 12.
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Restrictive Cardiomyopathies: The Importance of Noninvasive Cardiac Imaging Modalities in Diagnosis and Treatment-A Systematic Review.限制性心肌病:非侵入性心脏成像模态在诊断和治疗中的重要性——一项系统评价
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Pediatric Cardiomyopathies.小儿心肌病
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Differentiation of Constriction and Restriction: Complex Cardiovascular Hemodynamics.收缩与限制的区分:复杂心血管血液动力学。
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