儿童肌肉萎缩症的临床诊断和遗传分析。

IF 1 4区 医学 Q3 PEDIATRICS
Clinical Pediatrics Pub Date : 2025-05-01 Epub Date: 2024-08-28 DOI:10.1177/00099228241272029
Yanshu Xie, Jingsi Luo, Jingzi Zhong, Dan Lan
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引用次数: 0

摘要

90%以上的疑似肌营养不良症(MD)患者可通过多重结扎依赖性探针扩增(MLPA)结合靶向面板进行确诊,但也有少数患者无法确诊。这项研究共纳入了 312 名疑似肌营养不良症患者。研究人员进行了 MLPA 联合靶向肌病检测。结果为阴性的患者将接受全外显子组测序(WES)、全基因组测序(WGS)和/或 RNA 测序(RNA-seq)。共有275例被诊断为杜氏/贝克尔肌营养不良症(DMD/BMD),20例为其他类型的肌病或非肌肉疾病。6例女性DMD/BMD患者患有不同程度的典型DMD样症状,另有2例疑似性腺嵌合症。WES、WGS和/或RNA-seq的系统应用凸显了检测当前标准诊断程序所遗漏的变异的必要性。女性患者和嵌合携带者的鉴定对于预测复发风险和提供最佳遗传咨询至关重要。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Clinical Diagnosis and Genetic Analysis of Children With Muscular Dystrophies.

More than 90% suspected muscular dystrophy (MD) can be confirmed with multiplex ligation-dependent probe amplification (MLPA) combined with targeted panel, although there are a few that cannot be identified. A total of 312 suspected MD patients were enrolled into the study. The MLPA combined with a targeted myopathy panel were performed. Patients with negative results were subjected to whole exome sequencing (WES), whole genome sequencing (WGS), and/or RNA sequencing (RNA-seq). A total of 275 cases were diagnosed as Duchenne/Becker muscular dystrophy (DMD/BMD) and 20 cases were other types of myopathy or nonmuscular diseases. Six female DMD/BMD patients suffered from varying degrees of typical DMD-like symptoms and 2 others were suspected to be gonadal mosaicism. The systematic application of WES, WGS, and/or RNA-seq highlighted the need for the detection of variants missed by the current standard diagnostic procedures. The identification of female patients and mosaic carriers was crucial to predict the risk of recurrence and allow for optimal genetic counseling.

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来源期刊
Clinical Pediatrics
Clinical Pediatrics 医学-小儿科
CiteScore
2.10
自引率
6.20%
发文量
189
审稿时长
3-8 weeks
期刊介绍: Clinical Pediatrics (CLP) a peer-reviewed monthly journal, is a must read for the busy pediatrician. CLP contains state-of-the-art, accurate, concise and down-to earth information on practical, everyday child care topics whether they are clinical, scientific, behavioral, educational, or ethical.
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