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实施精准儿童心血管医学的途径。

A Path to Implement Precision Child Health Cardiovascular Medicine.

作者信息

Touma Marlin, Reemtsen Brian, Halnon Nancy, Alejos Juan, Finn J Paul, Nelson Stanley F, Wang Yibin

机构信息

Department of Pediatrics, Children's Discovery and Innovation Institute, University of California at Los Angeles, Los Angeles, CA, United States.

Cardiovascular Research Laboratory, University of California at Los Angeles, Los Angeles, CA, United States.

出版信息

Front Cardiovasc Med. 2017 Jun 1;4:36. doi: 10.3389/fcvm.2017.00036. eCollection 2017.

Abstract

Congenital heart defects (CHDs) affect approximately 1% of live births and are a major source of childhood morbidity and mortality even in countries with advanced healthcare systems. Along with phenotypic heterogeneity, the underlying etiology of CHDs is multifactorial, involving genetic, epigenetic, and/or environmental contributors. Clear dissection of the underlying mechanism is a powerful step to establish individualized therapies. However, the majority of CHDs are yet to be clearly diagnosed for the underlying genetic and environmental factors, and even less with effective therapies. Although the survival rate for CHDs is steadily improving, there is still a significant unmet need for refining diagnostic precision and establishing targeted therapies to optimize life quality and to minimize future complications. In particular, proper identification of disease associated genetic variants in humans has been challenging, and this greatly impedes our ability to delineate gene-environment interactions that contribute to the pathogenesis of CHDs. Implementing a systematic multileveled approach can establish a continuum from phenotypic characterization in the clinic to molecular dissection using combined next-generation sequencing platforms and validation studies in suitable models at the bench. Key elements necessary to advance the field are: first, proper delineation of the phenotypic spectrum of CHDs; second, defining the molecular genotype/phenotype by combining whole-exome sequencing and transcriptome analysis; third, integration of phenotypic, genotypic, and molecular datasets to identify molecular network contributing to CHDs; fourth, generation of relevant disease models and multileveled experimental investigations. In order to achieve all these goals, access to high-quality biological specimens from well-defined patient cohorts is a crucial step. Therefore, establishing a CHD BioCore is an essential infrastructure and a critical step on the path toward precision child health cardiovascular medicine.

摘要

先天性心脏病(CHD)影响约1%的活产婴儿,即使在医疗保健系统先进的国家,也是儿童发病和死亡的主要原因。除了表型异质性外,CHD的潜在病因是多因素的,涉及遗传、表观遗传和/或环境因素。清晰剖析潜在机制是建立个性化治疗的有力步骤。然而,大多数CHD尚未明确诊断出潜在的遗传和环境因素,更缺乏有效的治疗方法。尽管CHD的存活率在稳步提高,但在提高诊断精度和建立靶向治疗以优化生活质量并尽量减少未来并发症方面,仍有重大需求未得到满足。特别是,在人类中正确识别与疾病相关的基因变异一直具有挑战性,这极大地阻碍了我们描绘导致CHD发病机制的基因-环境相互作用的能力。实施系统的多层次方法可以建立一个连续体,从临床的表型特征到使用组合的下一代测序平台进行分子剖析,再到在合适的实验台上模型进行验证研究。推动该领域发展所需的关键要素包括:第一,正确描绘CHD的表型谱;第二,通过结合全外显子组测序和转录组分析来定义分子基因型/表型;第三,整合表型、基因型和分子数据集以识别导致CHD的分子网络;第四,生成相关疾病模型并进行多层次实验研究。为了实现所有这些目标,从明确界定的患者队列中获取高质量生物标本是关键一步。因此,建立一个CHD生物样本库是精准儿童健康心血管医学道路上必不可少的基础设施和关键步骤。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a9a3/5451507/f928acf3642b/fcvm-04-00036-g001.jpg

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