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应对儿童肺血管疾病表型分析的挑战。

Addressing the challenges of phenotyping pediatric pulmonary vascular disease.

作者信息

Goss Kara N, Everett Allen D, Mourani Peter M, Baker Christopher D, Abman Steven H

机构信息

Division of Allergy, Pulmonary and Critical Care Medicine, Department of Medicine, University of Wisconsin School of Medicine and Public Health, Madison, WI, USA.

Pediatric Cardiology, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD, USA.

出版信息

Pulm Circ. 2017 Jan 1;7(1):7-19. doi: 10.1086/689750. eCollection 2017 Mar.

Abstract

Pediatric pulmonary vascular disease (PVD) and pulmonary hypertension (PH) represent phenotypically and pathophysiologically diverse disease categories, contributing substantial morbidity and mortality to a complex array of pediatric conditions. Here, we review the multifactorial nature of pediatric PVD, with an emphasis on improved recognition, phenotyping, and endotyping strategies for pediatric PH. Novel tailored approaches to diagnosis and treatment in pediatric PVD, as well as the implications for long-term outcomes, are highlighted.

摘要

小儿肺血管疾病(PVD)和肺动脉高压(PH)在表型和病理生理方面属于不同的疾病类别,在一系列复杂的儿科病症中导致了相当高的发病率和死亡率。在此,我们回顾小儿PVD的多因素性质,重点关注小儿PH的识别、表型分型及内型分型策略的改进。文中强调了小儿PVD诊断和治疗的新型定制方法以及对长期预后的影响。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e5e3/5448545/c03edf681ef7/10.1086_689750-fig1.jpg

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