Bosentan for Treatment of Pediatric Idiopathic Pulmonary Arterial Hypertension: State-of-the-Art

被引：7

作者：

Wang, Yuchen ^{[1
,2
]}

Chen, Selena ^{[3
]}

Du, Junbao ^{[1
,4
]}

机构：

[1] Peking Univ, Hosp 1, Dept Pediat, Beijing, Peoples R China

[2] Peking Univ, Hlth Sci Ctr, Dept Clin Med, Beijing, Peoples R China

[3] Univ Calif San Diego, Div Biol Sci, La Jolla, CA 92093 USA

[4] Minist Educ, Key Lab Mol Cardiovasc Sci, Beijing, Peoples R China

来源：

FRONTIERS IN PEDIATRICS | 2019年 / 7卷

基金：

北京市自然科学基金;

关键词：

idiopathic pulmonary hypertension; target therapy; bosentan; pediatrics; pharmacology; CHILDREN; THERAPY; SURVIVAL; SILDENAFIL; SAFETY; PHARMACOKINETICS; EPOPROSTENOL; FORMULATION; EXPRESSION; EXPERIENCE;

D O I：

10.3389/fped.2019.00302

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

Idiopathic pulmonary arterial hypertension (IPAH) is a complex disease associated with progressive deterioration. Targeted therapy for IPAH has improved in the last several decades. However, there remain many challenges to current treatment of children with IPAH, including poor prognosis and a median survival of 0.8 years. Endothelin-1 (ET-1) appears to be a key mediator in the pathogenesis of IPAH, with elevated concentrations in the plasma. Bosentan, an endothelin receptor antagonist, has been confirmed in Food and Drug Administration (FDA) to effectively treat IPAH when administered in recent studies. This review focuses on related studies and advance of bosentan in the treatment of IPAH in children.

引用

页数：10

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