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

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 media...

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Detalles Bibliográficos
Autores principales: Wang, Yuchen, Chen, Selena, Du, Junbao
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2019
Materias:
Pediatrics
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6663967/
https://www.ncbi.nlm.nih.gov/pubmed/31396496
http://dx.doi.org/10.3389/fped.2019.00302
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author Wang, Yuchen
Chen, Selena
Du, Junbao
author_facet Wang, Yuchen
Chen, Selena
Du, Junbao
author_sort Wang, Yuchen
collection PubMed
description 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.
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spelling pubmed-66639672019-08-08 Bosentan for Treatment of Pediatric Idiopathic Pulmonary Arterial Hypertension: State-of-the-Art Wang, Yuchen Chen, Selena Du, Junbao Front Pediatr Pediatrics 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. Frontiers Media S.A. 2019-07-23 /pmc/articles/PMC6663967/ /pubmed/31396496 http://dx.doi.org/10.3389/fped.2019.00302 Text en Copyright © 2019 Wang, Chen and Du. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Pediatrics
Wang, Yuchen
Chen, Selena
Du, Junbao
Bosentan for Treatment of Pediatric Idiopathic Pulmonary Arterial Hypertension: State-of-the-Art
title Bosentan for Treatment of Pediatric Idiopathic Pulmonary Arterial Hypertension: State-of-the-Art
title_full Bosentan for Treatment of Pediatric Idiopathic Pulmonary Arterial Hypertension: State-of-the-Art
title_fullStr Bosentan for Treatment of Pediatric Idiopathic Pulmonary Arterial Hypertension: State-of-the-Art
title_full_unstemmed Bosentan for Treatment of Pediatric Idiopathic Pulmonary Arterial Hypertension: State-of-the-Art
title_short Bosentan for Treatment of Pediatric Idiopathic Pulmonary Arterial Hypertension: State-of-the-Art
title_sort bosentan for treatment of pediatric idiopathic pulmonary arterial hypertension: state-of-the-art
topic Pediatrics
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6663967/
https://www.ncbi.nlm.nih.gov/pubmed/31396496
http://dx.doi.org/10.3389/fped.2019.00302
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AT chenselena bosentanfortreatmentofpediatricidiopathicpulmonaryarterialhypertensionstateoftheart
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