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Pulmonary vascular development in congenital diaphragmatic hernia
Congenital diaphragmatic hernia (CDH) is a rare congenital anomaly characterised by a diaphragmatic defect, persistent pulmonary hypertension (PH) and lung hypoplasia. The relative contribution of these three elements can vary considerably in individual patients. Most affected children suffer primar...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
European Respiratory Society
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9488749/ https://www.ncbi.nlm.nih.gov/pubmed/29367409 http://dx.doi.org/10.1183/16000617.0104-2017 |
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author | Mous, Daphne S. Kool, Heleen M. Wijnen, Rene Tibboel, Dick Rottier, Robbert J. |
author_facet | Mous, Daphne S. Kool, Heleen M. Wijnen, Rene Tibboel, Dick Rottier, Robbert J. |
author_sort | Mous, Daphne S. |
collection | PubMed |
description | Congenital diaphragmatic hernia (CDH) is a rare congenital anomaly characterised by a diaphragmatic defect, persistent pulmonary hypertension (PH) and lung hypoplasia. The relative contribution of these three elements can vary considerably in individual patients. Most affected children suffer primarily from the associated PH, for which the therapeutic modalities are limited and frequently not evidence based. The vascular defects associated with PH, which is characterised by increased muscularisation of arterioles and capillaries, start to develop early in gestation. Pulmonary vascular development is integrated with the development of the airway epithelium. Although our knowledge is still incomplete, the processes involved in the growth and expansion of the vasculature are beginning to be unravelled. It is clear that early disturbances of this process lead to major pulmonary growth abnormalities, resulting in serious clinical challenges and in many cases death in the newborn. Here we provide an overview of the current molecular pathways involved in pulmonary vascular development. Moreover, we describe the abnormalities associated with CDH and the potential therapeutic approaches for this severe abnormality. |
format | Online Article Text |
id | pubmed-9488749 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | European Respiratory Society |
record_format | MEDLINE/PubMed |
spelling | pubmed-94887492022-11-14 Pulmonary vascular development in congenital diaphragmatic hernia Mous, Daphne S. Kool, Heleen M. Wijnen, Rene Tibboel, Dick Rottier, Robbert J. Eur Respir Rev Mini-review: Paediatric Year in Review Congenital diaphragmatic hernia (CDH) is a rare congenital anomaly characterised by a diaphragmatic defect, persistent pulmonary hypertension (PH) and lung hypoplasia. The relative contribution of these three elements can vary considerably in individual patients. Most affected children suffer primarily from the associated PH, for which the therapeutic modalities are limited and frequently not evidence based. The vascular defects associated with PH, which is characterised by increased muscularisation of arterioles and capillaries, start to develop early in gestation. Pulmonary vascular development is integrated with the development of the airway epithelium. Although our knowledge is still incomplete, the processes involved in the growth and expansion of the vasculature are beginning to be unravelled. It is clear that early disturbances of this process lead to major pulmonary growth abnormalities, resulting in serious clinical challenges and in many cases death in the newborn. Here we provide an overview of the current molecular pathways involved in pulmonary vascular development. Moreover, we describe the abnormalities associated with CDH and the potential therapeutic approaches for this severe abnormality. European Respiratory Society 2018-01-24 /pmc/articles/PMC9488749/ /pubmed/29367409 http://dx.doi.org/10.1183/16000617.0104-2017 Text en Copyright ©ERS 2018. https://creativecommons.org/licenses/by-nc/4.0/ERR articles are open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0. |
spellingShingle | Mini-review: Paediatric Year in Review Mous, Daphne S. Kool, Heleen M. Wijnen, Rene Tibboel, Dick Rottier, Robbert J. Pulmonary vascular development in congenital diaphragmatic hernia |
title | Pulmonary vascular development in congenital diaphragmatic hernia |
title_full | Pulmonary vascular development in congenital diaphragmatic hernia |
title_fullStr | Pulmonary vascular development in congenital diaphragmatic hernia |
title_full_unstemmed | Pulmonary vascular development in congenital diaphragmatic hernia |
title_short | Pulmonary vascular development in congenital diaphragmatic hernia |
title_sort | pulmonary vascular development in congenital diaphragmatic hernia |
topic | Mini-review: Paediatric Year in Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9488749/ https://www.ncbi.nlm.nih.gov/pubmed/29367409 http://dx.doi.org/10.1183/16000617.0104-2017 |
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