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Pulmonary vascular development in congenital diaphragmatic hernia
- Source :
- European Respiratory Review, 27(147):Unsp 170104. European Respiratory Society, European Respiratory Review, Vol 27, Iss 147 (2018)
- Publication Year :
- 2018
-
Abstract
- 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.
- Subjects :
- 0301 basic medicine
Pulmonary and Respiratory Medicine
Pulmonary Circulation
medicine.medical_specialty
Hypertension, Pulmonary
Neovascularization, Physiologic
Diaphragmatic breathing
Hemodynamics
Diaphragmatic defect
03 medical and health sciences
0302 clinical medicine
Risk Factors
030225 pediatrics
Internal medicine
Animals
Humans
Medicine
Lung hypoplasia
Lung
lcsh:RC705-779
business.industry
Congenital diaphragmatic hernia
lcsh:Diseases of the respiratory system
Prognosis
medicine.disease
Capillaries
Arterioles
030104 developmental biology
Cardiology
Respiratory epithelium
Gestation
Abnormality
Hernias, Diaphragmatic, Congenital
business
Signal Transduction
Subjects
Details
- ISSN :
- 09059180
- Database :
- OpenAIRE
- Journal :
- European Respiratory Review, 27(147):Unsp 170104. European Respiratory Society, European Respiratory Review, Vol 27, Iss 147 (2018)
- Accession number :
- edsair.doi.dedup.....ccbc188fb1db5042fd111d95df2fffad