Pulmonary vascular development in congenital diaphragmatic hernia.
Pulmonary vascular development in congenital diaphragmatic hernia.
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DOI：
10.1183/16000617.0104-2017
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发表时间：
2018-03-31
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影响因子：
--
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中科院分区：
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先天性腹股沟疝（CDH）是一种罕见的先天性异常，其特征是动脉缺损、持续性肺动脉高压（PH）和肺发育不全。这三种元素的相对贡献在个体患者中可能差异很大。大多数受影响的儿童主要患有相关的PH，其治疗方式有限，而且往往没有证据。与PH相关的血管缺陷，其特征是小动脉和毛细血管的肌化增加，在妊娠早期开始发展。肺血管的发育与气道上皮的发育是一体的。虽然我们的知识仍然是不完整的，在脉管系统的生长和扩张所涉及的过程开始被解开。很明显，这一过程的早期干扰导致主要的肺生长异常，导致严重的临床挑战，并在许多情况下导致新生儿死亡。在这里，我们概述了当前参与肺血管发育的分子途径。此外，我们还描述了与CDH相关的异常和这种严重异常的潜在治疗方法。先天性肺血管疾病起源于发育早期，导致临床挑战http://ow.ly/EFno30hBhbv
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. Congenital pulmonary vascular diseases originate early during development, leading to clinical challenges http://ow.ly/EFno30hBhbv
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影响因子：
21.1
作者：
Davenport AP;Hyndman KA;Dhaun N;Southan C;Kohan DE;Pollock JS;Pollock DM;Webb DJ;Maguire JJ
通讯作者：
Maguire JJ
DOI：
10.1152/ajplung.00050.2006
发表时间：
2007-02-01
影响因子：
4.9
作者：
Alejandre-Alcazar, Miguel A.;Kwapiszewska, Grazyna;Morty, Rory E.
通讯作者：
Morty, Rory E.
DOI：
10.1152/ajplung.00298.2004
发表时间：
2005-04-01
影响因子：
4.9
作者：
Chen, H;Sun, JP;Shi, W
通讯作者：
Shi, W
影响因子：
7.3
作者：
de Lagausie, P;de Buys-Roessingh, A;Berrebi, D
通讯作者：
Berrebi, D
影响因子：
2.1
作者：
Hillman NH;Kallapur SG;Jobe AH
通讯作者：
Jobe AH