先天性膈疝:不仅仅是肺部问题

E. D., Van der Veeken L, E. A, E. A, A. A., Deprest J
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引用次数: 0

摘要

先天性膈疝(CDH)是一种罕见的先天性异常,其特征是膈缺损,使腹部器官疝入胸腔。这导致肺发育不全,在出生时,患有CDH的儿童会出现呼吸窘迫和肺动脉高压。尽管有最佳的新生儿治疗,CDH仍然与高死亡率和发病率相关。在严重的情况下,胎儿干预(FETO)可能会改变这种疾病的自然进程。在此,我们描述了实施这种干预的基本原理、作用机制和技术。尽管有希望获得结果,但该技术仍处于左侧CDH的研究阶段。然而,生存率的提高可能是以发病率的增加为代价的。先天性CDH患儿长期和短期发病的风险增加,包括神经发育问题。到目前为止,神经系统疾病的严重程度和患病率仍然存在不确定性。此外,尚不确定这些问题是否在产前就已经存在,以及产前干预是否会影响这些问题。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Congenital Diaphragmatic Hernia: More than Just a Lung Problem
Congenital Diaphragmatic Hernia (CDH) is a rare congenital anomaly characterized by a defect in the diaphragm, which permits abdominal organs to herniate into the thorax. This causes lung hypoplasia and at birth, children with CDH experience respiratory distress and pulmonary hypertension. Despite optimal neonatal treatment, CDH is still associated with a high mortality and morbidity. In severe cases, Fetal Intervention (FETO) may alter the natural course of this disease. Herein we describe the rationale, action mechanism and technique to perform this intervention. Despite hope giving results, this technique remains investigational for left sided CDH. However, an increased survival may come at the cost of increased morbidity. Children born with CDH are at increased risk for long and short-term morbidity, including neurodevelopmental problems. Until now, there are still uncertainties about the severity and prevalence of neurologic morbidity. Furthermore, it remains uncertain if these problems are already present prenatally and if a prenatal intervention influence this.
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