Congenital Diaphragmatic Hernia: State-of-the-Art in Translating Experimental Research to the Bedside

Eur J Pediatr Surg DOI: 10.1055/s-0039-1693993Congenital diaphragmatic hernia (CDH) is a devastating disease that still carries a high mortality and morbidity rate. Poor outcomes for fetuses and infants with CDH are mainly related to pulmonary hypoplasia (PH) and pulmonary vascular remodeling that leads to pulmonary hypertension (PHTN). Over the last five decades, research efforts have focused on modeling CDH not only to study the pathophysiology of the diaphragmatic defect, pulmonary hypoplasia, and pulmonary hypertension, but also to identify therapies that would promote lung growth and maturation, and correct vascular remodeling. As CDH is a multifactorial condition whose etiology remains unknown, there is not a single model of CDH, rather several ones that replicate different aspects of this disease. While small animals like the mouse and the rat have mainly been used to uncover biological pathways underlying the diaphragmatic defect and poor lung growth, larger animals like the lamb and the rabbit models have been instrumental for pursuing medical and surgical interventions. Overall, the use of animal models has indeed advanced our knowledge on CDH and helped us test innovative therapeutic options. For example, the lamb model of CDH has been the paradigm for testing fetal surgical procedures, including tracheal occlusion, which has been translated to clinical use. In this review, we outline the induction protocols of CDH in animals with the use of chemicals, dietary chan...
Source: European Journal of Pediatric Surgery - Category: Surgery Authors: Tags: Review Article Source Type: research

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Conclusion A history of CDH is associated with higher rates of inhaled bronchodilator prescriptions, inhaled steroid prescriptions, and asthma-related physician visits from 0 to 5 years of age compared with age-matched controls. However, this difference resolves by 5 to 10 years of age. [...] Georg Thieme Verlag KG Stuttgart · New YorkArticle in Thieme eJournals: Table of contents  |  Abstract  |  Full text
Source: European Journal of Pediatric Surgery - Category: Surgery Authors: Tags: Original Article Source Type: research
Conclusions: Majority of the neonates with CDH at our center were >37 weeks and survival was 60%. The predictors of adverse outcome were low Apgar score, presence of moderate-to-severe PPHN, need for higher ventilatory settings, and shock. Antenatal detection of diaphragmatic hernia did not impact survival rates.
Source: Journal of Indian Association of Pediatric Surgeons - Category: Surgery Authors: Source Type: research
Conclusions: Our data indicate that miR-200b improves PH and decreases the incidence of CDH. Future studies will further exploit this newly discovered prenatal therapy for lung hypoplasia and CDH.
Source: Annals of Surgery - Category: Surgery Tags: ORIGINAL ARTICLES Source Type: research
Support on Extracorporeal oxygenation membrane (ECMO) represents the last therapeutic option in the management of respiratory failure and pulmonary hypertension refractory to treatment in patients with congenital diaphragmatic hernia (CDH).
Source: Journal of Pediatric Surgery - Category: Surgery Authors: Source Type: research
AbstractPurpose of reviewThis review aims to describe the assessment of pulmonary hypertension and ventricular function in neonates with congenital diaphragmatic hernia and the long-term follow-up of their pulmonary vascular disease.Recent findingsIn 2015, the pediatric pulmonary hypertension guidelines from the American Heart Association and American Thoracic Society suggested class I level of evidence B guidelines for routine evaluation of patients with congenital diaphragmatic hernia, including longitudinal care in an interdisciplinary pulmonary hypertension program and following the recommendations offered for all chil...
Source: Current Treatment Options in Cardiovascular Medicine - Category: Cardiology Source Type: research
AbstractBackgroundThe presence of a hernia sac in congenital diaphragmatic hernia (CDH) has been reported to be associated with higher lung volumes and better postnatal outcomes.ObjectiveTo compare prenatal imaging (ultrasound and MRI) prognostic measurements and postnatal outcomes of CDH with and without hernia sac.Materials and methodsWe performed database searches from January 2008 to March 2017 for surgically proven cases of CDH with and without hernia sac. All children had a detailed ultrasound (US) examination and most had an MRI examination. We reviewed the medical records of children enrolled in our Pulmonary Hypop...
Source: Pediatric Radiology - Category: Radiology Source Type: research
CONCLUSIONS: ECMO is an effective rescue means for refractory PPHN. Appropriate intervention timing, accurate coagulation, and volume management are important. PMID: 30607885 [PubMed - as supplied by publisher]
Source: World Journal of Pediatrics : WJP - Category: Pediatrics Authors: Tags: World J Pediatr Source Type: research
European J Pediatr Surg Rep 2018; 06: e100-e103 DOI: 10.1055/s-0038-1675377Lung hypoplasia and pulmonary hypertension (PH) in association with congenital diaphragmatic hernia (CDH) may cause fatal respiratory failure. Lung transplantation (Ltx) may represent an option for CDH-related end-stage pulmonary failure. The aim of this study is to report a patient with CDH who underwent Ltx or combined heart-lung transplantation (H-Ltx). Our patient was born at 33 weeks of gestation, with a prenatally diagnosed isolated left CDH. Twenty-four hours after birth, she underwent surgical repair of a type D defect (according to the CDH ...
Source: European Journal of Pediatric Surgery Reports - Category: Surgery Authors: Tags: Case Report Source Type: research
Conclusion This study demonstrates for the first time decreased NOSIP expression in the pulmonary vasculature of the nitrofen-induced CDH. These findings suggest that NOSIP underexpression may interfere with NO production, contributing to abnormal vascular remodeling and PPH. [...] Georg Thieme Verlag KG Stuttgart · New YorkArticle in Thieme eJournals: Table of contents  |  Abstract  |  Full text
Source: European Journal of Pediatric Surgery - Category: Surgery Authors: Tags: Original Article Source Type: research
Prostaglandin E1 (PGE) has been used to maintain ductus arteriosus patency and unload the suprasystemic right ventricle (RV) in neonates with congenital diaphragmatic hernia (CDH) and severe pulmonary hypertension (PH). Here we evaluate the PH response in neonates with CDH and severe PH treated with PGE.
Source: Journal of Pediatric Surgery - Category: Surgery Authors: Source Type: research
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