scholarly journals Congenital Diaphragmatic Hernia in Neonate: Experience in a Teaching Hospital

1970 ◽  
Vol 7 (1) ◽  
pp. 28-30 ◽  
Author(s):  
SE Khan ◽  
AKMZ Siddiq ◽  
M Nessa
Keyword(s):  
Pulmonary Hypertension ◽  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Birth Defect ◽  
Pulmonary Hypoplasia ◽  
Good Prognosis ◽  
Military Hospital ◽  
Paediatric Surgery ◽  
Stabilization Phase ◽  
Abdominal Route

Introduction: Congenital diaphragmatic hernia (CDH) is one of the major surgical causes of respiratory distress in neonates. Reported survival averages 60% but may be significantly lower. Pulmonary hypertension and pulmonary hypoplasia are recognised as two corner stones of the pathophysiology of CDH. Objective: Objective of the study was to evaluate the outcome of this birth defect in Bangladesh situation. Method: This retrospective study was carried out at the Department of Paediatric Surgery of Combined Military Hospital, Dhaka over a period of five years. During this period a total of 8 neonates of CDH were admitted in this hospital. All the data were collected from record sheet and were compiled. Result: Age of patients ranged from 1 day to 20 days. Out of 8 neonates 5 (62.50%) were male and 3 (37.50%) were female. All patients were diagnosed postnatally. All the 8 neonates had Bochdalek type of CDH. Seven patients (87.50%) had left sided hernia. Two patients (25%) died before operation in the stabilization phase while on ventilator and 6 (75%) were operated. Out of these 6 patients, 5 (left sided) were operated through abdominal route and 1 (right sided) was approached through thorax. Overall outcome was satisfactory in 5 neonates and one died. Conclusion: Early intervention can result good prognosis in CDH. Key words: Congenital diaphragmatic hernia; neonate; Bochdalek type DOI: http://dx.doi.org/10.3329/jafmc.v7i1.8623 JAFMC Bangladesh. Vol 7, No 1 (June) 2011; 28-30  

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

2019 ◽  
Vol 29 (04) ◽  
pp. 317-327
Author(s):  
Lina Antounians ◽  
Rebeca Lopes Figueira ◽  
Lourenço Sbragia ◽  
Augusto Zani
Keyword(s):  
Pulmonary Hypertension ◽  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Vascular Remodeling ◽  
Pulmonary Hypoplasia ◽  
Genetic Alterations ◽  
Diaphragmatic Defect ◽  
Morbidity Rate ◽  
Lung Growth ◽  
Mortality And Morbidity

AbstractCongenital 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 changes, genetic alterations, and surgical maneuvers, and we describe the studies that have translated experimental results to the bedside.

scholarly journals Amniotic fluid stem cell extracellular vesicles promote fetal lung branching and cell differentiation in experimental congenital diaphragmatic hernia

2022 ◽  
Author(s):  
Kasra Khalaj ◽  
Rebeca Lopes Figueira ◽  
Lina Antounians ◽  
Sree Gandhi ◽  
Matthew Wales ◽  
...  
Keyword(s):  
Amniotic Fluid ◽  
Congenital Diaphragmatic Hernia ◽  
Extracellular Vesicles ◽  
Diaphragmatic Hernia ◽  
Lung Development ◽  
Pulmonary Hypoplasia ◽  
Fetal Lung ◽  
Branching Morphogenesis ◽  
Neuroendocrine Cells ◽  
Beta Catenin

Pulmonary hypoplasia secondary to congenital diaphragmatic hernia (CDH) is characterized by impaired branching morphogenesis and differentiation. We have previously demonstrated that administration of extracellular vesicles derived from rat amniotic fluid stem cells (AFSC-EVs) rescues development of hypoplastic lungs at the pseudoglandular and alveolar stages in rodent models of CDH. Herein, we tested whether AFSC-EVs exert their regenerative effects at the canalicular and saccular stages, as these are translationally relevant for clinical intervention. To induce fetal pulmonary hypoplasia, we gavaged rat dams with nitrofen at embryonic day 9.5 and demonstrated that nitrofen-exposed lungs had impaired branching morphogenesis, dysregulated signaling pathways relevant to lung development (FGF10/FGFR2, ROBO/SLIT, Ephrin, Neuropilin 1, beta-catenin) and impaired epithelial and mesenchymal cell marker expression at both stages. AFSC-EVs administered to nitrofen-exposed lung explants rescued airspace density and increased the expression levels of key factors responsible for branching morphogenesis. Moreover, AFSC-EVs rescued the expression of alveolar type 1 and 2 cell markers at both canalicular and saccular stages, and restored markers of club, ciliated epithelial, and pulmonary neuroendocrine cells at the saccular stage. AFSC-EV treated lungs also had restored markers of lipofibroblasts and PDGFRA+ cells to control levels at both stages. EV tracking showed uptake of AFSC-EV RNA cargo throughout the fetal lung and an mRNA-miRNA network analysis identified that several miRNAs responsible for regulating lung development processes were contained in the AFSC-EV cargo. These findings suggest that AFSC-EV based therapies hold potential for restoring fetal lung growth and maturation in babies with pulmonary hypoplasia secondary to CDH.

scholarly journals Cellular Origin(s) of Congenital Diaphragmatic Hernia

2021 ◽  
Vol 9 ◽  
Author(s):  
Gabriëla G. Edel ◽  
Gerben Schaaf ◽  
Rene M. H. Wijnen ◽  
Dick Tibboel ◽  
Gabrielle Kardon ◽  
...  
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Birth Defect ◽  
Congenital Disorder ◽  
Diaphragmatic Defect ◽  
Lung Hypoplasia ◽  
Cellular Origin ◽  
Recent Developments ◽  
Mechanical Factors

Congenital diaphragmatic hernia (CDH) is a structural birth defect characterized by a diaphragmatic defect, lung hypoplasia and structural vascular defects. In spite of recent developments, the pathogenesis of CDH is still poorly understood. CDH is a complex congenital disorder with multifactorial etiology consisting of genetic, cellular and mechanical factors. This review explores the cellular origin of CDH pathogenesis in the diaphragm and lungs and describes recent developments in basic and translational CDH research.

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