Fibrillin-1 Expression Is Decreased in the Diaphragmatic Muscle Connective Tissue of Nitrofen-Induced Congenital Diaphragmatic Hernia

Eur J Pediatr Surg DOI: 10.1055/s-0036-1587586 Introduction Diaphragmatic morphogenesis depends on proper formation of muscle connective tissue (MCT) and underlying extracellular matrix (ECM). Fibrillin-1 is an essential ECM protein and crucial for the structural integrity of MCT in the developing diaphragm. Recently, mutations in the fibrillin-1 gene (FBN1) have been identified in cases of congenital diaphragmatic hernia (CDH), thus suggesting that alterations in FBN1 gene expression may lead to diaphragmatic defects. We designed this study to investigate the hypothesis that the diaphragmatic expression of fibrillin-1 is decreased in the MCT of nitrofen-induced CDH. Materials and Methods Time-mated rats were exposed to nitrofen or vehicle on gestational day 9 (D9). Fetal diaphragms (n = 72) were harvested on D13, D15, and D18, and divided into control and nitrofen-exposed specimens. Laser-capture microdissection was used to obtain diaphragmatic tissue cells. Gene expression levels of FBN1 were analyzed by qRT-PCR. Immunofluorescence-double-staining for fibrillin-1 and the mesenchymal marker Gata4 was performed to evaluate protein expression and localization. Results Relative mRNA expression of FBN1 was significantly decreased in pleuroperitoneal folds on D13 (3.39 ± 1.29 vs. 5.47 ± 1.92; p 
Source: European Journal of Pediatric Surgery - Category: Surgery Authors: Tags: Original Article Source Type: research

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Conclusions: we propose the description of large sample of pediatric patients with congenital bronchopulmonary malformations, followed from one Center. Chest CT and LTBS are important investigation in congenital airway anomalies.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Paediatric bronchology Source Type: research
ConclusionWe successfully adapted BaseScope ™ to detect circRNAs in rat biofluids: serum and amniotic fluid. We detected signal from probes targeted to circRNAs that are dysregulated in rat CDH. This work establishes the preliminary feasibility of circRNA detection in prenatal diagnostics.
Source: Pediatric Surgery International - Category: Surgery 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
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Source: European Journal of Pediatric Surgery - Category: Surgery Authors: Tags: Original Article Source Type: research
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Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Paediatric bronchology Source Type: research
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Source: Annals of the Royal College of Surgeons of England - Category: Surgery Authors: Tags: Ann R Coll Surg Engl Source Type: research
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Source: European Journal of Pediatric Surgery Reports - Category: Surgery Authors: Tags: Case Report Source Type: research
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Source: Pediatric Surgery International - Category: Surgery Source Type: research
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Source: European Journal of Pediatric Surgery - Category: Surgery Authors: Tags: Original Article Source Type: research
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Source: Surgical Endoscopy - Category: Surgery Source Type: research
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