Lung tissue mechanics predict lung hypoplasia in a rabbit model for congenital diaphragmatic hernia

2007 ◽  
Vol 42 (6) ◽  
pp. 505-512 ◽  
Author(s):  
Andreas W. Flemmer ◽  
Jacques C. Jani ◽  
Florian Bergmann ◽  
Oliver J. Muensterer ◽  
Denis Gallot ◽  
...  
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Lung Tissue ◽  
Diaphragmatic Hernia ◽  
Rabbit Model ◽  
Tissue Mechanics ◽  
Lung Hypoplasia

scholarly journals Increased TGF-β: a drawback of tracheal occlusion in human and experimental congenital diaphragmatic hernia?

2016 ◽  
Vol 310 (4) ◽  
pp. L311-L327 ◽  
Author(s):  
Aline Vuckovic ◽  
Susanne Herber-Jonat ◽  
Andreas W. Flemmer ◽  
Ina M. Ruehl ◽  
Carmela Votino ◽  
...  
Keyword(s):  
Extracellular Matrix ◽  
Congenital Diaphragmatic Hernia ◽  
Lung Tissue ◽  
Diaphragmatic Hernia ◽  
Tissue Mechanics ◽  
Sham Operation ◽  
Lung Growth ◽  
Tracheal Occlusion ◽  
Matrix Deposition ◽  
Extracellular Matrix Components

Survivors of severe congenital diaphragmatic hernia (CDH) present significant respiratory morbidity despite lung growth induced by fetal tracheal occlusion (TO). We hypothesized that the underlying mechanisms would involve changes in lung extracellular matrix and dysregulated transforming growth factor (TGF)-β pathway, a key player in lung development and repair. Pulmonary expression of TGF-β signaling components, downstream effectors, and extracellular matrix targets were evaluated in CDH neonates who died between birth and the first few weeks of life after prenatal conservative management or TO, and in rabbit pups that were prenatally randomized for surgical CDH and TO vs. sham operation. Before tissue harvesting, lung tissue mechanics in rabbits was measured using the constant-phase model during the first 30 min of life. Human CDH and control fetal lungs were also collected from midterm onwards. Human and experimental CDH did not affect TGF-β/Smad2/3 expression and activity. In human and rabbit CDH lungs, TO upregulated TGF-β transcripts. Analysis of downstream pathways indicated increased Rho-associated kinases to the detriment of Smad2/3 activation. After TO, subtle accumulation of collagen and α-smooth muscle actin within alveolar walls was detected in rabbit pups and human CDH lungs with short-term mechanical ventilation. Despite TO-induced lung growth, mediocre lung tissue mechanics in the rabbit model was associated with increased transcription of extracellular matrix components. These results suggest that prenatal TO increases TGF-β/Rho kinase pathway, myofibroblast differentiation, and matrix deposition in neonatal rabbit and human CDH lungs. Whether this might influence postnatal development of sustainably ventilated lungs remains to be determined.

Lung Tissue Blood Perfusion Changes Induced by in utero Tracheal Occlusion in a Rabbit Model of Congenital Diaphragmatic Hernia

2009 ◽  
Vol 26 (3) ◽  
pp. 137-142 ◽  
Author(s):  
Rogelio Cruz-Martinez ◽  
Oscar Moreno-Alvarez ◽  
Jordi Prat ◽  
Lucas Krauel ◽  
Xavier Tarrado ◽  
...  
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Lung Tissue ◽  
Diaphragmatic Hernia ◽  
Rabbit Model ◽  
Blood Perfusion ◽  
In Utero ◽  
Tracheal Occlusion

Possibility of application of prenatal ultrasound indexes in congenital diaphragmatic hernia in fetus to determine postnatal outcomes

2021 ◽  
Author(s):  
N.V. Mashinets
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Pleural Cavity ◽  
Prenatal Ultrasound ◽  
Lung Hypoplasia ◽  
Prenatal Period ◽  
Compression Index ◽  
Disease Group ◽  
Group I ◽  
Group Ii

Objectives. To assess the effectiveness of the use of prenatal ultrasound indexes in congenital diaphragmatic hernia of the fetus to determine the postnatal prognosis. Materials. The analysis of 95 observations of left-sided congenital diaphragmatic hernia of the fetus was carried out. In the prenatal period, the composition of organs displaced into the pleural cavity was determined, the heart compression index (HCI), O/E LHR according to Jani and DeKoninck, and QLI were calculated. Results. Survival rate of newborns was 57.9%, mortality rate was 42.1%. The newborns were divided into two groups depending on the outcome of the disease. Group I — surviving newborns (n = 55), group II — deceased patients (n = 40). In the analyzed groups, there were no statistical differences in the timing of delivery, birth weight of newborns, the severity of asphyxia after birth and the type of hernia. In group I, the intestinal loops and stomach were significantly more often identified in the pleural cavity in isolation, less often the liver. HCI corresponded to 1.3, Jani O/E LHR 45.7%, DeKoninck O/E LHR 38.7%, QLI 0.7. In group II, concomitant malformations, polyhydramnios and displacement of the liver into the pleural cavity were significantly more frequent. HCI was 1.5, Jani O/E LHR 38.6%, DeKoninck O/E LHR 32.0%, QLI 0.6. Conclusions. In predicting the outcome of the disease for a newborn, the most effective is a comprehensive assessment of the location of the liver, the heart compression index and the index of lung hypoplasia (O/E LHR according to Jani). The diagnostic accuracy of the method is 80%, the sensitivity is 74.4%, and the specificity is 83.3%.

scholarly journals Vascular and ventilatory mechanical responses in three different stages of pulmonary development in the rabbit model of congenital diaphragmatic hernia

2018 ◽  
Vol 33 (10) ◽  
pp. 879-888 ◽  
Author(s):  
Rebeca Lopes Figueira ◽  
Karina Miura da Costa ◽  
Ana Laura Marsico ◽  
Thamires Melchiades da Silva Milani ◽  
Walusa Assad Gonçalves ◽  
...  
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Rabbit Model ◽  
Mechanical Responses

scholarly journals Prenatal Diagnosis and Management of a Rare Central Tendon Defect Type of Congenital Diaphragmatic Hernia with a Massive Pericardial Effusion

2020 ◽  
Vol 2020 ◽  
pp. 1-5
Author(s):  
Inas Babic ◽  
Haifa Al-Jobair ◽  
Osama Al Towaijri ◽  
Huda Al-Shammary ◽  
Merna Atiyah ◽  
...  
Keyword(s):  
Pericardial Effusion ◽  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Neonatal Morbidity ◽  
Lung Hypoplasia ◽  
Defect Type ◽  
Tendon Defect ◽  
Prenatal Diagnostic ◽  
Large Pericardial Effusion ◽  
Prenatal Intervention

The central tendon defect type of congenital diaphragmatic hernia (CDH) is extremely rare and usually associated with a significant pericardial effusion. Prenatal diagnostic ultrasound features of this quite rare entity remain often overlooked or misdiagnosed. There is a dearth of literature about the role of prenatal intervention, often through an elective pericardiocentesis, for the prevention of lung hypoplasia and to decrease the overall neonatal morbidity and mortality. To the best of our knowledge, till date, there is only one case that was subjected to a prenatal intervention. Here, we present a second case of a central tendon defect type of CDH with a large pericardial effusion that was subjected to a prenatal transthoracic pericardiocentesis. Although smooth intubation and ventilation were performed immediately after birth, the infant suffered for several months from respiratory instability. Laparoscopic central tendon hernia repair was performed, and neonate was discharged home at seven months of age. Although prenatal pericardiocentesis may facilitate smoother postnatal intubation and ventilation, its broader effect on respiratory function is uncertain and still remains elusive.

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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