scholarly journals Lung tissue perfusion in congenital diaphragmatic hernia and association with the lung-to-head ratio and intrapulmonary artery pulsed Doppler

2010 ◽  
Vol 35 (5) ◽  
pp. 578-582 ◽  
Author(s):  
O. Moreno-Alvarez ◽  
R. Cruz-Martinez ◽  
E. Hernandez-Andrade ◽  
E. Done ◽  
O. Gómez ◽  
...  
2011 ◽  
Vol 29 (1) ◽  
pp. 101-107 ◽  
Author(s):  
Rogelio Cruz-Martínez ◽  
Oscar Moreno-Alvarez ◽  
Edgar Hernández-Andrade ◽  
Montserrat Castañón ◽  
Josep Maria Martínez ◽  
...  

1999 ◽  
Vol 45 (4, Part 2 of 2) ◽  
pp. 56A-56A
Author(s):  
Robert P Lemke ◽  
Grzegorz Sawicki ◽  
Cheung Po-Yin ◽  
Douglas W Allan ◽  
Richard Schulz ◽  
...  

2002 ◽  
Vol 283 (6) ◽  
pp. L1310-L1314 ◽  
Author(s):  
Randal P. Babiuk ◽  
John J. Greer

Congenital diaphragmatic hernia (CDH) is a significant clinical problem in which a portion of the diaphragmatic musculature fails to form, resulting in a hole in the diaphragm. Here we use animal models of CDH to test two hypotheses regarding the pathogenesis. First, the origin of the defect results from the malformation of the amuscular mesenchymal component of the primordial diaphragm rather than with the process of myogenesis. Second, the defect in the primordial diaphragmatic tissue is not secondary to defects in the developing lung. In c- met(−/−) mouse embryos, in which diaphragm muscle fibers do not form because of a defect in muscle precursor migration, the amuscular substratum forms fully. We show that a defect characteristic of CDH can be induced in the amuscular membrane. In Fgf10(−/−) mouse embryos that have lung agenesis we show that the primordial diaphragm does not depend on signals from lung tissue for proper development and that diaphragmatic malformation is a primary defect in CDH. These data suggest that the pathogenesis of CDH involves mechanisms fundamentally different from previously proposed hypotheses.


Neonatology ◽  
2013 ◽  
Vol 103 (3) ◽  
pp. 213-217 ◽  
Author(s):  
R.B. Van Loenhout ◽  
R.R. De Krijger ◽  
C.P. Van de Ven ◽  
I.W.J.M. Van der Horst ◽  
L.W.J.E. Beurskens ◽  
...  

2007 ◽  
Vol 42 (6) ◽  
pp. 505-512 ◽  
Author(s):  
Andreas W. Flemmer ◽  
Jacques C. Jani ◽  
Florian Bergmann ◽  
Oliver J. Muensterer ◽  
Denis Gallot ◽  
...  

2016 ◽  
Vol 310 (4) ◽  
pp. L311-L327 ◽  
Author(s):  
Aline Vuckovic ◽  
Susanne Herber-Jonat ◽  
Andreas W. Flemmer ◽  
Ina M. Ruehl ◽  
Carmela Votino ◽  
...  

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.


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