Congenital diaphragmatic hernia.

@article{Keijzer2010CongenitalDH,
  title={Congenital diaphragmatic hernia.},
  author={Richard Keijzer and Prem Lata Puri},
  journal={Seminars in pediatric surgery},
  year={2010},
  volume={19 3},
  pages={
          180-5
        }
}
Congenital diaphragmatic hernia (CDH) is a congenital anomaly consisting of a posterolateral defect in the diaphragm also known as a Bochdalek hernia. It occurs in 1 in 2000 to 3000 newborns and is associated with a variable degree of pulmonary hypoplasia (PH) and persistent pulmonary hypertension (PPH). Despite remarkable advances in neonatal resuscitation and intensive care and the new postnatal treatment strategies, many newborns with CDH continue to have high rates of mortality and… 
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We present a novel case of the association of right-sided Bochdalek hernia, a diaphragmatic life-threatening malformation, and Kartagener syndrome, which is characterized by congenital
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    Seminars in pediatric surgery
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TLDR
Critical findings in the areas of clinical and basic research that highlight the importance of genetics in the development of CDH are reviewed and practical information is provided that can aid physicians and surgeons as they evaluate and care for patients with isolated, nonisolated, and syndromic forms ofCDH.
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TLDR
The current molecular and genetic background of lung formation, as well as a reflection of this knowledge towards CDH, are discussed.
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TLDR
Key findings are examined that are likely to form the basis for future research in this field ofCDH, including evidence of the roles of specific genes in the development of CDH.
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TLDR
This review will provide a brief summary of diaphragm development and model organism work most relevant to human CDH and will primarily describe important human phenotypes associated withCDH and also provide recommendations for diagnostic evaluation of a fetus or infant with CDH.
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TLDR
Almost 75 percent of fetuses with congenital diaphragmatic hernia detected before birth die despite optimal postnatal care, and the neonatal outcome is related to the degree of pulmonary hypoplasia from in utero lung compression, which is determined by the timing and volume.
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TLDR
This work describes a new procedure intended to reduce the liver gradually so that the ductus venosus is never acutely kinked and more normal rib cage mechanics may be maintained.
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TLDR
The epidemiology and pathophysiology of human CDH, the metabolism of retinoids and the implications ofretinoids in the development of the diaphragm and lung are described and the existing evidence of a disruption of the retinoid-signaling pathway in CDH is described.
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TLDR
A review of recent advances in human and mouse genetics that have led to the identification of genes involved in CDH discusses potential mechanisms underlying the seemingly random combination of diaphragmatic, pulmonary, cardiovascular, and gonadal defects in patients with CDH.
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