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- W2609826259 endingPage "128" @default.
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- W2609826259 abstract "The outcomes of patients diagnosed with congenital diaphragmatic hernia (CDH) have recently improved. However, mortality and morbidity remain high, and this is primarily caused by the abnormal lung development resulting in pulmonary hypoplasia and persistent pulmonary hypertension. The pathogenesis of CDH is poorly understood, despite the identification of certain candidate genes disrupting normal diaphragm and lung morphogenesis in animal models of CDH. Defects within the lung mesenchyme and interstitium contribute to disturbed distal lung development. Frequently, a disturbance in the development of the pleuroperitoneal folds (PPFs) leads to the incomplete formation of the diaphragm and subsequent herniation. Most candidate genes identified in animal models have so far revealed relatively few strong associations in human CDH cases. CDH is likely a highly polygenic disease, and future studies will need to reconcile how disturbances in the expression of multiple genes cause the disease. Herein, we summarize the available literature on abnormal lung development associated with CDH." @default.
- W2609826259 created "2017-05-05" @default.
- W2609826259 creator A5011688601 @default.
- W2609826259 creator A5076325102 @default.
- W2609826259 creator A5087290454 @default.
- W2609826259 date "2017-06-01" @default.
- W2609826259 modified "2023-10-01" @default.
- W2609826259 title "Abnormal lung development in congenital diaphragmatic hernia" @default.
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