Stephen L. Gans Distinguished Overseas Lecture. The neural crest in pediatric surgery
| Autor: | Juan A, Tovar |
|---|---|
| Rok vydání: | 2007 |
| Předmět: |
Patched Receptors
Receptors Retinoic Acid Cardiovascular Abnormalities Receptors Cell Surface Tretinoin Zinc Finger Protein GLI1 Enteric Nervous System Receptors G-Protein-Coupled Cell Movement Neoplasms Humans Abnormalities Multiple Cell Lineage Hedgehog Proteins Child Esophageal Atresia Hernia Diaphragmatic Homeodomain Proteins Genes Homeobox Infant Newborn Infant Syndrome Smoothened Receptor Branchial Region Neural Crest Child Preschool Face Blood Vessels Hernias Diaphragmatic Congenital Pigmentation Disorders Signal Transduction Transcription Factors |
| Zdroj: | Journal of pediatric surgery. 42(6) |
| ISSN: | 1531-5037 |
| Popis: | This review highlights the relevance of the neural crest (NC) as a developmental control mechanism involved in several pediatric surgical conditions and the investigative interest of following some of its known signaling pathways.The participation of the NC in facial clefts, ear defects, branchial fistulae and cysts, heart outflow tract and aortic arch anomalies, pigmentary disorders, abnormal enteric innervation, neural tumors, hemangiomas, and vascular anomalies is briefly reviewed. Then, the literature on clinical and experimental esophageal atresia-tracheoesophageal fistula (EA-TEF) and congenital diaphragmatic hernia (CDH) is reviewed for the presence of associated NC defects. Finally, some of the molecular signaling pathways involved in both conditions (sonic hedgehog, Hox genes, and retinoids) are summarized.The association of facial, cardiovascular, thymic, parathyroid, and C-cell defects together with anomalies of extrinsic and intrinsic esophageal innervation in babies and/or animals with both EA-TEF and CDH strongly supports the hypothesis that NC is involved in the pathogenesis of these malformative clusters. On the other hand, both EA-TEF and CDH are observed in mice mutant for genes involved in the previously mentioned signaling pathways.The investigation of NC-related molecular pathogenic pathways involved in malformative associations like EA-TEF and CDH that are induced by chromosomal anomalies, chemical teratogens, and engineered mutations is a promising way of clarifying why and how some pediatric surgical conditions occur. Pediatric surgeons should be actively involved in these investigations. |
| Databáze: | OpenAIRE |
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