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Defective neural crest development gives rise to a number of congenital
malformations. The term neurocristopathies collectively refers to these
syndromes, tumors, and dimorphologies of neural crest tissue. The neurocristopathies
often involve the adrenal medulla, endocrine organs of neural crest
origin, and Schwann cells of the ANS. Neurocristopathies are not limited to these regions - there is a great
diversity of disorders associated with the crest cells because they
play important roles in an eclectic mix of body tissues. Typically, there are two major categories of neurocristopathies: defects in neural crest migration and tumors of neural crest. (Carlson, 259) Tumors of neural crest include: Pheochrtomocyoma: a tumor of chromaffin tissue of the
adrenal medulla Defects of Migration: Waardenburg's syndromeTrunk Neural Crest Hirshsprung's disease Cranial Neural Crest Aorticopulmonary septation defect of heart Cleft Lip, cleft palate. Frontonasal dysplasia Di George syndrome - hyperthyroidism, thyroid deficiency, thymic dysplasia. Dental anomalies. Trunk & Cranial CHARGE association Other Defects |
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