Synonyms
Congenital adrenal hyperplasia; 21‐hydroxylase deficiency
Definition
Congenital adrenal hyperplasia is a group of inborn errors of metabolism arising from enzyme defects in the biosynthesis pathways of adrenal corticosteroids, resulting in inadequate production of glucocorticoids and mineralocorticoids and excess production of adrenal androgens.
Pathology/Histopathology
The adrenal cortex produces three principle groups of corticosteroids: mineralocorticoids responsible for electrolyte balance, glucocorticoids necessary for carbohydrate mobilization and potentiation of catecholamine action in response to stress, and adrenal androgens necessary for growth and sexual development. Adrenal corticosteroid production begins with the uptake of serum cholesterol and transport into the mitochondrium of the cells of the adrenal cortex, under the stimulation of adrenocorticotropic hormone (ACTH), gonadotropins, and steroidogenic acute regulatory protein (StAR). Hormone production...
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Bibliography
Miller WL (1997) The adrenal cortex and its disorders. In: Brook C, Clayton P, Brown R (eds) Brook's Clinical Pediatric Endocrinology. Blackwell Publishing. pp 293–351
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Daneman A (2004) The adrenal gland. In: Kuhn JP, Slovis TL, Haller JO (eds) Caffey's Paediatric Diagnostic Imaging. Mosbypages 239–251
Al‐Alwan I, Navarro O, Daneman D et al (1999) Clinical utility of adrenal ultrasonography in the diagnosis of congenital adrenal hyperplasia. J Pediatr 13571–75
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© 2008 Springer-Verlag Berlin Heidelberg New York
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Arthur, R. (2008). Adrenogenital Syndrome. In: Baert, A.L. (eds) Encyclopedia of Diagnostic Imaging. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-35280-8_59
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DOI: https://doi.org/10.1007/978-3-540-35280-8_59
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