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Adrenomedullary Dysplasia and Hypofunction in Patients with Classic 21-Hydroxylase Deficiency
Ist Teil von
The New England journal of medicine, 2000-11, Vol.343 (19), p.1362-1368
Ort / Verlag
Boston, MA: Massachusetts Medical Society
Erscheinungsjahr
2000
Link zum Volltext
Quelle
Alma/SFX Local Collection
Beschreibungen/Notizen
Congenital adrenal hyperplasia is characterized clinically by prenatal virilization and genital ambiguity in newborn girls, postnatal virilization in both boys and girls, and adrenal insufficiency with or without salt wasting.
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Biochemically, the disorder is characterized by impaired production of cortisol with or without impaired production of aldosterone, chronic stimulation of the adrenal cortex by corticotropin, and overproduction of cortisol precursors and androgens. The most common cause of congenital adrenal hyperplasia is 21-hydroxylase deficiency, with an incidence of approximately 1 case per 15,000 live births worldwide.
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Despite adequate treatment with glucocorticoid and mineralocorticoid replacement, children with the classic or severe . . .