CVAH (congenital virilizing adrenal hyperplasia): a syndrome produced by a genetically transmitted enzymatic defect in the functioning of the adrenal cortices of males and females, which induces varying degrees of insufficiency of cortisol and aldosterone and excesses of adrenal androgen and pituitary adrenocorticotropin (ACTH). Abnormal function of the adrenal cortex starts in fetal life and, unless treated, continues chronically after birth. Females born with the syndrome have ambiguous genitalia and, if they survive without salt loss and dehydration, undergo severe virilization. Males are usually not recognized at birth, but if they survive, will prematurely develop pubertally during the early years of life. In the severe form of the disease, untreated, mortality rate is almost 100 percent for both sexes. Treatment with glucocorticoids and in some cases also with salt-retaining hormone is lifesaving and prevents untimely and, in girls, incongruous postnatal virilization. Plastic surgery is needed to feminize the genitalia. With appropriate therapy, prognosis for survival and good physical and mental health is excellent. Synonym, CAH; Adrenogenital syndrome

Dictionary of Sexology Project: Main Index

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