[Management of congenital adrenal hyperplasia].
This review deals with the current problems in the management of classical 21-hydroxylase deficiency from the fetal life to the puberty. The clinical consequences of 21-hydroxylase deficiency reflect the disordered physiology--impaired secretion of glucocorticoids and mineralocorticoids, and excessive secretion of androgens. Current therapy is intended to correct the disordered physiology by replacing mineralocorticoid and glucocorticoid hormones, thereby reducing the ACTH-driven increase in adrenal androgen secretion. Treated patients should expect a normal life span and reproductive potential. This can be achieved by careful attention to regular measurements of clinical parameters and biochemical indices of control.
Sólyom
J
J
II. Gyermekklinika, Semmelweis Orvostudományi Egyetem, Budapest.
D000305:Adrenal Cortex Hormones / Q000378:metabolism / Q000627:therapeutic use*; D000312:Adrenal Hyperplasia, Congenital / Q000175:diagnosis / Q000188:drug therapy / Q000201:enzymology*; D000309:Adrenal Insufficiency / Q000188:drug therapy / Q000209:etiology; D000728:Androgens / Q000378:metabolism; D005260:Female; D005938:Glucocorticoids / Q000172:deficiency; D006801:Humans; D007231:Infant, Newborn; D008901:Mineralocorticoids / Q000172:deficiency; D011247:Pregnancy; D011296:Prenatal Diagnosis; D012733:Sex Differentiation
D000305:Adrenal Cortex Hormones; D000728:Androgens; D005938:Glucocorticoids; D008901:Mineralocorticoids
A congenitalis adrenalis hyperplasia kezeléséröl.