Disproportionate suppression of dehydroepiandrosterone sulfate (DHEAS) in treated patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.
Serum concentrations of dehydroepiandrosterone sulfate (DHEAS) were measured in 28 patients (18 females, 10 males) with congenital adrenal hyperplasia due to 21-hydroxylase deficiency who were treated with oral hydrocortisone (non-salt losers) or hydrocortisone and 9-alpha-fluorohydrocortisone (salt-losers). Adequacy of therapy was assessed by clinical findings, determination of bone age, urinary excretion of 17-ketosteroids, and serum concentration of 17-hydroxyprogesterone. These allowed the separation of patients into three groups: poorly controlled, adequately controlled and overtreated. Individual values for serum levels of DHEAS were compared to mean normal values for age. In the adequately controlled and overtreated patients, mean serum concentrations of DHEAS were significantly lower than normal values for age (P less than 0.05). In the poorly treated patients, the mean serum concentration of DHEAS was not significantly different from normal values for age (P = 0.50). These data indicate that the serum concentration of DHEAS is overly suppressed in treated patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. This finding suggests that measurement of the serum levels of DHEAS has limited value in assessing the adequacy of therapy in this disease.
D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000097:blood / Q000188:drug therapy* / Q000209:etiology; D000367:Age Factors; D002648:Child; D003687:Dehydroepiandrosterone / Q000031:analogs & derivatives* / Q000037:antagonists & inhibitors / Q000097:blood; D019314:Dehydroepiandrosterone Sulfate; D005260:Female; D005438:Fludrocortisone / Q000627:therapeutic use; D005500:Follow-Up Studies; D006801:Humans; D006854:Hydrocortisone / Q000627:therapeutic use; D008297:Male; D006899:Mixed Function Oxygenases / Q000172:deficiency
D003687:Dehydroepiandrosterone; D019314:Dehydroepiandrosterone Sulfate; D006899:Mixed Function Oxygenases; D005438:Fludrocortisone; D006854:Hydrocortisone