[Adrenal hyperplasia of late onset: optimization of therapy by measuring the circadian variation of serum 17-OH-progesterone].

A 19 yr-old female patient with the diagnosis of late onset adrenal hyperplasia was treated since age 15 with different glucocorticoid preparations and dosage schedules plus spironolactone. In spite of a very good response in terms of amelioration of their hirsutism she experienced cushingoid manifestations associated with adrenal suppression. To overcome these side effects the patient was placed on hydrocortisone 20 mg at 8 AM plus spironalactone 50 mg q.i.d. Cushingoid features vanished and response to cosyntropin (ACTH 250 ug i.m.) was reestablished. To better ascertain the effects of this treatment we studied the circadian variation in serum 17-OH progesterone after hydrocortisone was administered at 8 AM and compared it with circadian variations under basal conditions or after late-evening (11 PM) administration of hydrocortisone, 20 mg. The early morning administration of hydrocortisone was unable to prevent the nocturnal elevation of 17-OH-progesterone in spite of normal levels from 9.30 AM to 3 AM. This nocturnal peak was associated with a slightly blunted nocturnal elevation of serum cortisol. In contrast, the late evening administration of hydrocortisone was able to suppress 17-OH-progesterone to within normal levels during all day. Serum cortisol during late evening therapy was not different from that observed during early morning administration (12.2 +/- 13.1 vs 9.9 +/- 11.3 micrograms/dl, p = 0.53), yet the corresponding 17-OH-progesterone levels were much lower (0.8 +/- 0.6 vs 5.9 +/- 6.9 ng/ml). We conclude that individualization of therapy is essential in patients with late-onset adrenal hyperplasia.(ABSTRACT TRUNCATED AT 250 WORDS)