Hiperplasia suprarrenal de expresión tardía: optimización de la terapia midiendo la variación circadiana de la 17-OH-progesterona sérica / Late onset adrenal hyperplasia: optimization of treatment by assesment of the circadian variation in serum 17-OH-progesterone

ABSTRACT

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 spsironulactone. In spite of a very tgood response in terms of amelioration of ther hursutism she experienced cushingoid manifestations associated with, adrenal suppression. To overcome these side effects the patient was placed ons hydrocortisone 20 mg at 8 AM plus spironallactone 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 administered at 8 AM and compared it with curcadian variations under basal conditions or after late-evening (11 PM) adminsitration of hydrocortisosne, 20 mg. The early morning adminstration of hydro cortisone 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 adminsitration of hidrocortisone was able to supress 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 adminstration (12.2 ñ 13.1 vs 9.9 ñ 11.3 ug/dl,p = 0.53), yet the corresponding 17-OH-progesterone levels were much lower (0.8 ñ 0.6 vs 5.9 ñ 6.9 ng/ml. We conclude that individualization of therapy is essential in patients with lateosnt adrenal