SUCCESSFUL METYRAPONE |[lpar]|M|[rpar]| TREATMENT FOR CUSHING'S DISEASE |[lpar]|C D|[rpar]| IN AN ADOLESCENT BOY

摘要

A 14 year old boy presented with growth retardation and delayed gonadarche in presence of adrenarche, discrete obesity of trunk and face, delayed bone age, osteoporosis and biochemical hypercortisolism: plasma Cortisol (F)24h-profile was elevated but hypopulsatile (mean 22.1 ng/ml ± 13 %) and without diumal variation; urinary free F and 20α -dihydro-F were increased (86.4 and 370 μg/ 24h). C T-scans of pituitary and adrenals were unconspicuous. Normal A C T it-levels (8 a.m.:31.3 pg/ml) and maintenance of a circadian rhythm were not entirely consistent with C D, but good F-suppnession by 0.5 mg Dexamethasone or 1500 mg M and adrenal stimulation by A C T H and C R F -via A C T H- indicated a central origin of hypercortisolism. 24h-secretion of gonadotropins was suppressed and hypopulsatile; H G H was reduced during sleep and after G H R H -stimulation. After a 4-month-therapy with 2 × 250 mg M, the following changes were measured: decrease of mean 24h plasma F (3.7 ng/ml ± 37%) and D H E A S (4406 vs 3333 ng/ml); slight elevation of A C T H (40.1 pg/ml) and increase of testosterone (0.59 vs 4.9 ng/ml, LH (1.8 vs 7.1 ng/ml), F S H (3.9 vs 6.7 ng/ml) and H G H (max. nocturnal peak: 10.5 vs 18 ng/ml). Normal renine activity and high somatomedin C were unchanged. Clinically the patient exhibiteded catch-up growth and an increase of testicular volume witliout adverse M-effects.Conclusion: If surgery is not feasible, M may be an effective longterm medication for hypercortisolism in adolescents.