Abstract
Cushing's syndrome is frequently associated with growth retardation and decreased gonadal function. So far, the underlying mechanisms of these disturbances are poorly understood. An 18 year old boy is described who was referred because of delayed puberty (bone age 12 years) and short stature (115.5 cm). The patient had a typical cushingoid habitus. His testes were preadolescent in size (4-5 ml), and the testosterone was low (18 ng/dl). 24h plasma Cortisol levels were consistently high (32.3 ± 5 μg/dl) with episodic increases. ACTH was elevated (126 ± 44.6 pg/ml), and the cortisol spikes could be related to concomitant ACTH bursts. 24h profiles of LH and HGH exhibited low levels (LH, 2.52 ± 0.51 mU/ml; HGH, 0.58 ± 0.5 ng/ml), and the pulsatile secretion was completely abolished. HGH failed to respond to arginine infusion, insulin-induced hypoglycemia, and GRF1-44. LHRH-stimulation produced a prepubertal increase of LH: 0 min, 2.95 mU/ml; 30 min, 8.2 mU/ml. Surgery revealed an ACTH-producing microadenoma of the pituitary. 3 months after complete extirpation of the tumor LH and HGH showed normal pulsatile secretion patterns (9 pulses per 24 h), and testosterone had begun to normalize (414 ng/dl). After 1 year of observation, testicular size was 12-15 ml, and growth velocity was 10.6 cm/year. It is suggested that the delay of puberty and growth was due to the suppression of the pulsatile secretion of LH and HGH, caused by the hypercortisolism.
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Joost, S., Heidemann, P. & Stubbe, P. SUPPRESSION OF THE PULSATILE SECRETION PATTERN OF LH AND HGH IN AN ADOLESCENT BOY WITH CUSHING'S DISEASE. Pediatr Res 23, 129 (1988). https://doi.org/10.1203/00006450-198801000-00166
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DOI: https://doi.org/10.1203/00006450-198801000-00166
