Abstract
Sexual precocity arises frequently from a CNS disturbance but its association with hypothalamic hypopituitarism Is most unusual. We report 5 girls with SOD and multiple tropic hormone deficiencies; all were GH and ACTH def. and 2/5 had sexual precocity while 3 were prepubertal and responded to IV GnRH. This finding contrasts sharply with other patients with multiple tropic hormone def. who have severely blunted or absent responses to GnRH. Case 1 diagnosed at 2 mos. with ADH def., presented at 7 4/12 yrs with midpubertal development, menarche, bone age 13.5 yrs, height age 9 yrs, and growth increment of 20 cm/last 2 yrs. GH and ACTH def. were documented by arginine/L-dopa and IV metapyrone tests. Basal serum Prl values were 31-42 ng/ml. Pelvic exam was normal and brain scan showed no mass. Case 2 presented at 16 yrs before GnRH testing was available, with short stature (HA 8 yrs), optic nerve dysplasia and regular menstrual periods since 10 yrs. Case 5 also was ADH def.; only case 3 was TSH def.
These Interesting cases illustrate selective retention of gonadotropin secretion despite presumed anterior hypothalamic dysfunction. Their congenital anomalies may have resulted in sexual precocity by interference with tonic inhibitory effects of the CNS.
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Huseman, C., Kelch, R., Hopwood, N. et al. SEXUAL PRECOCITY IN ASSOCIATION WITH SEPTO-OPTIC DYSPLASIA (SOD) AND HYPOTHALAMIC HYPOPITUITARISM. Pediatr Res 11, 426 (1977). https://doi.org/10.1203/00006450-197704000-00341
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DOI: https://doi.org/10.1203/00006450-197704000-00341
