TY - JOUR
T1 - Luteinizing hormone-releasing hormone analog treatment of boys with hypothalamic hamartoma and true precocious puberty
AU - Comite, Florence
AU - Pescovitz, Ora H.
AU - Rieth, Kenneth G.
AU - Dwyer, Andrew J.
AU - Hench, Karen
AU - McNemar, Ann
AU - Loriaux, D. Lynn
AU - Cutler, Gordon B.
PY - 1984/11
Y1 - 1984/11
N2 - A long-acting analog of LRH (LRHa) has been shown to suppress pituitary gonadotropin and estradiol secretion to prepubertal levels in girls with idiopathic true precocious puberty. We treated six boys, aged 1–6 yr, with true precocious puberty due to hypothalamic hamartoma for 6–24 months with daily sc injections of LRHa. The patients had enlarged testes (6–25 ml), Tanner stage II-IV pubic hair, facial and axillary hair, increased growth rate, and an advanced bone age. Frequent erections occurred in all patients. Computed tomography of the head showed abnormalities characteristic of hypothalamic hamartoma (0.5–3 cm in diameter) in each boy. Each patient had measurable LH and FSH levels, with pulsed nocturnal secretion, and pubertal LH and FSH responses to LRH. Serum testosterone testosterone was in the range for normal adult men (200–600 ng/dl). LRHa significantly decreased basal LH (P < 0.005) and FSH levels (P < 0.01), LRH-stimulated gonadotropin levels (P < 0.005), and serum testosterone levels (P < 0.005). Testis size decreased significantly (P < 0.005). Annualized growth velocity (centimeters per yr) decreased significantly compared to the pretreatment growth rate (P < 0.01). Bone age advancement per yr slowed significantly during the course of LRHa treatment (P < 0.01). Pubic hair, facial hair, and erections decreased in all patients. LRHa is an effective treatment for boys with precocious puberty associated with hypothalamic hamartoma. Chronic therapy will be required, however, to assess the ultimate effect of LRHa.
AB - A long-acting analog of LRH (LRHa) has been shown to suppress pituitary gonadotropin and estradiol secretion to prepubertal levels in girls with idiopathic true precocious puberty. We treated six boys, aged 1–6 yr, with true precocious puberty due to hypothalamic hamartoma for 6–24 months with daily sc injections of LRHa. The patients had enlarged testes (6–25 ml), Tanner stage II-IV pubic hair, facial and axillary hair, increased growth rate, and an advanced bone age. Frequent erections occurred in all patients. Computed tomography of the head showed abnormalities characteristic of hypothalamic hamartoma (0.5–3 cm in diameter) in each boy. Each patient had measurable LH and FSH levels, with pulsed nocturnal secretion, and pubertal LH and FSH responses to LRH. Serum testosterone testosterone was in the range for normal adult men (200–600 ng/dl). LRHa significantly decreased basal LH (P < 0.005) and FSH levels (P < 0.01), LRH-stimulated gonadotropin levels (P < 0.005), and serum testosterone levels (P < 0.005). Testis size decreased significantly (P < 0.005). Annualized growth velocity (centimeters per yr) decreased significantly compared to the pretreatment growth rate (P < 0.01). Bone age advancement per yr slowed significantly during the course of LRHa treatment (P < 0.01). Pubic hair, facial hair, and erections decreased in all patients. LRHa is an effective treatment for boys with precocious puberty associated with hypothalamic hamartoma. Chronic therapy will be required, however, to assess the ultimate effect of LRHa.
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U2 - 10.1210/jcem-59-5-888
DO - 10.1210/jcem-59-5-888
M3 - Article
C2 - 6434587
AN - SCOPUS:0021231714
SN - 0021-972X
VL - 59
SP - 888
EP - 892
JO - Journal of Clinical Endocrinology and Metabolism
JF - Journal of Clinical Endocrinology and Metabolism
IS - 5
ER -