Pescovitz O H, Comite F, Hench K, Barnes K, McNemar A, Foster C, Kenigsberg D, Loriaux D L, Cutler G B
J Pediatr. 1986 Jan;108(1):47-54. doi: 10.1016/s0022-3476(86)80767-3.
Between 1979 and 1983, 129 children (95 girls) with precocious puberty were referred to the National Institutes of Health and received treatment for at least 6 months with the long-acting LHRH analogue D-Trp6-Pro9-NEt-LHRH. The majority (107 of 129) of the children had central precocious puberty mediated by activation of the hypothalamic-pituitary-gonadal axis in association with hypothalamic hamartomas (24 of 107) or other central nervous system lesions (21 of 107), or idiopathic precocious puberty (62 of 107). Hypothalamic hamartomas or other central nervous system lesions were a frequent cause of central precocious puberty in girls (27 of 87), but idiopathic precocious puberty was still the most frequent diagnosis (63%). Idiopathic precocious puberty was uncommon in boys (6%). The patients with peripheral precocious puberty included six girls with McCune-Albright syndrome and six boys with familial male precocious puberty. These children had peripheral sex steroid secretion in the absence of hypothalamic-pituitary-gonadal axis maturation. The children with combined peripheral and central precocious puberty included nine children with congenital adrenal hyperplasia and one girl with a virilizing adrenal tumor. In the patients with central precocious puberty or combined peripheral and central precocious puberty, LHRHa therapy caused suppression of gonadotropin and sex steroid levels (P less than 0.001), stabilization or regression of secondary sexual characteristics, and decreases in growth rate and in the rate of bone age maturation (P less than 0.005). Patients with peripheral precocious puberty, however, had no significant change in gonadotropin or sex steroid levels, growth rate, or the rate of bone age maturation, and no improvement in secondary sexual characteristics. Thus, LHRHa is an effective treatment of central precocious puberty and combined peripheral and central precocious puberty, but is ineffective in the therapy of peripheral precocious puberty.
1979年至1983年间，129名性早熟儿童（95名女孩）被转诊至美国国立卫生研究院，并接受长效促黄体生成素释放激素类似物D-Trp6-Pro9-NEt-LHRH治疗至少6个月。大多数（129名中的107名）儿童患有中枢性性早熟，其由下丘脑-垂体-性腺轴激活介导，伴有下丘脑错构瘤（107名中的24名）或其他中枢神经系统病变（107名中的21名），或特发性性早熟（107名中的62名）。下丘脑错构瘤或其他中枢神经系统病变是女孩中枢性性早熟的常见原因（87名中的27名），但特发性性早熟仍是最常见的诊断（63%）。特发性性早熟在男孩中不常见（6%）。外周性早熟患者包括6名患有McCune-Albright综合征的女孩和6名患有家族性男性性早熟的男孩。这些儿童在无下丘脑-垂体-性腺轴成熟的情况下出现外周性类固醇分泌。合并外周性和中枢性性早熟的儿童包括9名先天性肾上腺皮质增生症患儿和1名患有男性化肾上腺肿瘤的女孩。在中枢性性早熟或合并外周性和中枢性性早熟的患者中，促性腺激素释放激素类似物（LHRHa）治疗导致促性腺激素和性类固醇水平受到抑制（P<0.001），第二性征稳定或消退，生长速率和骨龄成熟速率降低（P<0.005）。然而，外周性早熟患者的促性腺激素或性类固醇水平、生长速率或骨龄成熟速率无显著变化，第二性征也无改善。因此，LHRHa是治疗中枢性性早熟以及合并外周性和中枢性性早熟的有效方法，但对外周性早熟治疗无效。
