Neuroendocrinology. 2018;106(3):203-210. doi: 10.1159/000477584. Epub 2017 May 30.
Hypothalamic hamartoma (HH) represents the commonest cause of organic central precocious puberty (CPP). Follow-up of these patients in adulthood is scarce.
To describe the anthropometric, metabolic, and reproductive parameters of patients with CPP due to HH before and after treatment with gonadotropin-releasing hormone analog (GnRHa).
We performed a retrospective and cross-sectional study in a single tertiary center including 14 patients (7 females) with CPP due to HH.
The mean duration of GnRHa treatment was 7.7 ± 2.4 years in boys and 7.9 ± 2.1 years in girls. GnRHa treatment was interrupted at the mean chronological age (CA) of 12.1 ± 1.1 years in boys and 10.7 ± 0.5 years in girls. At the last visit, the mean CA of the male and female patients was 21.5 ± 3.2 and 24 ± 3.9 years, respectively. Eleven of the 14 patients reached normal final height (FH) (standard deviation score -0.6 ± 0.9 for males and -0.6 ± 0.5 for females), all of them within the target height (TH) range. The remaining 3 patients had predicted height within the TH range. The mean body mass index and the percentage of body fat mass was significantly higher in females, with a higher prevalence of metabolic disorders. All patients presented normal gonadal function in adulthood, and 3 males fathered a child.
All patients with CPP due to HH reached normal FH or near-FH. A higher prevalence of overweight/obesity and hypercholesterolemia was observed in the female patients. Finally, no reproductive disorder was identified in both sexes, indicating that HH per se has no deleterious effect on the gonadotropic axis in adulthood.
下丘脑错构瘤(HH)是导致特发性中枢性性早熟(CPP)的最常见原因。这些患者在成年后的随访资料较为缺乏。
描述经促性腺激素释放激素类似物(GnRHa)治疗后 CPP 患者的人体测量学、代谢和生殖参数。
我们在一家三级中心进行了一项回顾性和横断面研究,共纳入 14 例(7 例女性)因 HH 导致 CPP 的患者。
男孩 GnRHa 治疗的平均持续时间为 7.7 ± 2.4 年,女孩为 7.9 ± 2.1 年。GnRHa 治疗在男孩平均年龄(CA)为 12.1 ± 1.1 岁、女孩为 10.7 ± 0.5 岁时中断。末次随访时,男性和女性患者的平均 CA 分别为 21.5 ± 3.2 岁和 24 ± 3.9 岁。14 例患者中有 11 例达到正常最终身高(FH)(男孩标准偏差评分 -0.6 ± 0.9,女孩 -0.6 ± 0.5),均在靶身高(TH)范围内。其余 3 例患者的预测身高在 TH 范围内。女性的平均体重指数和体脂百分比显著更高,代谢紊乱的患病率更高。所有患者在成年后均表现出正常的性腺功能,其中 3 名男性生育了孩子。
所有因 HH 导致 CPP 的患者均达到正常 FH 或接近 FH。女性患者超重/肥胖和高胆固醇血症的患病率更高。最后,两性均未发现生殖障碍,表明 HH 本身对成年后性腺轴没有不良影响。