Long-Term Outcomes of Patients with Central Precocious Puberty due to Hypothalamic Hamartoma after GnRHa Treatment: Anthropometric, Metabolic, and Reproductive Aspects

被引:20
作者
Ramos, Carolina O.
Latronico, Ana C.
Cukier, Priscilla
Macedo, Delanie B.
Bessa, Danielle S.
Cunha-Silva, Marina
Arnhold, Ivo J.
Mendonca, Berenice B.
Brito, Vinicius N.
机构
[1] Univ Sao Paulo, Fac Med, Disciplina Endocrinol & Metabol, Unidade Endocrinol Desenvolvimento, Sao Paulo, Brazil
[2] Hosp Clin Sao Paulo, Lab Hormonios Genet Mol LIM 42, Sao Paulo, Brazil
关键词
Central precocious puberty; Hypothalamic hamartoma; Gonadotropin-releasing hormone analogs; Final height; Body composition and gonadal function; HORMONE ANALOG THERAPY; BODY-MASS INDEX; FINAL HEIGHT; DIAGNOSIS; GIRLS; DISCONTINUATION; EXPERIENCE; MUTATIONS; EPILEPSY; CHILDREN;
D O I
10.1159/000477584
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Background: Hypothalamic hamartoma (HH) represents the commonest cause of organic central precocious puberty (CPP). Follow-up of these patients in adulthood is scarce. Objective: 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). Methods: We performed a retrospective and cross-sectional study in a single tertiary center including 14 patients (7 females) with CPP due to HH. Results: 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. Conclusion: All patients with CPP due to HH reached normal FH or near-FH. A higher prevalence of overweight/obesity and hypercholes-terolemia 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. (c) 2017 S. Karger AG, Basel
引用
收藏
页码:203 / 210
页数:8
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