Treatment of central precocious puberty and early puberty with GnRH analog in girls with Williams-Beuren syndrome

被引:4
作者
Spielmann, Sarah [1 ]
Partsch, Carl Joachim [2 ]
Gosch, Angela [3 ]
Pankau, Rainer [4 ]
机构
[1] MVZ Pro Cura GmbH, D-22111 Hamburg, Germany
[2] Endokrinol Hamburg, Hamburg, Germany
[3] Hsch Angew Wissensch Munchen, Munich, Germany
[4] Ostseeklin Boltenhagen, D-23946 Ostseebad Boltenhagen, Germany
关键词
precocious puberty; treatment; Williams-Beuren syndrome (WBS); FINAL HEIGHT; RANDOMIZED-TRIAL; STATURAL GROWTH; CHILDREN;
D O I
10.1515/jpem-2014-0393
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Objective: Little has been published on treatment of precocious puberty in girls with Williams-Beuren syndrome (WBS), a condition occurring frequently in this group. We analyzed our own data on growth/age at menarche of now adult female patients with WBS being diagnosed with central precocious puberty or early puberty. Data of patients treated with gonadotropin-releasing hormone (GnRH) analog (n=13) were compared with those not treated (con-trol group, n=11). Patients: Longitudinal data on the somatic development of 24 now adult female patients were analyzed. Results: Medium final height was 157.2 +/- 6.5 cm compared to 151.4 +/- 5.6 cm in the control group. No significant difference could be found in the discrepancy of genetic target height and final height. Prepubertally girls were normal weight in both groups; in adulthood the majority of patients were overweight/obese. Menarche commenced 11 months after cessation of therapy. Conclusion: As already known from other studies, hormonal suppression via GnRH analog was well tolerated.
引用
收藏
页码:1363 / 1367
页数:5
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