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Abstract：
Puberty in children with Prader-Willi syndrome (PWS) is usually delayed and/or incomplete but in some patients premature/early adrenarche is observed. We assessed the premature adrenarche (PA) in PWS patients during the recombinant human growth hormone (rhGH) therapy and influence of PA on the course of central puberty (CP), rhGH efficacy and safety, and patients\' metabolic state. Forty-nine PWS patients were treated with rhGH, 11 presented with PA (group 1) and 14 had normal course of adrenarche (group 2). PA was observed in 22.5% of the PWS children treated with rhGH. The mean time between the rhGH start and the adrenarche, the rhGH dose, the growth velocity and the insulin-like growth factor 1 SD (IGF1 SD) during the treatment, as well as the time of CP, final height SD and BMI SD were similar in both groups. There were also no significant differences in the metabolic assessment-the oral glucose tolerance test (OGTT) and lipid profile results. PA may be a part of the clinical picture of PWS, apart from hypogonadotrophic hypogonadism and it seems to have no influence on CP in PWS patients. The rhGH efficacy and safety were comparable in the patients with PA and the normal course of adrenarche.
摘要：
Prader-Willi综合征（PWS）儿童的青春期通常是延迟和/或不完整的，但在某些患者中观察到过早/早期的肾上腺素。我们评估了PWS患者在重组人生长激素（rhGH）治疗期间的早熟肾上腺素（PA）以及PA对中央青春期（CP）过程的影响，rhGH的疗效和安全性，和患者的代谢状态。49名PWS患者接受rhGH治疗，11例表现为PA（第1组），14例表现为正常的肾上腺素（第2组）。在接受rhGH治疗的PWS儿童中，有22.5％观察到PA。rhGH开始和肾上腺素之间的平均时间，rhGH剂量，治疗期间的生长速度和胰岛素样生长因子1SD（IGF1SD），以及CP的时间，两组最终身高SD和BMISD相似。代谢评估-口服葡萄糖耐量试验（OGTT）和血脂谱结果也没有显着差异。PA可能是PWS临床表现的一部分，除了促性腺激素低，似乎对PWS患者的CP没有影响。在PA患者和正常过程中，rhGH的疗效和安全性具有可比性。
