Abstract：
OBJECTIVE: Children with Prader-Willi Syndrome (PWS) may develop premature pubarche (PP). We investigated the frequency of PP, and its potential precursors and sequelae, in PWS.
METHODS: A chart review of children with PWS treated at our institution between 1990 and 2021 was performed. PP was defined as Tanner stage 2 (TS2) pubic hair in girls <8 and boys <9 years old. Demographic, anthropometric, and laboratory data were collected to assess predisposing factors and consequences of PP in comparison to patients with PWS who had normal pubarche (NP).
RESULTS: Analysis included 43 children with PWS, 23 (53.5%) with PP and 20 (46.5%) with NP. Median age at pubarche was 7.0 years in PP group and 10.0 years in NP group. Age at pubarche was not correlated with age of recombinant human growth hormone (rhGH) initiation, body mass index (BMI) z-score, or homeostasis model assessment of insulin resistance (HOMA-IR) at pubarche. BMI z-score at pubarche was modestly correlated with degree of pubarchal BA advancement (p = 0.033). Those with PP were more likely to have a lower high-density lipoprotein (HDL) (1.05 mmol/L vs. 1.41 mmol/L in the NP group, p = 0.041). The difference between target and final height did not differ between groups (p = 0.507).
CONCLUSIONS: PP is common in PWS but does not compromise final height in comparison to the NP group. Obesity and insulin resistance were not associated with PP in children with PWS, contrary to what has been seen in obese children without PWS.
METHODS: A chart review of children with PWS treated at our institution between 1990 and 2021 was performed. PP was defined as Tanner stage 2 (TS2) pubic hair in girls <8 and boys <9 years old. Demographic, anthropometric, and laboratory data were collected to assess predisposing factors and consequences of PP in comparison to patients with PWS who had normal pubarche (NP).
RESULTS: Analysis included 43 children with PWS, 23 (53.5%) with PP and 20 (46.5%) with NP. Median age at pubarche was 7.0 years in PP group and 10.0 years in NP group. Age at pubarche was not correlated with age of recombinant human growth hormone (rhGH) initiation, body mass index (BMI) z-score, or homeostasis model assessment of insulin resistance (HOMA-IR) at pubarche. BMI z-score at pubarche was modestly correlated with degree of pubarchal BA advancement (p = 0.033). Those with PP were more likely to have a lower high-density lipoprotein (HDL) (1.05 mmol/L vs. 1.41 mmol/L in the NP group, p = 0.041). The difference between target and final height did not differ between groups (p = 0.507).
CONCLUSIONS: PP is common in PWS but does not compromise final height in comparison to the NP group. Obesity and insulin resistance were not associated with PP in children with PWS, contrary to what has been seen in obese children without PWS.
摘要：
目的：Prader-Willi综合征（PWS）患儿可能会出现性早熟（PP）。我们调查了PP的频率，及其潜在的前体和后遗症，在PWS中。
方法：对1990年至2021年在我们机构治疗的PWS儿童进行了图表回顾。PP定义为<8岁的女孩和<9岁的男孩的Tanner2期(TS2)阴毛。人口统计,人体测量学,并收集了实验室数据，以评估PP的诱发因素和后果，与具有正常耻骨（NP）的PWS患者相比。
结果：分析包括43名PWS儿童，23(53.5%)与PP和20(46.5%)与NP。PP组青春期中位年龄为7.0岁，NP组为10.0岁。青春期的年龄与重组人生长激素（rhGH）起始的年龄无关，体重指数（BMI）z评分，或体内平衡模型评估胰岛素抵抗（HOMA-IR）在阴部。青春期的BMIz评分与青春期的BA进展程度适度相关（p=0.033）。那些有PP的人更可能有较低的高密度脂蛋白（HDL）（1.05mmol/L与NP组1.41mmol/L,p=0.041)。目标身高和最终身高之间的差异在组间没有差异(p=0.507)。
结论：PP在PWS中很常见，但与NP组相比，不会损害最终身高。肥胖和胰岛素抵抗与PWS儿童的PP无关。与没有PWS的肥胖儿童相反。
方法：对1990年至2021年在我们机构治疗的PWS儿童进行了图表回顾。PP定义为<8岁的女孩和<9岁的男孩的Tanner2期(TS2)阴毛。人口统计,人体测量学,并收集了实验室数据，以评估PP的诱发因素和后果，与具有正常耻骨（NP）的PWS患者相比。
结果：分析包括43名PWS儿童，23(53.5%)与PP和20(46.5%)与NP。PP组青春期中位年龄为7.0岁，NP组为10.0岁。青春期的年龄与重组人生长激素（rhGH）起始的年龄无关，体重指数（BMI）z评分，或体内平衡模型评估胰岛素抵抗（HOMA-IR）在阴部。青春期的BMIz评分与青春期的BA进展程度适度相关（p=0.033）。那些有PP的人更可能有较低的高密度脂蛋白（HDL）（1.05mmol/L与NP组1.41mmol/L,p=0.041)。目标身高和最终身高之间的差异在组间没有差异(p=0.507)。
结论：PP在PWS中很常见，但与NP组相比，不会损害最终身高。肥胖和胰岛素抵抗与PWS儿童的PP无关。与没有PWS的肥胖儿童相反。
