Predictors of reproductive and non-reproductive outcomes of gonadotropin mediated pubertal induction in male patients with congenital hypogonadotropic hypogonadism (CHH).
Predictors of reproductive and non-reproductive outcomes of gonadotropin mediated pubertal induction in male patients with congenital hypogonadotropic hypogonadism (CHH).
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DOI：
10.1007/s40618-021-01556-x
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发表时间：
2021-11
影响因子：
5.4
通讯作者：
Bonomi M
中科院分区：
文献类型：
--
作者：
Cangiano B;Goggi G;Federici S;Bresesti C;Cotellessa L;Guizzardi F;Vezzoli V;Duminuco P;Persani L;Bonomi M
目的探讨促性腺激素诱导的先天性性腺功能低下症(CHH)青春期后睾丸反应和非生殖结局(身高、体重比例)的预测因素。一项对CHH男性患者青春期诱导的回顾分析，这些患者接受了联合应用促性腺激素(FSH和hCG)。19名CHH患者在促性腺激素刺激前和刺激期间的临床和激素评估；16名患者还接受了促性腺激素刺激后的精液分析。诱导24个月后睾丸体积较小的增加与：(I)隐睾症；(Ii)阳性遗传背景；(Iii)完全型CHH显著相关。我们发现与24个月内给予hCG的累积剂量没有显著相关性。我们发现与精液分析结果没有关联，可能是因为精子数量较少。身体比例失调的衡量标准(优节状生境和成人与目标身高之差：deltaSDSth)与以下因素密切相关：(I)青春期诱导开始时的年龄；(Ii)诱导期间的生长持续时间；(Iii)初始骨龄。诱导期的生长持续时间与之前注射睾丸素和部分形式的CHH有关。这项研究表明，强烈的遗传背景和隐睾症，作为宫内生活以来GnRH完全缺乏的指标，是CHH患者睾丸对促性腺激素刺激反应的负面预测因素。身体比例失调与诱导过程中的治疗延迟和生长持续时间有关，而这显然与先前的雄激素释放呈负相关。网上版载有补充材料，可在10.1007/s40618-021-01556-x查阅。
To investigate predictors of testicular response and non-reproductive outcomes (height, body proportions) after gonadotropin-induced puberty in congenital hypogonadotropic hypogonadism (CHH). A retrospective analysis of the puberty induction in CHH male patients, undergoing an off-label administration of combined gonadotropin (FSH and hCG). Clinical and hormonal evaluations before and during gonadotropin stimulation in 19 CHH patients genotyped by Targeted Next Generation Sequencing for CHH genes; 16 patients underwent also semen analysis after gonadotropins. A lesser increase in testicular volume after 24 months of induction was significantly associated with: (I) cryptorchidism; (II) a positive genetic background; (III) a complete form of CHH. We found no significant correlation with the cumulative dose of hCG administered in 24 months. We found no association with the results of semen analyses, probably due to the low numerosity. Measures of body disproportion (eunuchoid habitus and difference between adult and target height: deltaSDSth), were significantly related to the: (I) age at the beginning of puberty induction; (II) duration of growth during the induction; (III) initial bone age. The duration of growth during induction was associated with previous testosterone priming and to partial forms of CHH. This study shows that a strong genetic background and cryptorchidism, as indicators of a complete GnRH deficiency since intrauterine life, are negative predictors of testicular response to gonadotropin stimulation in CHH. Body disproportion is associated with a delay in treatment and duration of growth during the induction, which is apparently inversely related to previous androgenization. The online version contains supplementary material available at 10.1007/s40618-021-01556-x.
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