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Premature Pubarche before One Year of Age: Distinguishing between Mini-Puberty Variants and Precocious Puberty

机译:一岁前的过早青春期:区分小青春期变体和早熟青春期

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Background The aim of this study was to facilitate the distinction between the benign “mini-puberty of early infancy” and precocious puberty (PP). Material and Methods We compared 59 patients (21 boys and 38 girls) seen for pubic hair development before one year of age diagnosed as mini-puberty to 13 patients (2 boys) in whom pubertal development before one year revealed a PP. Results The boys with mini-puberty presented with pubic hair development and prepubertal testicular volume, with low plasma testosterone concentrations. Their gonadotropin responses to gonadotropin releasing hormone (GnRH) test showed predominant luteinising hormone increase in 9/13. The girls presented with pubic hair development that was accompanied by breast development in 47% of cases, with low plasma estradiol concentrations. Their gonadotropin responses showed predominant follicle-stimulating hormone increase in the 17 evaluated.The patients with PP had organic central PP (5 hypothalamic hamartoma) or idiopathic central PP (n=6), or peripheral PP (one ovarian tumor and one congenital adrenal hyperplasia). The diagnosis was challenging only in 3 girls with idiopathic central PP presenting with prepubertal plasma estradiol concentrations and responses to GnRH test. Conclusions The diagnosis of PP was easily determined based on the clinical presentation and the pubertal concentrations of testosterone in boys or of estradiol in girls, as was the diagnosis of central or peripheral origin of PP based on gonadotropin response to the GnRH test. Once PP is excluded, these patients need careful follow–up and physician consultation is needed if clinical pubertal signs progress.
机译:背景技术这项研究的目的是促进区分良性的“婴儿早期青春期”和性早熟(PP)。材料和方法我们将59岁(21岁男孩和38名女孩)在一岁之前被诊断为青春期发育的阴毛发育与13岁(2岁男孩)的一年前青春期发育揭示PP的患者进行了比较。结果迷你青春期男孩表现为阴毛发育和青春期前睾丸体积,血浆睾丸激素浓度低。他们的促性腺激素对促性腺激素释放激素(GnRH)测试的反应表明,主要的促黄体生成激素增加9/13。在47%的病例中,这些女孩表现为耻骨发育并伴有乳房发育,血浆雌二醇浓度较低。他们的促性腺激素反应显示在所评估的17位中主要是刺激卵泡的激素增加.PP患者患有器质性中枢性PP(5个下丘脑错构瘤)或特发性中枢性PP(n = 6)或周围性PP(一个卵巢肿瘤和一个先天性肾上腺增生) )。诊断仅对3名特发性中枢性PP的女孩具有青春期前血浆雌二醇浓度和对GnRH测试的反应具有挑战性。结论根据临床表现和男孩中睾丸激素的青春期浓度或女孩中雌二醇的青春期浓度可以容易地确定PP的诊断,基于对GnRH试验的促性腺激素反应对PP的中枢或外周起源的诊断也很容易。一旦排除了PP,这些患者需要仔细随访,如果临床青春期体征发展,则需要医生咨询。

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