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首页> 外文期刊>Endocrinology, Diabetes & Metabolism Case Reports >A case of Kallmann syndrome associated with a non-functional pituitary microadenoma
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A case of Kallmann syndrome associated with a non-functional pituitary microadenoma

机译:一例非功能性垂体微腺瘤相关的Kallmann综合征

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SummaryKallmann syndrome (KS) is a form of hypogonadotropic hypogonadism in combination with a defect in sense of smell, due to abnormal migration of gonadotropin-releasing hormone-producing neurons. We report a case of a 17-year-old Tunisian male who presented with eunuchoid body proportions, absence of facial, axillary and pubic hair, micropenis and surgically corrected cryptorchidism. Associated findings included anosmia. Karyotype was 46XY and hormonal measurement hypogonadotropic hypogonadism. MRI of the brain showed bilateral agenesis of the olfactory bulbs and 3.5?mm pituitary microadenoma. Hormonal assays showed no evidence of pituitary hypersecretion.Learning points:The main clinical characteristics of KS include hypogonadotropic hypogonadism and anosmia or hyposmia.MRI, as a non-irradiating technique, should be the first radiological step for investigating the pituitary gland as well as abnormalities of the ethmoid, olfactory bulbs and tracts in KS.KS may include anterior pituitary hypoplasia or an empty sella syndrome. The originality of our case is that a microadenoma also may be encountered in KS. Hormonal assessment indicated the microadenoma was non-functioning. This emphasizes the importance of visualizing the pituitary region in KS patients to assess for hypoplastic pituitary malformations or adenomas.
机译:小结卡尔曼综合症(KS)是促性腺激素减退性性腺功能减退症的一种形式,伴随着嗅觉缺陷,归因于促性腺激素释放激素产生神经元的异常迁移。我们报告了一例17岁的突尼斯男性患者,其表现为太监体比例,面部,腋毛和阴毛,微阴茎和手术矫正隐睾症。相关发现包括失眠。核型为46XY,激素测量性腺功能减退性腺功能减退。脑部MRI显示双侧嗅球发育不全和3.5?mm垂体微腺瘤。激素测定未显示垂体过度分泌的证据。学习要点:KS的主要临床特征包括性腺功能低下性腺功能减退症和失眠或低渗症。MRI作为一种非放射技术,应该是研究垂体及异常腺体的第一步放射学步骤。 KS的筛骨,嗅球和尿道可能包括垂体前叶发育不全或空蝶鞍综合征。我们病例的独创性是在KS中也可能遇到微腺瘤。激素评估表明微腺瘤无功能。这强调了在KS患者中观察垂体区域以评估垂体发育不良或腺瘤发育的重要性。

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