Bolu S E, Tasar M, Uçkaya G, Gönül E, Deniz F, Ozdemir I C
Department of Endocrinology and Metabolism, School of Medicine, Gühane Military Medical Academy, Ankara, Türkiye.
J Endocrinol Invest. 2004 Dec;27(11):1029-33. doi: 10.1007/BF03345305.
Idiopathic hypogonadotropic hypogonadism (IHH) is a well-known disorder apart from its pathogenesis, which is still mostly unclear, even though a diverse subgroup of patients with hypogonadotropic hypogonadism and hyposmia/anosmia--the Kallman syndrome--have been partly linked to a mutated gene, known as kal-1 gene. In this study, we aimed to evaluate the sellar region of patients with IHH on magnetic resonance (MR). Pituitary MR of 120 male patients with IHH, diagnosed by a thorough endocrinologic assessment, were compared with pituitary MR of 49 healthy cases selected randomly who underwent detailed endocrinologic and neurologic evaluation and were assessed as healthy. Patients with IHH were diagnosed with microadenomas and irregularly contrasting pituitary (ICP), 18.2 and 10.7%, respectively. Although some anatomic variations were seen in healthy controls, microadenomas and ICP had solely been observed in patients with IHH and none in controls. Intact appearence of hypophysis in patients with IHH was significantly lower than in randomly selected healthy male subjects (p = 0.021). Mean infundibulum width of hypophysis and transverse diameter of posterior hypophysis were significantly broader in patients with IHH than in controls (both having p < 0.001), while mean hypophysis height and volume did not differ between groups. Results showed unusual incidence of pituitary abnormalities on pituitary MR in male patients with IHH. In conclusion, MR imaging is particularly useful in defining the morphological aspects of the hypothalamo - pituitary region in some endocrine disorders and other researchers might want to bear our findings in mind when performing MR evaluation of similar patient subgroups.
特发性低促性腺激素性性腺功能减退症(IHH)是一种广为人知的疾病,但其发病机制仍大多不明,尽管低促性腺激素性性腺功能减退症伴嗅觉减退/嗅觉丧失的不同亚组患者——卡尔曼综合征——已部分与一种名为kal-1基因的突变基因相关联。在本研究中,我们旨在通过磁共振(MR)评估IHH患者的蝶鞍区。对120例经全面内分泌评估诊断为IHH的男性患者的垂体MR进行了比较,这些患者与49例随机选择的健康对照者的垂体MR进行了比较,后者接受了详细的内分泌和神经学评估并被评估为健康。IHH患者中分别有18.2%和10.7%被诊断为微腺瘤和垂体形态不规则(ICP)。虽然在健康对照者中观察到一些解剖变异,但仅在IHH患者中观察到微腺瘤和ICP,对照者中未观察到。IHH患者垂体外观完整的比例显著低于随机选择的健康男性受试者(p = 0.021)。IHH患者垂体柄平均宽度和垂体后叶横径显著宽于对照者(两者p均<0.001),而垂体平均高度和体积在两组之间无差异。结果显示,IHH男性患者垂体MR上垂体异常的发生率异常。总之,MR成像在确定某些内分泌疾病下丘脑 - 垂体区域的形态学方面特别有用,其他研究人员在对类似患者亚组进行MR评估时可能需要牢记我们的发现。