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首页> 外文期刊>Journal of Clinical and Diagnostic Research >Clinicohormonal Parameters as a PrimaryStep to Differentiate Normosmic IdiopathicHypogonadotropic Hypogonadism andKallmann Syndrome in a Tertiary CareHospital in Eastern Indi
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Clinicohormonal Parameters as a PrimaryStep to Differentiate Normosmic IdiopathicHypogonadotropic Hypogonadism andKallmann Syndrome in a Tertiary CareHospital in Eastern Indi

机译：临床潜在参数作为一种小学，以区分Normosmic IdiopathichynogoOnodopoOnoStropic Hypoconadism和Kallmann综合征在东部的第三次Carehospitali

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Idiopathic hypogonadotropic hypogonadism is a rare gonadal dysgenesis in which puberty does not take place naturally. It occurs due to insufficient pulsatile secretion of Gonadotrophin-Releasing Hormone (GnRH) and the resulting Follicle-Stimulating Hormone (FSH) and Luteinising Hormone (LH) deficiency leads to absence of or delayed sexual maturation. Kallmann syndrome is an uncommon genetic disorder characterised by hypogonadotropic hypogonadism associated with anosmia or hyposmia. When anosmia is absent, the same is referred as normosmic Idiopathic Hypogonadotropic hypogonadism (nIHH).Aim: To find out the significant differences between Kallmann syndrome and nIHH based on clinical features and biochemical assessment as a primary measure to initiate the treatment early.Materials and Methods: This hospital based cross-sectional observational cohort study was conducted in Department of Endocrinology, Nilratan Sircar Medical College and Hospital, Kolkata, India. The study was done on 55 cases of IHH presenting to the department with delayed secondary sexual characteristics.Results: Out of these 55 cases, 45 (81.8%) were of nIHH and only 10 (18.2%) cases were of Kallmann Syndrome. It was found that both the conditions show male predominance. Smell abnormalities were present only in Kallmann group. The level of serum testosterone was significantly higher (p<0.05) in nIHH subjects (mean-35.59 ng/dL) than patients with Kallmann Syndrome (mean-14.90 ng/dL). Patients with Kallmann syndrome showed significantly reduced pubic and axillary hair development and absence of gonadal development.Conclusion: Absence of puberty with anosmia/hyposmia with low serum FSH and LH, drastically reduced serum testosterone, are factors that point towards the diagnosis of Kallmann syndrome even in absence of genetic study, which is helpful for initiation of hormone replacement therapy for treatment.
机译：特发性低血管同时性低因素是一种罕见的性腺功能因子，其中Puerty不会自然地发生。它由于促性腺激素释放激素（GNRH）的脉动分泌不足，并且所得的卵泡刺激激素（FSH）和刻度引发激素（LH）缺乏导致性成熟而导致缺乏或延迟的性成熟。 Kallmann综合征是一种罕见的遗传疾病，其特征在于与Anosmia或hyposmia相关的低血糖同向增不作用性。当Anosmia不存在时，相同的是常规发作性低因素过度动力学（NIHH）.aum：根据临床特征和生物化学评估，以临床特征和生物化学评估为主要措施，了解Kallmann综合征和Nihh之间的显着差异。方法：该医院横截面观察队列研究是在内分泌学，尼尔拉特兰Sircar医学院和医院，加尔各答，印度加州山区进行。这项研究是在第55例IHH呈现给部门的二级性特征。结果：在这55例中，45例（81.8％）是NIHH，只有10（18.2％）的病例是Kallmann综合征。结果发现，条件都显示出男性优势。异味异常只存在于Kallmann组。血清睾酮水平在NIHH受试者（平均-35.59ng / dl）明显高（P <0.05），比Kallmann综合征患者（平均-14.90 ng / dl）。 Kallmann综合征患者显着降低了耻骨和腋毛发育和缺乏性腺发育。结论：患有低血清FSH和LH的血清症和LH的青春痘，血清睾酮的缺失甚至是血清睾酮的因素甚至在没有遗传学研究的情况下，这有助于启动激素替代治疗治疗。

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