Физиологическое действие гипоталамо-гипо-физарно-гонадной оси основывается на пульсиру-ющей гипоталамической секреции гонадолиберина (ГнРГ), нарушение которой является причиной ред-ких заболеваний, объединяемых под термином ги-погонадизм. Отсутствие ГнРГ приводит к дезакти-вации рецептора на поверхности гонадотрофов аде-ногипофиза и выпадению стимулирующего дей-ствия на синтез и высвобождение гонадотропинов (ЛГ и ФСГ) в общий кровоток. Дефицит ЛГ и ФСГ обусловливает отсутствие гаметогенеза и синтеза половых стероидов. Таким образом, нормальное функционирование репродуктивной системы зави-сит от скоординированной работы трех звеньев: ар-куатных ядер гипоталамуса, гонадотрофов гипофиза и гонад (рис. 1).В зависимости от сроков возникновения разли-чают врожденный и приобретенный гипогонадизм, а в зависимости от уровня поражения -первичный (поражение самих гонад) и вторичный (гипоталамо-гипофизарный) гипогонадизм. Существует также классификация, разделяющая поражения гипофиза и гипоталамуса на вторичный и третичный уровень
Central hypogonadism (CH) is a rare endocrine disorder caused by the disfunction of production, secretion and/or biological action of gonadotropin-releasing hormone (GnRH), which is the main hormonal regulator of hypothalamo-pituitarygonadal axis in human. Female CH is important medical and social concern due to large amount of infertile couples. Etiological structure of this condition is heterogeneous and diff ers between congenital and acquired forms. Congenital forms have a genetic predisposition: currently about 50 genes associated with CH have been found. However, genetic basis can be identifi ed just in half of CH cases. Speaking about acquired forms of CH, important to pay attention on hypothalamo-pituitary area condition. In case of intact state the functional form of CH can be diagnosed, the presence of structural disorders in this area speaks in favor of the organic cause of CH. In this review are summarized current knowledge in the fi eld of etiology and pathogenesis of female central hypogonadism.
Background: Central hypogonadism (CH) is a syndrome characterized by low levels of peripheral sex steroid hormones due to the lack of central (hypothalamic-pituitary) regulation of reproductive system. In females, CH clinically manifests by amenorrhea, anovulation, and infertility. The classical diagnostic criteria of CH in the absence of organic disease of hypothalamic-pituitary region (“idiopathic” CH) include low gonadotropin levels; however, their levels within the reference ranges do not exclude CH. Moreover, reference ranges for these parameters are different between laboratories. Thus, currently no clear laboratory diagnostic criteria for female CH are available.Aim: To determine the diagnostic value of luteinizing hormone (LH) and follicle-stimulating hormone (FSH) basal levels for the confirmation of CH diagnosis in women and to identify cutoffs of basal gonadotropins levels, which can be considered as diagnostic criteria for female CH.Materials and methods: This cross-sectional study included 87 women: 49 with confirmed CH, aged 18 to 36 years (median, 24 [Q21; Q29]), and 38 healthy fertile women with regular menstrual cycles aged 21 to 45 years (median, 23 [Q23; Q28]). In all subjects, LH, FSH, estradiol, testosterone, prolactin, and free thyroxin levels were measured by chemiluminiscent immunoassay.Results: LH, FSH, estradiol, and prolactin levels in the CH patients were significantly lower than those in healthy subjects. The ROC analysis showed that LH level ≤ 1.95 ME/l indicated the central genesis of hypogonadism with sensitivity of 81.25% and specificity of 91.89%. Basal FSH level ≤ 5.075 ME/l had a 70.00% sensitivity and 77.14% specificity for CH diagnosis.Conclusion: Basal LH level ≤ 1.95 ME/l measured by chemiluminiscent immunoassay can be considered as an idiopathic CH diagnostic criterion in female with amenorrhea due to the hypoestrogenemia with sensitivity of > 80% and specificity > 90%.
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