Clinical Data Analysis Of 17 Idiopathic Hypogonadotropic Hypogonadism

Objective: To retrospectively study the clinical data of patients with idiopathic hypogonadotropic hypogonadism（IHH）treated in the Department of Endocrinology and Metabolism of Lanzhou University Second Hospital from August 2012 to March2021,which mainly included: Basic clinical characteristics,laboratory examination results,IHH related metabolic diseases and their influencing factors,treatment methods,treatment effects were observed and analyzed to deepen and popularize the understanding of IHH,provide timely and reasonable diagnosis and treatment for IHH,and improve the quality of life and fertility of patients with IHH.Methods: Collection,analysis,the general clinical data of 17 cases of IHH patients,non-reproductive clinical features,analysis of different hormone levels in patients with sexual development level differences and the correlation between hormone levels and secondary development,synchronous analysis IHH patients with insulin resistance index,metabolic correlation fatty liver disease and the incidence of bone metabolic index and influencing factors,Imaging examination results such as pituitary MRI were analyzed.Finally,according to the follow-up results,the therapeutic effects of patients were analyzed and compared.Results: Among the 17 IHH patients enrolled,all of them were male,and 13（76.47%）were patients with normal sense of smell hypogonadotropic hypogonadism（n IHH）.There were 4 patients（23.53%）with Kallmann syndrome（KS）,mainly due to secondary sex sign retardation,cryptorchidism,male mammary gland development and progressive weight increase.Most of the patients were at Tanner I～ Ⅱ stage of masculinity development.The included patients were also accompanied by unilateral renal dysplasia,renal developmental malformation,hydronephrosis,syndactyly,amblyopia,hearing loss and congenital megacolon and other non-reproductive developmental malformations.IHH patients were also prone to osteoporosis and even pathologic fracture.Tanner staging was positively correlated with luteinizing hormone（LH）and testosterone（T）.There were no statistically significant differences in age,BMI and testicular volume between the KS group and the n IHH group（P >0.05）;there were statistically significant differences in height at first visit between the two groups（P < 0.05）;there were no statistically significant differences in FSH,LH and T hormone levels between the two groups（P > 0.05）.However,the levels of FSH,LH and T in both groups were lower than the normal reference range.Insulin resistance was found in 9 patients,and there was a positive correlation between insulin resistance and BMI in IHH patients.Testosterone replacement therapy can promote and maintain masculinity characteristics.After 6-8 months of h CG/HMG treatment,the hormone levels were basically normal,the level of secondary sexual characteristics was almost close to adults,and the voice became deeper,but there was no Adam’s apple development.After 1 year of Gn RH pump treatment,spermatogenesis effect was obvious.Conclusion:（1）IHH is rare in clinic,if missing the stage of sexual development,it will affect development and fertility.（2）Timely,reasonable and personalized treatment can improve the adolescent development and fertility function of patients.