男性Kallmann综合征与特发性低促性腺激素性性腺功能减退症的性腺轴功能评价  

Evaluation of Gonadal Axis Function in Male Patients with Kallmann Syndrome and Idiopathic Hypogonadotropic Hypogonadism.

作　　者：张娜娜 王琼 戚瑞 李晓苗 Zhang Nana;Wang Qiong;Qi Rui(Department of Endocrinology,Xijing Hospital,Air Force Medical University,Xi an,Shanxi 710032,China.)

机构地区：[1]空军军医大学西京医院内分泌代谢科,陕西西安710032

出　　处：《四川医学》2023年第9期963-967,共5页Sichuan Medical Journal

摘　　要：目的评价男性Kallmann综合征(KS)与特发性低促性腺激素性性腺功能减退症(IHH)的性腺轴功能。方法分析我科2013年至2021年50例KS及61例IHH患者的临床表现及GnRH、HCG兴奋试验特点。结果两组患者均表现为性发育不良,第二性征缺失,睾丸阴茎小及低促性腺激素性性腺功能减退。KS患者伴嗅觉减退或缺失。KS组平均睾丸体积、基础FSH及LH值均低于IHH组,组间比较差异有统计学意义。GnRH兴奋试验:KS组的LH峰值3.13 IU/L,FSH峰值3.31 IU/L。IHH组的LH峰值4.76 IU/L,FSH峰值3.68 IU/L。HCG兴奋试验:T峰值在72 h,KS组的T峰值62.82 ng/dl,IHH组的T峰值118.44 ng/dl。KS组的LH及T峰值均低于IHH组,组间比较差异有统计学意义。隐睾组的GnRH兴奋试验与HCG兴奋试验的峰值均低于与正常睾丸组,两组间差异有统计学意义。结论KS与IHH患者均表现为低促性腺激素性性腺功能减退,KS患者伴嗅觉减退或缺失,两组在基础FSH、LH值及GnRH及HCG兴奋试验中的峰值差异有统计学意义。睾丸的状态一定程度反映了性腺轴的储备功能。Objective To evaluate gonadal axis function of male Kallmann syndrome(KS)and idiopathic hypogonadotropic hypogonadism(IHH).Methods From 2013 to 2021,clinical manifestations and GnRH/HCG stimulation tests of 50 patients with KS and 61 patients with IHH in our department were summarized.Results Both groups showed sexual dysplasia,deficiency of secondary sexual characteristics,small testis and penis and hypogonadotropic hypogonadism.KS was with Hyposmia or anosmia.The average testicular volume in KS group was smaller than that in IHH group,basal FSH and LH in KS group were lower than that in IHH group and there were significant differences.GnRH stimulation test was that in KS group,peak values of LH was 3.13IU/L and FSH was 3.31 IU/L.In IHH group,peak values of LH was 4.76 IU/L and FSH was 3.68 IU/L.T peak of HCG stimulation test was 72 h,T peak of KS group was 62.82 ng/dl and T peak of IHH group was 118.44 ng/dl.Peak values of LH and T in IHH patients were lower than those in KS patients,and there were significant differences.Peak values of GnRH stimulation test and HCG stimulation test in cryptorchidism group were lower than those in normal testis group,and there was significant difference.Conclusion Both patients with KS and IHH show hypogonadotropic hypogonadism.KS Patients would be with Hyposmia or anosmia.There are significant differences in the basic FSH,LH and GnRH/HCG stimulation tests.The state of testis reflects the reserve function of the gonadal axis to some extent.

关 键 词：KALLMANN综合征 特发性低促性腺激素性性腺功能减退症 性发育不良 嗅觉减退 

分 类 号：R584.2[医药卫生—内分泌]