摘要
目的探讨成年发生的特发性低促性腺激素性性腺功能减退症(AIHH)的诊疗特点并提供诊疗思路。方法收集就诊于南方医科大学南方医院内分泌代谢科的3例男性AIHH患者的临床、实验室、影像学及完整随访资料。结果 3例患者均为男性,平均年龄39(22~40)岁,2例以"乳房增大,性功能下降"为主诉入院,1例以"性功能下降"为主诉入院。体格检查:阴茎长度6(5~6)cm,双侧睾丸体积7.96(4.70~8.82)mL;基础性激素水平:睾酮0.32(0.24~2.96)ng/mL、卵泡刺激素0.56(0.1~0.75)mIU/mL、黄体生成素0.69(0.1~1.03)mIU/mL;垂体前叶分泌的其他激素水平未见异常;下丘脑-垂体区域磁共振成像(MRI)提示其中1例患者存在垂体微腺瘤。3例患者选择促性腺激素释放激素(GnRH)脉冲泵或促性腺激素序贯治疗,1例患者在使用GnRH脉冲泵治疗后下丘脑-垂体-性腺(HPG)轴功能逆转并持续1年,但随后仍不可逆性降低。结论 AIHH同时具有成年起病及特发性低促性腺激素的特点,生育力改善仍是此类患者的重要需求,GnRH脉冲泵或促性腺激素治疗对AIHH患者具有良好的治疗效果但疗效具有波动性,应及时进行生育力保存。
Objective To investigate the clinical characteristics and offer diagnostic and therapeutic approaches for adult-onset idiopathic hypogonadotropic hypogonadism(AIHH).Methods Clinical,laboratory,and imaging data,as well as follow-up information,of three male patients diagnosed with AIHH at the Department of Endocrinology and Metabolism of Nanfang Hospital,Southern Medical University,were systematically reviewed and analyzed.Results All three patients were male,with a median age of 39 years(range,22 to 40).Two patients reported symptoms of enlarged breasts and reduced sexual function,while one case solely reported a decline in sexual function.Physical examination showed that the median length of the penis was 6 cm(range,5 to 6 cm),and the bilateral testicular volume was 7.96 mL(4.70-8.82 mL).Basal hormone levels at the time of initial visit to our hospital as follows:the median testosterone level was 0.32 ng/mL(0.24-2.96 ng/mL),median follicle stimulating hormone(FSH)level was 0.56 mIU/mL(0.1-0.75 mIU/mL),and the median luteinizing hormone(LH)level was 0.69 mIU/mL(0.1-1.03 mIU/mL).The levels of other hormones secreted by the anterior pituitary gland were normal.Hypothalamic-pituitary magnetic resonance imaging(MRI)showed that 1 patient had a pituitary microadenoma.Three patients were treated with pulsatile GnRH or gonadotropins,one of which had hypothalamic-pituitary-gonadal(HPG)axis function reversal after GnRH pulse pump therapy and lasted for 1 year,but then still had irreversible reduction.Conclusion AIHH is marked by adult-onset disease and idiopathic hypogonadism.Enhancing fertility remains a critical requirement for these patients.Pulsatile GnRH treatment or gonadotropin therapy,as viable treatments,exhibit therapeutic effects,albeit with occasional fluctuations.Therefore,the emphasis lies in the timely consideration of fertility preservation.
作者
骆静
蒲美岑
黄奕娟
王丹
邹梦晨
范新钊
何美男
谢翠华
薛耀明
曹瑛
Luo Jing;Pu Meicen;Huang Yijuan;Wang Dan;Zou Mengchen;Fan Xinzhao;He Meinan;Xie Cuihua;Xue Yaoming;Cao Ying(Department of Endocrinology and Metabolism,Nanfang Hospital of Southern Medical University,Guangzhou 510515,China)
出处
《中华内分泌代谢杂志》
CAS
CSCD
北大核心
2024年第1期5-10,共6页
Chinese Journal of Endocrinology and Metabolism
