关键词: adolescents hyperandrogenism ovarian hyperthecosis pre-menopausal virilisation

Mesh : Humans Female Adolescent Hyperandrogenism / drug therapy pathology Ovarian Diseases / pathology drug therapy complications Gonadotropin-Releasing Hormone / agonists analogs & derivatives Testosterone / blood Prognosis Hirsutism / drug therapy etiology

来  源:   DOI:10.1515/jpem-2024-0223

Abstract:
OBJECTIVE: Ovarian hyperthecosis (OHT) is a rare cause of severe hyperandrogenism in the adolescent age group. We describe two case reports, and present an approach to management in this age group based on a review of the literature.
METHODS: Patient A presented at age 13 years with a 2 year history of androphonia and hirsuitism. Her testosterone level was elevated at 8.3 nmol/L, and there was marked enlargement of her ovaries bilaterally. There were no focal adrenal or ovarian lesions identified on imaging. She was treated with a gonadotropin releasing hormone (GnRH) agonist and spironolactone with biochemical and clinical improvement. Patient B presented at age 14 years with secondary amenorrhoea, and a 2 year history of androphonia, hirsutism and androgenetic alopecia. Her testosterone level was 12 nmol/L, and a pelvic ultrasound revealed numerous follicles in each ovary which were otherwise normal in size. She was managed with GnRH agonist initially, and now continues on a combined oral contraceptive pill.
CONCLUSIONS: Ovarian hyperthecosis needs to be considered in pre-menopausal women presenting with severe hyperandrogenism, after exclusion of androgen-producing adrenal and ovarian tumours. The principles of management in this age group are gonadotropin suppression and hormone replacement.
摘要:
目标:卵巢增生病(OHT)是青少年年龄组中严重高雄激素血症的罕见原因。我们描述了两个病例报告,并在文献综述的基础上提出了这个年龄段的管理方法。
方法:患者A在13岁时出现,有2年的安隆病和多毛症病史。她的睾酮水平升高到8.3nmol/L,她的卵巢两侧明显增大。影像学上没有发现局灶性肾上腺或卵巢病变。她接受了促性腺激素释放激素(GnRH)激动剂和螺内酯治疗,生化和临床改善。患者B在14岁时出现继发性闭经,并有2年的安罗汉病病史,多毛症和雄激素性脱发。她的睾酮水平是12nmol/L,盆腔超声显示每个卵巢中有许多大小正常的卵泡。她最初是用GnRH激动剂治疗的,现在继续服用联合口服避孕药。
结论:在出现严重高雄激素血症的绝经前妇女中,需要考虑卵巢增生。排除产生雄激素的肾上腺和卵巢肿瘤后。该年龄组的治疗原则是促性腺激素抑制和激素替代。
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