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一种针对家族性部分脂肪营养不良所致自发性卵巢过度刺激综合征的有前景的治疗方法:促性腺激素释放激素类似物联合囊肿抽吸术。

A promising treatment for spontaneous ovarian hyperstimulation syndrome due to familial partial lipodystrophy: GnRH analogs combined with cyst aspiration.

作者信息

Gökçay Canpolat Asena, Aslan Batuhan, Şükür Yavuz Emre

机构信息

Department of Endocrinology and Metabolism, Ankara University School of Medicine, Ankara, Turkey.

Department of Obstetrics and Gynaecology, Ankara University School of Medicine, Ankara, Turkey.

出版信息

Hormones (Athens). 2023 Dec;22(4):741-745. doi: 10.1007/s42000-023-00469-z. Epub 2023 Jul 25.

Abstract

PURPOSE

To present a patient with familial partial lipodystrophy (FPLD) and polycystic ovary syndrome (PCOS) who was admitted with spontaneous ovarian hyperstimulation syndrome (OHSS)-like extremely enlarged ovaries, which was successfully treated using gonadotropin-releasing hormone analogs and abdominal cyst aspiration in combination.

METHOD

This is a descriptive case report of a single patient with FPLD and PCOS.

RESULTS

Clinical improvement was achieved 6 months after therapy besides progressive reduction in total testosterone and DHEAS. Furthermore, there was a significant improvement in hyperinsulinemia and hypertriglyceridemia. Additionally, reduction in the size of ovarian cysts, reduction in the size and number of localizations of acanthosis nigricans, reduction in scores of mFGS, and weight loss were also observed.

CONCLUSION

Although there are few reports in the literature describing the association between PCOS with FPLD, management of this novel spontaneous OHSS-like condition has not yet been clearly defined. In the case of extremely enlarged multicystic ovaries and severe hyperandrogenemia, GnRH analogs may be considered to prevent ovarian enlargement and reduce hyperandrogenemia, especially when other treatment options are inappropriate.

摘要

目的

报告一例患有家族性部分脂肪营养不良(FPLD)和多囊卵巢综合征(PCOS)的患者,该患者因自发性卵巢过度刺激综合征(OHSS)样的卵巢极度肿大入院,采用促性腺激素释放激素类似物和经腹囊肿抽吸联合治疗取得成功。

方法

这是一例关于患有FPLD和PCOS的单一患者的描述性病例报告。

结果

治疗6个月后临床症状改善,同时总睾酮和硫酸脱氢表雄酮(DHEAS)逐渐降低。此外,高胰岛素血症和高甘油三酯血症也有显著改善。另外,还观察到卵巢囊肿大小减小、黑棘皮病的大小和部位数量减少、改良的Ferriman-Gallwey评分(mFGS)降低以及体重减轻。

结论

尽管文献中很少有关于PCOS与FPLD关联的报道,但这种新型的自发性OHSS样病症的管理尚未明确界定。对于多囊卵巢极度肿大和严重高雄激素血症患者,尤其是在其他治疗选择不适用时,可考虑使用GnRH类似物来预防卵巢肿大并降低高雄激素血症。

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