结论:Hair-AN综合征,多毛症的共存,胰岛素抵抗(IR)和黑棘皮病,构成了一种罕见的病态实体。它的特点是临床和生化高雄激素血症伴有严重的胰岛素抵抗,慢性无排卵和代谢异常。字面上,HAIR-AN代表多囊卵巢综合征(PCOS)的极端情况。在日常实践中,HAIR-AN的管理构成了对现有药物的治疗挑战。具体来说,二甲双胍给药不能显着改善IR的程度,而长期服用口服避孕药与代谢谱恶化有关。利拉鲁肽和艾塞那肽,与二甲双胍合用,已被用于治疗严重肥胖的PCOS妇女,结果令人满意。基于这个概念,我们给5名有头发的女性开了利拉鲁肽.在所有参与者中,IR的程度有了显著改善,脂肪沉积,观察雄激素水平和月经周期模式,最小的体重减轻。此外,一名妇女在利拉鲁肽治疗期间怀孕,生下一个健康的孩子。因此,我们得出的结论是,利拉鲁肽是HAIR-AN妇女管理的有效替代方案。
结论:HAIR-AN管理具有挑战性,经典治疗方案无效。从字面上看,Hair-AN综合征,多毛症的共存,胰岛素抵抗和黑棘皮病,代表多囊卵巢综合征的极端情况。在Hair-AN的情况下,利拉鲁肽是一种有效和安全的选择。
CONCLUSIONS: HAIR-AN syndrome, the coexistence of Hirsutism, Insulin Resistance (IR) and Acanthosis Nigricans, constitutes a rare nosologic entity. It is characterized from clinical and biochemical hyperandrogenism accompanied with severe insulin resistance, chronic anovulation and metabolic abnormalities. Literally, HAIR-AN represents an extreme case of polycystic ovary syndrome (PCOS). In everyday practice, the management of HAIR-AN constitutes a therapeutic challenge with the available pharmaceutical agents. Specifically, the degree of IR cannot be significantly ameliorated with metformin administration, whereas oral contraceptives chronic administration is associated with worsening of metabolic profile. Liraglutide and exenatide, in combination with metformin, have been introduced in the management of significantly obese women with PCOS with satisfactory results. Based on this notion, we prescribed liraglutide in five women with HAIR-AN. In all participants a significant improvement regarding the degree of IR, fat depositions, androgen levels and the pattern of menstrual cycle was observed, with minimal weight loss. Furthermore, one woman became pregnant during liraglutide treatment giving birth to a healthy child. Accordingly, we conclude that liraglutide constitutes an effective alternative in the management of women with HAIR-AN.
CONCLUSIONS: HAIR-AN management is challenging and classic therapeutic regimens are ineffective. Literally HAIR-AN syndrome, the coexistence of Hirsutism, Insulin Resistance and Acanthosis Nigricans, represents an extreme case of polycystic ovary syndrome. In cases of HAIR-AN, liraglutide constitutes an effective and safe choice.