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Lancet Diabetes Endocrinol:库欣综合征药物治疗前沿进展

2019-01-26 贾朝娟 环球医学

20世纪50年代,因为缺乏有效治疗手段,库欣综合征患者的平均存活仅4.6年。半个多世纪后的今天,随着医学知识和技术的不断更新和飞速发展,库欣综合征患者的寿命得到大幅延长,但死亡率仍是正常人群的1.7~4.8倍。及时诊断和治疗库欣综合征非常重要。2018年7月,发表在《Lancet Diabetes Endocrinol》的一篇综述对库欣综合征药物治疗最新进展情况进行了阐述。

20世纪50年代,因为缺乏有效治疗手段,库欣综合征患者的平均存活仅4.6年。半个多世纪后的今天,随着医学知识和技术的不断更新和飞速发展,库欣综合征患者的寿命得到大幅延长,但死亡率仍是正常人群的1.7~4.8倍。及时诊断和治疗库欣综合征非常重要。2018年7月,发表在《Lancet Diabetes Endocrinol》的一篇综述对库欣综合征药物治疗最新进展情况进行了阐述。

库欣综合征与多系统疾病相关,当治疗欠佳时,会增加死亡率。若手术治疗不成功,药物治疗则是患者唯一的选择。

药物治疗可分为垂体导向药物、类固醇合成抑制剂和糖皮质激素受体拮抗剂。过去10年,每个种类的药物都有了新的发展。用卡麦角林或帕瑞肽或同时使用两者,靶向促肾上腺皮质激素腺瘤的多巴胺和生长抑素受体,多达40%患者的皮质醇生成可得到控制。

促肾上腺皮质腺瘤的潜在新靶点包括表皮生长因子受体,细胞周期蛋白依赖性激酶和热休克蛋白90。奥西洛克斯塔特和左旋酮康唑目前是新的类固醇生成抑制剂,目前正在进行多中心研究。CORT125134是一种正在进行研究的新的选择性糖皮质激素受体拮抗剂。

作者总结了各种形式库欣综合征的药物治疗选择,关注了新涌现的药物和药物靶点,有望成为库欣综合征患者新的潜在有效个性化药物治疗。

原始出处:

Feelders RA, Newell-Price J, Pivonello R, et al. Advances in the medical treatment of Cushing's syndrome. Lancet Diabetes Endocrinol. 2018 Jul 19. pii: S2213-8587(18)30155-4. doi: 10.1016/S2213-8587(18)30155-4.

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    2019-09-01 howi
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背景:库欣综合征(CS)是具有挑战性的诊断。随着特定人群CS发病率的增加,但是对CS的常规筛查仍具有种种问题。为了减少延迟诊断和改善疾病预后,在高危人群中进行新的简单的CS筛查方法是必要的。目的:基于临床症状和生化结果,开发和验证一个简单的评分系统,预测CS。方法:观察性、前瞻性、多中心研究纳入了353例患者。所有患者均进行深夜唾液皮质醇(LNSC)和小剂量地塞米松抑制试验(DST)。以诊断或排除

FDA批准内源性库欣综合征治疗药Korlym(米非司酮)

近日,FDA批准Korlym(米非司酮)用于控制成人内源性库欣综合征的高血糖症(血糖水平过高),该药物的适应症是具有2型糖尿病或者葡萄糖耐受不良,已行手术但血糖仍然偏高,或者不适合做手术的库欣综合征病人。这是首个被批准用于治疗该疾病的药物。内源性库欣综合征是一种抑制性和少见的多系统性疾病,主要影响年龄介于25岁至40岁之间。该疾病是由过高的皮质醇激素水平引起的,皮质醇是一种甾体激素,由肾上腺产生,

当库欣综合征遇上妊娠

28岁女性,因“皮肤紫纹、血压升高伴宫内孕5个月余”入院。经鉴别诊断,患者为库欣综合征肾上腺腺瘤合并妊娠。据悉,到2004年,通过查询Cochrane图书馆(cochrane ibrary)和PubMed,全球只报道库欣综合征合并妊娠136例。对于此病如何进行诊断?又该如何进行治疗?

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