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Cushing Syndrome

内分泌

关键词内分泌 疾病 糖皮质激素分泌过多

词汇介绍

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解析

Cushing   英 /'kuʃiŋ/

       n. 库欣(姓氏)

       Objective To reinvestigate the value of overnight low-dose dexamethasone suppression test in the diagnosis of Cushing syndrome. 目的重新探讨过夜小剂量地塞米松抑制试验对库欣综合征的诊断价值。

 

Syndrome   英 /'sɪndrəʊm/   美 /'sɪndrəm/

       n. [临床] 综合症状;并发症状;校验子;并发位

               n. (Syndrome)人名;(英)辛德罗姆

       The researchers found that some of the people with this syndrome lack a gene for BDNF and have correspondingly low blood levels of the substance. 研究人员发现患有这种综合症的一些人缺少表达BDNF的基因,并且血液中这种物质的水平相对较低。

概述

库欣综合征是由于长期接触糖皮质激素(如皮质醇)引起的体征和症状的集合。症状和体征可能包括高血压,腹部肥胖,但手臂和腿部较细,红色妊娠纹,红色圆脸,肩部脂肪肿块,肌肉无力,骨骼脆弱,痤疮和皮肤愈合不良。女性可能会出现毛发旺盛和月经不调。偶尔可能会出现情绪变化,头痛和疲倦感诊断方法①24h尿游离皮质醇测试;②深夜唾液皮质醇测试;③低剂量地塞米松抑制试验(LDDST);④地塞米松-CRH试验。治疗方法垂体瘤最常见的治疗方法是手术切除肿瘤。使用特殊的显微镜和精细仪器,外科医生通过鼻孔或上唇下方的开口

Osilodrostat Is a Potential Novel Steroidogenesis Inhibitor for the Treatment of Cushing Syndrome: An In Vitro Study复制标题

Osilodrostat是一种潜在的新型类固醇生成抑制剂,用于治疗库欣综合征: 一项体外研究

发表时间:2019-04-24

影响因子:5.6

作者: Sara G Creemers

期刊:J Clin Endocrinol Metab

Cushing syndrome (CS) is characterized by chronic exposure to excess glucocorticoids, resulting in substantial multisystem morbidity and, when untreated, increased mortality. ACTH-dependent CS can be caused by a corticotroph pituitary adenoma [Cushing disease (CD)] or, more rarely, by ectopic ACTH secretion by a neuroendocrine tumor (ectopic ACTH syndrome). ACTH-independent CS is in most cases caused by a unilateral cortisol-producing adrenocortical adenoma (ACA) and less frequently by an adrenocortical carcinoma (ACC) or bilateral adrenal hyperplasia. The first-line treatment modality in all types of CS is surgery. There are several conditions, however, such as surgical failure, metastatic or occult disease, or high surgical risk, in which other treatment modalities such as medical therapy are indicated. Traditionally, medical treatment options for CS can be divided into three categories: (i) pituitary-targeting drugs (i.e., pasireotide and cabergoline); (ii) glucocorticoid receptor blockers (e.g., mifepristone); and (iii) adrenocortical steroidogenesis inhibitors that directly suppress cortisol production via inhibition of steroidogenic enzymes. Two of the most frequently used steroidogenesis inhibitors are metyrapone and ketoconazole. Although it is known that metyrapone selectively inhibits the last step in the cortisol biosynthesis via inhibition of CYP11B1 (11b-hydroxylase), it also inhibits CYP11B2 (aldosterone synthase). More recently, in vitro data obtained in a rodent model even suggested that metyrapone has greater potency to inhibit CYP11B2, a feature not previously recognized. Ketoconazole, originally developed as an antifungal agent, is known to inhibit several steps in adrenal steroid synthesis. Although several medical therapies are currently available, not all patients respond and many patients experience side effects. Metyrapone can cause hypertension, edema, hypokalemia, acne, and hirsutism resulting from an increase of mineralocorticoid precursors and adrenal androgens. The most important adverse events of ketoconazole include hepatotoxicity and gastrointestinal symptoms.

译文

库欣综合征(CS)的特征在于长期暴露于过量的糖皮质激素,导致大量的多系统发病率,并且当未治疗时,死亡率增加。 ACTH依赖性CS可由促肾上腺皮质激素垂体腺瘤[库欣病(CD)]引起,或者更罕见地由神经内分泌肿瘤(异位ACTH综合征)分泌的异位ACTH引起。在大多数情况下,ACTH非依赖性CS由单侧皮质醇产生的肾上腺皮质腺瘤(ACA)引起,并且不太常由肾上腺皮质癌(ACC)或双侧肾上腺增生引起。所有类型CS的一线治疗方式是手术。然而,有几种情况,例如手术失败,转移性或隐匿性疾病,或高手术风险,其中指出了其他治疗方式,例如药物治疗。传统上,CS的医疗选择可分为三类:(i)垂体靶向药物(即pasireotide和cabergoline); (ii)糖皮质激素受体阻滞剂(例如米非司酮); (iii)肾上腺皮质类固醇生成抑制剂,其通过抑制类固醇生成酶直接抑制皮质醇的产生。两种最常用的类固醇生成抑制剂是甲吡酮和酮康唑。虽然已知甲吡酮通过抑制CYP11B1(11b-羟化酶)选择性地抑制皮质醇生物合成的最后一步,但它也抑制CYP11B2(醛固酮合成酶)。最近,在啮齿动物模型中获得的体外数据甚至表明甲吡酮具有更强的抑制CYP11B2的效力,这是之前未被认识到的特征。最初开发为抗真菌剂的酮康唑可抑制肾上腺类固醇合成的几个步骤。虽然目前有几种医学疗法可用,但并非所有患者都有反应,许多患者都有副作用。由于盐皮质激素前体和肾上腺雄激素的增加,Metyrapone可引起高血压,水肿,低钾血症,痤疮和多毛症。酮康唑最重要的不良事件包括肝毒性和胃肠道症状。