内分泌
词汇介绍
拓展阅读
解析
Cushing 英 /'kuʃiŋ/
释 义 n. 库欣（姓氏）
例 句 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/
释 义 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
例 句 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
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接近脑下垂体。可转介到专门从事此类手术的中心。
产生异位ACTH的肿瘤，异位肿瘤的首选治疗方法是手术切除。如果肿瘤是癌性的并且已经扩散，可能需要化学疗法，放射疗法或其他癌症治疗。降低皮质醇水平的药物也可能是治疗的一部分。如果其他治疗失败，外科医生可能不得不切除肾上腺以控制库欣综合征。
肾上腺肿瘤，手术切除肾上腺肿瘤是最常见的治疗方法。一些罕见疾病在肾上腺中引起许多结节，需要手术切除两个腺体。如果肾上腺都被切除了，将需要终身服用药物来代替肾上腺产生的皮质醇和其他激素。
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
影响指数：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
