Androgens in Cushing's Syndrome.

2区 医学 Q2 Medicine Frontiers of Hormone Research Pub Date : 2019-01-01 DOI:10.1159/000494904
G. Arnaldi, M. Martino
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引用次数: 11

Abstract

Although polycystic ovary syndrome (PCOS) is the most common androgen excess disorder, screening for Cushing's Syndrome (CS) should be considered in women with PCOS phenotype, particularly if they are also affected by other disturbances that increase their pretest probability (e.g., osteoporosis/bone fractures). Approximately 70-80% of women with CS present menstrual abnormalities, and PCOS findings are found in 46% of these patients. Diagnostic efforts should strengthen if the clinical picture is severe or of rapid onset in order to ensure the earliest and most appropriate treatment. If the diagnosis of CS is challenging, its differentiation from PCOS is not outdone: isolated PCOS may be associated to hypothalamic-pituitary-adrenal axis disruption, leading to false-positive results in screening tests. Because of this overlap, the diagnosis of CS is initially missed or delayed. Diagnostic utility of serum androgen assessment is controversial, but the widespread use of high-performance liquid chromatography and gas chromatography-mass spectrometry for urinary steroid profiling is showing promising results. According to the role of adrenocorticotropic hormone (ACTH) in adrenal androgen secretion, it is not surprising that the levels of dehydroepiandrosterone, dehydroepiandrosterone-sulfate, and androstenedione (A4) are generally elevated or in the upper normal range in patients with ACTH-dependent CS. Conversely, adrenal androgens are generally low in patients with cortisol-secreting adrenocortical adenoma. However, androgen-secreting adrenal tumors (adenoma and carcinoma) can be also associated with severe hyperandrogenism. Regression of hypercortisolism after treatment causes disappearance of hyperandrogenism. However, signs of androgen excess may be detectable in well-controlled CS as a result of ACTH compensatory response to certain adrenal steroidogenesis inhibitors.
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库欣综合征中的雄激素。
虽然多囊卵巢综合征(PCOS)是最常见的雄激素过量疾病,但PCOS表型的女性应考虑库欣综合征(CS)筛查,特别是如果她们还受到其他干扰的影响,这些干扰会增加其检前概率(例如骨质疏松症/骨折)。大约70-80%的CS患者出现月经异常,其中46%的患者出现多囊卵巢综合征。如果临床表现严重或发病迅速,应加强诊断工作,以确保尽早和最适当的治疗。如果CS的诊断具有挑战性,其与PCOS的鉴别也不例外:孤立的PCOS可能与下丘脑-垂体-肾上腺轴断裂有关,导致筛查试验的假阳性结果。由于这种重叠,CS的诊断最初被遗漏或延迟。血清雄激素评估的诊断效用是有争议的,但广泛使用高效液相色谱和气相色谱-质谱分析尿类固醇显示出有希望的结果。根据促肾上腺皮质激素(ACTH)在肾上腺雄激素分泌中的作用,在ACTH依赖性CS患者中,脱氢表雄酮、脱氢表雄酮硫酸酯和雄烯二酮(A4)水平普遍升高或处于正常上限。相反,肾上腺雄激素在分泌皮质醇的肾上腺皮质腺瘤患者中通常较低。然而,分泌雄激素的肾上腺肿瘤(腺瘤和癌)也可能与严重的高雄激素症有关。治疗后高皮质醇症消退导致高雄激素症消失。然而,由于ACTH对某些肾上腺甾体生成抑制剂的代偿反应,雄激素过量的迹象可能在控制良好的CS中被检测到。
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来源期刊
Frontiers of Hormone Research
Frontiers of Hormone Research 医学-内分泌学与代谢
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期刊介绍: A series of integrated overviews on cutting-edge topics New sophisticated technologies and methodological approaches in diagnostics and therapeutics have led to significant improvements in identifying and characterizing an increasing number of medical conditions, which is particularly true for all aspects of endocrine and metabolic dysfunctions. Novel insights in endocrine physiology and pathophysiology allow for new perspectives in clinical management and thus lead to the development of molecular, personalized treatments. In view of this, the active interplay between basic scientists and clinicians has become fundamental, both to provide patients with the most appropriate care and to advance future research.
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