Best Practice & Research Clinical Endocrinology & Metabolism ( IF 6.1 ) Pub Date : 2021-03-15 , DOI: 10.1016/j.beem.2021.101521 Osamah A Hakami 1 , Shahzada Ahmed 2 , Niki Karavitaki 1
Endogenous Cushing’s syndrome (CS) is a rare endocrine disorder characterised by excess cortisol secretion due to either ACTH-dependent conditions [commonly an ACTH-producing pituitary adenoma (Cushing’s disease)] or ACTH-independent causes (with most common aetiology being a benign adrenal adenoma). Overall, the annual incidence of CS ranges between 1.8-3.2 cases per million population. Mortality in active CS is elevated compared to the general population, and a number of studies support the view that survival is also compromised even after apparent successful treatment. The main cause of death is cardiovascular disease highlighting the negative impact of cortisol excess on cardiovascular risk factors. Early diagnosis and prompt treatment of the cortisol excess, as well as vigilant monitoring and stringent control of cardiovascular risk factors are key elements for the long-term prognosis of these patients.
中文翻译:
库欣综合征的流行病学和死亡率
内源性库欣综合征(CS)是一种罕见的内分泌失调,其特征是由于ACTH依赖性疾病[通常是产生ACTH的垂体腺瘤(库欣病)]或ACTH非依赖性原因(最常见的病因是良性肾上腺)腺瘤)。总体而言,CS的年发病率范围为每百万人口1.8-3.2例。与普通人群相比,活动性CS的死亡率有所提高,许多研究支持这样的观点,即即使在明显的成功治疗后,存活率也会受到损害。死亡的主要原因是心血管疾病,突显了皮质醇过多对心血管危险因素的负面影响。皮质醇过多的早期诊断和及时治疗,