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库欣综合征:快速证据综述。

Cushing's Syndrome: Rapid Evidence Review.

机构信息

University of Tennessee Family Medicine Residency Program, Jackson, TN.

University of Tennessee Health Science Center, Jackson, TN.

出版信息

Am Fam Physician. 2024 Sep;110(3):270-280.

Abstract

Cushing's syndrome is a rare, multisystemic disease caused by chronic exposure to supraphysiologic levels of cortisol. Prolonged hypercortisolism is associated with significant multisystem morbidity and mortality and decreased quality of life. Diagnosis of Cushing's syndrome is often delayed by several years due to its insidiously progressive course, diverse clinical presentation, overlap of symptoms with many common conditions, and testing complexity. Exogenous glucocorticoid use must be excluded as the primary etiology. Excessive endogenous cortisol production can be caused by an overproduction of adrenocorticotropic hormone (ACTH) through pituitary tumors or ectopic sources (ACTH-dependent cases), or it can be caused by autonomous cortisol overproduction by the adrenal glands (ACTH-independent cases). The recommended diagnostic approach includes appropriate screening, confirmation of hypercortisolism, and determination of etiology. First-line treatment is surgical removal of the source of cortisol overproduction. Lifelong posttherapy monitoring is required to treat comorbidities and detect recurrence.

摘要

库欣综合征是一种罕见的多系统疾病,由慢性暴露于皮质醇的超生理水平引起。长期的皮质醇过多与显著的多系统发病率和死亡率以及生活质量下降有关。由于其隐匿性进展过程、多种临床表现、与许多常见疾病的症状重叠以及检测的复杂性,库欣综合征的诊断常常延迟数年。必须排除外源性糖皮质激素的使用作为主要病因。内源性皮质醇的过度产生可由于通过垂体肿瘤或异位来源的促肾上腺皮质激素 (ACTH) 过度产生引起(ACTH 依赖性病例),也可由于肾上腺自主皮质醇过度产生引起(ACTH 非依赖性病例)。推荐的诊断方法包括适当的筛查、确认皮质醇过多症和确定病因。一线治疗是手术切除皮质醇过度产生的来源。需要终身的治疗后监测来治疗合并症和检测复发。

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