库欣综合症:快速证据审查。
Cushing's Syndrome: Rapid Evidence Review.
发表日期:2024 Sep
作者:
David L Maness, Grant Studebaker, Christopher M Knight
来源:
AMERICAN FAMILY PHYSICIAN
摘要:
库欣综合征是一种罕见的多系统疾病,由长期暴露于超生理水平的皮质醇引起。长期皮质醇增多症与显着的多系统发病率和死亡率以及生活质量下降有关。由于库欣综合征的病程隐匿、临床表现多样、症状与许多常见病症重叠以及检测的复杂性,库欣综合征的诊断常常会延迟数年。必须排除外源性糖皮质激素的使用作为主要病因。内源性皮质醇产生过多可能是由于垂体肿瘤或异位来源(ACTH 依赖性病例)过度产生促肾上腺皮质激素(ACTH)引起的,也可能是肾上腺自主皮质醇过度产生(ACTH 非依赖性病例)引起的。推荐的诊断方法包括适当的筛查、皮质醇增多症的确认和病因的确定。一线治疗是手术切除皮质醇过量产生的根源。需要终身治疗后监测来治疗合并症并检测复发。
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.