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Cushing syndrome

delete2025-01-23
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PRE
AI
L
Lynnette K. Nieman *
N
Newell-Price, John
D
Drouin, Jacques
T
Takahashi, Yutaka
DOI:10.1038/s41572-024-00588-wdelete
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摘要

摘要

En 中文
库欣综合征(CS)是由外源性或内源性糖皮质激素过度暴露引起的一系列体征和症状。内源性CS是由单侧或双侧肾上腺(肾上腺CS)皮质醇分泌增多,或垂体肿瘤(库欣病(CD))或非垂体肿瘤(异位ACTH分泌)导致促肾上腺皮质激素(ACTH)分泌升高,进而刺激皮质醇过度产生所致。CS与严重的多系统功能损害相关,包括心血管和代谢功能受损、感染及神经精神障碍,这些显著降低了患者的生活质量。由于肺栓塞、感染、心肌梗死和中风等原因,CS患者的死亡率升高。临床表现多样,由于部分CS的体征和症状在普通人群中较为常见,因此诊断可能需要累积多个特征后才会被考虑。指南建议对疑似CS患者进行24小时尿皮质醇、睡前唾液皮质醇和/或1毫克地塞米松抑制试验筛查。随后,确定CS的病因至关重要,因为它影响治疗方案。所有内源性CS病因的一线治疗均为手术切除病因组织,包括ACTH依赖性CS的促肾上腺皮质激素瘤或异位肿瘤,或肾上腺CS的单侧或双侧肾上腺切除术。二线治疗包括对任何CS病因的类固醇生成抑制剂、针对CD的垂体放射治疗(可联合或不联合类固醇生成抑制剂),以及针对ACTH依赖性CS病因的双侧肾上腺切除术。
Keyword:
CORTICOTROPIN-RELEASING HORMONE
NIGHT SALIVARY CORTISOL
LONG-TERM REMISSION
NODULAR ADRENOCORTICAL-DISEASE
DEXAMETHASONE-SUPPRESSION TEST
CLINICAL-PRACTICE GUIDELINES
RECEPTOR GENE-EXPRESSION
PITUITARY-ADRENAL AXIS
URINARY FREE CORTISOL
GLUCOCORTICOID-RECEPTOR

期刊

N
Nature Reviews Disease Primers
IF:
60.6
论文数:
648
被引数:
3.8W

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