关键词: ACTH Cortisol Cushing’s disease adrenal Cushing’s syndrome adrenalectomy biochemical control ectopic Cushing’s syndrome hypercortisolism medical treatment pituitary surgery radiotherapy

来  源:   DOI:10.1210/clinem/dgae337

Abstract:
BACKGROUND: Normalization of hypercortisolism is essential to reduce morbidity and mortality in patients with Cushing\'s syndrome (CS). The aim of this analysis was to assess biochemical control rates in patients with Cushing\'s disease (CD), ectopic Cushing\'s syndrome (ECS) and adrenal Cushing\'s syndrome (ACS).
METHODS: Patients with confirmed CS (n= 296) treated in a single tertiary care center were retrospectively analysed (185 CD, 27 ECS, 84 uni- and bilateral ACS).
RESULTS: Firstline treatment led to biochemical control in 82% of the patients. Time to biochemical control (median, IQR) was longer in CD (11.0 weeks, 5.6-29.8; p< 0.05) than in ACS (7.7 weeks, 4.1-17.1) and ECS (5.6 weeks, 4.1-23.3). Disease persistence or recurrence after first-line therapy was observed more often in CD (24% and 18%; p< 0.05) than in ECS (15% and 15%) and ACS (6% and 4%). Total time in hypercortisolism since diagnosis was significantly shorter in patients with CD diagnosed since 2013, after specialized patient care was implemented, compared to patients diagnosed before 2013 (13.5 weeks, vs. 26.1 weeks; p< 0.0070). Control of hypercortisolism at last follow up (76 months, 38-163) was achieved in 94% of patients with ACS, 100% of patients with ECS and 92% of patients with CD.
CONCLUSIONS: Biochemical control can be achieved in most patients with different subtypes of CS within a reasonable time frame. Control of hypercortisolism has improved over time.
摘要:
背景:皮质醇增多症的正常化对于降低库欣综合征(CS)患者的发病率和死亡率至关重要。这项分析的目的是评估库欣病(CD)患者的生化控制率,异位库欣综合征(ECS)和肾上腺库欣综合征(ACS)。
方法:回顾性分析在单一三级护理中心治疗的确诊CS患者(n=296)(185CD,27ECS,84单和双边ACS)。
结果:一线治疗导致82%的患者生化控制。生化控制时间(中位数,IQR)在CD中更长(11.0周,5.6-29.8;p<0.05)比ACS(7.7周,4.1-17.1)和ECS(5.6周,4.1-23.3)。与ECS(15%和15%)和ACS(6%和4%)相比,在CD(24%和18%;p<0.05)中观察到一线治疗后疾病持续或复发的频率更高。在实施专门的患者护理后,自2013年以来诊断为CD的患者自诊断以来皮质醇增多症的总时间明显缩短,与2013年之前诊断的患者相比(13.5周,vs.26.1周;p<0.0070)。末次随访时皮质醇增多症的控制(76个月,38-163)在94%的ACS患者中实现,100%的ECS患者和92%的CD患者。
结论:在合理的时间范围内,大多数患有不同亚型CS的患者可以实现生化控制。随着时间的推移,皮质醇增多症的控制有所改善。
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