Institute of Medicine, Sahlgrenska Academy, University of Gothenburg and Department of Endocrinology, Sahlgrenska University Hospital, Gröna Stråket 8, SE-413 45 Gothenburg, Sweden.
Eur J Endocrinol. 2013 Oct 4;169(5):R139-52. doi: 10.1530/EJE-13-0534. Print 2013 Nov.
One hundred years have passed since Harvey Williams Cushing presented the first patient with the syndrome that bears his name. In patients with Cushing's syndrome (CS), body composition and lipid, carbohydrate and protein metabolism are dramatically affected and psychopathology and cognitive dysfunction are frequently observed. Untreated patients with CS have a grave prognosis with an estimated 5-year survival of only 50%. Remission can be achieved by surgery, radiotherapy and sometimes with medical therapy. Recent data indicate that the adverse metabolic consequences of CS are present for years after successful treatment.In addition, recent studies have demonstrated that health-related quality of life and cognitive function are impaired in patients with CS in long-term remission. The focus of specialised care should therefore be not only on the diagnostic work-up and the early postoperative management but also on the long-term follow-up. In this paper, we review the long-term consequences in patients with CS in remission with focus on the neuropsychological effects and discuss the importance of these findings for long-term management. We also discuss three different phases in the postoperative management of surgically-treated patients with CS, each phase distinguished by specific challenges: the immediate postoperative phase, the glucocorticoid dose tapering phase and the long-term management. The focus of the long-term specialised care should be to identify cognitive impairments and psychiatric disorders, evaluate cardiovascular risk, follow pituitary function and detect possible recurrence of CS.
哈维·威廉姆斯·库欣 (Harvey Williams Cushing) 教授提出以他的名字命名的综合征患者已经过去了 100 年。在库欣综合征 (CS) 患者中，身体成分以及脂质、碳水化合物和蛋白质代谢受到显著影响，同时常伴有精神病理学和认知功能障碍。未经治疗的 CS 患者预后极差，估计 5 年生存率仅为 50%。通过手术、放疗，有时还可以通过药物治疗来实现缓解。最近的数据表明，CS 的不良代谢后果在成功治疗后多年仍然存在。此外，最近的研究表明，长期缓解的 CS 患者的健康相关生活质量和认知功能受损。因此，专业护理的重点不仅应放在诊断性检查和术后早期管理上，还应放在长期随访上。在本文中，我们回顾了缓解期 CS 患者的长期后果，重点关注神经心理学影响，并讨论这些发现对长期管理的重要性。我们还讨论了手术治疗 CS 患者的术后管理的三个不同阶段，每个阶段都有特定的挑战：术后即刻阶段、糖皮质激素剂量递减阶段和长期管理阶段。长期专业护理的重点应该是识别认知障碍和精神障碍、评估心血管风险、随访垂体功能以及检测 CS 可能的复发。
