Berr Christina M, Di Dalmazi Guido, Osswald Andrea, Ritzel Katrin, Bidlingmaier Martin, Geyer Lucas L, Treitl Marcus, Hallfeldt Klaus, Rachinger Walter, Reisch Nicole, Blaser Rainer, Schopohl Jochen, Beuschlein Felix, Reincke Martin
Medizinische Klinik und Poliklinik IV (C.M.B., G.D.D., A.O., K.R., M.B., N.R., J.S., F.B., M.R.), Institut für Klinische Radiologie (L.L.G., M.T.), Chirurgische Klinik und Poliklinik-Innenstadt (K.H.), 80336 München, Germany; Klinik für Neurochirurgie, Klinikum Grosshadern (W.R.), Ludwig-Maximilians-Universität München, 81377 München, Germany; and Institut für Medizinische Statistik und Epidemiologie (R.B.), Klinikum rechts der Isar der TU München, 81675 München, Germany.
J Clin Endocrinol Metab. 2015 Apr;100(4):1300-8. doi: 10.1210/jc.2014-3632. Epub 2014 Dec 29.
Successful tumor resection in endogenous Cushing's syndrome (CS) results in tertiary adrenal insufficiency requiring hydrocortisone replacement therapy.
The aim was to analyze the postsurgical duration of adrenal insufficiency of patients with Cushing's disease (CD), adrenal CS, and ectopic CS.
We performed a retrospective analysis based on the case records of 230 patients with CS in our tertiary referral center treated from 1983-2014. The mean follow-up time was 8 years.
We included 91 patients of the three subtypes of CS undergoing curative intended surgery and documented followup after excluding cases with persistent disease, pituitary radiation, concurrent adrenostatic or somatostatin analog treatment, and malignant adrenal disease.
The probability of recovering adrenal function within a 5 years followup differed significantly between subtypes (P = .001). It was 82% in ectopic CS, 58% in CD and 38% in adrenal CS. In the total cohort with restored adrenal function (n = 52) the median time to recovery differed between subtypes: 0.6 years (interquartile range [IQR], 0.03-1.1 y) in ectopic CS, 1.4 years (IQR, 0.9-3.4 y) in CD, and 2.5 years (IQR, 1.6-5.4 y) in adrenal CS (P = .002). In CD the Cox proportional-hazards model showed that the probability of recovery was associated with younger age (hazard ratio, 0.896; 95% confidence interval, 0.822-0.976; P = .012), independently of sex, body mass index, duration of symptoms, and basal ACTH and cortisol levels. There was no correlation with length and extend of hypercortisolism or postoperative glucocorticoid replacement doses.
Time to recovery of adrenal function is dependent on the underlying etiology of CS.
内源性库欣综合征（CS）成功进行肿瘤切除术后会导致三发性肾上腺功能不全，需要氢化可的松替代治疗。
分析库欣病（CD）、肾上腺性CS和异位性CS患者术后肾上腺功能不全的持续时间。
我们基于1983年至2014年在我们三级转诊中心接受治疗的230例CS患者的病例记录进行了回顾性分析。平均随访时间为8年。
我们纳入了91例接受根治性手术的CS三种亚型患者，排除了患有持续性疾病、垂体放疗、同时进行肾上腺抑制或生长抑素类似物治疗以及肾上腺恶性疾病的病例，并记录了随访情况。
在5年随访期内恢复肾上腺功能的概率在各亚型之间存在显著差异（P = 0.001）。异位性CS为82%，CD为58%，肾上腺性CS为38%。在肾上腺功能恢复的总队列（n = 52）中，各亚型恢复的中位时间不同：异位性CS为0.6年（四分位数间距[IQR]，0.03 - 1.1年），CD为1.4年（IQR，0.9 - 3.4年），肾上腺性CS为2.5年（IQR，1.6 - 5.4年）（P = 0.002）。在CD中，Cox比例风险模型显示，恢复概率与年龄较小相关（风险比，0.896；95%置信区间，0.822 - 0.976；P = 0.012），与性别、体重指数、症状持续时间以及基础促肾上腺皮质激素和皮质醇水平无关。与皮质醇增多症的持续时间和程度或术后糖皮质激素替代剂量无关。
肾上腺功能恢复的时间取决于CS的潜在病因。
