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一组库欣病患者的长期缓解率和复发率:长期随访的必要性。

Long-term remission and recurrence rate in a cohort of Cushing's disease: the need for long-term follow-up.

作者信息

Aranda G, Enseñat J, Mora M, Puig-Domingo M, Martínez de Osaba M J, Casals G, Verger E, Ribalta M T, Hanzu F A, Halperin I

机构信息

Department of Endocrinology and Nutrition, Hospital Clinic, Barcelona, Spain.

出版信息

Pituitary. 2015 Feb;18(1):142-9. doi: 10.1007/s11102-014-0567-8.

DOI:10.1007/s11102-014-0567-8
PMID:24748528
Abstract

OBJECTIVE

Transsphenoidal surgery is the procedure of choice in Cushing disease (CD), with immediate post-operative remission rates ranging between 59 and 94% and recurrence rates between 3 and 46%, both depending upon the definition criteria and the duration of the follow-up. Our aim was to assess the rate of remission, recurrence and persistence of the disease after the first treatment and to identify predictors of remission in the CD population of our center.

METHODS

Retrospective cohort study of the patients diagnosed of CD and with follow-up in our center between 1974 and 2011. We analyzed 41 patients (35 women and 6 men) with a mean age at diagnosis of 34 ± 13 years. The mean follow-up was 14 ± 10 years (range 1-37 years) and the median of follow-up period was 6.68 years.

RESULTS

Thirty-five (85.4%) patients underwent transsphenoidal surgery as first treatment option. Histopathological evidence of a pituitary adenoma was registered in 17 (48.5%) patients. Thirty-two (78%) patients achieved disease remission after the first treatment, 21 (65.6%) of them presented disease recurrence. Persistent disease was observed in 9 (22%) patients. Twelve (29.3%) subjects developed post-surgical adrenal insufficiency, 7 of which (70%) achieved stable remission. Two parameters were found to be significant predictors of remission after the first treatment: age at disease diagnosis and the development of adrenal insufficiency (cortisol <3 μg/dl) in the immediate post-operative state.

CONCLUSIONS

We report a high recurrence rate, at least partially attributable to the long follow-up time. Early post-surgery adrenal insufficiency predicts remission. Hypopituitarism was also very prevalent, and strongly associated with radiotherapy. These results lead us to the conclusion that CD needs a life-long strict follow-up.

摘要

目的

经蝶窦手术是库欣病(CD)的首选治疗方法,术后立即缓解率在59%至94%之间,复发率在3%至46%之间,这两者均取决于定义标准和随访时间。我们的目的是评估首次治疗后疾病的缓解率、复发率和持续率,并确定我们中心CD患者群体中缓解的预测因素。

方法

对1974年至2011年在我们中心诊断为CD并接受随访的患者进行回顾性队列研究。我们分析了41例患者(35例女性和6例男性),诊断时的平均年龄为34±13岁。平均随访时间为14±10年(范围1至37年),随访期中位数为6.68年。

结果

35例(85.4%)患者接受经蝶窦手术作为首选治疗方案。17例(48.5%)患者有垂体腺瘤的组织病理学证据。32例(78%)患者在首次治疗后实现疾病缓解,其中21例(65.6%)出现疾病复发。9例(22%)患者疾病持续存在。12例(29.3%)患者出现术后肾上腺功能不全,其中7例(70%)实现稳定缓解。发现两个参数是首次治疗后缓解的显著预测因素:疾病诊断时的年龄和术后即刻出现肾上腺功能不全(皮质醇<3μg/dl)。

结论

我们报告了较高的复发率,至少部分归因于较长的随访时间。术后早期肾上腺功能不全可预测缓解。垂体功能减退也非常普遍,且与放疗密切相关。这些结果使我们得出结论,CD需要终身严格随访。

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