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促肾上腺皮质激素依赖性皮质醇增多症患者行双侧肾上腺切除术:预测因子、生物标志物和结局。

Synchronous bilateral adrenalectomy in ACTH-dependent hypercortisolism: predictors, biomarkers and outcomes.

机构信息

Pituitary Unit, Divisione di Endocrinologia, Fondazione Policlinico Universitario A. Gemelli IRCCS - Università Cattolica del Sacro Cuore, 00168, Roma, Italy.

Department of Endocrine and Metabolic Surgery, Fondazione Policlinico Universitario A. Gemelli IRCCS - Università Cattolica del Sacro Cuore, 00168, Roma, Italy.

出版信息

Endocrine. 2019 Dec;66(3):642-649. doi: 10.1007/s12020-019-02091-9. Epub 2019 Oct 3.

Abstract

INTRODUCTION

Hypercortisolism requires a prompt therapeutic management to reduce the risk of development of a potential fatal emergency. A synchronous bilateral adrenalectomy (SBA) is effective in recovering hypercortisolism. However, specific indications for an SBA are not available. We aimed to evaluate the outcome of patients who underwent an SBA and to identify biomarkers able to predict the requirements of an SBA.

PATIENTS AND METHODS

A mono-centric and longitudinal study was conducted on 19 consecutive patients who underwent SBA for ACTH-dependent hypercortisolism between December 2003 and December 2017. This study population was compared to two control groups composed of patients cured after the resection of the ACTH secreting pituitary adenoma (Group A: 44 patients) and of the ACTH-secreting neuroendocrine tumours (Group B: 8 patients).

RESULTS

Short- or long-term SBA complications or the recurrence of hypercortisolism did not occur. A single patient experienced Nelson syndrome. Clinical features after SBA showed improvement in the glico-metabolic assessment, hypertension, bone metabolism and the occurrence of hypokalaemia and infections. The younger the age at the time of Cushing's disease diagnosis, the longer the duration of active hypercortisolism, higher values of plasmatic ACTH and Cortisol (1 month after pituitary neurosurgery) and higher values of Ki67 in pituitary adenomas were detected in this study population as compared to Group A.

CONCLUSIONS

SBA is an effective and safe treatment for patients with unmanageable ACTH-dependent hypercortisolism. A multidisciplinary team in a referral centre with a high volume of patients is strongly recommended for the management of these patients and the identification of patients, for better surgical timing.

摘要

介绍

皮质醇增多症需要及时进行治疗以降低潜在致命并发症的发生风险。双侧肾上腺切除术(SBA)是治疗皮质醇增多症的有效方法。然而,目前还没有明确的 SBA 适应证。我们旨在评估接受 SBA 的患者的预后,并确定能够预测 SBA 需求的生物标志物。

患者和方法

进行了一项单中心、纵向研究,共纳入 19 例 2003 年 12 月至 2017 年 12 月期间因 ACTH 依赖性皮质醇增多症而行 SBA 的连续患者。该研究人群与两个对照组进行比较,对照组由接受 ACTH 分泌性垂体腺瘤切除术(A 组:44 例)和 ACTH 分泌性神经内分泌肿瘤切除术(B 组:8 例)后治愈的患者组成。

结果

SBA 术后无短期或长期并发症,也无皮质醇增多症复发。1 例患者发生纳尔逊综合征。SBA 后临床特征显示糖代谢、高血压、骨代谢和低钾血症及感染的改善。与 A 组相比,该研究人群中皮质醇增多症诊断时年龄较小、活跃性皮质醇增多症持续时间较长、术后 1 个月时血浆 ACTH 和皮质醇水平较高、垂体腺瘤中 Ki67 水平较高。

结论

SBA 是治疗不可控制的 ACTH 依赖性皮质醇增多症的有效且安全的方法。建议在有大量患者的转诊中心成立多学科团队,以便更好地管理这些患者并确定手术时机。

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