在慢性肾脏病背景下诊断库欣病：病例报告及文献复习。

Diagnosing Cushing's disease in the context of chronic kidney disease: a case report and literature review.

机构信息

Rhinology and Skull Base Research Group, Applied Medical Research Centre, University of New South Wales, Sydney, Australia.

St Vincent's Clinical School, Faculty of Medicine, University of New South Wales, Sydney, Australia.

出版信息

Eur J Endocrinol. 2019 Oct;181(4):K29-K35. doi: 10.1530/EJE-19-0326.

DOI:10.1530/EJE-19-0326

PMID:31382242

Abstract

OBJECTIVE

The diagnosis of Cushing's disease (CD) is particularly challenging in patients with chronic kidney disease (CKD) due to abnormalities of the hypothalamo-pituitary-adrenal axis associated with the latter. This case report presents discrepant biochemical findings in a patient with CKD who was subsequently diagnosed with CD, and outlines principles which may guide the definitive diagnosis of CD in this context.

METHODS

The case of a patient with Stage 4 CKD who underwent transsphenoidal surgery for pituitary-dependent CD is presented. A literature review was conducted to identify similar cases and characterise features of hypothalamo-pituitary-adrenal axis dysfunction in CKD.

RESULTS

The patient discussed herein presented with markedly elevated plasma adrenocorticotrophic hormone (ACTH) due to a pituitary macroadenoma, with normal 24-h urine free cortisol (24-UFC) but abnormal overnight dexamethasone suppression testing and elevated midnight salivary cortisol. He experienced biochemical remission after undergoing transsphenoidal adenomectomy. A literature review revealed that CKD can be associated with elevated serum cortisol, reduced UFC and elevated plasma ACTH. Only four other cases of CD being diagnosed in a patient with CKD have been published. The loss of a circadian rhythm of cortisol secretion was the most common feature among all cases.

CONCLUSIONS

To establish a definitive diagnosis of CD in the context of pre-existing CKD, the absence of circadian rhythms of cortisol and ACTH is a more sensitive indicator than 24-UFC and low-dose dexamethasone suppression testing.

摘要

目的

由于慢性肾脏病（CKD）相关的下丘脑-垂体-肾上腺轴异常，库欣病（CD）的诊断在 CKD 患者中尤其具有挑战性。本病例报告介绍了一名 CKD 患者的生化检查结果不一致，随后被诊断为 CD，并概述了在这种情况下可能指导 CD 明确诊断的原则。

方法

介绍了一名 4 期 CKD 患者，该患者因垂体依赖性 CD 而行经蝶窦手术。进行了文献回顾，以确定类似病例并描述 CKD 中下丘脑-垂体-肾上腺轴功能障碍的特征。

结果

本文讨论的患者因垂体大腺瘤而出现明显升高的血浆促肾上腺皮质激素（ACTH），24 小时尿游离皮质醇（24-UFC）正常，但 overnight dexamethasone suppression testing 异常和午夜唾液皮质醇升高。他在接受经蝶窦腺瘤切除术后面临生化缓解。文献回顾显示，CKD 可伴有血清皮质醇升高、UFC 降低和血浆 ACTH 升高。仅发表了其他 4 例 CKD 患者被诊断为 CD 的病例。所有病例中最常见的特征是皮质醇分泌的昼夜节律丧失。