Turgut Didem, Piskinpasa Serhan Vahit, Keskin Havva, Agbaht Kemal, Coskun Yenigun Ezgi, Dede Fatih
Baskent University Ankara Hospital, Department of Internal Medicine, Division of Nephrology, Ankara, Turkey.
Iskenderun State Hospital, Division of Nephrology, Hatay, Turkey.
Medeni Med J. 2021;36(1):52-57. doi: 10.5222/MMJ.2021.93902. Epub 2021 Mar 26.
Systemic amyloidosis may affect many organs, and may cause endocrinologic problems which may result in adrenal insufficiency. However, assessment of adrenocortical reserve is challenging in amyloidosis patients with renal involvement. We aimed to evaluate adrenocortical reserve with various methods of cortisol measurement to determine any occult clinical condition.
Patients with renal amyloidosis and healthy subjects were evaluated in this cross-sectional study. Basal cortisol, corticosteroid-binding globulin (CBG), and albumin levels were measured. Serum free cortisol (cFC) level was calculated. Cortisol response tests performed after ACTH stimulation test (250 μg, intravenously) were evaluated, and free cortisol index (FCI) was calculated.
Twenty renal amyloidosis patients, and 25 healthy control subjects were included in the study. Patients and control subjects had similar median serum baseline cortisol levels [258 (126-423) vs 350 (314-391) nmol/L, p=0.169)] whereas patients' stimulated cortisol levels at the 60 minute were lower [624 (497-685) vs 743 (674-781) nmol/L, p=0.011)]. The 60-minute total cortisol levels of 8 of the 20 (40%) amyloidosis patients were <500 nmol/L, but only three of these 8 patients had stimulated FCI <12 nmol/mg suggesting an adrenal insufficiency (15%).
ACTH stimulation test and cortisol measurements should be considered in renal amyloidosis patients with severe proteinuria to avoid false positive results if only ACTH stimulation test is used. It will be appropriate to evaluate this group of patients together with estimated measurements as FCI.
系统性淀粉样变性可能累及多个器官,并可能导致内分泌问题,进而引发肾上腺功能不全。然而,对于合并肾脏受累的淀粉样变性患者,评估肾上腺皮质储备具有挑战性。我们旨在通过多种皮质醇测量方法评估肾上腺皮质储备,以确定是否存在隐匿的临床状况。
在这项横断面研究中,对肾淀粉样变性患者和健康受试者进行了评估。测量了基础皮质醇、皮质类固醇结合球蛋白(CBG)和白蛋白水平。计算血清游离皮质醇(cFC)水平。评估促肾上腺皮质激素(ACTH)刺激试验(静脉注射250μg)后进行的皮质醇反应试验,并计算游离皮质醇指数(FCI)。
本研究纳入了20例肾淀粉样变性患者和25例健康对照受试者。患者和对照受试者的血清基线皮质醇水平中位数相似[258(126 - 423)对350(314 - 391)nmol/L,p = 0.169],而患者在60分钟时的刺激后皮质醇水平较低[624(497 - 685)对743(674 - 781)nmol/L,p = 0.011]。20例(40%)淀粉样变性患者中有8例的60分钟总皮质醇水平<500 nmol/L,但这8例患者中只有3例刺激后的FCI<12 nmol/mg,提示肾上腺功能不全(15%)。
对于重度蛋白尿的肾淀粉样变性患者,应考虑进行ACTH刺激试验和皮质醇测量,以避免仅使用ACTH刺激试验时出现假阳性结果。将这组患者与FCI等估计测量值一起评估是合适的。