An Unusual Cause of Hypokalemia to Consider.

JCEM case reports Pub Date : 2025-02-17 eCollection Date: 2025-03-01 DOI:10.1210/jcemcr/luaf026
Inés Borrego-Soriano, Yanieli Hernández-Perdomo, Begoña Rivas, Paola Parra-Ramírez, Óscar Moreno-Domínguez, Cristina Vega-Cabrera
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Abstract

Apparent mineralocorticoid excess syndrome is a rare disorder that can be acquired through inhibition of the enzyme 11 β-hydroxysteroid dehydrogenase type 2 by various substances such as bile acids. We report the case of a 61-year-old woman presenting with painless jaundice. Computed tomography demonstrated a pulmonary as well as a pancreatic tumor, with multiple metastases and dilated bile ducts. Laboratory findings showed persistent hypokalemia despite aggressive enteral and parenteral supplementation, as well as hypertension, metabolic alkalosis, and elevated cholestasis enzymes. Urinary potassium excretion was inappropriately high. Plasma aldosterone concentration was 0.97 ng/dL (26.91 pmol/L) (reference range, 2.21-25.30 ng/dL [61.31-701.82 pmol/L]) and direct renin concentration was 3.9 mIU/L (2.1 ng/L) (reference range, 4.4-46.1 mIU/L [2.53-27.42 ng/L]). Endogenous hypercortisolism was ruled out. After the placement of a metal biliary stent via endoscopic retrograde cholangiopancreatography and the subsequent decrease in cholestasis enzyme levels, potassium levels, hypertension, and metabolic alkalosis gradually normalized. The case was ultimately diagnosed as apparent mineralocorticoid excess syndrome resulting from 11 β-hydroxysteroid dehydrogenase type 2 inhibition caused by elevated bile acids secondary to malignant obstruction of the biliary tract.

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考虑低钾血症的不寻常原因。
表观矿皮质激素过量综合征是一种罕见的疾病,可通过各种物质(如胆汁酸)抑制酶11 β-羟基类固醇脱氢酶2型而获得。我们报告的情况下,一个61岁的妇女提出无痛性黄疸。计算机断层扫描显示肺和胰腺肿瘤,多发转移和胆管扩张。实验室结果显示,尽管有积极的肠内和肠外补充,但仍存在持续的低钾血症,以及高血压、代谢性碱中毒和胆汁淤积酶升高。尿钾排泄量过高。血浆醛固酮浓度为0.97 ng/dL (26.91 pmol/L)(参考范围为2.21 ~ 25.30 ng/dL [61.31 ~ 701.82 pmol/L]),直接肾素浓度为3.9 mIU/L (2.1 ng/L)(参考范围为4.4 ~ 46.1 mIU/L [2.53 ~ 27.42 ng/L])。排除内源性高皮质醇血症。经内窥镜逆行胆管造影术置入金属胆道支架后,胆汁淤积酶水平、钾水平、高血压、代谢性碱中毒逐渐降低。该病例最终被诊断为胆汁酸升高继发于胆道恶性梗阻引起的11 β-羟基类固醇脱氢酶2型抑制所致的明显矿皮质激素过量综合征。
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