Extraordinarily high aldosterone, 901.0 ng/dL, in a patient with primary aldosteronism: an insight into the underlying mechanism

Endocrine Journal

Yosuke Okubo⁴  Katsuko Shirotori²  Toru Aizawa³  Yuto Yamazaki⁵  Hironobu Sasano⁵  Kazuhiro Oguchi⁶  Mitsuhisa Komatsu⁴  Tetsuo Nishikawa¹  Yasuto Nakasone³  Yuka Sato³  Keishi Yamauchi³  Tsuyoshi Matsushita⁷ 

[1] Endocrinology and Diabetes Center, Yokohama Rosai Hospital, Yokohama, Japan;Department of Nephrology, Aizawa Hospital, Matsumoto, Japan;Diabetes Center, Aizawa Hospital, Matsumoto, Japan;Division of Diabetes, Endocrinology and Metabolism, Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Japan;Department of Pathology, Tohoku University Graduate School of Medicine, Sendai, Japan;Positron Imaging Center, Aizawa Hospital, Matsumoto, Japan;Department of Radiology, Shinshu University School of Medicine, Matsumoto, Japan

关键词: Aldosteronoma;    Hyperreninemia;    KCNJ5 mutation;   

DOI  :  10.1507/endocrj.EJ15-0398

学科分类：内分泌与代谢学

来源: Japan Endocrine Society

【 摘 要 】

References(19)A 43-yr-old hypertensive male was admitted due to hypokalemia (1.8 mEq/L) and renal dysfunction (eGFR, 20.0 mL/min/1.73 m2).His plasma aldosterone was 901.0 ng/dL, plasma renin activity 5.7 ng/mL/hr, and aldosterone/renin activity ratio 158.Angiotensin II (AII) was 0.7 pg/mL, ACTH <1.0 pg/mL, and cortisol 21.6 μg/dL.Liquid chromatography-tandem mass spectrometry analysis showed that aldosterone (104 times the control) as well as its precursors were significantly elevated in the patient’s plasma.A left adrenal (4-cm-diameter) tumor with 131I-Adosterol® uptake was found and removed.Four days later, plasma aldosterone and renin activity had dropped to 7.73 ng/dL and 1.6 ng/mL/hr, respectively.However, they rose to 24.0 ng/dL and 10.9 ng/mL/hr, respectively, by Day 102.Nevertheless, magnetic resonance angiography found no evidence of a renovascular lesion.The tumor was a benign adrenocortical adenoma composed predominantly of clear cells positive for 17α-hydroxylase, [hydroxy-delta-5-steroid dehydrogenase, 3 beta- and steroid delta-isomerases], and aldosterone synthase.A quantitative real-time polymerase chain reaction analysis of the tumor cells revealed that expression of the gene encoding aldosterone synthase was 85 times the control level.In addition, the tumor cells harbored G151R mutation of the inward rectifying potassium channel subfamily j, member 5 gene.The striking overexpression of aldosterone synthase by the tumor cells was considered the primary mechanism for the extravagant overproduction of aldosterone in this case.This overexpression may have resulted from integration of signals from AII and forced membrane depolarization due to the potassium channel mutation.

【 授权许可】

Unknown