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

Yosuke Okubo, Yuka Sato, Yasuto Nakasone, Katsuko Shirotori, Kazuhiro Oguchi, Tsuyoshi Matsushita, Tetsuo Nishikawa, Yuto Yamazaki, Hironobu Sasano, Mitsuhisa Komatsu, Keishi Yamauchi, Toru Aizawa

Research output: Contribution to journalArticlepeer-review

1 Citation (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)127-133
Number of pages7
Journalendocrine journal
Volume63
Issue number2
DOIs
Publication statusPublished - 2016 Feb 29

Keywords

  • Aldosteronoma
  • Hyperreninemia
  • KCNJ5 mutation

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Endocrinology

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