SHM Converge Scientific Abstract ePoster Gallery-Unrecognized Pseudohypoaldosteronism: When the K Is Not O-K

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This case describes a 12-month-old girl with recurrent severe hyperkalemia and non-anion gap metabolic acidosis. She initially presented after outpatient labs showed potassium of 7.1 mEq/L, and repeat testing in the emergency department confirmed potassium of 7.3 mEq/L. She had normal vital signs and no ECG changes. Despite treatment with kayexalate, intravenous furosemide, IV fluids, and a low-potassium diet, her hyperkalemia persisted.<br /><br />Her evaluation was complicated by a broad differential, including renal tubular acidosis, hypoaldosteronism, adrenal insufficiency, gastrointestinal losses, and excess potassium intake. An adenovirus infection was identified, but the electrolyte abnormalities did not resolve as expected. Additional workup showed low renin, low-normal aldosterone, normal kidney function, and a low transtubular potassium gradient, indicating impaired renal potassium excretion.<br /><br />These findings supported a diagnosis of pseudohypoaldosteronism type II, a rare disorder characterized by impaired renal secretion of both potassium and hydrogen, leading to hyperkalemia and non-anion gap metabolic acidosis despite normal glomerular filtration. The patient responded rapidly to hydrochlorothiazide, with correction of potassium and bicarbonate levels. Outpatient attempts to wean the medication led to recurrence of hyperkalemia, while potassium remained normal on therapy.<br /><br />The case highlights the importance of recognizing pseudohypoaldosteronism type II in hospitalized pediatric patients with persistent hyperkalemia. Key clues include hyperkalemia, normal renal function, low renin, and non-anion gap metabolic acidosis. Early diagnosis is important because thiazide diuretics can effectively prevent complications.

Asset Subtitle

Lauren McIntosh

Meta Tag

Author List Lauren McIntosh, Sheila Swartz, Swathi Prasad

Category Clinical Vignettes

Concept Pseudohypoaldosteronism Type II

Concept Hyperkalemia

Concept Normal Anion Gap Metabolic Acidosis

Concept Renin

Concept Transtubular Potassium Gradient

Distinguished Non-Finalist

Presenter Organization Medical College of Wisconsin

Presenting Author Lauren McIntosh

Track Pediatric

Keywords

hyperkalemia

non-anion gap metabolic acidosis

pseudohypoaldosteronism type II

pediatric case

low renin

aldosterone

renal potassium excretion

hydrochlorothiazide

adenovirus infection

thiazide diuretics

Pseudohypoaldosteronism Type II

Hyperkalemia

Normal Anion Gap Metabolic Acidosis

Renin

Transtubular Potassium Gradient