Abstract: SA-PO0331
Hypokalemic Nephropathy with Zebra Bodies: Fabry vs. Mimic?
Session Information
- AKI: Case Reports - Drug/Toxin Injury, Crystals, Obstruction, and Unusual Presentations
October 24, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Acute Kidney Injury
- 102 AKI: Clinical, Outcomes, and Trials
Authors
- Gutierrez-Silva, Jean Carlos, BayCare Health System, Tampa, Florida, United States
- Ostolaza, Otto, BayCare Health System, Tampa, Florida, United States
Introduction
Hypokalemic nephropathy is an underrecognized and potentially reversible cause of acute kidney injury from chronic potassium depletion. Podocyte myelinosomes, classically associated with Fabry disease, may also occur in acquired phospholipidosis, creating a diagnostic dilemma.
Case Description
A 46-year-old woman with hypertension and chronic alcohol use presented with lower extremity weakness, pain, and a flat erythematous rash. Labs showed severe hypokalemia (2.3 mmol/L) and creatinine of 14.7 mg/dL, with prior AKI and intermittent hypokalemia since 2005.
Urine potassium remained elevated during hypokalemia, with a TTKG of 8.16, consistent with renal potassium wasting despite suppressed renin–aldosterone activity; magnesium 1.6, and CPK 136. Bicarbonate ranged 17–22 mmol/L previously (nadir 13) and was 28 mmol/L on admission, suggesting dynamic acid–base changes rather than fixed distal RTA. Autoimmune serologies were negative, with no relevant medication exposures.
Kidney biopsy showed acute tubular injury with epithelial vacuolization consistent with hypokalemic nephropathy and podocyte myelinosomes, suggestive of Fabry disease. Renal function improved with electrolyte repletion (creatinine 3.17 mg/dL).
Discussion
Severe hypokalemia is a reversible, underrecognized cause of biopsy-proven AKI driven by renal potassium wasting and tubular dysfunction, often exacerbated by chronic alcohol use. Alkalosis without persistent acidosis supports a dynamic tubular disorder rather than distal RTA.
Chronic potassium depletion causes tubular injury via intrarenal vasoconstriction, impaired angiogenesis, and direct tubular cell injury, resulting in ischemia, inflammation, and characteristic vacuolization.
Podocyte myelinosomes expand the differential diagnosis to Fabry disease, acquired phospholipidosis, and other lysosomal disorders. Although historically considered pathognomonic of Fabry disease, zebra bodies may also occur in other conditions. Normal α-galactosidase A activity and lyso-Gb3 argue against classic Fabry disease but do not exclude it in heterozygous females. GLA gene sequencing is ongoing.
This case highlights that zebra bodies require clinicopathologic correlation and that chronic hypokalemia can cause significant, potentially reversible kidney injury. It also illustrates how a kidney biopsy can reveal unexpected underlying disorders, including Fabry disease, in patients with atypical presentations.