1. Decreased Renal Expression of H(+)-ATPase and Pendrin in a Patient with Distal Renal Tubular Acidosis Associated with Sjögren's Syndrome.
- Author
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Kim HY, Kim SS, Bae EH, Ma SK, and Kim SW
- Subjects
- Acidosis, Renal Tubular etiology, Adult, Antibodies, Female, Humans, Sjogren's Syndrome complications, Sjogren's Syndrome metabolism, Treatment Outcome, Acidosis, Renal Tubular pathology, Diuretics therapeutic use, Hypokalemia pathology, Kidney metabolism, Kidney Tubules, Distal metabolism, Potassium Citrate therapeutic use, Proton-Translocating ATPases metabolism, Sjogren's Syndrome pathology
- Abstract
A 31-year-old woman with no significant past medical or family history was admitted with complaints of general weakness. Laboratory tests revealed: serum potassium 3.0 mEq/L, arterial blood pH 7.28, serum bicarbonate 17.8 mEq/L and urinary pH 7.0. Double-labeling confocal fluorescence microscopy using H(+)-ATPase and pendrin antibodies demonstrated a decreased expression of these proteins in the patient's renal collecting duct compared to normal controls. Anti-Sjögren's-syndrome-related antigen A (Anti-Ro/SS-A) and anti-Sjögren's syndrome type B (anti-La/SS-B) antibodies were strongly positive with very high titers, consistent with Sjögren's syndrome. We present a case of distal renal tubular acidosis-associated Sjögren's syndrome with a defect in H(+)-ATPase and pendrin in the renal collecting duct.
- Published
- 2015
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