title: Decreased Renal Expression of H(+)-ATPase and Pendrin in a Patient with Distal Renal Tubular Acidosis Associated with Sjögren's Syndrome.
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.
authors.affiliation: Department of Internal Medicine, Chonnam National University Medical School, Korea.
authors.forename: Ha Yeon
authors.initials: HY
authors.lastname: Kim
authors.forename: Sung Sun
authors.initials: SS
authors.lastname: Kim
authors.forename: Eun Hui
authors.initials: EH
authors.lastname: Bae
authors.forename: Seong Kwon
authors.initials: SK
authors.lastname: Ma
authors.forename: Soo Wan
authors.initials: SW
authors.lastname: Kim
mesh_terms: D000141:Acidosis, Renal Tubular / Q000209:etiology / Q000473:pathology*; D000328:Adult; D000906:Antibodies; D004232:Diuretics / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007008:Hypokalemia / Q000473:pathology*; D007668:Kidney / Q000378:metabolism*; D007686:Kidney Tubules, Distal / Q000378:metabolism*; D019357:Potassium Citrate / Q000627:therapeutic use*; D006180:Proton-Translocating ATPases / Q000378:metabolism*; D012859:Sjogren's Syndrome / Q000150:complications / Q000378:metabolism / Q000473:pathology*; D016896:Treatment Outcome
chemical_list: D000906:Antibodies; D004232:Diuretics; D006180:Proton-Translocating ATPases; D019357:Potassium Citrate