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Case Reports in Nephrology
Volume 2017 (2017), Article ID 8596169, 3 pages
Case Report

Successful Management of Refractory Type 1 Renal Tubular Acidosis with Amiloride

1Department of Internal Medicine, University of Maryland School of Medicine, Baltimore, MD, USA
2Division of Nephrology, University of Maryland Medical Center Midtown Campus, Baltimore, MD, USA
3Department of Medicine, Division of Nephrology, Johns Hopkins School of Medicine, Baltimore, MD, USA
4Department of Epidemiology, Johns Hopkins Bloomberg School of Public Health, Baltimore, MD, USA
5Nephrology Center of Maryland, Baltimore, MD, USA

Correspondence should be addressed to Patrick Oguejiofor

Received 14 July 2016; Revised 2 December 2016; Accepted 14 December 2016; Published 3 January 2017

Academic Editor: Salih Kavukcu

Copyright © 2017 Patrick Oguejiofor et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


A 28-year-old female with history of hypothyroidism, Sjögren’s Syndrome, and Systemic Lupus Erythematosus (SLE) presented with complaints of severe generalized weakness, muscle pain, nausea, vomiting, and anorexia. Physical examination was unremarkable. Laboratory test showed hypokalemia at 1.6 mmol/l, nonanion metabolic acidosis with HCO3 of 11 mmol/l, random urine pH of 7.0, and urine anion gap of 8 mmol/l. CT scan of the abdomen revealed bilateral nephrocalcinosis. A diagnosis of type 1 RTA likely secondary to Sjögren’s Syndrome was made. She was started on citric acid potassium citrate with escalating dosages to a maximum dose of 60 mEq daily and potassium chloride over 5 years without significant improvement in serum K+ and HCO3 levels. She had multiple emergency room visits for persistent muscle pain, generalized weakness, and cardiac arrhythmias. Citric acid potassium citrate was then replaced with sodium bicarbonate at 15.5 mEq every 6 hours which was continued for 2 years without significant improvement in her symptoms and electrolytes. Amiloride 5 mg daily was added to her regimen as a potassium sparing treatment with dramatic improvement in her symptoms and electrolyte levels (as shown in the figures). Amiloride was increased to 10 mg daily and potassium supplementation was discontinued without affecting her electrolytes. Her sodium bicarbonate was weaned to 7.7 mEq daily.