title: Successful Management of Refractory Type 1 Renal Tubular Acidosis with Amiloride.
abstract: 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 HCO3of 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 HCO3levels. 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.
authors.affiliation: Department of Internal Medicine, University of Maryland School of Medicine, Baltimore, MD, USA.
authors.forename: Patrick
authors.identifier: 0000-0001-8725-1440
authors.initials: P
authors.lastname: Oguejiofor
authors.affiliation: Department of Internal Medicine, University of Maryland School of Medicine, Baltimore, MD, USA.
authors.forename: Robert
authors.initials: R
authors.lastname: Chow
authors.affiliation: Division of Nephrology, University of Maryland Medical Center Midtown Campus, Baltimore, MD, USA.
authors.forename: Kenneth
authors.initials: K
authors.lastname: Yim
authors.affiliation: Department of Medicine, Division of Nephrology, Johns Hopkins School of Medicine, Baltimore, MD, USA; Department of Epidemiology, Johns Hopkins Bloomberg School of Public Health, Baltimore, MD, USA; Nephrology Center of Maryland, Baltimore, MD, USA.
authors.forename: Bernard G
authors.initials: BG
authors.lastname: Jaar