Successful Management of Refractory Type 1 Renal Tubular Acidosis with Amiloride

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...

Full description

Saved in:
Bibliographic Details
Main Authors: Patrick Oguejiofor, Robert Chow, Kenneth Yim, Bernard G. Jaar
Format: Article
Language:English
Published: Wiley 2017-01-01
Series:Case Reports in Nephrology
Online Access:http://dx.doi.org/10.1155/2017/8596169
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1832551466985848832
author Patrick Oguejiofor
Robert Chow
Kenneth Yim
Bernard G. Jaar
author_facet Patrick Oguejiofor
Robert Chow
Kenneth Yim
Bernard G. Jaar
author_sort Patrick Oguejiofor
collection DOAJ
description 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.
format Article
id doaj-art-6781923aadd34883a503d7fbfd1d2137
institution Kabale University
issn 2090-6641
2090-665X
language English
publishDate 2017-01-01
publisher Wiley
record_format Article
series Case Reports in Nephrology
spelling doaj-art-6781923aadd34883a503d7fbfd1d21372025-02-03T06:01:19ZengWileyCase Reports in Nephrology2090-66412090-665X2017-01-01201710.1155/2017/85961698596169Successful Management of Refractory Type 1 Renal Tubular Acidosis with AmiloridePatrick Oguejiofor0Robert Chow1Kenneth Yim2Bernard G. Jaar3Department of Internal Medicine, University of Maryland School of Medicine, Baltimore, MD, USADepartment of Internal Medicine, University of Maryland School of Medicine, Baltimore, MD, USADivision of Nephrology, University of Maryland Medical Center Midtown Campus, Baltimore, MD, USADepartment of Medicine, Division of Nephrology, Johns Hopkins School of Medicine, Baltimore, MD, USAA 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.http://dx.doi.org/10.1155/2017/8596169
spellingShingle Patrick Oguejiofor
Robert Chow
Kenneth Yim
Bernard G. Jaar
Successful Management of Refractory Type 1 Renal Tubular Acidosis with Amiloride
Case Reports in Nephrology
title Successful Management of Refractory Type 1 Renal Tubular Acidosis with Amiloride
title_full Successful Management of Refractory Type 1 Renal Tubular Acidosis with Amiloride
title_fullStr Successful Management of Refractory Type 1 Renal Tubular Acidosis with Amiloride
title_full_unstemmed Successful Management of Refractory Type 1 Renal Tubular Acidosis with Amiloride
title_short Successful Management of Refractory Type 1 Renal Tubular Acidosis with Amiloride
title_sort successful management of refractory type 1 renal tubular acidosis with amiloride
url http://dx.doi.org/10.1155/2017/8596169
work_keys_str_mv AT patrickoguejiofor successfulmanagementofrefractorytype1renaltubularacidosiswithamiloride
AT robertchow successfulmanagementofrefractorytype1renaltubularacidosiswithamiloride
AT kennethyim successfulmanagementofrefractorytype1renaltubularacidosiswithamiloride
AT bernardgjaar successfulmanagementofrefractorytype1renaltubularacidosiswithamiloride