A Case of Pseudo-Bartter Syndrome with Severe Metabolic Alkalosis

INTRODUCTION: The Pseudo-Bartter Syndrome, unlike the Bartter and Gitelman Syndromes, is characterized with metabolic alkalosis without a tubular defect. We aimed to summarize the management of metabolic alkalosis by discussing a case of Pseudo-Bartter Syndrome with severe metabolic alkalosis.

CASE: A 35-year-old female was admitted to emergency with nausea, vomiting, anuria and altered consciousness. She had suffered from attacks of vomiting for the last two years that had increased in severity for the last ten days and her urine volume had decreased progressively ending in anuria for the last 48 hours. Her physical examination was normal except distorted orientation for time and place. Abnormal laboratory findings were: urea:160mg/dl, creatinin:12.02mg/dl, Na:145mmol/L, K:3.67mmol/L, Cl:73mmol/ L, pH:7.62, HCO3 :56.9mmol/L, pCO2 :58mmHg, uric acid:16.4mg/ dl, albumin:3.27gr/dl and phosphorus:9.5mg/dl. Ultrasonography showed normal sized kidneys with increased echogenity. She needed hemodialysis due to anuria and uremia despite improved metabolic alkalosis (pH:7.38 and HCO3 -:46) with isotonic fluid replacement. Her urine flow started on the tenth day (urine chloride concentration:17meq/L); urea and creatinine levels decreased progressively without hemodialysis. Upper gastrointestinal endoscopy showed an ulcer causing pyloric obstruction; pathology was consistent with signet ring cell carcinoma. She was accepted as inoperable and followed with palliative measures.

DISCUSSION: The etiology of metabolic alkalosis includes gastrointestinal H+ and Cl- loss, diuretic use (hipovolemia, chloride loss), HCO3 - treatment, movement of H+ ions intracellularly (hypokalemia), loss of gastric content in patients with achlorhydria and profuse sweating in cystic fibrosis. Our patient didnot have diarhea, profuse sweating or history of laxative use. The Bartter and Gitelman Syndromes were excluded due to her age, low urine chloride level, good response to isotonic fluid and presence of a definite etiology. She was diagnosed with pyloric stenosis, Pseudo-Bartter syndrome due to Cl- and H+ loss, hypovolemia and acute renal failure.

CONCLUSION: The underlying etiology must be evaluated and treatment should be programmed accordingly when dealing with metabolic alkalosis.