The Spitzer-Weinstein Syndrome: One Form of Type IV Renal Tubular Acidosis and Its Response to Indapamide: A Case Report

Abstract

Spitzer-Weinstein is rare disorder characterized by thiazide responsive hyperkalemia and normal anion gap metabolic acidosis, similar to Gordon syndrome.The hyperfunction of thiazide–sensitive Na-Cl cotransporter (TSC) is the main pathophysiological mechanism. We present a 21-year-old male with normal blood pressure, persistently elevated serum potassium, and metabolic asidosis. The diagnosis of Spitzer-Weinstein syndrome was made by clinical pictures and thiazide test. After using 1.5 mg indapamide from a group of thiazide diuretics, his serum potassium decreased from 6.68 mmol/L to 3.54 mmol/L and the daily urine potassium excretion increased from 13.2 mmol to 34.1 mmol. This patient then took indapamide 1.5 mg daily. The persistent hyperkalemia and metabolic acidosis were corrected. Thiazide, a powerful inhibitor of TSC, proved to be a useful tool for the diagnosis and treatment of Spitzer-Weinstein syndrome.