Abstract
Two male patients were found to have Southeast Asian ovalocytosis - one incidentally and the other one during the investigations of the aetiology for hypokalemic paralysis. Southeast Asian ovalocytosis is found almost exclusively in Southeast Asia (1, 2). Familial renal tubular acidosis can co-exist with south East Asian ovalocytosis in the same patient (2). Anionexchanger 1 gene AE1 mutation is the underlying pathology and both can originate from the same mutation (2). This can be asymptomatic as in case 1 and can be symptomatic as in case 2. Symptomatic patients need oral potassium and bicarbonate replacement and follow up. After initial clinical assessment and investigations of case 2, he was given intravenous potassium replacement. The correction of metabolic acidosis was made by giving oral sodium bicarbonate 600mg twice daily. Eventually he made full recovery and did not develop further similar episodes.