Abstract
Hypokalemia is a rare but important cause of rhabdomyolysis in children. Unlike typical rhabdomyolysis, hyperkalemia may be absent because the triggering factor is hypokalemia, potentially delaying diagnosis. We report a five-year-old boy with hypoplastic left heart syndrome after a Fontan procedure who developed severe hypokalemia and rhabdomyolysis following prolonged diarrhea while receiving loop diuretics. He presented with acute lower extremity weakness and inability to stand. Laboratory evaluation revealed potassium 1.9 mmol/L and creatine kinase 9,013 U/L, peaking at 32,908 U/L. Urinalysis showed myoglobinuria, and electrocardiography demonstrated ST depression and prominent U waves. Intravenous potassium replacement and diuretic adjustment resulted in rapid clinical recovery without acute kidney injury. A review of previously reported pediatric cases suggests that potassium depletion from gastrointestinal losses or renal wasting predisposes children to rhabdomyolysis. Early recognition and prompt electrolyte correction are essential.