Andrio Palayukan,Novalin Sumendap
Mitra Masyarakat Hospital
Background : Bartter syndrome is a group of clinical manifestations due to an autosomal recessive genetic disorder with one characteristic of hypokalemia beside metabolic alkalosis and hyperreninemia with normal blood pressure. The reported prevalence of Bartter syndrome was 1 in 1,000,000.
Case Presentation Summary : A 13-year-old girl came to the emergency room with a chief complaint of weakness in both legs. Patient claimed to be able to spend almost 5 liter of mineral water in one day. Family history of similar complaints were denied. Complaints have been experienced since 4 years ago. Several investigations performed last year revealed hypokalemia (2.4 mmol/L), metabolic alkalosis (pH 7.50), and hyperreninemia (14.0 ng/mL/hr) with normal aldosterone level (6.2 ng/dL). Vital signs: Blood Pressure 100/70 mmHg, Heart Rate 88 per minute, Respiratory Rate 22 per minute, temperature 36oC. Physical examination: drooping mouth, decreased muscle strength (movement against gravity but not resistance). Her full blood count was normal. Electrolyte examination: sodium 133 mmol/L, potassium 2.0 mmol/L, and chloride 103 mmol/L. Patient was treated with 30 mEq of potassium every 6 hours intravenously, K L-aspartate 600 mg every 8 hours and consumption of high potassium foods (tomatoes, bananas). She was discharged after 2 days of treatment. She was able to walk and the muscle strength was back to normal. From the electrolyte examination, the level of Potassium was 2.8 mmol /L.
Learning Points/Discussion : Correction of hypokalemia is the primary goal of medical therapy in Bartter Syndrome. Intravenous administration of potassium is required to increase plasma potassium levels in severe hypokalemia. Patients also require oral potassium supplementation and consumption of foods with high potassium levels. The tubular defect in Bartter syndrome is incurable so that lifetime treatment is required. Bartter syndrome has a good prognosis when handled quickly.