Successful treatment with two siblings affected classic Bartter syndrome

Case presentation Two siblings were admitted to National Hospital of Pediatrics with chief complaints of motor-development delay, growth retardation and failure of thrive. The 1 child a 39 month old boy with his birth weight of 1.9 kg presented with his height of 69 cm (-7.5 SD), his weight of 7.5 kg (-4.4 SD). The 2 child – an 18 months old girl presented with her height of 58 cm (-7.8 SD), her weight of 5.8 kg (-3.4 SD). They both developed polyuria (6ml/kg/hour), polydipsia, chronic dehydration and motor delay that they could not stand and walk but had normal intelligence. The investigations revealed hypokalemic metabolic alkalosis (PH: 7.5 – 7.51; pCO2: 45.9 56.8 mmHg; HCO3: 36.6 45.7 mmol/l; serum potassium levels: 1.8 – 2.1 mmol/l), hyponatremia (125 – 128 mmol/l), hypochloremia (67 – 84 mmol/l), normal calcemia, and normal calciuria. They were treated with potassium supplement, indomethacin (2.5 mg/kg/ day). After 15 months of treatment: height, weight of the 1 boy and 2 girl were 94 cm (increasing 25cm; -3 SD), 12kg (increasing 4.5kg; -3 SD) and 84 cm (increasing 26 cm; -2.4 SD), 11 kg (increasing 5.2 kg; -1.3 SD), respectively. Their plasma electrolyte became normal after 2 weeks of treatment.