Abstract
The neonatal form of Bartter syndrome is characterized by intrauterine onset of polyuria leading to severe polyhydramnios. We report a case of a 31 weeks, male baby was born by emergency Caesarean Section due to severe polyhydramnios (AFI 54). Postnatally, baby had polyuria, dehydration, hyponatremia, hypokalemia and hypochloremic metabolic alkalosis. Possibility of neonatal Bartter syndrome was supported by high serum renin and aldosterone levels. Correction of electrolytes and dehydration along with indomethacin constituted the treatment. This syndrome is reported to make paediatricians aware about the severe neonatal form of the disease.J Nepal Paediatr Soc 2016;36(2):204-207.
Highlights
Bartter syndrome is an autosomal recessive disorder characterized by severe polyuria and defective reabsorption of chloride in Thick Ascending limb of Loop of Henle
Fetal polyuria leads to polyhydramnios and preterm delivery
Polyuria can lead to life-threatening dehydration[1,2].The disease is characterized by hypokalemia, hypochloremia, metabolic alkalosis, hyperplasia of juxtaglomerular apparatus, hyperreninaemia, secondary hyperaldosteronism and normal blood pressure
Summary
Polyuria can lead to life-threatening dehydration[1,2].The disease is characterized by hypokalemia, hypochloremia, metabolic alkalosis, hyperplasia of juxtaglomerular apparatus, hyperreninaemia, secondary hyperaldosteronism and normal blood pressure. Baby had polyuria with urine output of 5 ml/Kg/hour on day1of life which increased to 7 ml/Kg/hour on day 3 and 11.7% weight loss. Diagnosis of neonatal Bartter was considered as baby had metabolic alkalosis, hypochloremia, hyponatremia, hypokalemia and normocalcaemia. As the serum renin and aldosterone levels were raised, diagnosis of neonatal Bartter’s syndrome was confirmed on the 5thdayof life. Nasogastric tube feeds were gradually hiked and baby achieved full nasogastric tube feeds on day 10 of life. Baby required sodium replacement up to 6 mEq/kg/day and potassium up to 8 mEq/kg/ day. Baby improved over the two weeks and at present two months old, thriving with adequate weight gain, developmentally normal and without nephrocalcinosis on USG
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