Long-Term Follow-Up of a Girl with the Neonatal Form of Bartter's Syndrome.

Abstract

Abstract.We followed up a girl with the neonatal form of Bartter's syndrome for sixteen years and determined the sensitivity to angiotensin II before and during the indomethacin treatment.A 4-monthold girl was admitted to our hospital, because of severe hypokalemia and growth retardation. Initially we treated her with spironolactone and potassium supplements. This treatment increased plasma potassium levels and her growth. At the age of one year she was diagnosed as having Bartter's syndrome.Since then she has been treated with indomethacin at an initial dose of 3 mg/kg/day combined with spironolactone and potassium. After the start of the indomethacin treatment, her growth increased dramatically, and her final height was normal adult height. Her puberty developed normally and menarche occurred at the age of 12 years. Levels of serum sodium, chloride, plasma aldosterone and urinary prostaglandin E2 were also normalized. Levels of angiotensin I and II were improved but not within the normal range, but plasma potassium levels slightly decreased after plasma aldosterone levels were normalized and did not change during the treatment period. Plasma renin activity remained high until about the age of 8 years, after which it decreased to almost within the normal range. At 5 months after the start of indomethacin (3 mg/kg/day), her vascular sensitivity to angiotensin II had been improved, and after 2 years and 5 months, her vascular sensitivity was further improved. At this time renin activity had decreased after angiotensin II infusion, but plasma aldosterone did not change. At the age of 16 years (dose of indomethacin: 0.5 mg/kg/day), plasma aldosterone increased after angiotensin II infusion. These data suggest that indomethacin and spironolactone are effective treatments for the neonatal form of Bartter's syndrome, especially during childhood.

Bartter Syndrome Type 3: Phenotype-Genotype Correlation and Favorable Response to Ibuprofen

“…Futhermore, for the past few years, more and more studies have raised that, on the basis of salt substitution, prostaglandin synthetase inhibitors may play an important part in improving growth retardation ( 18 – 20 ), which was also suggested in case 1. According to long-term follow-up studies in Bartter Syndrome, with appropriate treatment, patients with Bartter syndrome can achieve normal electrolyte values and growth parameters ( 21 , 22 ), as we also observed in case 1 regarding the electrolytes while growth retardation remained. Case 2 however still showed electrolyte imbalance, alkalosis and growth retardation even after treatment with salt substitution and prostaglandin synthetase inhibitors.…”

Successful management of an extreme example of neonatal hyperprostaglandin-E syndrome (Bartter???s syndrome) with the new cyclooxygenase-2 inhibitor rofecoxib

“…However, it does not correct the primary chloride resorption defect or improve the urinary concentration defect because indomethacin does not affect the ion channels (2,10,11,13,14). Normal growth and development is feasible for most patients treated with regular indomethacin therapy (12,14,(17)(18)(19)(20)(21)(22)(23).…”

Nephrocalcinosis and Placental Findings in Neonatal Bartter Syndrome

“…On the contrary, our findings suggest that nephrocalcinosis may be inevitable because IND was not effective in our case. We checked the usage dose of IND in the previous reports 5,6,10. It was around 2 to 3 mg/kg/d, which was too much for us, because we usually use it in dosages of 0.2 mg/kg for patent ductus arteriosus therapy.…”