Successful indomethacin treatment of two paediatric patients with severe tubulopathies. A boy with an unusual hypercalciuria and a girl with cystinosis.
Two children were followed for severe congenital tubulopathies: a boy presented an excessive sodium, calcium and water excretion; a girl had cystinosis and a De Toni-Debré-Fanconi syndrome. These renal defects were both associated with increased levels of plasma renin activity and aldosterone, and excessive urinary PGE1 production. They had been unresponsive to therapeutic attempts. Only indomethacin treatment was successful in reversing the biochemical abnormalities and improving the growth pattern.
D000450:Aldosterone / Q000097:blood; D001834:Body Water / Q000378:metabolism; D002118:Calcium / Q000652:urine*; D002675:Child, Preschool; D002986:Clinical Trials as Topic; D003554:Cystinosis / Q000188:drug therapy*; D005198:Fanconi Syndrome / Q000188:drug therapy; D005260:Female; D006801:Humans; D007213:Indomethacin / Q000627:therapeutic use*; D007684:Kidney Tubules / Q000503:physiopathology; D008297:Male; D011458:Prostaglandins E / Q000652:urine; D015499:Renal Tubular Transport, Inborn Errors / Q000188:drug therapy*; D012083:Renin / Q000097:blood; D012964:Sodium / Q000378:metabolism
D011458:Prostaglandins E; D000450:Aldosterone; D012964:Sodium; D012083:Renin; D002118:Calcium; D007213:Indomethacin