Congenital pseudohypoaldosteronism: case report and review. Effect of indomethacin during sodium chloride depletion.
A newborn boy (birth weight 2550 g) presented from the first days of life with poor drinking, moderate vomiting and persistent weight loss. On hospital admission at age 4 weeks, there were severe dehydration, dystrophy and electrolyte disturbances (Na 107, Cl 80, K 5,4 mval/l). The usual causes of salt wasting were excluded, but plasma renin activity, plasma aldosterone and urinary aldosterone-18-glucuronide were markedly increased. DOCA had no salt-retaining effect, but a sodium chloride supplement of 3 g per day improved the clinical condition dramatically and normalized the electrolyte values. With this treatment, plasma renin activity and aldosterone were normal or almost normal beyond the age of 6.5 months, but urinary aldosterone-18-glucuronide remained slightly increased. Considerable augmentation of the plasma renin activity and of urinary aldosterone-18-glucuronide, but no clear salt loss were induced by spironolactone. With salt restriction, there was evidence for marked salt loss. Its progress could be inhibited by administration of indomethacin. Since indomethacin inhibits the synthesis of prostaglandins with saluretic activity, it is probable that the prostaglandins participate in the pathogenesis of the salt wasting in pseudohypoaldosteronism.
D000309:Adrenal Insufficiency / Q000175:diagnosis*; D000450:Aldosterone / Q000097:blood / Q000172:deficiency* / Q000652:urine; D001835:Body Weight / Q000187:drug effects; D002712:Chlorides / Q000097:blood; D003900:Desoxycorticosterone / Q000627:therapeutic use; D003937:Diagnosis, Differential; D004032:Diet; D006801:Humans; D007213:Indomethacin / Q000627:therapeutic use*; D007231:Infant, Newborn; D008297:Male; D011188:Potassium / Q000097:blood; D012083:Renin / Q000097:blood; D012964:Sodium / Q000097:blood / Q000652:urine; D012965:Sodium Chloride / Q000378:metabolism*; D013148:Spironolactone / Q000627:therapeutic use
D002712:Chlorides; D013148:Spironolactone; D003900:Desoxycorticosterone; D012965:Sodium Chloride; D000450:Aldosterone; D012964:Sodium; D012083:Renin; D011188:Potassium; D007213:Indomethacin