Abstract
A congenital subtype of hypokalemic tubular disorders in 5 children (age 8 m to 4 ys) is described with many features resembling Bartter's syndrome. Additional features are: Prenatal onset with polyhydramnios and premature labor; failure to thrive; life threatening crises of fever, diarrhea, renal electrolyte and water wasting (however with normal renal chloride reabsorption); hypercalciuria, nephrocalcinosis and osteopenia. Renal PGE2 activity was monitored by mass spectrometric determination of urinary PGE2, and systemic PGE2 activity by a major urinary metabolite (PGE-M). The pathogenetic role of PGE2 is supported by withdrawal of PG synthesis inhibitors (PGSI) indomethacin or aspirin.
Prolonged indomethacin treatment significantly improved the affected children's general condition. We conclude that renal as well as systemic PGE2 hyperactivity ranks high in the pathogenetic chain of events in this complex tubular disorder.
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Seyberth, H., Kühl, P., Schweer, H. et al. 1632 THE ROLE OF PROSTAGLANDIN E2 (PGE2) IN A CONGENITAL HYPOKALEMIC TUBULAR DISORDER WITH HYPERCALCIURIA AND PREMATURITY. Pediatr Res 19, 382 (1985). https://doi.org/10.1203/00006450-198504000-01656
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DOI: https://doi.org/10.1203/00006450-198504000-01656