The role of cyclooxygenases and prostanoid receptorsin furosemide-like salt losing tubulopathy: the hyperprostaglandin E syndrome.
Hyperprostaglandin E syndrome/antenatal Bartter syndrome is characterized by NaCl wasting and volume depletion, juxtaglomerula hypertrophy, hyperreninism and secondary hyperaldosteronism. Primary causes are mutations in the gene for Na-K-2Cl-cotransporter, NKCC2, or for potassium channel, ROMK, responsible for medullary NaCl malabsorption. Most intriguing aspect of the syndrome is the association with a massively increased renal prostaglandin production which contributes substantially to the clinical picture of the patients. Therefore the term hyperprostaglandin E syndrome has been introduced. It is unclear how prostaglandins aggravate the NaCl transport deficiency. Aspects to prostaglandin synthesis and receptor-mediated function within the kidney in patients suffering from hyperprostaglandin E syndrome/antenatal Bartter syndrome will be discussed.
Nüsing
R M
RM
Department of Pediatrics, Philipp's University, Marburg, Germany.
Seyberth
H W
HW
D001477:Bartter Syndrome / Q000503:physiopathology*; D004231:Diuresis; D006801:Humans; D007668:Kidney / Q000378:metabolism; D011451:Prostaglandin-Endoperoxide Synthases / Q000502:physiology*; D011458:Prostaglandins E / Q000096:biosynthesis; D018078:Receptors, Prostaglandin E / Q000502:physiology*
D011458:Prostaglandins E; D018078:Receptors, Prostaglandin E; D011451:Prostaglandin-Endoperoxide Synthases