Role of prostaglandins in hyperprostaglandin E syndrome and in selected renal tubular disorders
- 55 Downloads
Renal and systemic prostanoid activity was assessed in various renal tubular disorders, using mass spectrometric determination of urinary excretion rates of primary prostaglandins (PGE2, PGF2α, PGI2, and TXA2) and their systemically produced index metabolites. Only PGE2 levels (normal range: 2.0–16.4 ng/h per 1.73 m2) are elevated in Bartter syndrome (median: 43.4, range: 6.7–166.3), nephrogenic diabetes insipidus (46.2, 12.1–1290), Fanconi syndrome (96.6, 19.3–135.5), and in a complex tubular disorder in premature infants (40.7, 22.3–132.1), for which the term hyperprostaglandin E syndrome has been introduced. In this disorder with a Bartter-syndrome-like tubulopathy, the systemic features of the disease such as fever, diarrhoea and osteopenia with hypercalciuria were associated with increased systemic PGE2 activity. In most patients the urinary excretion rate of the systemic index metabolite of PGE2 (PGE-M) was markedly elevated (1028, 285–4709; normal range: 104–664 ng/h per 1.73 m2). Hypercalciuria per se was associated neither with increased renal nor with systemic PGE2 hyperactivity. Most problems in infants with hyperprostaglandin E syndrome could be controlled by long-term indomethacin treatment in contrast to the moderate and partial effect of this treatment in patients with Fanconi syndrome. Thus increased PGE2 synthesis plays a major role in the pathogenesis of hyperprostaglandin E syndrome, while in Fanconi syndrome PGE2 hyperactivity in the kidney is a secondary event and only aggravates the water and electrolyte wastage.
Key wordsProstaglandins Tubular disorders Bartter syndrome Nephrogenic diabetes inspidus Hypercalciuria Fanconi syndrome Hyperprostaglandin E syndrome
Unable to display preview. Download preview PDF.
- 4.Gill JR (1985) Bartter's syndrome. In: Gonick HC, Buckalew VM (eds) Renal tubular disorders: pathophysiology, diagnosis and management. Dekker, New York, Basel, pp 457–473Google Scholar
- 8.Catella F, Nowak J, FitzGerald GA (1986) Measurement of renal and non-renal eicosanoid synthesis. Am J Med 81 [suppl 2B]: 23–29Google Scholar
- 9.Güllner H, Bartter FC, Cerletti C, Smith JB, Gill JR (1979) Prostacyclin overproduction in Bartter's syndrome. Lancet II: 2: 767–768Google Scholar
- 10.Patrono C, Cinotti GA, Ciabattoni G, Pugliese F (1980) Prostaglandins, thromboxanes, and renal physiology: introduction. In: Remuzzi G, Mecca G, de Gaetano G (eds) Hemostasis, prostaglandins, and renal disease. Raven Press, New York, pp 151–157Google Scholar
- 11.Watson ML, Branch RA, Gill JR, Pates JA, Brash AR (1983) Systemic prostaglandin I2 synthesis is normal in patients with Bartter's syndrome. Lancet II: 1: 368–369Google Scholar
- 12.Seyberth HW, Müller H, Soeding K, Wille L, Hackenthal E (1983) Urinary excretion rate of 6-keto-prostaglandin Ftα as an index of circulating prostacyclin. Adv Prostaglandin Thromboxane-Leukotriene Res 11: 533–538Google Scholar
- 15.Rascher W, Rosendahl W, Henrichs IA, Maier R, Seyberth HW (1987) Congenital nephrogenic diabetes insipidus: vasopressin and prostaglandins in response to treatment with hydrochlorothiazide and indomethacin. Pediatr Nephrol 1: 487–492Google Scholar