Implications of disorders of purine metabolism for the kidney and the urinary tract.
The spectrum of kidney and urinary tract disorders related to purines comprises acute hyperuricosuric nephropathy, chronic urate nephropathy and urolithiasis. Two factors in the development of acute hyperuricosuric nephropathy are increased uric acid concentration and low pH in the tubular fluid. Chronic urate nephropathy still possess several problems: incidence (although this seems to be decreasing, presumably owing to effective prevention), the source of interstitial urate, the cause of the interstitial deposition of urate, and the role of urate deposits in the pathogenesis of the interstitial nephropathy. The relation of the experimental nephropathy to the pathogenesis of chronic urate nephropathy in the human is not yet clear but a model is proposed according to which interstitial urate derives from two sources: hyperuricaemic plasma and hyperuricosuric tubular fluid. Urolithiasis related to purines leads to uric acid-urate stones, xanthine stones, 2,8-dihydroxyadenine stones, iatrogenic xanthine and oxipurinol stones, and possibly calcium stones. Pathogenetic factors in uric acid lithiasis are hyperuricosuria (whether due to an inborn enzyme abnormality or of unknown aetiology) and low urinary pH; oliguria is a contributory factor. There remain several open questions about uric acid lithiasis: incidence, the shift of its location from lower to upper urinary tract, the interplay of pathogenetic factors, and the role of compounds which inhibit crystallization.
D000228:Adenine Phosphoribosyltransferase / Q000172:deficiency; D000493:Allopurinol / Q000627:therapeutic use; D000818:Animals; D002118:Calcium / Q000378:metabolism; D006073:Gout / Q000378:metabolism; D006801:Humans; D006863:Hydrogen-Ion Concentration; D007041:Hypoxanthine Phosphoribosyltransferase / Q000172:deficiency; D007669:Kidney Calculi / Q000453:epidemiology / Q000378:metabolism; D007674:Kidney Diseases / Q000378:metabolism* / Q000503:physiopathology; D007676:Kidney Failure, Chronic / Q000378:metabolism; D010117:Oxypurinol / Q000378:metabolism; D011687:Purines / Q000378:metabolism*; D012964:Sodium / Q000378:metabolism; D012995:Solubility; D014527:Uric Acid / Q000378:metabolism*; D014570:Urologic Diseases / Q000378:metabolism*; D014969:Xanthine Oxidase / Q000172:deficiency; D014970:Xanthines / Q000378:metabolism
D011687:Purines; D014970:Xanthines; D014527:Uric Acid; D000493:Allopurinol; D012964:Sodium; D014969:Xanthine Oxidase; D000228:Adenine Phosphoribosyltransferase; D007041:Hypoxanthine Phosphoribosyltransferase; D010117:Oxypurinol; D002118:Calcium