Urate Deposition and Stone Formation in the Kidney in Renal Hypouricemia

  • O. Sperling
Conference paper


Renal hypouricemia is associated with various degrees of hyperuricosuria. This abnormality is the cause of two types of pathological manifestation: uric acid urolithiasis and uric acid nephropathy associated with acute renal failure. In the following, I shall elaborate on the various types of renal hypouricemia, on the mechanism of the renal tubular defect, and on the clinical pathological manifestations.


Uric Acid Urate Transport Urate Excretion Urate Deposition Renal Hypouricemia 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Sperling O (1989) Hereditary renal hypouricemia. In: Scriver CR, Beaudet AK, Sly WS and Valle D (eds) The Metabolic Basis of Inherited Disease 6th edition, McGraw-Hill, p 2605Google Scholar
  2. 2.
    Wallis IA, EAGLE RI (1957) The adult Fanconi syndrome. II. Review of eighteen cases. Amer J Med 22: 13–23PubMedCrossRefGoogle Scholar
  3. 3.
    Baron DN, Dent CE, Harris H, Hard EW, Jebson JB (1956) Hereditary Pellagra-like skin rash with temporary cerebellar ataxia, constant renal aminoaciduria and other bizzare biochemical features. Lancet 271: 421–428PubMedCrossRefGoogle Scholar
  4. 4.
    Beck IH (1979) Hypouricemia in the syndrom of inappropriate secretion of antidiuretic hormone. New Engl J Med 301: 538–530Google Scholar
  5. 5.
    Smithline N, Kassirer JP, Cohen JJ (1976) Light-chain nephropathy. N Engl J Med 294: 71–74PubMedCrossRefGoogle Scholar
  6. 6.
    Bennett JS, Bond J, Singer I, Gottlieb AJ (1952) Hypouricemia in Hodgkin’s disease. Ann Intern Med 76: 751–756Google Scholar
  7. 7.
    Weinstein B, Irreverre F, Watkin DM (1965) Lung carcinoma, hypouricemia and aminoaciduria. Am J Med 39: 520–526PubMedCrossRefGoogle Scholar
  8. 8.
    Chisholm JJ Jr, Harrison HAC, Everlein WR, Harrison HE (1955) Amino-aciduria, hypohosphatemia and rickets in lead poisoning. In: Lawson RB, Weech AA, Smyth FS, Kennedy RIJ, Wilson JI, Zuelzer WW (eds) American Journal of Diseases of Children, Chicago American Medical Association, p 159Google Scholar
  9. 9.
    Gross JM (1963) Fanconi syndrome (adult type) developing secondary to the ingestion of outdated tetracycline. Ann Intern Med 48: 523–528Google Scholar
  10. 10.
    Schlosstein L, Kippen I, Bluestone R, Whitehouse MW, Klineberg JR (1974) Association between hypouricemia and jaundice. Ann Rheum Dis 33: 308 —312PubMedCrossRefGoogle Scholar
  11. 11.
    Michelis MF, Warms PC, Fusco RD, Davis BB (1974) Hypouricemia and hyperuricosuria in Laennec cirrhosis. Arch Intern Med 134: 681–683PubMedCrossRefGoogle Scholar
  12. 12.
    Berliner RW, Hilton JG, Tu TF, Kennedy JR TJ (1950) The renal mechanism for urate excretion in man. J Clin Invest 29: 396–401PubMedCrossRefGoogle Scholar
  13. 13.
    Gutman AB, Tu TF, Berger L (1959) Tubular secretion of urate in man. J Clin Invest 38: 1778–1781PubMedCrossRefGoogle Scholar
  14. 14.
    Yu TF, Berger L, Stone DJ, Wolf J, Gutman AB (1957) Effect of pyrazinamide and pyrazinoic acid on urate clearance and other discrete renal functions. Proc Soc Exp Biol (N. Y. ) 96: 264–267Google Scholar
  15. 15.
    Yu TF, Berger L, Gutman AB (1961) Suppression of tubular secretion of urate by pyrazinamide in the dog. Proc Soc Exp Biol (N. Y.) 107: 905 —908Google Scholar
  16. 16.
    Steele TH, Rieselbach RE (1967) The renal mechanism for urate homeostasis in normal man. Amer J Med 43: 868–875PubMedCrossRefGoogle Scholar
  17. 17.
    Abramson RG, Levitt MF (1975) Micropuncture study of uric acid in rat kidney. Am J Physiol 228: 1597–1605PubMedGoogle Scholar
  18. 18.
    Wyngaarden JB, Kelley WN (1983) Gout. In: Stanbury JB, Wyngaarden JB, Fredrickson DS, Goldstein JL, Brown MS (eds) The Metabolic Basis of Inherited Disease. McGraw-Hill Book Company, New York, 1983, p 1043Google Scholar
  19. 19.
    Kahn AM, Weinman EJ (1985) Urate transport in the proximal tubule: in-vivo and vesicle studies. Am J Physiol 249: F789–798PubMedGoogle Scholar
  20. 20.
    Roch-Ramel F, Weiner IM (1973) Excretion of urate by the kidney of Cebus monkeys: A micropuncture study. Am J Physiol 224: 1369–1374PubMedGoogle Scholar
  21. 21.
    de Rougemont D, Henchoz M, Roch-Ramel F (1976) Renal urate excretion at various plasma concentrations in the rat: a free-flow micropuncture study. Am J Physiol 231: 387–392PubMedGoogle Scholar
  22. 22.
    Weiner IM, Fanelli GM JR (1975) Renal urate excretion in animal models. Nephron 14: 33–47PubMedCrossRefGoogle Scholar
  23. 23.
    Praetorius E, Kirk JE (1950) Hypouricemia with evidence for tubular elimination of uric acid. J Lab Clin Med 35: 865–868PubMedGoogle Scholar
  24. 24.
    Podevin R, Ardaillou R, Paillard F, Fontannele J, Richet G (1981) Etude chez l’ homme de la cinetique d’ apparition dans purine de l’acide urique 2 4C. Nephron 5: 134–140CrossRefGoogle Scholar
  25. 25.
    Weinman EJ, Sansom SC, Steplock DA, Sheth AU, Knight TF, Senekjian HO (1980) Secretion of urate in the proximal convoluted tubule of the rat. Am J Physiol 239: F393 —F387Google Scholar
  26. 26.
    Shimomura A, Chonko A, Tanner RM, Edwards R, Grantham JJ (1981) Nature of urate transport in isolated rabbit proximal tubules. Am J Physiol 241: F565 — F578PubMedGoogle Scholar
  27. 27.
    Gutman AB, Yu TF (1961) A three-component system for regulation of renal excretion of uric acid in man. Trans Assoc Am Physicians 74: 353–364PubMedGoogle Scholar
  28. 28.
    Steele TH, Boner G (1973) Origins of the uricosuric response. J Clin Invest 52: 1368–1375PubMedCrossRefGoogle Scholar
  29. 29.
    Steele TH (1973) Urate secretion in man: The pyrazinamide suppression test. Ann Intern Med 79: 734–737PubMedGoogle Scholar
  30. 30.
    Diamond HS, Paolini JS (1973) Evidence for a post-secretory reabsorptive site for uric acid in man. J Clin Invest 52: 1491–1499PubMedCrossRefGoogle Scholar
  31. 31.
    Rieselbach RE, Steele TH (1974) Influence of the kidney upon urate homeostasis in health and disease. Am J Med 56: 665–675PubMedCrossRefGoogle Scholar
  32. 32.
    Grantham JJ, Chonko AM (1986) Renal handling of organic anions and cations; metabolism and excretion of uric acid. In: Brenner BM (ed) The Kidney (3rd ed.) Saunders Company, Philadelphia, p 663–700Google Scholar
  33. 33.
    Harkness RA, Nicol AD (1969) Plasma uric acid levels in children. Arch Dis Child 44: 773–778PubMedCrossRefGoogle Scholar
  34. 34.
    Stapelton FB (1983) Renal uric acid clearance in human neonates. J Pediatr 103: 290 — 294CrossRefGoogle Scholar
  35. 35.
    Wolfson WO, Hunt HJ, Levine E, Gutterman HS, Cohn C, Rosenberg EF, Huddlestun B, Kadota IC (1949) The transport and excretion of uric acid in man V. A sex differential in urate metabolism with a note on clinical and laboratory findings in gouty women. J Clin Endocrinol 9: 749–767CrossRefGoogle Scholar
  36. 36.
    Steele TH, Oppenheimer S (1969) Factors affecting urate excretion following diuretic administration in man. Am J Med 47: 563 —573CrossRefGoogle Scholar
  37. 37.
    Steele TH, Manuel MA, Boner G (1975) Diuretics, urate excretion and sodium reabsorption: A test of acetazolamide and urinary alkalinization. Nephron 14: 48–61CrossRefGoogle Scholar
  38. 38.
    Emmerson BT (1978) Abnormal urate excretion associated with renal and systemic disorders, drugs and toxins. In: Kelley WN, Weiner IM (eds) Handbook of Experimental Pharmacology, Uric Acid. Springer-Verlag, Berlin, Vol 51, p 287Google Scholar
  39. 39.
    Yu TF, Gutman AB (1959) Study of the paradoxical effects of salicylate in low, intermediate and high dosage on the renal mechanisms for excretion of urate in man. J Clin Invest 38: 1298–1315PubMedCrossRefGoogle Scholar
  40. 40.
    Stewart RJ, Chonko AM (1981) Pharmacologic inhibition of urate transport across perfused and non-perfused rabbit proximal straight tubules. Kidney Int 19: 258Google Scholar
  41. 41.
    Meisel AD, Diamond HS (1977) Hyperuricosuria in the Fanconi syndrome. Am J Med Sci 273: 109–115PubMedCrossRefGoogle Scholar
  42. 42.
    Wilson DB, Goldstein NP (1973) Renal urate excretion in patients with Wilson’s disease. Kidney Int 4: 331–336PubMedCrossRefGoogle Scholar
  43. 43.
    Gibson T, Sims HP, Jimenez SA (1976) Hypouricemia and increased renal urate clearance associated with hyperparathyroidism. Ann Rheum Dis 35: 372–376PubMedCrossRefGoogle Scholar
  44. 44.
    Weinberger A, Weinberger A, Sperling 0, Ben-Bassat M, Kaplan I, Pinkhas J (1977) Increased uric acid clearance in patients with burns. Biomedicine Express 27: 277–278PubMedGoogle Scholar
  45. 45.
    Kay NE, Gotleib AJ (1973) Hypouricemia in Hodgkin’s disease: Report of an additional case. Cancer 32: 1508— 1511PubMedCrossRefGoogle Scholar
  46. 46.
    Sperling 0, Boer P, Weinberger A, de Vries A (1975) Transport into erythrocytes and intestinal absorption of uric acid in hereditary renal hypouricemia. Biomedicine 23: 157–159PubMedGoogle Scholar
  47. 47.
    Vinay P, Gatterean A, Moulin B, Gougoux A, Lemieux G (1983) Normal urate transport into erythrocytes in familial renal hypouricemia and in Dalmatian dog. Can Med Assoc J 128: 545–549PubMedGoogle Scholar
  48. 48.
    Akaoka I, Nishizawa T, Yano E, Kamatani N, Nishida T, Sasaki S (1977) Renal urate excretion in five cases of hypouricemia with an isolated renal defect of urate transport. J Rheumat 4: 86–94Google Scholar
  49. 49.
    Kawabe K, Muryama T, Akaoka I (1976) A case of uric acid renal stone with hypouricemia caused by tubular reabsorption defect of uric acid. J Urol 116: 690–692PubMedGoogle Scholar
  50. 50.
    Takeda E, Kuroda T, Ito M, Toshima K, Watanabe T, Ito M, Naiko E, Yokota I, Huwang TJ, Miyao M (1985) Hereditary renal hypouricemia in children. J Pediat 107: 71–74PubMedCrossRefGoogle Scholar
  51. 51.
    Sperling 0, de Vries A (1964) Studies on the etiology of uric acid lithiasis. Part II: Solubility of uric acid in urine specimens from normal subjects and patients with idiopathic uric lithiasis. J Urol 92: 331–334PubMedGoogle Scholar
  52. 52.
    Sperling O, Kedem O, de Vries A (1966) Etiologie de la lithiase urique. I. Solubilite de l’ acid urique et de l’urate de sodium en solution tampon. Rev Franc d’Etudes Clin et Biol 11: 40–48Google Scholar
  53. 53.
    Coe FL, Strauss AL, Tembe V, Le Dun S (1980) Uric acid saturation in calcium nephrolithiasis. Kidney Int 17: 662–668PubMedCrossRefGoogle Scholar
  54. 54.
    Teotia M, Sutor J (1971) Crystallization of ammonium acid urate and other uric acid derivatives from urine. Brit J Urol 43: 381–386PubMedCrossRefGoogle Scholar
  55. 55.
    Teotia M, Teotia SPS (1977) Kidney and bladder stones in India. Postgrad Med J 53: (supp 2) 41–48PubMedGoogle Scholar
  56. 56.
    Sperling 0, de Vries A, Kedem O (1965) Studies on the etiology of uric acid lithiasis. IV. Urinary non-dialyzable substances in idiopathic uric acid lithiasis. J Urol 94: 286–292PubMedGoogle Scholar
  57. 57.
    Sperling 0 (1990) Uric acid nephrolithiasis. In: Wickham JEA and Buck AC (eds) Renal tract stone: Metabolic basis and clinical practice, Churchill Livingstone, LondonGoogle Scholar
  58. 58.
    Erley Ch MM, Hirschberg RR, Hoefer W, Schaefer K (1989) Acute renal failure due to uric acid nephropathy in a patient with renal hypouricemia. Klin Wochenschr 67: 308–312PubMedCrossRefGoogle Scholar
  59. 59.
    Ishikawa I, Sakurai Y, Masuzaki S, Sugishita N, Shinoda A, Shikura N (1990) Exersice-induced acute renal failure in 3 patients with renal hypouricemia. Japanese J Nephrology 32: 923–928Google Scholar
  60. 60.
    Ishikawa I, Onoudi Z, Yuri T et al. (1982) Acute renal failure with severe loin pain and patchy renal vasoconstrictions. In: Eliahou HE (ed) Acute Renal Failure. Libbey, London, p 224Google Scholar

Copyright information

© Springer Verlag, Berlin Heidelberg 1991

Authors and Affiliations

  • O. Sperling

There are no affiliations available

Personalised recommendations