[Renal hypouricemia]

Nihon Rinsho. 1996 Dec;54(12):3337-42.
[Article in Japanese]


Renal hypouricemia (serum urate < 2.0 mg/dl) is the inborn disorder due to the impaired tubular urate transport resulted in an increased urate excretion. The inheritance shows an autosomal recessive trait. The prevalence of renal hypouricemia is 0.15% in the outpatients. Based on the 4-component model, by using the pharmacological inhibitor, renal hypouricemia is classified into the 4 types as follows, 1) defective presecretory reabsorption, 2) defective postsecretory reabsorption, 3) enhanced urate secretion, and 4) both defective presecretory and postsecretory reabsorption including the three subtypes. Although renal hypouricemia has asymptomatic as usual, acute renal failure and urolithiasis (hematuria) have been reported as the complication. We further introduced the novel pathophysiology of the renal hypouricemia such as the aciduria correlating to the accelerated urate excretion and the hyperoxipurinemia in this review.

Publication types

  • English Abstract
  • Review

MeSH terms

  • Acute Kidney Injury / etiology
  • Biological Transport
  • Kidney Tubules / metabolism
  • Purine-Pyrimidine Metabolism, Inborn Errors* / classification
  • Purine-Pyrimidine Metabolism, Inborn Errors* / genetics
  • Uric Acid / metabolism
  • Uric Acid / urine*
  • Urinary Calculi / etiology


  • Uric Acid