Gout and hyperuricemia associated with sickle-cell anemia.
Since the initial description, in 1958, of gouty arthritis occurring in association with SCA, more than 12 cases have been reported. The high proportion of women and the relatively young ages are noteworthy. Since 1968, studies of patients with SCA have shown a high prevalence of hyperuricemia, beginning during childhood. The initial event in the development of hyperuricemia presumably is increased synthesis of nucleic acids occurring as part of the erythropoietic response to hemolysis. Catabolism of the nucleic acids generates urate. Increased production of UA normally is compensated for by increased urinary excretion of UA. This response occurs in patients with SCA, but during the third decade of life hyperuricosuria can be reduced, probably by damage to the renal tubules caused by infarction and hypoxia resulting from sickling. Impairment of the compensatory renal response leads to more severe and sustained hyperuricemia, and gouty arthritis may then develop. A number of questions about hyperuricemia and gout in SCA remain unanswered. The prevalence of gout among patients with SCA, both in general and in relation to age and sex, has not been determined. The relationships between specific aspects of SCA and of hyperuricemia and gout need to be determined. These include any effect of sickle cell crises on SUA and attacks of gout, and correlation of abnormalities in renal handling of urate with other indices of tubular function and with the pathologic anatomy of the kidney. Finally, it is important to learn whether hyperuricemia and hyperuricosuria contribute to the renal manifestations of SCA; if so, allopurinol might be useful in the prevention and treatment of the renal disease.
D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications*; D001168:Arthritis / Q000150:complications*; D005260:Female; D006073:Gout / Q000150:complications*; D006461:Hemolysis; D006801:Humans; D008297:Male; D008875:Middle Aged; D009696:Nucleic Acids / Q000378:metabolism; D014527:Uric Acid / Q000097:blood* / Q000378:metabolism
D009696:Nucleic Acids; D014527:Uric Acid