Predicted reciprocal serum creatinine at age 10 years as a measure of renal function in children with nephropathic cystinosis treated with oral cysteamine.
The predicted reciprocal creatinine at age 10 years (PRC10), a parameter of renal function based upon the linear relationship between reciprocal serum creatinine and age, incorporates age, serum creatinine, and rate of renal deterioration into a single term. PRC10 measurements were employed to assess renal function in children with nephropathic cystinosis treated with oral cysteamine, a cystine-depleting agent. In 71 children receiving oral cysteamine for at least 1 year, PRC10 decreased linearly with initial serum creatinine concentration. This indicated that, although established renal damage in cystinosis was irreversible, early intervention with cysteamine therapy could favorably alter the rate of glomerular deterioration. In other analyses, mean PRC10 was shown to increase with duration of cysteamine therapy and extent of leukocyte cystine depletion. The predicted reciprocal creatinine value at a certain age can be useful in analyzing the effects of therapeutic intervention in a disease with a relatively uniform rate of renal deterioration.
Gahl
W A
WA
Human Genetics Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892.
Schneider
J A
JA
Schulman
J D
JD
Thoene
J G
JG
Reed
G F
GF
D000284:Administration, Oral; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D003404:Creatinine / Q000097:blood*; D003543:Cysteamine / Q000008:administration & dosage / Q000627:therapeutic use; D003554:Cystinosis / Q000097:blood* / Q000188:drug therapy; D006801:Humans; D007677:Kidney Function Tests / Q000379:methods*
D003543:Cysteamine; D003404:Creatinine