Table 1. Serum Urate Concentration and 24-Hour Urinary Uric Acid Excretion in PRS Superactivity

PhenotypeSerum Urate
(mg/dL or µmol/L) 1
Urinary Uric Acid
(mg/24 hrs) 2
Infantile onsetIncreasedSignificantly elevated
Juvenile/adult onsetIncreasedSignificantly elevated
Normal adult male >12y3.5-7.2 mg/dL or 210-430 µmol/L 3250-750 mg or 1.5-4.5 mmol per 24 hours; 4
200-500 mmol urate/mol creatinine
Normal adult female2.6-6.0 mg/dL or 150-360 µmol/LSame as adult male
Normal child ≤12y2.0-5.5 mg/dL or 120-330 µmol/L300-1400 mmol urate/mol creatinine
Normal infant <2y2.0-5.5 mg/dL or 120-330 µmol/L300-1800 mmol urate/mol creatinine

1. Serum and urine urate ranges are divided into child and adult. Recommended values here are for one specialist referral center; age and urate cutoffs vary locally.

2. The ratio of urinary uric acid to creatinine concentration may be more helpful for screening purposes. PRS superactivity values are typically greater than twofold the upper limit of normal.

3. Male and female adult serum urate ranges differ.

4. “Normal” values vary by age and weight. Values given in table are for a “standard” diet with no medications influencing serum urate levels.

From: Phosphoribosylpyrophosphate Synthetase Superactivity

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Pagon RA, Adam MP, Ardinger HH, et al., editors.
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