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Organic acid analysis is used for the early detection/ exclusion and for the follow-up of inherited disorders of amino acid and organic acid metabolism. Urinary organic acid concentrations in 417 healthy Caucasian children (1 day to 17 years of age) were determined after liquid solid extraction, as their trimethylsilyl derivatives, by gas chromatography and mass spectrometry. Concentrations of most of the organic acids adjusted for creatinine tend to decrease with age. No differences were found between gender except for the Krebs cycle intermediates in the older age groups. In neonates, the immaturity of the neonatal kidney led to a much larger variation of organic acid levels when related to creatinine. The low number of subjects (n = 36-52) per age class resulted in large 95% confidence intervals of the percentiles used for decision. This must be taken into account when using the data for exclusion or diagnosis of disorders.

Aim-To evaluate whether the urinary creatinine concentration is a reliable reference value to standardise urinary solute excretion in a spot urine sample during the first week of life. Methods-Spontaneously voided urine specimens were obtained in 48 healthy, full term neonates, aged 1 to 6 days (median 2.4) and in 168 healthy older children with a median age of 1.5 years (range 1 month to 3 years). In 62% of the children two urine samples were available with an interval of 2 to 4 (neonates) and 7 days (older children). Results-In neonates both the urinary creatinine concentration and the urinary creatinine:osmolality ratios were significantly higher than in the older children, and were spread over a wider range. During the first postnatal week of life the mean urinary creatinine and urinary creatinine: osmolality ratio values in the first urine samples were also significantly higher than in the second samples. In children aged between 1 month and 3 years of age, these data were remarkably stable without any significant changes between repeat urine samples. Conclusions-The urinary creatinine concentration during the first days of life is high and variable, even when corrected for urinary osmolality. This is the opposite of what is found in older children and adults. Urinary creatinine and the urinary creatinine:osmolality ratio, therefore, cannot be used to standardise the urinary excretion of solutes in the first week of life. (Arch Dis Child Fetal Neonatal Ed 1999;80:F240-F242) Keywords: spot urine sample; urinary creatinine; osmolality ratio; urinary solute excretion Without invasive techniques such as bladder catheterisation, accurate urinary collections are notoriously diYcult to obtain in young children, especially in neonates. It is therefore understandable that methods which eliminate the need for the gold standard of timed urine collections are constantly being sought. Several studies have used the urinary solute:creatinine ratio in a single urine specimen to quantify the urinary excretion of various substances in neonates, infants, and children.