Analysis of succinylacetone, as a Girard T derivative, in urine and dried bloodspots by flow injection electrospray ionization tandem mass spectrometry.

Flow injection electrospray ionization tandem mass spectrometric methods for succinylacetone (SA) in 250 µL urine, using d5-SA as internal standard, and in 3 mm dried bloodspots, using 13C4-SA as internal standard, are described. Selectivity and sensitivity of analysis is achieved by the use of a mono-Girard T derivative. Measured SA infant urine normal range (n = 20) is 0.013–0.27 µmol/mmol creatinine. Measured SA newborn bloodspot normal range (n = 152) is 0–0.30 µmol/L. Bloodspots from children with hepatorenal tyrosinemia type 1, and kept at room temperature for up to 7 years, afforded SA concentrations of 0.9–5.7 µmol/L. Copyright © 2006 John Wiley & Sons, Ltd.