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 mu L urine, using d(5)-SA as internal standard, and in 3 mm dried bloodspots, using C-13(4)-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 mu mol/mmol creatinine. Measured SA newborn bloodspot normal range (n = 152) is 0-0.30 mu mol/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 mu mol/L. Copyright (c) 2006 John Wiley & Sons, Ltd.