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

David W Johnson, Rosemarie Gerace, Enzo Ranieri, Minh-Uyen Trinh, Ralph Fingerhut

Index: Rapid Commun. Mass Spectrom. 21(1) , 59-63, (2007)

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Abstract

Flow injection electrospray ionization tandem mass spectrometric methods for succinylacetone (SA) in 250 microL 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 micromol/mmol creatinine. Measured SA newborn bloodspot normal range (n=152) is 0-0.30 micromol/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 micromol/L.Copyright (c) 2006 John Wiley & Sons, Ltd.