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 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.
Johnson
David W
DW
Department of Genetic Medicine, Women's and Children's Hospital, 72 King William Rd., North Adelaide, South Australia, 5006 Australia. david.johnson@adelaide.edu.au
Gerace
Rosemarie
R
Ranieri
Enzo
E
Trinh
Minh-Uyen
MU
Fingerhut
Ralph
R
D001622:Betaine / Q000031:analogs & derivatives* / Q000737:chemistry; D002648:Child; D004912:Erythrocytes / Q000737:chemistry; D017022:Flow Injection Analysis; D006537:Heptanoates / Q000097:blood* / Q000652:urine*; D006801:Humans; D007202:Indicators and Reagents; D007223:Infant; D007231:Infant, Newborn; D007664:Ketosteroids / Q000032:analysis; D021241:Spectrometry, Mass, Electrospray Ionization; D020176:Tyrosinemias / Q000097:blood / Q000175:diagnosis / Q000652:urine
D006537:Heptanoates; D007202:Indicators and Reagents; D007664:Ketosteroids; D001622:Betaine; C020804:succinylacetone; C012332:Girard's reagent T