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Please use this identifier to cite or link to this item: http://hdl.handle.net/123456789/18420

Title: Tandem mass spectrometric assay of succinylacetone in urine for the diagnosis of hepatorenal tyrosinemia.
Authors: Rashed S, M.
Al-Ahaidib Y, L.
Al-Dirbashi Y, O.
Al Amoudi, M.
Al-Sayed M, M.
Rahbeeni, Z.
Al-Hassnan, Z.
Al-Dbaas, A.
Al-Owain, M.
Ni Luanaigh, M.
Keywords: Tyrosinemia; Succinylacetone; LC–MS/MS
تاريخ النشر: 2005
Publisher: Elsevier
Citation: Analytical Biochemistry. 339(2):310-7.
Abstract: We describe an isotope dilution liquid chromatography-tandem mass spectrometry (LC-MS/MS) method for the determination of succinylacetone (SA) in urine for the diagnosis of hepatorenal tyrosinemia (HT1). The method used 15N-labeled 5(3)-methyl-3(5)-isoxazole propionic acid as internal standard. Urine samples were oximated with hydroxylamine hydrochloride at 80 degrees C, extracted by solvent-solvent extraction, and followed by derivatization of the butyl ester. The butylated isoxazole derivatives of SA and its internal standard were detected and quantified using positive ion electrospray LC-MS/MS with selected reaction monitoring. The turnaround time between injections was 10 min. Calibration curves were linear over the range of 0.0633-63.3 micromol/L. The intra- and interday assay variations were less than 7%. Mean recoveries of SA at three different concentrations ranged from 96 to 109%. During the course of this study, we identified 12 new patients with HT1 and applied this method to follow up the treatment of 4 of these patients as well as previously diagnosed HT1 patients.
URI: http://hdl.handle.net/123456789/18420
ISSN: 0003-2697 DOI: 10.1016/j.ab.2005.01.014
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