Determination of succinylacetone in dried blood spots and liquid urine as a dansylhydrazone by liquid chromatography tandem mass spectrometry

• Published in: Journal of chromatography. B, Analytical technologies in the biomedical and life sciences Vol. 831; no. 1; pp. 274 - 280
• Main Authors: Al-Dirbashi, Osama Y., Rashed, Mohamed S., Brink, Herman J. Ten, Jakobs, Cornelis, Filimban, Najlaa, Al-Ahaidib, Lujane Y., Jacob, Minnie, Al-Sayed, Moeen M., Al-Hassnan, Zuhair, Faqeih, Eissa
• Format: Journal Article
• Language: English
• Published: Amsterdam Elsevier B.V 02.02.2006; Elsevier Science
• Subjects: Analysis; Analytical, structural and metabolic biochemistry; Biological and medical sciences; Chromatography, High Pressure Liquid - methods; Dansyl Compounds - chemistry; Dansylhydrazine; Fundamental and applied biological sciences. Psychology; General pharmacology; Hepatorenal tyrosinemia; Heptanoates - blood; Heptanoates - chemistry; Heptanoates - urine; Humans; Hydrazines - chemistry; Infant, Newborn; Mass Spectrometry - methods; Medical sciences; Neonatal Screening - methods; Newborn screening; Pharmacology. Drug treatments; Specimen Handling; Succinylacetone; Tandem mass spectrometry; Tyrosine; Tyrosinemias - diagnosis; Tyrosine; Newborn screening; Dansylhydrazine; Tandem mass spectrometry; Hepatorenal tyrosinemia; Succinylacetone; Human; Urine; Biological fluid; Tyrosinemia; Metabolic diseases; Liquid chromatography; Dansylhydrazine: Tandem mass spectrometry; Enzymopathy; Blood; Genetic disease; Screening; Newborn; Aminoacid; Mass spectrometry MS/MS; Aminoacid disorder; Quantitative analysis; Hepatorenal tyrosinemia: Tyrosine: Succinylacetone
• ISSN: 1570-0232; 1873-376X
• DOI: 10.1016/j.jchromb.2005.12.010

Succinylacetone (SA) is a specific marker for the inherited metabolic disease, hepatorenal tyrosinemia. We developed a stable-isotope dilution liquid chromatography tandem mass spectrometry for the determination of SA in dried blood spots (DBS) and liquid urine using a 13C 4-SA as internal standard. SA was extracted, converted to the butyl ester and derivatized with dansylhydrazine (Dns-H). Calibration curves in DBS and urine calibrators were linear up to 100 and 30 μM, respectively. At a signal-to-noise ratio of 3, the limits of detection in DBS and urine were 0.2 and 0.005 μM, respectively. Total run time was 5 min. Intra- and inter-assay precision expressed as coefficient of variation were better than 9.1% with more than 96% recovery. The method was applied retrospectively and prospectively for the diagnosis of hepatorenal tyrosinemia and for follow-up of patients under treatment.