Abstract
The excretion of ketones other than acetone has been described in a few reports of patients with the ketotic hyperglycinemia syndrome. Our unexpected finding of butanone, 2-and 3-pentanone in the urine of Gompertz' patient with methylcrotonylglycinuria led us to modify our gaschromatographic(GC)method to allow quantitative determination. The neutral dinitrophenylhydrazones (up to C6) are separated by isothermal GC under the following conditions: column 180 cm, 2 mm ID with Dexsil 300 3% on 100/120 Supelcoport; N2 46ml/min, 48 psi; oven215°C, injector 250°C,detector 300°C. The limit of detection is appr.100 picomoles, the FID response linear up to at least 10 nmoles injected. We have found that normal children not only excrete acetaldehyde and acetone, but also small amounts of butanone and 2-pentanone. These latter compounds and 3-pentanone are markedly increased in methylmalonic acidemia. Aldehydes and ketones in biological fluids and breath have been neglected up to now. However they might be biologically active and their increase could indicate blocks in the intermediary metabolism.
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Bachmann, C., Baumgartner, R. & Wick, H. Quantitative determination of aldehydes and ketones in metabolic disorders. Pediatr Res 8, 907 (1974). https://doi.org/10.1203/00006450-197411000-00062
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DOI: https://doi.org/10.1203/00006450-197411000-00062