Propionylcarnitine excretion is not affected by metronidazole administration to patients with disorders of propionate metabolism

S. P. Burns, R. A. Iles, J. M. Saudubray, R. A. Chalmers

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7 Citations (Scopus)


Propionylcarnitine (PC) excretion has been measured during a clinical trial of metronidazole therapy in two patients with propionic acidaemia and two patients with methylmalonic aciduria. All patients were in good metabolic control and were receiving L-carnitine. While total propionate excretion was reduced by up to 40% in all four patients during metronidazole therapy, the excretion of propionylcarnitine remained largely unchanged. PC comprised up to 80% of total propionate excretion in patients with propionic acidaemia. Conclusion. These results suggest an extra-hepatic source and/or differing compartmentation for PC formation from those for the production of other metabolites of propionyl-CoA.
Original languageEnglish
Pages (from-to)31-35
Number of pages5
JournalEuropean Journal of Pediatrics
Issue number1
Publication statusPublished - Jan 1996
Externally publishedYes


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