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  • Title: Propionylcarnitine excretion is not affected by metronidazole administration to patients with disorders of propionate metabolism.
    Author: Burns SP, Iles RA, Saudubray JM, Chalmers RA.
    Journal: Eur J Pediatr; 1996 Jan; 155(1):31-5. PubMed ID: 8750807.
    Abstract:
    UNLABELLED: 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.
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