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

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

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.
LanguageEnglish
Pages31-35
Number of pages5
JournalEuropean Journal of Pediatrics
Volume155
Issue number1
DOIs
Publication statusPublished - Jan 1996
Externally publishedYes

Fingerprint

propionylcarnitine
Metronidazole
Propionates
Propionic Acidemia
Carnitine
Clinical Trials

Cite this

@article{0a9b191f8e0b433a809e03928c65b7a1,
title = "Propionylcarnitine excretion is not affected by metronidazole administration to patients with disorders of propionate metabolism",
abstract = "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.",
keywords = "H NMR spectroscopy, disorders of propionate metabolism, L-carnitine, metronidazole, propionylcarnitine",
author = "Burns, {S. P.} and Iles, {R. A.} and Saudubray, {J. M.} and Chalmers, {R. A.}",
year = "1996",
month = "1",
doi = "10.1007/BF02115623",
language = "English",
volume = "155",
pages = "31--35",
journal = "European Journal of Pediatrics",
issn = "0340-6199",
publisher = "Springer Verlag",
number = "1",

}

Propionylcarnitine excretion is not affected by metronidazole administration to patients with disorders of propionate metabolism. / Burns, S. P.; Iles, R. A.; Saudubray, J. M.; Chalmers, R. A.

In: European Journal of Pediatrics, Vol. 155, No. 1, 01.1996, p. 31-35.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Propionylcarnitine excretion is not affected by metronidazole administration to patients with disorders of propionate metabolism

AU - Burns, S. P.

AU - Iles, R. A.

AU - Saudubray, J. M.

AU - Chalmers, R. A.

PY - 1996/1

Y1 - 1996/1

N2 - 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.

AB - 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.

KW - H NMR spectroscopy

KW - disorders of propionate metabolism

KW - L-carnitine

KW - metronidazole

KW - propionylcarnitine

UR - http://www.scopus.com/inward/record.url?scp=0030065271&partnerID=8YFLogxK

U2 - 10.1007/BF02115623

DO - 10.1007/BF02115623

M3 - Article

VL - 155

SP - 31

EP - 35

JO - European Journal of Pediatrics

T2 - European Journal of Pediatrics

JF - European Journal of Pediatrics

SN - 0340-6199

IS - 1

ER -