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Carnitine homeostasis in the organic acidurias.

Publication ,  Journal Article
Roe, CR; Millington, DS; Kahler, SG; Kodo, N; Norwood, DL
Published in: Prog Clin Biol Res
1990

The detection of acylcarnitines by FAB-MS in human physiological fluids has added another valuable diagnostic tool for the recognition of specific metabolic diseases. The newly developed technique of FAB-MS/MS, which embodies the principles of tandem mass spectrometry, affords a quantum leap in specificity, enabling the detection and quantification of acylcarnitines in concentrations well within the physiological ranges in urine, blood and tissue. The therapeutic use of L-carnitine appears to offer protection against the harmful consequences of catabolism. A reduction in the number of clinical episodes requiring hospitalization has been observed in most cases. When such occurrences have necessitated intravenous fluids, the addition of L-carnitine to the IV infusate has led to dramatic reduction in recovery times. It has also been shown that there is no discernable toxicity associated with carnitine therapy, even under high-dose IV conditions.

Duke Scholars

Published In

Prog Clin Biol Res

ISSN

0361-7742

Publication Date

1990

Volume

321

Start / End Page

383 / 402

Location

United States

Related Subject Headings

  • Metabolism, Inborn Errors
  • Mass Spectrometry
  • Humans
  • Homeostasis
  • Carnitine
 

Citation

APA
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ICMJE
MLA
NLM
Roe, C. R., Millington, D. S., Kahler, S. G., Kodo, N., & Norwood, D. L. (1990). Carnitine homeostasis in the organic acidurias. Prog Clin Biol Res, 321, 383–402.
Roe, C. R., D. S. Millington, S. G. Kahler, N. Kodo, and D. L. Norwood. “Carnitine homeostasis in the organic acidurias.Prog Clin Biol Res 321 (1990): 383–402.
Roe CR, Millington DS, Kahler SG, Kodo N, Norwood DL. Carnitine homeostasis in the organic acidurias. Prog Clin Biol Res. 1990;321:383–402.
Roe, C. R., et al. “Carnitine homeostasis in the organic acidurias.Prog Clin Biol Res, vol. 321, 1990, pp. 383–402.
Roe CR, Millington DS, Kahler SG, Kodo N, Norwood DL. Carnitine homeostasis in the organic acidurias. Prog Clin Biol Res. 1990;321:383–402.

Published In

Prog Clin Biol Res

ISSN

0361-7742

Publication Date

1990

Volume

321

Start / End Page

383 / 402

Location

United States

Related Subject Headings

  • Metabolism, Inborn Errors
  • Mass Spectrometry
  • Humans
  • Homeostasis
  • Carnitine