CARNITINE REDUCES FASTING KETOGENESIS IN PATIENTS WITH DISORDERS OF PROPIONATE METABOLISM

Jon A. Wolff, Le Phuc Thuy, Richard Haas, James Edwin Carroll, Christina Prodanos, William L. Nyhan

Research output: Contribution to journalArticle

23 Citations (Scopus)

Abstract

Patients with disorders of propionate metabolism have low plasma levels of free carnitine and excrete higher than normal quantities of esterified carnitine. The response to a 19 h fast was assessed as a physiological index of carnitine deficiency. In patients with propionic acidaemia and methylmalonic acidaemia a substantial ketogenesis developed in response to fasting. Supplementation with L-carnitine significantly reduced this ketogenic response.

Original languageEnglish (US)
Pages (from-to)289-291
Number of pages3
JournalThe Lancet
Volume327
Issue number8476
DOIs
StatePublished - Feb 8 1986

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Carnitine
Propionates
Fasting
Propionic Acidemia

ASJC Scopus subject areas

  • Medicine(all)

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CARNITINE REDUCES FASTING KETOGENESIS IN PATIENTS WITH DISORDERS OF PROPIONATE METABOLISM. / Wolff, Jon A.; Thuy, Le Phuc; Haas, Richard; Carroll, James Edwin; Prodanos, Christina; Nyhan, William L.

In: The Lancet, Vol. 327, No. 8476, 08.02.1986, p. 289-291.

Research output: Contribution to journalArticle

Wolff, Jon A. ; Thuy, Le Phuc ; Haas, Richard ; Carroll, James Edwin ; Prodanos, Christina ; Nyhan, William L. / CARNITINE REDUCES FASTING KETOGENESIS IN PATIENTS WITH DISORDERS OF PROPIONATE METABOLISM. In: The Lancet. 1986 ; Vol. 327, No. 8476. pp. 289-291.
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