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 Scopus citations

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

ASJC Scopus subject areas

  • Medicine(all)

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    Wolff, J. A., Thuy, L. P., Haas, R., Carroll, J. E., Prodanos, C., & Nyhan, W. L. (1986). CARNITINE REDUCES FASTING KETOGENESIS IN PATIENTS WITH DISORDERS OF PROPIONATE METABOLISM. The Lancet, 327(8476), 289-291. https://doi.org/10.1016/S0140-6736(86)90826-3