Clinical Trial
doi: 10.1016/s0140-6736(86)90826-3.
Carnitine reduces fasting ketogenesis in patients with disorders of propionate metabolism
- PMID: 2868163
- DOI: 10.1016/s0140-6736(86)90826-3
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Clinical Trial
Carnitine reduces fasting ketogenesis in patients with disorders of propionate metabolism
Lancet.
.
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.
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