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L-Carnitine treatment in glutaric aciduria type I

Department of Pathology (Drs. Seccombe and James), University of British Columbia and Shaughnessy Hospital, Lipid Research Group, Vancouver, British Columbia, Canada; and the Department of Pediatrics (Dr. Booth), University of Manitoba and Children's Hospital, Winnipeg, Manitoba, Canada.

Patients with organic aciduria may have a higher than normal requirement for L-carnitine. In a patient with type I glutaric aciduria, serum total L-carnitine levels were 8.5 µM (normal, 54.4 ± 14.2 [2 SD] µM). After treatment with L-carnitine for 1 month, serum levels of both free and total L-carnitine were normal with an acyl-to-total ratio of 0.18. The fractional clearance rates of free and acylcarnitine were increased fourfold by treatment. Acetylcarnitine accounted for a lower than normal percentage of acylcarnitine recovered in serum and urine. Data suggest that this patient may have been carnitine-deficient.

Address correspondence and reprint requests to Dr. Seccombe, Department of Pathology, Shaughnessy Hospital, 4500 Oak Street, Vancouver, BC, Canada V6H 3N1.

Supported by a grant from the British Columbia Health Care Research Foundation. L. James was supported on this project by a B.C. Heart Studentship.

Accepted for publication May 30, 1985.

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