L-carnitine treatment in glutaric aciduria type I.
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 microM (normal, 54.4 +/- 14.2 [2 SD] microM). 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.
D002331:Carnitine / Q000097:blood / Q000172:deficiency / Q000627:therapeutic use*; D002675:Child, Preschool; D005260:Female; D005977:Glutarates / Q000652:urine*; D006801:Humans; D013997:Time Factors
D005977:Glutarates; C035736:glutaric acid; D002331:Carnitine