Relative carnitine insufficiency in children with type I diabetes mellitus.

Recognizing the similarity of type I diabetes mellitus to inborn errors of metabolism that have responded to carnitine therapy, we initiated a study of 54 children with type I diabetes mellitus. Examining a fasting blood sample for levels of carnitine, glucose, and glycosylated hemoglobin A1c, and a urine sample for levels of ketones and glucose, we found 13 children were deficient of free carnitine (less than 20 mumol/L) and 30 had elevated acyl carnitine levels (greater than 11 mumol/L). Statistical tests confirmed a significant difference between the diabetic population and normal population for reduced free carnitine, elevated acyl carnitine, and an elevated ratio of acyl carnitine to free carnitine. Also, a significant correlation was found between the levels of urine glucose and ketones and the level of acyl carnitine. Our data indicate that carnitine deficiency and relative insufficiency may be an overlooked component in the management of diabetes.