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Carnitine deficiency.

Carnitine is a naturally occurring amino acid derivative produced by the body and also found in foods such as red meat, dairy products and breast milk. Carnitine helps convert consumed food into energy by facilitating long-chain fatty acid entry into the mitochondria. This substance helps infants gain weight, builds muscle and organ tissues, and cleanses the body by removing cellular waste products. It is absolutely essential in keeping the body's metabolism running smoothly.

There are instances, however, in which carnitine is not present in sufficient amounts or is completely lacking, posing an obvious health concern to the individual. Often, when such a deficiency is suspected it is found to affect infants and children. The most common diagnoses related to carnitine deficiency include primary systemic carnitine deficiency and secondary carnitine deficiency. There have also been several noteworthy clinical studies documenting levocarnitine deficiency secondary to treatment with some anticonvulsant medications.

It is estimated that as many as 375,000 infants and children could suffer from carnitine deficiency but only 10,000 are prescribed treatment each year. This may be due to the fact that metabolic disorders are considered by pediatricians to be among the most difficult childhood diseases to diagnose and recognize. Carnitine deficiency is a secondary disorder to many metabolic disorders that can be life-threatening. There are approximately 100 to 150 metabolic disorders that can cause carnitine deficiency.

Levocarnitine (Carnitor[R]) is indicated for the treatment of primary systemic carnitine deficiency, as well as acute and chronic treatment of patients with an inborn error in metabolism that results in a secondary carnitine deficiency. Carnitor[R] is the only version of levocarnitine approved by the Food and Drug Administration for the treatment of carnitine deficiency. Other dietary and health supplement L-carnitine products have not been approved for safety, efficacy, or big-availability. It should be noted that D. L-carnitine, sold in health food stores as vitamin B, competitively inhibits L-carnitine and can cause a deficiency.

The dosing of Carnitor[R] is dependent upon clinical response. Carnitor[R] is available as an injection (1g/5ml), solution (100mg/ml) and in tablet form (330mg).

Possible adverse effects of supplemental levocarnitine may include "fishy" body odor, transient nausea and diarrhea. Monitoring should include periodic blood chemistries, vital signs, plasma carnitine concentrations, and overall clinical condition.

Levocarnitine is a valuable and potentially life-saving medication. There is little question that there is a condition known as primary systemic carnitine deficiency for which carnitine supplementation is essential. There are also a number of metabolic disorders or inborn errors of metabolism regarded as a `secondary type of deficiency' where supplementation of carnitine has proven highly beneficial.

For more information on carnitine deficiency and other metabolic disorders, contact the National Organization for Rare Disorders (NORD), PO. Box 8923, New Fairfield, CT 06812-1783; or the Association for Babies with Carnitine Deficiency (ABCD), 720 Enterprise Drive, Oak Brook, IL 60521, or 800-554-ABCD. For more information about the prescription drug Carnitor[R], contact sigma-tau Pharmaceuticals, Inc., at 1-800-447-0169 (Medical Affairs Dept.)
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Title Annotation:Mitochondrial/Metabolic Disorders, part 1; Mitochondrial Diseases
Author:Coley, Crystal; Legino, R.L.
Publication:The Exceptional Parent
Date:Jun 1, 1997
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