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© 1985 American Academy of Neurology Carnitine deficiency, organic acidemias, and Reye's syndromeDepartments of Neurology and Pediatrics, University of Colorado School of Medicine, Denver, CO. Relative carnitine deficiency is important in the pathophysiology of several disorders, including Reye's syndrome and organic acidemias. In acute clinical crises, carnitine serves as a "buffer," trapping toxic acyl compounds. Mitochondria1 failure develops in carnitine deficiency when there is insufficient tissue carnitine available to buffer toxic acyl-CoA metabolites. Toxic levels of acyl-Co A impair the citrate cycle, gluconeogenesis, the urea cycle, and fatty-acid oxidation. Carnitine replacement therapy is safe and induces excretion of toxic acyl groups in the urine. Address correspondence and reprint requests to Dr. Stumpf, Director of Pediatric Neurology, Northwestern University, Children's Memorial Hospital, 2300 Children's Plaza, Chicago, IL 60614. Supported by a Clinical Research Grant From the Muscular Dystrophy Association: a grant from the National Foundation-March of Dimes; an NIH Program Project Grant HD08315, Center Grant HD04024, and National Research Service Award Grant HD07096-01, all from NICHD; and Pediatric and Adult CRC Grant RR00069 from the General Clinical Research Centers Program of the Division of Research Resources. Accepted for publication October 17, 1984.
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