Biotinidase Deficiency Medication

Updated: Jul 27, 2018
  • Author: Germaine L Defendi, MD, MS, FAAP; Chief Editor: Maria Descartes, MD  more...
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Medication Summary

Biotin is the drug of choice (DOC) for biotinidase deficiency.


Vitamins and cofactors

Class Summary

Organic substances are required by the body in small amounts for various metabolic processes. They are used clinically for the prevention and treatment of specific deficiency states. Biotin is the DOC for biotinidase deficiency.


An essential coenzyme in fat metabolism and in other carboxylation reactions. Biotin deficiency may result in the urinary excretion of organic acids and changes in skin and hair. Functions as a coenzyme or a prosthetic group in all 4 of the body's carboxylases. Each of these carboxylases maintain critical roles in intermediary metabolism. In these enzymes, biotin serves as a carrier for CO2.