Introduction:
Biotin deficiency:
Biotin deficiency may occur with excess consumption of raw egg whites or egg white protein due to content of avidin which binds biotin rendering it useless. It also occurs in multiple carboxylase deficiency in which a defect results in holocarboxylase synthetase or biotinidase deficiency resulting in improper metabolism of biotin (4).
Screening of the newborn for multiple carboxylase deficiency:
It is important to ensure as a parent that a newborn is screened for multiple carboxylase deficiency because neurologic symptoms may be irreversible. Biotin is readily available as a supplement in pill form which corrects the deficiency. Pregnant patients with multiple carboxylase deficiency and their infants have a higher risk of sequelae from biotin deficiency.
Common Biotin Containing Foods (1,2)
(no daily intake guidance has been established)
Swiss chard
Egg yolk
Milk
Liver
Leafy green vegetables
Peanuts
Brewer’s yeast
Sardines
Legumes
Soybeans
Bananas
Whole grains
Biotin Supplements
Also produced by intestinal flora
Assessment and Plan: Biotin
- Ensure your newborn is screened for multiple carboxylase deficiency at birth. Ensure adequate intake of biotin during pregnancy. Patients with multiple carboxylase deficiency require biotin supplementation.
- Do not consume raw egg whites or egg white protein in excess.
References:
1.Roth, K. S. Biotin in clinical medicine–a review. Am.J Clin.Nutr. 1981;34(9):1967-1974.
2.Biotin. The Free Medical Dictionary. http://medical-dictionary.thefreedictionary.com/biotin. Retrieved 2013-1-27.
3.Cole H, Reynolds TR, Lockyer JM, Buck GA, Denson T, Spence JE, Hymes J, Wolf B Human serum biotinidase. cDNA cloning, sequence, and characterization. J Biol Chem. 1994;269(9):6566. http://www.ncbi.nlm.nih.gov/pubmed/7509806
4.Baykal T, Gokcay G, Gokdemir Y, Demir F, Seckin Y, Demirkol M, Jensen K, Wolf B.Asymptomatic adults and older siblings with biotinidase deficiency ascertained by family studies of index cases. J Inherit Metab Dis. 2005;28(6):903-12. http://www.ncbi.nlm.nih.gov/pubmed/16435182