PEDIATRICS Vol. 68 No. 4 October 1981, pp. 553-558
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Clinical and Metabolic Abnormalities in a Boy with Dietary Deficiency of Biotin

Lawrence Sweetman PhD1, Linda Surh 1, Herman Baker PhD1, Raymond M. Peterson MD1, and William L. Nyhan MD, PhD1

1 Department of Pediatrics, University of California, San Diego, La Jolla; The Regional Center for the Mentally Retarded, San Diego, California; and College of Medicine and Dentistry of New Jersey, East Orange

Dietary deficiency of biotin was documented in an 11-year-old retarded boy as a consequence of a dietary prescription containing raw eggs. Clinical manifestations were alopecia totalis and an erythematous, exfoliative dermatosis. Metabolic characteristics included increased excretion of 3-methylcrotonylglycine, 3-hydroxyisovaleric acid, 3-hydroxypropionic acid, methylcitric acid, and lactic acid, as well as a propensity for the development of ketosis. The activities of propionyl coenzyme A carboxylase and 3-methyicrotonyl coenzyme A carboxylase in extracts of leukocytes were deficient. Treatment with biotin and the removal of raw eggs, which contain the biotin-binding protein, avidin, from the diet led to the reversal of all of the clinical and metabolic manifestations observed.

Submitted on August 12, 1980
Accepted on January 14, 1981




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