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Clinical and metabolic abnormalities in a boy with dietary deficiency of biotin.

Abstract
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-methylcrotonyl 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.
AuthorsL Sweetman, L Surh, H Baker, R M Peterson, W L Nyhan
JournalPediatrics (Pediatrics) Vol. 68 Issue 4 Pg. 553-8 (Oct 1981) ISSN: 0031-4005 [Print] United States
PMID7322688 (Publication Type: Case Reports, Journal Article, Research Support, U.S. Gov't, Non-P.H.S., Research Support, U.S. Gov't, P.H.S.)
Chemical References
  • Citrates
  • Egg Proteins
  • Hydroxy Acids
  • Propionates
  • beta-methylcrotonylglycine
  • 2-methylcitric acid
  • Biotin
  • Carboxy-Lyases
  • Ligases
  • Carbon-Carbon Ligases
  • methylcrotonoyl-CoA carboxylase
  • Methylmalonyl-CoA Decarboxylase
  • Glycine
Topics
  • Alopecia (etiology)
  • Biotin (deficiency)
  • Carbon-Carbon Ligases
  • Carboxy-Lyases (deficiency)
  • Child
  • Citrates (urine)
  • Diet (adverse effects)
  • Egg Proteins (adverse effects)
  • Facial Dermatoses (etiology)
  • Glycine (analogs & derivatives, urine)
  • Humans
  • Hydroxy Acids (urine)
  • Ligases (deficiency)
  • Male
  • Methylmalonyl-CoA Decarboxylase
  • Propionates (deficiency)
  • Skin Diseases (etiology)

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