Document Detail


Clinical and metabolic abnormalities in a boy with dietary deficiency of biotin.
MedLine Citation:
PMID:  7322688     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
L Sweetman; L Surh; H Baker; R M Peterson; W L Nyhan
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, U.S. Gov't, Non-P.H.S.; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Pediatrics     Volume:  68     ISSN:  0031-4005     ISO Abbreviation:  Pediatrics     Publication Date:  1981 Oct 
Date Detail:
Created Date:  1982-03-26     Completed Date:  1982-03-26     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  0376422     Medline TA:  Pediatrics     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  553-8     Citation Subset:  AIM; IM    
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MeSH Terms
Descriptor/Qualifier:
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*
Grant Support
ID/Acronym/Agency:
HD04608/HD/NICHD NIH HHS; RR-00827/RR/NCRR NIH HHS
Chemical
Reg. No./Substance:
0/Citrates; 0/Egg Proteins; 0/Hydroxy Acids; 0/Propionates; 33008-07-0/beta-methylcrotonylglycine; 56-40-6/Glycine; 58-85-5/Biotin; 6061-96-7/2-methylcitric acid; EC 4.1.1.-/Carboxy-Lyases; EC 4.1.1.41/Methylmalonyl-CoA Decarboxylase; EC 6.-/Ligases; EC 6.4.-/Carbon-Carbon Ligases; EC 6.4.1.4/methylcrotonoyl-CoA carboxylase

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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