title: Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency with inadvertent caries in infants.
abstract: BACKGROUND
  Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD) is a rare systemic disease that is associated with early tooth decay.
  
  
  METHODS
  This report describes the case of a 3-year-old boy suffering from LCHADD. At the time of referral, extensive carious lesions of the subject's maxillary dentition necessitated the surgical removal of eight teeth. Preventive treatment for LCHADD involves a regular oral intake of glucose that is vital for the survival of the affected individual. In young infants, the glucose solution needs to be administered as often as every 3 h in order to prevent hypoglycaemia, leading to a local environment similar to that experienced in nursing bottle syndrome. While nursing bottle syndrome can be resolved by eliminating the sugar substrate and curtailing the feeding sessions, these alternatives are not available in cases of LCHADD.
  
  
  CONCLUSIONS
  This report highlights this rare disease and emphasizes its dire consequences for the dentition. Prophylactic recommendations for high-risk children are reviewed. Familiarity with LCHADD allows this high-risk group of patients to be identified, and thus, ensures diligent prophylactic action.
authors.affiliation: Department of Oral and Maxillofacial Surgery, University Hospital Hamburg-Eppendorf, Hamburg, Germany. blake@uke.uni-hamburg.de
authors.forename: Felix
authors.initials: F
authors.lastname: Blake
authors.forename: Marco
authors.initials: M
authors.lastname: Blessmann
authors.forename: Heiner
authors.initials: H
authors.lastname: Werle
authors.forename: Lei
authors.initials: L
authors.lastname: Li
authors.forename: Ali
authors.initials: A
authors.lastname: Gbara
mesh_terms: D015094:3-Hydroxyacyl CoA Dehydrogenases / Q000172:deficiency*; D002675:Child, Preschool; D003731:Dental Caries / Q000209:etiology*; D005500:Follow-Up Studies; D005947:Glucose / Q000009:adverse effects / Q000627:therapeutic use; D006801:Humans; D007003:Hypoglycemia / Q000188:drug therapy / Q000201:enzymology; D008052:Lipid Metabolism, Inborn Errors / Q000188:drug therapy / Q000201:enzymology; D063989:Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase; D008297:Male
chemical_list: D015094:3-Hydroxyacyl CoA Dehydrogenases; D063989:Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase; D005947:Glucose