Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency with inadvertent caries in infants.
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Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency with inadvertent caries in infants. / Blake, Felix; Blessmann, Marco; Werle, Heiner; Li, Lei; Gbara, Ali.
in: INT J PAEDIATR DENT, Jahrgang 17, Nr. 1, 1, 2007, S. 72-74.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency with inadvertent caries in infants.
AU - Blake, Felix
AU - Blessmann, Marco
AU - Werle, Heiner
AU - Li, Lei
AU - Gbara, Ali
PY - 2007
Y1 - 2007
N2 - BACKGROUND: Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD) is a rare systemic disease that is associated with early tooth decay. CASE REPORT: 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. CONCLUSION: 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.
AB - BACKGROUND: Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD) is a rare systemic disease that is associated with early tooth decay. CASE REPORT: 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. CONCLUSION: 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.
M3 - SCORING: Zeitschriftenaufsatz
VL - 17
SP - 72
EP - 74
JO - INT J PAEDIATR DENT
JF - INT J PAEDIATR DENT
SN - 0960-7439
IS - 1
M1 - 1
ER -