Long-term use of investigational β-Hydroxybutyrate salts in children with multiple acyl-CoA dehydrogenase or pyruvate dehydrogenase deficiency

Long-term use of investigational β-Hydroxybutyrate salts in children with multiple acyl-CoA dehydrogenase or pyruvate dehydrogenase deficiency

Several disorders of energy metabolism have been treated with exogenous ketone bodies. The benefit of this treatment is best documented in multiple acyl-CoA dehydrogenase deficiency (MADD) (MIM#231680). One might also expect ketone bodies to help in other disorders with impaired ketogenesis or in co...

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Bibliographic Details
Main Authors: Andrew A.M. Morris, Bernard Cuenoud, Philippe Delerive, Helen Mundy, Bernd C. Schwahn
Format: Article
Language:English
Published: Elsevier 2024-09-01
Series:Molecular Genetics and Metabolism Reports
Subjects:
Glutaric aciduria II
MADD
β-Hydroxybutyrate
Ketone bodies
Pyruvate dehydrogenase deficiency
Online Access:http://www.sciencedirect.com/science/article/pii/S2214426924000570

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http://www.sciencedirect.com/science/article/pii/S2214426924000570

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