25 top medical experts on Alpha-ketoglutarate dehydrogenase deficiency across 2 countries. This is based on an objective analysis of their Scientific Publications, Clinical Trials, Medicare, and NIH Grants.
- Alpha-ketoglutarate dehydrogenase deficiency: A rare, genetic, inborn error of metabolism disorder characterized by neonatal-onset of developmental delay, hypotonia, hepatomegaly, lactic acidemia, increased creatine kinase levels, elevated alpha-ketoglutaric acid in urine, and a decreased plasma beta-hydroxybutyrate-to-acetoacetate ratio. Pyruvate dehydrogenase deficiency can be associated, leading to hypoglycemia and neurologic anomalies, including seizures.
- Clinical guidelines are the recommended starting point to understand initial steps and current protocols in any disease or procedure:
- Synonyms: 2 alpha ketoglutarate dehydrogenase deficiency, 2-Ketoglutarate Dehydrogenase Deficiency, Oxoglutaric Aciduria, Oxoglutaricaciduria
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