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Dicarboxylic acids are those fatty acids with two carboxyl groups on each side examples are malonic, adipic, oxalic, pimeric, suberic acids.

They accumilate when fatty acid oxidation exceeds the capacity of mitochondria.

Clinical situations in addition to MCAD deficiency that have elevated dicarboxylic acids are:

  • Fasting
  • DKA
  • Neonatal Mitochondrial immaturity
  • Side effects of anticonvulsants such specifically Phenytoin
Reference:
Dicarboxylic Aciduria, Significance and prognostic indications
 
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