[Dr. N Elham]

The finding dicarboxilic acidemia is a classic finding of MCAD ( medium chain fatty acy co A dehydrogenase ) deficiency.
What is/are dicarboxilic acid(s) and how is/are it/they produced?

 

[lee-usmle]

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