Tags: Disease.
Propionic acidemia also known as propionic aciduria propionyl-CoA carboxylase deficiency and ketotic glycinemia is an autosomal recessive metabolic disorder classified as a branched-chain organic acidemia.The disorder presents in the early neonatal period with progressive encephalopathy. Death can occur quickly due to secondary hyperammonemia infection cardiomyopathy or basal ganglial stroke.Propionic acidemia is a rare disorder that is inherited from both parents.