Elevated accumulation and excretion of glycine accompanied by ketosis that is secondary to methylmalonicacidemia, isovalericacidemia and other organic acidemias, as distinguished from nonketotic hyperglycinemia.

Propionic acidemia, also known as propionic aciduria or propionyl-CoA carboxylase deficiency (PCC deficiency), is a rare autosomal recessive metabolic disorder, classified as a branched-chain organic acidemia. The disorder presents in the early neonatal period with poor feeding, vomiting, lethargy, and lack of muscle tone. Without treatment, death can occur quickly, due to secondary hyperammonemia, infection, cardiomyopathy, or brain damage..