Conditions / Genetic

propionic acidemia

info · Genetic · ICD-10: E71.121

An organic acidemia that involes a nonfunctional propionyl CoA carboxylase affecting conversion of amino acids and fats into sugar for energy.

Signs and symptoms

  • Increased level of hippuric acid in urine
  • Global developmental delay
  • Lethargy
  • Acute encephalopathy
  • Dystonia
  • Short stature
  • Seizure
  • Propionyl-CoA carboxylase deficiency
  • Hepatomegaly
  • Osteoporosis

Also known as: GLYCINEMIA, KETOTIC; KETOTIC HYPERGLYCINEMIA; ketotic II glycinemia; ketotic glycinemia; propionic aciduria