Important

It is possible that the main title of the report Glutaricaciduria II is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

• GA II
• Glutaric Acidemia II
• Glutaric Aciduria II
• Glutaricacidemia II

Disorder Subdivisions

• GA II
• Glutaric Acidemia II
• Glutaric Aciduria II
• Glutaricacidemia II
• Multiple Acyl-CoA Dehydrogenase Deficiency
• Glutaricaciduria, Type IIB
• Ethylmalonic Adipicaciduria
• GA IIB

General Discussion

There are two forms of Glutaricaciduria II which occur during different stages of life. They are both forms of organic acidemias which are a group of metabolic disorders characterized by excess acid in the blood and urine.

1) Glutaricaciduria IIA (GA IIA), Neonatal Form of Glutaricaciduria II. This neonatal form of Glutaricaciduria II is a very rare, sex-linked hereditary disorder characterized by large amounts of glutaric and other acids in blood and urine. Some researchers believe the disorder is caused by a defect in the breakdown of acyl-CoA compounds.

2) Glutaricaciduria IIB (GA IIB; Ethylmalonic Adipicaciduria), Adult Form of Glutaricaciduria II.This milder form of the disorder is inherited as an autosomal recessive trait. Acidity of the body tissues (metabolic acidosis), and a low blood sugar level (hypoglycemia) without an elevated level of ketones in body tissues (ketosis), occur during adulthood. Large amounts of glutaric acid in the blood and urine are caused by a deficiency of the enzyme "multiple acyl- CoA dehydrogenase."