Glutaric aciduria type II

Glutaric aciduria type II, also known as multiple acyl-CoA dehydrogenase deficiency (MADD), is an inherited metabolic disorder characterized by the body's inability to process fats and proteins properly. This leads to the accumulation of organic acids, including glutaric acid, in the blood and urine, causing a range of symptoms from mild to severe.

This code is appropriate for patients diagnosed with Glutaric aciduria type II, confirmed through biochemical testing (e.g., elevated glutaric acid and other acylcarnitines in blood, organic acids in urine) and/or genetic testing. Documentation should clearly specify "type II" or "multiple acyl-CoA dehydrogenase deficiency" to support its use.

• Ensure documentation specifies "type II" to differentiate from other forms of glutaric aciduria.
• Do not code this with E72.3 (Glutaric aciduria type 1 NOS) as per Excludes1 note.
• Consider sequencing this code as a primary diagnosis when it is the main reason for the encounter, especially for ongoing management or diagnostic workup.