Isobutyrylglycinuria (IBG)



Isobutyrylglycinuria (IBG) is an inherited condition in which the body is unable to break down certain proteins, which can lead to a harmful amount of organic acids and other toxins in the body. Symptoms of the condition vary. IBG is a very rare condition. Signs of IBG include delayed growth, sleeping longer or more often, tiredness, pale skin, trouble regulating body temperature, and difficulty breathing. Many of these signs may occur when a baby eats foods that the body cannot break down. If IBG is identified early and treatment is begun, individuals with the condition may be able to lead healthy lives. 

Condition Type:



Isobutyrylglycinuria (IBG) is extremely rare. The exact number of children affected by IBG is unknown. Fewer than 30 cases have been reported.

More Information for Parents:
Also known as:
  • IBG
  • IBD deficiency
  • Isobutyryl-CoA dehydrogenase deficiency
  • Isobutyryl-coenzyme A dehydrogenase deficiency
  • Acyl-CoA dehydrogenase family – member 8
  • ACAD8 deficiency

Core Conditions