Carnitine-acylcarnitine translocase deficiency

Carnitine-acylcarnitine translocase deficiency

Definition

Carnitine-acylcarnitine translocase (CACT) deficiency is a life-threatening, inherited disorder of fatty acid oxidation which usually presents in the neonatal period with severe hypoketotic hypoglycemia, hyperammonemia, cardiomyopathy and/or arrhythmia, hepatic dysfunction, skeletal muscle weakness, and encephalopathy.

Also known as CACT deficiency, carnitine-acylcarnitine translocase deficiency — per MONDO

Also identified as