SLC25A13 and Hyperammonemia: Only a few patients with citrin deficiency develop CTLN2, which is characterized by serious and recurrent hyperammonemia, liver steatosis, and neuropsychiatric symptoms such as sudden aberrant behavior and disturbance of consciousness after >10 years.[3] It is triggered by alcohol intake, excessive carbohydrate ingestion, sepsis, fasting, medication, or surgery,[4] and has a poor prognosis without liver transplantation despite intensive treatment.