Currently, it is diet management. For NICCD patients, lactose-free formula supplemented with MCT is recommended [Tazawa et al. 2004, Ohura et al. 2007]. For post-NICCD patients, they should adhere to a low carbohydrate, high-protein, high-fat diet. MCT is also reported to be helpful and is recommended to be included in the patient’s diet during the “silent period”. For Adolescent and Adult Citrin Deficiency (AACD)* patients, they should also follow a low carbohydrate, high-protein, high-fat diet in addition to prescriptions such as sodium pyruvate, arginine, and/or MCT.
* Previously known as Citrullinemia type 2 (CTLN2)
