Why do citrin deficiency patients need to take MCT?

Citrin deficiency patients cannot tolerate much carbohydrates which is why they develop a natural food aversion against foods that are high in carbohydrates. They instead take a lot of food that are high in fat and protein to gain energy, but the usual fat (long-chain fat) may increase health issues later on. Protein is not very efficient in generating energy.

MCT (medium-chain fat) is different from the usual fat in a way it directly provides energy to the liver. This will be important for citrin deficiency patients as they have energy deficiency in the liver. As MCT is not commonly found in foods, citrin deficiency patients are recommended to take MCT oil as supplements. 

MCT has been used as treatment for NICCD and AACD*. Additionally, MCT oil has also been reported to be effective for citrin deficiency patients during the compensatory phase.

*Adolescent and adult-onset citrin deficiency (AACD) – formerly known as CTLN2

Recommended Dosage of MCT
  1. 1g/kg – According to Professor Kimitoshi Nakamura, Professor and Chairman of the Department of Pediatrics at the Graduate School of Medical Sciences at Kumamoto University
  2. If patients are not able to tolerate this dosage due to stomach upset, please adjust the dosage accordingly. 
  3. Please consult your doctor for the usage
Recommended Dosage of MCT
  1. 1g/kg – According to Professor Kimitoshi Nakamura, Professor and Chairman of the Department of Pediatrics at the Graduate School of Medical Sciences at Kumamoto University
  2. If patients are not able to tolerate this dosage due to stomach upset, please adjust the dosage accordingly. 
  3. Please consult your doctor for the usage.

Useful tips on how to take MCT 

  1. MCT enriched formula / MCT supplemented breast milk (please consult your doctor for dosage) is recommended for NICCD patients 
  2. MCT oil cannot be heated for cooking but MCT powder is available for cooking.
  3. Post NICCD:
    • It is best to take MCT oil with your meals, in divided portions throughout the day 
    • Some suggested methods of consuming MCT oil are as follows: 
      • Blend it in your smoothie or drink with a blender 
      • Mix it in your salad or add it to boiled vegetables 
      • Add it to your soup and stir it well
      • Dip it with bread