The ideal PFC ratio depends on the condition of the patient. It is ok not to calculate it every time.

On normal days, if the patient is healthy as usual, the nutritional intake will naturally be well-balanced, if the patient consumes food they prefer in the amount they prefer.

A survey report conducted in Japan shows the average PFC ratio for citrin deficiency patients was 20:50:30 (Japan is known to consume a higher portion of carbohydrates compared to western countries). This ratio is derived from a study conducted by Dr. Yoshiyuki Okano which you can read more about here.

It will be ideal if one meal has a combination of dishes with a variety of ingredients that meets the balanced PFC. Changing the method of cooking (grill↔deep fry), the amount cooked, and ingredients used can help to adjust the nutritional balance. Adding MCT is another way to adjust the balance.

If for some reason, the PFC ratio of one meal is imbalanced (for example the patient took a lot of carbohydrates at lunch), the patient is likely to adjust the nutritional balance at dinner naturally by reducing the amount of carbohydrates and vice versa. Please leave it up to the patient but maintain careful monitoring.

* There have been reported cases of AACD* onset following a load of carbohydrate intake (ex. a bag of candy) at once, or long-term excessive carbohydrate consumption (ex. lifestyle change such as marriage), probably because the self-protection function in the patient was somehow impaired. Please be careful with consuming too much carbohydrate.

Please consult your dietitian periodically to review your diet.

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