Maintain a low carbohydrate, high protein, high fat diet

It is important to keep a low carbohydrate, high protein and high fat diet. This tendency will become obvious when infants begin eating more solid food and gain more of their energy from food rather than milk. This serves as a very important function to compensate for the metabolic issues of citrin deficiency in the form of a ‘food preference’. Patients tend to naturally keep to a PFC ratio that is comfortable for them with the food that they prefer to eat. In other words, patients may stay healthy by following their own choice of food.

Please pay careful attention to consuming too much carbohydrates. Consuming a significant amount of carbohydrates (sugar) all at once and over-consumption of carbohydrates (sugar) over a long period of time can lead to the onset of CTLN2.

For your reference, based on the 2019 Citrin Deficiency Guidelines published by the Japan Society of Inherited Metabolic Disorders, the recommended PFC ratio for citrin deficiency patients is Protein: 15% – 25%, Fat: 40% – 50%, Carbohydrate: 30% – 40%. Try to take a variety of sources of fat and avoid relying on animal fat by actively using more healthy fat such as vegetable-oriented fat like olive oil.

If a fixed meal is provided, such as during school lunch, some arrangements may be necessary. Please learn more here.

Parents are strongly advised to monitor and teach their children from early childhood, by allowing them to be aware of their condition and letting them manage their diet by themselves to help them prepare for future independence. Patients are advised to independently manage their diets through preparing their own food or finding means to have their food ready by adolescence.

Take 3 main meals and snacks in between

The energy tends to become insufficient for patients with citrin deficiency as they have metabolism issues. It is very important to load energy frequently by eating breakfast, lunch, dinner, and having snacks in between for patients at any age.