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Diet | Nutrients | Keep yourself up-to-date

Diet

Diet

As your child’s diet shifts from infant milk to food, there will be a couple of things to remember to help your child grow, stay energetic, and healthy:

Enough food and enough energy intake

At any age, patients with citrin deficiency can easily have energy deficiency due to some issues of the metabolic mechanism, and a shortage of energy can cause fatigue and dizziness.

Make sure your children eat breakfast, lunch, and dinner, and take snacks in between to secure the intake of enough food and to keep the break between meals short. This routine also helps to reduce the risk of them taking a lot of carbohydrates at one go. Please encourage them to keep to this routine as it will help them manage their own health better when they grow up.

Examples of snacks: cheese, yogurt, milk, deep-fried chicken, etc.

Patients are recommended to add MCT to your diet as it directly provides energy to the liver. This is important for citrin deficiency patients as they have energy deficiency in the liver.  MCT has also been reported to be effective for citrin deficiency patients during the compensatory phase. As MCT is not commonly found in foods, citrin deficiency patients are recommended to take MCT oil as supplements. To learn more about MCT, please visit What is MCT? for more information. 

High protein, high fat, and low carbohydrates

During infancy, the patients rely on milk supplemented with  MCT for their energy intake. Because the milk supplemented with MCT maintains a good energy balance (PFC ratio) for them, they are able to intake enough energy.

But as they grow bigger, the energy source shifts to food rather than milk. The total energy composition of general meals is very different from milk. It will be less protein, less fat, and more carbohydrate compared to what milk offers.

As it is not easy for citrin deficiency patients to convert carbohydrates to energy, patients tend to feel sick when they consume too much carbohydrates. As such they begin to pick and prefer food with higher amounts of fat and protein which are the other energy sources, to secure enough energy intake. Please read here for the guideline of amount and variety of meal to prepare.

Because the ideal nutritional balance of patients is different from the general balance, it looks like they have a strong preference for food, but they are just trying to main a well-balanced diet for themselves.

The appropriate energy balance may be somewhat different in each patient. Please consult your doctor. For your reference, the average energy component ratio (PFC ratio) of citrin patients in Japan is:

The average energy component ratio (PFC ratio) of patients in Japan

The average energy component ratio (PFC ratio) of patients in Japan

Protein: 20%, Fat: 50%, Carbohydrate: 30%

The above is also the recommended energy balance in Japan. Please read more here (Dr. Okano’s Analysis of dietary intake in citrin-deficient patients: Towards the prevention of CTLN2 – Citrin Foundation).

The PFC ratio can be calculated using the amount of protein, fat, and carbohydrate on the Nutrition Facts table usually found on the back of the food package. Please read here to learn how to calculate the energy balance.

To show you the PFC balance of some foods,

White rice

Protein: 6%, Fat: 2%, Carbohydrate: 92%

Fried chicken

Protein: 26%, Fat: 58%, Carbohydrate: 16%

Whole milk

Protein: 20%, Fat: 50%, Carbohydrate: 30%

It is better to balance the nutritional intake at every meal to help sustain a healthy state for the patients.

When a child is still young, they may claim they want more sugar than they can actually process in their body. Please watch your child carefully. 

Seasonings and Vegetables

According to a survey done in Japan, most patients seem to like just salt & pepper — or something simple for seasonings. They are sensitive to sweet-tasting seasonings and tend to avoid anything that tastes sweet to them when it may not be particularly sweet to others. Patients may not like vegetables if the vegetables contain a higher amount of carbohydrates or if they are cooked with certain seasonings. Conversely, they tend to like vegetables with higher protein such as tomato, broccoli, or asparagus. It is advisable for children to continue eating at least a small portion of vegetables that are low in carbohydrates even if they do not like them.

Nutrients

Nutrients

Protein

Protein

Protein is also an important source of energy, and it is a nutrient that constructs our bodies.

There are many kinds of protein available, and it is better for us to eat a variety of food so we can take in as many kinds of protein as possible. A lot of protein is found in eggs, chicken breast, fish, milk, cheese, yogurt, tofu, and beans.

Milk is a favorite for many patients with citrin deficiency but for those who do not prefer it or who have an allergy to dairy products, it can be substituted with soy milk or other soy products.

Fat

Fat

There are various kinds of fat, but they are all important sources of energy. Ribs, chicken thighs, sausage, bacon, oil, butter, and whip cream are examples of food that contains a lot of fat.

Patients with citrin deficiency like fatty food and consume a lot of such food to gain energy from it. Their favorite meat is usually chicken thigh, and their favorite fish is salmon but serving various kinds of meat and fish will help balance other nutrients.

It would be healthier if olive oil is used for deep-frying and regular cooking oil is used for pan-frying.

However, consuming too much food that are high in usual fat (long-chain fats) may cause other health issues so patients are recommended to consume some MCT instead. MCT is different from the usual fat as it directly provides energy to the liver. This is important for citrin deficiency patients as they have energy deficiency in the liver.   Please read more on MCT here.

Carbohydrate (sugar)

Carbohydrate (sugar)

Carbohydrates and sugar are similar, and it is one of the nutrients that produce energy for us.

Examples of food that contain high proportions of carbohydrates are: rice, bread, noodles, fruits, sweets, etc.

Please note that vegetables and meat that you may not think have carbohydrates, do contain some carbohydrates.

Taking too much carbohydrates all at once tends to cause nausea, headache, or stomachache in patients with citrin deficiency. Watch your child carefully, and do not force them to eat any more than he/she wants especially when it comes to food containing high amounts of carbohydrates. Children may be young, but their bodies know how much they can take.

If they are willing to take more carbohydrates than usual, please keep them under careful supervision. It may be because their body needs a little more carbohydrates for balance.

Keep yourself up to date

Keep yourself up to date

Research in science and medicine will consistently yield new findings and developments. It is important that you keep yourself up-to-date on the latest findings and information to enhance your or your child’s health.

Citrin Foundation aims to support patients by funding research in citrin deficiency directly and being on top of the latest developments in scientific and treatment development regarding citrin deficiency with our network of scientists and doctors around the world.

Please join our patient community here, and we will send you the latest updates on developments and treatments when they are available, as well as invitations for events we host and tips to enhance the quality of patients’ life.

Your registration will also help doctors and scientists to further understand the condition and will be helpful for our efforts in developing treatments.

Please be rest assured that your information will be kept confidential under our Personal Data Privacy Policy.

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