Infants
0 – 1 years old
Infants
0 – 1 years old
Diagnosis
Diagnosis
Results from newborn mass screenings or clinical symptoms such as cholestasis, prolonged jaundice, or whitish stools usually lead to the diagnosis of ‘Neonatal Intrahepatic Cholestasis caused by Citrin Deficiency (NICCD)’, which is confirmed by genetic testing.
NICCD is one of the diseases caused by the genetic condition of citrin deficiency. It usually goes away naturally through appropriate treatments around the age of 1.
Finding the genetic condition enables an early start to treatment.
Nursing
Nursing
For infants diagnosed with NICCD, MCT enriched formula is usually recommended as they cannot metabolize most nutrients of breast milk or regular formula milk well, but please always consult your doctor for the appropriate formula to meet your baby’s needs. MCT has also been used as treatment for NICCD.
If MCT enriched formula is not available, supplementation of MCT to regular formula or breast milk is recommended. Please always consult your doctor for the recommended dosage and follow the nursing advice they provide.
Please learn more about MCT here and learn more about possible treatments and tips to manage the condition here.
Baby food (early to mid-term)
Baby food (early to mid-term)
Baby food can be introduced at the general appropriate timing with food that is generally suitable for their age if they are already treated with MCT enriched milk/MCT supplement and other necessary treatments prescribed by the doctor. You might have heard that patients with citrin deficiency do not eat a lot of carbohydrates and may be worried about feeding your baby carbohydrates. If your baby drinks MCT-enriched formula or takes enough MCT, this food preference may only start to be seen when babies eat more food as the source of energy and balance of nutrients substantially changes.
Baby food (mid to late-term)
Baby food (mid to late-term)
Infants are getting used to food and will begin taking more energy from food around this time. This is also the time when some of you start to see some changes in your infant’s reaction towards food. For example, you may see your baby rejecting pumpkins and sweet potatoes when they were his/her favorite, or only eating a reduced amount of carbohydrates. You may start seeing the food preference anytime during early infancy.
These changes in reactions arise because there was a change in the nutritional balance since your baby started to eat food. In earlier terms, when the energy source was solely from milk with MCT supplemented, their nutrition was well-balanced for citrin deficiency, and they did not need to adjust their diet. But as they start to take more energy from food other than milk in later terms, your baby will start balancing the nutrition him/herself by showing food preferences.
The metabolic system of citrin deficiency makes it a little difficult to get energy from carbohydrates. Instead, patients try to get energy from protein and fat, and for this purpose, they unconsciously build dietary preferences to like food with higher protein and fat content and avoid food with higher carbohydrate content. This preference serves as a natural and special function to manage and secure sufficient energy that they need.
Please feed your baby what they like, and do not force them to eat food with a high carbohydrate content any more than he/she wants.
It may be a good idea to introduce whole milk into your baby’s diet around the age of 1 if no allergy was detected when it was used in the preparation of baby food. Milk provides a very good nutritional balance for citrin deficiency patients after foods are introduced to their diet, and there are many patients who consume a lot of milk through adulthood. It is also recommended to include grated cheese or sesame seeds, etc. into the baby’s food. Please make sure to consult your doctor for any of these food introductions.