Most children with Alagille syndrome need to take vitamin supplements. Because children with Alagille syndrome have difficulty absorbing fat, they may develop fat-soluble vitamin (A, D, E and K) deficiencies if they do not receive proper supplementation. They may also require extra calcium and zinc. The best way for a child with Alagille syndrome to receive the vitamins that he or she needs is to take separate vitamin supplements for each vitamin. Blood levels of vitamins should be monitored and supplements should be adjusted accordingly. Be sure to talk to your child's doctor about what vitamin supplements your child may need. Also, don't give give your child any additional vitamins without first checking with the healthcare team, because too much Vitamin A can be toxic to the liver.
Vitamin A is required for normal vision, cellular differentiation, reproduction, growth and proper immune function. Vitamin A is found primarily in selected foods of animal origin, especially liver and dairy products including whole milk, cheese and butter, as well as fish such as tuna and sardines. Signs and symptoms of vitamin A deficiency include growth retardation, increased susceptibility to infection and night blindness. Vitamin A can be toxic to the liver so individuals with AGS need to be careful about oversupplementation Careful monitoring of blood levels is essential to avoid toxicity.
Vitamin D is extremely important for bone health. Without enough vitamin D, children's bones can become thin, brittle and soft and rickets can develop. Vitamin D is found in foods such as milk and fatty fishes and can also be made by the body when it is exposed to sunlight. There are different forms of vitamin D and the proper form for children with cholestasis is cholecalciferol. Vitamin D levels (25 [OH] D) should be checked every few months, and supplementation should be adjusted to make sure that levels are at the high end of normal. Your child's vitamin D levels should also be checked during different seasons, because levels tend to be higher in the summer due to more sunlight exposure.
Vitamin E protects cells against the effects of free radicals, which are potentially damaging by-products of the body's metabolism. Vitamin E deficiency can cause decreased reflexes, weakness, wide gait (walking with the legs far apart) and hemolytic anemia. Children with cholestasis on large doses of vitamin E (either alpha tocopherol or alpha tocopherol acetate) can still have a vitamin E deficiency. The best vitamin E form for preventing or reversing this deficiency is the liquid form: tocopheryl polyethylene glycol 1000 succinate (TPGS), because the body absorbs it more readily. TGPS also enhances vitamin D absorption. Your child's vitamin E levels should be checked regularly.
Vitamin K is important for bone health and clotting. Children with vitamin K deficiency usually bruise and bleed more easily. Vitamin K is found in cabbage, cauliflower, spinach, and other green leafy vegetables, cereals, soybeans and other vegetables. Children whose blood clotting times can't be normalized with oral vitamin K may need intramuscular (into the muscle) vitamin K injections.
ADEK is a multivitamin supplement designed for people with malabsorptive conditions. It comes in pills or liquid and is all-in-one formula contains high levels of fat-soluble Vitamins A, D, E and K in water-miscible form, plus water-soluble vitamins and zinc.
Calcium is the mineral that makes up bones and keeps them strong. If you don't get enough calcium from the food you eat, your body automatically takes the calcium you need from your bones. Calcium is most widely found in dairy foods but is also fortified foods including certain brands of orange juices. Fractures are common in children with Alagille syndrome. If your child has low calcium intake, low bone density and/or severe fat malabsorption, he or she should take calcium supplements. A bone density test (called a DEXA scan) — a noninvasive test — can measure your child's bone health and help determine if he or she needs more calcium.
Zinc is an essential mineral that is found in almost every cell. Zinc supports a healthy immune system, is needed for wound healing and helps maintain a sense of taste and smell. Zinc also supports normal growth and development during childhood and adolescence. Zinc deficiency most often occurs when zinc intake is inadequate or poorly absorbed, when there are increased losses of zinc from the body, or when the body's requirement for zinc increases. Signs of zinc deficiency include growth retardation, hair loss, diarrhea, delayed sexual maturation and loss of appetite. Your child may have his or her blood zinc levels tested. While these levels aren't very effective at indicating zinc deficiency, currently there is no better alternative. If your child's zinc levels are low, he or she may benefit from zinc supplementation.
Reviewed by: Alisha Rovner
Date: November 2005