Children's liver diseases
What it is:
Children can fall prey to virtually any form of liver disease including viral hepatitis, fatty liver disease and liver cancer. There are however certain liver diseases that affect primarily children and are often diagnosed shortly after birth. Some are linked to defective genes, such as alpha-1 antitrypsin deficiency and tyrosinemia, while others like biliary atresia have unknown causes. What they all have in common however, is that they impair the functions of the liver, interfere with a child’s growth and development and in some cases can be fatal.
What we know:
Biliary atresia leads to blockage in the bile ducts leading out of the liver. If the condition is left untreated, it can result in severe liver damage and death before the age of three. Currently biliary atresia is the leading cause of liver disease-related death in infants in Canada. Many of these deaths could be prevented if children were diagnosed early within a critical window for performing the Kasai procedure – a surgical treatment that involves attaching a piece of the intestine directly to the liver (thereby bypassing the defective bile ducts) to re-establish bile flow. Although the Kasai is not necessarily a cure, 50 percent of babies who have it in the first 30 days will not need a liver transplant. After 90 days, the percentage drops to 20 per cent.
What the CLF is doing:
- When something is wrong with a baby’s liver, the first indication may be the colour of her stool. In partnership with the CLF, Dr. Rick Schreiber (BC Children’s Hospital, British Columbia) is testing a stool colour card that will allow new parents to track and record information about their baby’s stool in the first month of life. The hope is that the cards will become the basis of a national home-based, cost-effective screening program that will ensure biliary atresia is identified and treated at the earliest possible stage.
- Thanks to a CLF research grant, Dr. Diana Mager (University of Alberta, Alberta) is studying how to help babies with digestive related liver diseases like biliary atresia whose bodies cannot process the nutrients – protein, fat, minerals and vitamins – from the food they eat. These infants suffer a form of malnutrition (called ‘protein energy malnutrition’ or PEM) that can be devastating to their developing brains. Dr. Mager hopes that supplying them with extra amino acids prior to liver transplants will help ensure they do not experience serious delays in physical and mental development post-transplant.
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