The research funded by The Liver Foundation for Kids in 2001, is
based in finding the mechanisms leading to biliary atresia (the most
common, severe liver disease in infants). Biliary atresia is a disease
that begins around the time of birth and progresses to the destruction
of the bile ducts. The destruction of the bile ducts is mediated by
inflammatory injury. The focus of the research is why the inflammation
occurs and why it progresses. Inflammation is caused by some outside
stimulus or autonomously. Autonomous inflammation is the cause of
autoimmune disease, where the bodys immune system attacks and
attempts to destroy part of itself. Current theories suggest that
infection may stimulate an immune response, which confuses the body
into attacking itself.
The research funded theorizes that biliary atresia is, in fact, an
autoimmune disease and through studying immune systems of babies with
biliary atresia will attempt to identify the mechanism of biliary
atresia and how it might be interrupted and treated medically.
Dr. Cara Mack is spearheading this research, which has, since our
initial support, been further funded by National Institute of Health.
The support provided in 2002, has gone to research headed by Dr. Peter
Whittington, into the study of inflammation of the liver, diseases
such as autoimmune hepatitis, infectious hepatitis, and various biliary
diseases fall into this category.
The steps bridging the initial disease to the ensuing cirrhosis are
unknown as are the steps leading to inflammation and eventual scarring
of the liver. This new research focuses on the role of a newly found
cytokine,osteopontin, (a chemical messenger that calls In inflammatory
cells and activates scar production). It is theorized that osteopontin
may play an important role in many pediatric liver diseases, most
notably, autoimmune hepatitis,biliary atresia and Alagilles
syndrome.
It is important to note that any research that leads to a greater
understanding of the liver is critical to the development of treatments
for all types of liver disease.
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