Biliary atresia is the most serious liver disease of infancy and childhood, accounting for 50% of pediatric liver transplants and 10% of all transplants in adults. It is the most rapidly fibrosing liver disease and, unfortunately, its cause is still unknown. Doctors at Johns Hopkins are working hard to improve the outlook for children with this disease by a multi-pronged approach: pilot projects with stool color cards given to mothers of newborns to call attention to abnormally pale stools and the need to get medical help to rule out biliary atresia; basic laboratory studies to determine the molecular pathway of the scarring process and how to block it; clinical epidemiology studies to look for viral causes; and antifibrotic treatments to improve the outlook once the diagnosis is made. Much more research is needed to understand, treat and ultimately prevent this disease.
Over the past 25 years, the Pediatric Liver Center in the Division of Gastroenterology and Nutrition at the Johns Hopkins Children’s Center treated more than 1,000 young patients with a variety of acute and chronic liver diseases, including biliary atresia. Severe liver disease is often life-threatening and may necessitate liver transplantation, which can be both life-saving and problematic. By offering a comprehensive approach to the diagnosis and management of pediatric liver disease, the physicians at the Pediatric Liver Center have two major research goals: preventing the need for transplant by developing treatments for hepatitis and biliary atresia; and maximizing the success of transplants for liver failure of all causes. The team at the Pediatric Liver Center is committed to helping children suffering from liver disease, along with their families, lead better lives.
News releases from Johns Hopkins Children's Center website:
For patients suffering from rare forms of liver disease, finding other people with the same illness can be tough. Even doctors studying these illnesses can have trouble finding enough patients for their research.
Liver Space, a new Facebook application led by pediatric gastroenterologist Douglas Mogul, seeks to address these problems by linking both groups through Facebook. Users sign up by visiting apps.facebook.com/liverspace and creating a profile. Notifications about new Liver Space content are then automatically posted to the user’s Facebook account. It also offers self-care tools, like lab results tracking and the ability to connect with others nearby who have the same disease.
2016 Hopkins Medicine -- Health Library Article: How is a liver, biliary, or pancreatic disorder diagnosed?
July 2015: Small Study Affirms Accuracy of Free Mobile App that Screens for Liver Disease in Newborns
October 2014: Poop Color Screening Could Prevent Deaths, Avert Liver Transplants and Lower Treatment Costs in Babies with Rare Liver Disease
April 2014: The Color of Poop: Stool Guide, Mobile App to Speed up Diagnoses of Life-Threatening Liver Condition in Newborns