High-dose methylprednisolone-induced hepatitis in a patient with multiple sclerosis : A case report and brief review of literature 199

Background: Biliary atresia (BA) is a progressive inflammatory destructive process of the bile ducts occurring in about one of every 20,000 live births. If left untreated, biliary atresia can lead to liver failure. The only effective treatments for BA at the moment are the Kasai operation and liver transplantation. Kasai portoenterostomy increases the survival of children with BA and postpones subsequent liver transplantation. Because long-term survival is rare, there is not much known about the long-term efficacy of the Kasai operation. Methods: The aim of this review was to study the outcome of patients with BA who survived more than 20 years on their native liver. We performed a systematic search on PubMed using MeSH terms for articles describing the long-term outcomes of patients with biliary atresia. We searched for patients who have lived at least 20 years with their native liver and we registered the number of complications. The endpoints identified in these articles were: death, cholangitis, portal hypertension and gastro intestinal bleeding. Results: From 53 articles we included 14 articles for analysis. In total 184 patients were above the age of 20 years. Of these 162 patients, 88% (162/184) were still alive with their native liver and 60.5% (98/162) were suffering from liver-related complications. Conclusions: It is possible for patients with biliary atresia to survive more than 20 years on their native liver after undergoing the Kasai operation during early infancy. However, 60.5% of the long-term survivors alive on their native liver end up suffering from progressive liver-related complications. K e y W o r d s Biliary atresia/surgery, cholangitis, follow-up, porto enterostomy, survival rate i n t r o d U C t i o n Biliary atresia (BA) is a progressive inflammatory destructive (obliterative) process of the bile ducts. It is characterised by a perinatal complete obstruction of all or part of the extrahepatic bile ducts and is always associated with abnormalities of the intrahepatic biliary tree. If left untreated, biliary atresia will lead to liver failure. Biliary atresia is a rare condition with a prevalence of about one patient per 20,000 live births; patients are destined for a fatal outcome if they are not treated during the first few months of life. The only effective treatments for BA at present are hepatic portoenterostomy (Kasai operation) and liver transplantation. The Kasai operation was introduced in 1959, and consists of constructing a new bile drainage system, generally by creating an anastomosis of the jejunum by a Roux-en-Y loop to the porta hepatis region to re-establish a connection between the intrahepatic bile ducts and the intestine. If successful, Kasai portoenterostomy increases the survival of children with BA and consequently postpones subsequent liver transplantation. Studies have reported 20-year survival rates in patients with their native liver of 21%, 25%, 22%, 23% and 44%. However, most long-term survivors develop complications. Until the age of 18, BA patients are generally managed by paediatricians or paediatric surgeons. After the age of 18 these patients are referred to and managed by gastroenterologists. It is