Biliary Atresia

Abstract

Biliary atresia is a rare disease of unclear etiology, in which obstruction of the biliary tree causes severe cholestasis leading to cirrhosis and ultimately death if left untreated. Biliary atresia is the leading cause of neonatal cholestasis and the most frequent indication for pediatric liver transplantation. Any infant with persistent jaundice beyond 2 weeks of life needs to be evaluated for biliary atresia with fractionation of the bilirubin into conjugated and unconjugated portions. Early performance of a hepatoportoenterostomy in the first 45 days of life to restore bile flow and lessen further damage to the liver is thought to optimize outcome. Despite surgery, progressive liver scarring occurs, and 80% of patients with biliary atresia will require liver transplantation during childhood.

Regulation of bile acid metabolism in biliary atresia: reduction of FGF19 by Kasai portoenterostomy and possible relation to early outcome

“…It is caused by progressive obliteration of extrahepatic bile ducts but also involves intrahepatic bile ducts. The aetiology of the disease remains unclear, but left untreated it will lead to liver failure and death within 2 years of age . First‐line treatment to restore bile flow and thereby stop progression of the disease is the surgical procedure of Kasai portoenterostomy (KPE), where a Roux‐en‐Y loop of the intestine is anastomosed to the liver .…”

“…First‐line treatment to restore bile flow and thereby stop progression of the disease is the surgical procedure of Kasai portoenterostomy (KPE), where a Roux‐en‐Y loop of the intestine is anastomosed to the liver . This procedure improves cholestasis in about half of the cases, but 70–80% of the affected children will still require liver transplantation before adulthood .…”

Large-scale proteomics identifies MMP-7 as a sentinel of epithelial injury and of biliary atresia

“…This rare disease results from a rapidly progressive inflammatory and fibrosing injury to extrahepatic bile ducts that interrupts the flow of bile and produces severe liver injury in otherwise healthy infants. With an onset of pathological jaundice limited to a highly reproducible window in the first 3–4 months of life of affected infants, the early diagnosis and surgical intervention are critical for the restoration of bile flow and improvement in short-term outcome (7, 9, 10). However, neonatal jaundice is a common sign for several clinical syndromes.…”

“…However, neonatal jaundice is a common sign for several clinical syndromes. Clinical algorithms have been proposed to differentiate BA from other causes of neonatal cholestasis (7, 11, 12), but they are imprecise and have the potential to delay the diagnosis and treatment (12). …”

Factors associated with the need for intraoperative packed red cells transfusion in pediatric liver transplant patients

“…The main indications for liver transplantation in pediatric patients are cholestatic diseases, mainly biliary atresia. 8,9 According to the European Liver Transplant Registry, 74% of recipients under 2 years of age and more than 40% of older children had cholestasis. A substantial percentage of these patients were malnourished (28%), signifying the severity of the Massive bleeding is defined as the loss of more than 50% of blood volume in the first 3 hours of surgery.…”