Biliary Atresia Sample Clauses

Biliary Atresia. NSC has similarities with a more common condition, biliary atresia (BA). Both involve inflammation and narrowing of the bile ducts, and lead to liver failure. NSC could in fact be one form of BA, particularly as it is generally accepted that the latter is not one condition at all, but rather several different conditions with a similar ultimate phenotype. At King’s College Hospital NSC is diagnosed in only one or two newborn infants every year, while there are as many as twenty new cases of BA in the same time period. BA is also a high GGT cholangiopathy, affecting extra and intrahepatic bile ducts and manifesting with prolonged cholestasis, progressive fibrosis and pronounced inflammatory obliteration of the extrahepatic biliary system during the first few weeks of life. Early surgery in the form of a Kasai portoenterostomy is the treatment of choice with about 40-60% of the patients clearing their jaundice (Xxxxxxxxx et al. 1997, Xxxxxxx et al. 2009). LT is the treatment of choice for the patients who remain cholestatic or develop portal hypertension, with bleeding varices refractory to endoscopic treatment. The incidence of the disease is between 1:8,000-18,000 live births (Xxxxxxxx et al. 2008). The pathogenesis of the disease remains unclear with some patients developing cirrhosis very early on in the neonatal period, while others have milder liver histological changes. The best outcome with their native liver is reported in infants who undergo a Kasai portoenterostomy under the age of 43 days (Xxxxxxxxx et al. 2011).