Outcomes of biliary atresia in the Nordic countries – a multicenter study of 158 patients during 2005–2016

Abstract

Level II.

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 .…”

Outcome for biliary atresia patients treated at a low-volume centre

“…It is possible that a more systematic ultrasonographic examination might have demonstrated findings highly suggestive of BA in some patients which could have been referred to surgery without any further investigations. In our center, percutaneous liver biopsies are not undertaken to diagnose BA because interpretation of biopsies may be difficult and takes time (7). Therefore, either ERCP or PCC have been performed.…”

“…One Norwegian patient has been successfully operated at another hospital in the study period, but data from this patient is not included in this series. Some of the data from patients operated between January 2005 and June 2016 have recently presented in a multicentre Nordic study (7). Clearance of jaundice was defined as postoperative plasma bilirubin level < 20 mol/l within six months after PE.…”

A paradigm shift in the intention‐to‐transplant children with biliary atresia: Outcomes of 101 cases and a review of the literature

“…These kinds of studies were scarce in the literature, but the information was provided by various registries. Table suggests that the outcomes of BA children are lower than one thought, with an overall intent‐to‐treat survival of 83%‐90% at 5 years, and probably much lower, for those who are in need of a transplant before the age of 1.…”

“…Because the disease onset is in the neonatal period and because Kasai portoenterostomy surgery has a limited success rate, around 50% of the infants born with BA need a liver replacement before the age of 5, in order to prevent serious complications and death . In a cohort of 14 452 children transplanted in Europe between 1968 and 2017 (data from the ELTR), BA was the indication for 39% of the primary LT, setting BA as the leading cause in children.…”