BACKGROUND: Biliary atresia is a progressive biliary injury which occurs only in infants. AIMS: To review the experience of patients surviving into adulthood without the need for liver transplantation in childhood. METHODS: A multicentre review of patients with biliary atresia treated surgically who survived into adulthood without the need for transplantation. RESULTS: Twenty-two patients were identified across four centres. Median age at the last follow-up was 25 years (range: 18-46), and 21 patients had clinical features of portal hypertension. At last follow-up values of liver enzymes varied from normal to 15 × the upper limit of normal (ULN) for ALT (median 2.11 × ULN) and 9 × the ULN for ALP (median 2.02 × ULN). Six patients had a serum bilirubin > 50 μmol/l. Pruritus and jaundice were noted in 8 of 20 patients (40%) and 11 of 22 patients (50%) respectively. Thirteen patients (59.1%) were shown to have imaging features of sclerosing cholangitis, with strictures of intrahepatic bile duct(s) (IHBD), dilatation of IHBD (n = 8), or stone(s) within the IHBD (n = 5). A history of presumed bacterial cholangitis was present in 11 patients (50%). Successful pregnancies were recorded in three of fourteen female patients. Four patients underwent transplant between the ages of 20-27 years. Twenty-one patients (95.5%) were alive, including 18 (81.8%) with their native liver at the time of last follow-up. CONCLUSIONS: Some patients treated for biliary atresia will survive into adulthood with their native liver, but commonly with secondary biliary disease including cholangitis and portal hypertension.
BACKGROUND:Biliary atresia is a progressive biliary injury which occurs only in infants. AIMS: To review the experience of patients surviving into adulthood without the need for liver transplantation in childhood. METHODS: A multicentre review of patients with biliary atresia treated surgically who survived into adulthood without the need for transplantation. RESULTS: Twenty-two patients were identified across four centres. Median age at the last follow-up was 25 years (range: 18-46), and 21 patients had clinical features of portal hypertension. At last follow-up values of liver enzymes varied from normal to 15 × the upper limit of normal (ULN) for ALT (median 2.11 × ULN) and 9 × the ULN for ALP (median 2.02 × ULN). Six patients had a serum bilirubin > 50 μmol/l. Pruritus and jaundice were noted in 8 of 20 patients (40%) and 11 of 22 patients (50%) respectively. Thirteen patients (59.1%) were shown to have imaging features of sclerosing cholangitis, with strictures of intrahepatic bile duct(s) (IHBD), dilatation of IHBD (n = 8), or stone(s) within the IHBD (n = 5). A history of presumed bacterial cholangitis was present in 11 patients (50%). Successful pregnancies were recorded in three of fourteen female patients. Four patients underwent transplant between the ages of 20-27 years. Twenty-one patients (95.5%) were alive, including 18 (81.8%) with their native liver at the time of last follow-up. CONCLUSIONS: Some patients treated for biliary atresia will survive into adulthood with their native liver, but commonly with secondary biliary disease including cholangitis and portal hypertension.
Authors: Vicky Lee Ng; Barbara H Haber; John C Magee; Alexander Miethke; Karen F Murray; Sonia Michail; Saul J Karpen; Nanda Kerkar; Jean P Molleston; Rene Romero; Philip Rosenthal; Kathleen B Schwarz; Benjamin L Shneider; Yumirle P Turmelle; Estella M Alonso; Averell H Sherker; Ronald J Sokol Journal: J Pediatr Date: 2014-07-09 Impact factor: 4.406
Authors: Bjarte Fosby; Espen Melum; Kristian Bjøro; William Bennet; Allan Rasmussen; Ina Marie Andersen; Maria Castedal; Michael Olausson; Christina Wibeck; Mette Gotlieb; Henrik Gjertsen; Leena Toivonen; Stein Foss; Heikki Makisalo; Arno Nordin; Truls Sanengen; Annika Bergquist; Marie E Larsson; Gunnar Soderdahl; Greg Nowak; Kirsten Muri Boberg; Helena Isoniemi; Susanne Keiding; Aksel Foss; Pål-Dag Line; Styrbjörn Friman; Erik Schrumpf; Bo-Göran Ericzon; Krister Höckerstedt; Tom H Karlsen Journal: Scand J Gastroenterol Date: 2015-06 Impact factor: 2.423