OBJECTIVE: To determine the outcomes of Canadian children with biliary atresia. STUDY DESIGN: Health records of infants born in Canada between January 1, 1985 and December 31, 1995 (ERA I) and between January 1, 1996 and December 31, 2002 (ERA II) who were diagnosed with biliary atresia at a university center were reviewed. RESULTS: 349 patients were identified. Median patient age at time of the Kasai operation was 55 days. Median age at last follow-up was 70 months. The 4-year patient survival rate was 81% (ERA I = 74%; ERA II = 82%; P = not significant [NS]). Kaplan-Meier survival curves for patients undergoing the Kasai operation at age < or = 30, 31 to 90, and > 90 days showed 49%, 36%, and 23%, respectively, were alive with their native liver at 4 years (P < .0001). This difference continued through 10 years. The 2- and 4-year post-Kasai operation native liver survival rates were 47% and 35% for ERA I and 46% and 39% for ERA II (P = NS). A total of 210 patients (60%) underwent liver transplantation; the 4-year transplantation survival rate was 82% (ERA I = 83%, ERA II = 82%; P = NS). CONCLUSIONS: This is the largest outcome series of North American children with biliary atresia at a time when liver transplantation was available. Results in each era were similar. Late referral remains problematic; policies to ensure timely diagnosis are required. Nevertheless, outcomes in Canada are comparable to those reported elsewhere.
OBJECTIVE: To determine the outcomes of Canadian children with biliary atresia. STUDY DESIGN: Health records of infants born in Canada between January 1, 1985 and December 31, 1995 (ERA I) and between January 1, 1996 and December 31, 2002 (ERA II) who were diagnosed with biliary atresia at a university center were reviewed. RESULTS: 349 patients were identified. Median patient age at time of the Kasai operation was 55 days. Median age at last follow-up was 70 months. The 4-year patient survival rate was 81% (ERA I = 74%; ERA II = 82%; P = not significant [NS]). Kaplan-Meier survival curves for patients undergoing the Kasai operation at age < or = 30, 31 to 90, and > 90 days showed 49%, 36%, and 23%, respectively, were alive with their native liver at 4 years (P < .0001). This difference continued through 10 years. The 2- and 4-year post-Kasai operation native liver survival rates were 47% and 35% for ERA I and 46% and 39% for ERA II (P = NS). A total of 210 patients (60%) underwent liver transplantation; the 4-year transplantation survival rate was 82% (ERA I = 83%, ERA II = 82%; P = NS). CONCLUSIONS: This is the largest outcome series of North American children with biliary atresia at a time when liver transplantation was available. Results in each era were similar. Late referral remains problematic; policies to ensure timely diagnosis are required. Nevertheless, outcomes in Canada are comparable to those reported elsewhere.
Authors: Sanjiv Harpavat; Philip J Lupo; Loriel Liwanag; John Hollier; Mary L Brandt; Milton J Finegold; Benjamin L Shneider Journal: J Pediatr Gastroenterol Nutr Date: 2018-06 Impact factor: 2.839
Authors: Melissa Leyva-Vega; Jennifer Gerfen; Brian D Thiel; Dorota Jurkiewicz; Elizabeth B Rand; Joanna Pawlowska; Diana Kaminska; Pierre Russo; Xiaowu Gai; Ian D Krantz; Binita M Kamath; Hakon Hakonarson; Barbara A Haber; Nancy B Spinner Journal: Am J Med Genet A Date: 2010-04 Impact factor: 2.802
Authors: Marcello Napolitano; Stéphanie Franchi-Abella; Maria Beatrice Damasio; Thomas A Augdal; Fred Efraim Avni; Costanza Bruno; Kassa Darge; Damjana Ključevšek; Annemieke S Littooij; Luisa Lobo; Hans-Joachim Mentzel; Michael Riccabona; Samuel Stafrace; Seema Toso; Magdalena Maria Woźniak; Gianni Di Leo; Francesco Sardanelli; Lil-Sofie Ording Müller; Philippe Petit Journal: Pediatr Radiol Date: 2020-11-17