Bárbara Neto1, Mariana Borges-Dias1, Eunice Trindade2, José Estevão-Costa1, José Miguel Campos1. 1. Department of Pediatric Surgery, Centro Hospitalar São João, Faculdade de Medicina da Universidade do Porto, Porto, Portugal. 2. Department of Pediatrics, Centro Hospitalar São João, Faculdade de Medicina da Universidade do Porto, Porto, Portugal.
Abstract
INTRODUCTION: Biliary atresia is the main cause of death by hepatic failure and the main indication for liver transplant in children. This study aims to analyze the population with this diagnosis, treated between 2000 and 2015 at Hospital de São João. MATERIAL AND METHODS: Descriptive, observational, and retrospective study, including the patients with biliary atresia, diagnosed and treated between January 1, 2000 and December 31, 2015. We analyzed epidemiologic, clinical, biochemical, and image data, as well as registered complications and present status. RESULTS: Eighteen patients were evaluated. The median age at time of Kasai portoenterostomy was 63 days of life, with better prognosis for those patients who had surgery before 72 days. The procedure was successful in 2/3 of cases. There was a significant association between recurrent cholangitis and survival. Five cases of transplant and 2 deaths, one of them after transplant, were registered. Survival with native liver was 77.8%, 72.2%, and 64.2% at 1, 5, and 10 years of follow-up, respectively. DISCUSSION: The presentation and evolution of patients was similar to other studies. However, there was a higher surgical success and survival rates at 5 and 10 years of follow-up than most series. Age at surgery and recurrence of cholangitis were the only factors significantly related to prognosis. CONCLUSION: In spite of the low number of patients (1,125/year), our results were similar to those of other reference centers.
INTRODUCTION: Biliary atresia is the main cause of death by hepatic failure and the main indication for liver transplant in children. This study aims to analyze the population with this diagnosis, treated between 2000 and 2015 at Hospital de São João. MATERIAL AND METHODS: Descriptive, observational, and retrospective study, including the patients with biliary atresia, diagnosed and treated between January 1, 2000 and December 31, 2015. We analyzed epidemiologic, clinical, biochemical, and image data, as well as registered complications and present status. RESULTS: Eighteen patients were evaluated. The median age at time of Kasai portoenterostomy was 63 days of life, with better prognosis for those patients who had surgery before 72 days. The procedure was successful in 2/3 of cases. There was a significant association between recurrent cholangitis and survival. Five cases of transplant and 2 deaths, one of them after transplant, were registered. Survival with native liver was 77.8%, 72.2%, and 64.2% at 1, 5, and 10 years of follow-up, respectively. DISCUSSION: The presentation and evolution of patients was similar to other studies. However, there was a higher surgical success and survival rates at 5 and 10 years of follow-up than most series. Age at surgery and recurrence of cholangitis were the only factors significantly related to prognosis. CONCLUSION: In spite of the low number of patients (1,125/year), our results were similar to those of other reference centers.
Authors: Daniel H Leung; Amrita Narang; Charles G Minard; Girish Hiremath; John A Goss; Ross Shepherd Journal: Liver Transpl Date: 2016-11 Impact factor: 5.799
Authors: Mark Davenport; J De Ville de Goyet; M D Stringer; G Mieli-Vergani; D A Kelly; P McClean; L Spitz Journal: Lancet Date: 2004-04-24 Impact factor: 79.321