British paediatric surveillance unit study of biliary atresia: outcome at 13 years
| Autor: | Giorgina Mieli-Vergani, Deirdre Kelly, Carla Lloyd, Alastair Baker, PJ McKiernan |
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| Rok vydání: | 2009 |
| Předmět: |
Pediatrics
medicine.medical_specialty medicine.medical_treatment Portoenterostomy Hepatic Liver transplantation Gastroenterology Actuarial survival National cohort Cohort Studies Biliary atresia Biliary Atresia Internal medicine Surveys and Questionnaires medicine Humans Child Survival rate business.industry Follow up studies Infant medicine.disease United Kingdom Liver Transplantation Transplantation Survival Rate Treatment Outcome Child Preschool Pediatrics Perinatology and Child Health business Ireland Cohort study Follow-Up Studies |
| Zdroj: | Journal of pediatric gastroenterology and nutrition. 48(1) |
| ISSN: | 1536-4801 |
| Popis: | Background: Little information is available on contemporary, prospectively collected data on the long-term outcome of national cohorts of children with biliary atresia. Objective: This study aimed to describe the current outcome of a national cohort of children with biliary atresia. Patients and Methods: All 93 cases of biliary atresia in the United Kingdom and Ireland diagnosed between March 1993 and February 1995 were followed up prospectively. Results: A total of 91 children underwent Kasai portoenterostomy in 15 individual centres. Only 2 centres treated more than 5 children annually. Median age at last follow-up was 12 years (range 0.25–14). Fifteen children (16%) have died: 10 after unsuccessful portoenterostomy, 1 of sepsis after successful portoenterostomy, and 4 after liver transplantation. Forty-two (45%) underwent liver transplantation at a median age of 1 year (range 0.5–9), with 90% survival. All 41 children with failed portoenterostomy (and 2 without portoenterostomy) died or underwent liver transplantation at a median age of 0.8 years (range 0.25–6.5). When the portoenterostomy was successful, 40 of 50 patients (80%) are alive without liver transplantation. The 13-year actuarial survival without liver transplantation is 43.8% overall and is better in children treated at centres that treat more than 5 cases yearly (54% vs 27.3%, P = 0.005). Conclusions: If the portoenterostomy is successful, then few children with biliary atresia will need transplantation before adolescence. Children with biliary atresia should be treated in experienced centres to maximize the chance of successful surgery. |
| Databáze: | OpenAIRE |
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