Biliary Atresia in Northwest Iran: Epidemiologic Features and Long-Term Outcome

Background: Biliary atresia is a rare distractive cholangiopathy in childhood and the most common cause of liver transplantation in pediatrics. Present study is the first report of biliary atresia and its incidence and outcome from northwest of Iran. Methods: In this retrospective cohort study, all patients with biliary atresia admitted to the Tabriz Children's Hospital from March 2006 to March 2016were included. Demographical, clinical and preclinical data of all patients are extracted from the hospital records. Information about the outcome after Kasai portoenterostomy was gathered by reviewing the progress notes or by phone call from the parents. The Mann-Whitney U and chi-square or Fisher's exact tests were used for comparison of continuous and categorical variables respectively. Survival curves and tables, Kaplan-Meier method and Cox regression were used for survival analysis. Results: The incidence rate of biliary atresia was 1/13280 live births. Isolated form of biliary atresia was seen in 85.1% of cases. Splenic malformation was not observed in our patients. The rate of successful Kasai Portoenterostomy was 42.9%. Survival analysis showed that 1, 2, 3, 5, and 10-year native liver survival rate was 73%, 56%, 54%, 40% and 31%, respectively. Median overall survival rate was 36 months [95%CI: 2.89 - 69.1]. Successful Kasai Portoenterostomy and clearance of jaundice was the only factor that affected survival rate. Conclusions: The incidence rate of biliary atresia in Northwest of Iran was lower than that in other Asian countries. Like other Asian countries, spleen anomaly was rare in our patients. The success of Kasai Portoenterostomy and survival rates of our cases were lower than that in other Asian countries.