Clinical and Laboratory Outcomes in Biliary Atresia: Insights from the Shiraz Pediatric Liver Cirrhosis Cohort.

OBJECTIVES: Biliary atresia is a progressive cholangiopathy in neonates leading to liver failure and often requiring liver transplant. Although surgical interventions like the Kasai portoenterostomy offer temporary relief, the disease continues to be the leading cause of liver transplant. This study aimed to evaluate the demographic, clinical, and laboratory characteristics of these patients and the effects of these characteristics on outcomes. MATERIALS AND METHODS: This cohort study included 167 pediatric patients (aged <18 years ) with confirmed biliary atresia, selected from the Shiraz Pediatric Liver Cirrhosis Cohort Study between 2018 and March 2024. We analyzed data collected from the pediatric liver cirrhosis registry and assessed patient outcomes by comparing survivors versus those who did not survive. RESULTS: Among the 167 patients, 86 (51.5 % ) died and 81 (48.5 % ) survived. Those who died were significantly younger at enrollment (15.47 vs 36.31 months old ) and had higher internal normalized ratio, prolonged partial thromboplastin time, and elevated liver enzymes compared with survivors. The Kasai portoenterostomy was performed in 77.8 % of patients, with no significant difference in its distribution between survivors and patients who did not survive. CONCLUSIONS: Although most children had a Kasai surgery and over one - third had a liver transplant, a significant number did not survive. To improve outcomes for children with biliary atresia, better perioperative and postoperative care, aggressive treatment of liver dysfunction, and robust nutritional support are essential.