[Biliary Atresia Registry: First Report in Argentina].

Abstract

Introduction: Biliary atresia is a disease with high morbidity and mortality, characterized by a fibrosclerosis and obstruction of the biliary ducts. Treatment is surgical and includes hepatic portoenterostomy and liver transplant. Early diagnosis is essential to ensure access to hepatic portoenterostomy within the first 30 - 45 days of life. In our country, we do not have national data on this pathology, which motivated the Hepatology Committee of the Argentine Society of Pediatrics to create a registry for this purpose. This report presents the results of incorporating a first center into the registry. This study allowed us to characterize a population of patients diagnosed with biliary atresia and treated at a high-complexity center in Argentina.

Objective: The objective of this study was to describe the clinical, surgical, and evolutionary characteristics of the registered patients.

Material and methods: This observational, descriptive, and retrospective study included patients diagnosed with biliary atresia treated at the Dr. Juan P. Garrahan Pediatric Hospital, born between January 1, 2015, and January 1, 2020. The diagnosis was based on clinical, biochemical, radiological, histological, and surgical findings. Hepatic portoenterostomy was performed according to Kasai technique or one of its variants.

Results: Ninety-one patients from various regions of Argentina and abroad were included in the study. Of these, 55 children underwent surgical treatment (32 at the high-complexity institution and 23 at other centers). Sixty-three patients received liver transplants (31 as a first intervention and 32 after failure of hepatic portoenterostomy). There was a delay in the age of the patients at the time of diagnosis, and overall survival exceeded 80%, in coincidence with other case series.

Conclusions: This is the first study in our country on biliary atresia that includes demographic, clinical-surgical, diagnostic, treatment, and short-term outcome data.