Biliary Atresia -⁠ Incidence and Therapeutic Results in Czech Republic

Overview

Introduction:
Biliary atresia (BA) is the result of a destructive idiopathic inflammatory process which affectsintra -⁠ and extrahepatic biliary pathways. It leads to their obliteration, to the development of fibrosis or even biliarycirrhosis. The incidence of the disease is 1 : 10 -⁠ 15 000 liveborn neonates. In ca 10 -⁠ 20% it is associated with furtherextrahepatic anomalies.Objective: The investigation is focused on the epidemiology and subsequent course of the disease in childrenwith BA born during the last 4.5 years in the CR (from Jan. 1 1998 -⁠ June 30 2002).Patients: The First Paediatric Clinic Charles University, Second Medical Faculty and Faculty Hospital Motoladmitted during that period a total of 24 children with BA (16 girls and 8 boys). In all patients at the Clinic ofPaediatric Surgery in the above department portoenteroanastomosis according to Kasai was performed at the ageof 4 -⁠ 15 weeks (mean age 8.5 weeks), in 13/24 (54%) before the age of 8 weeks. Other anomalies were in 4/24(16.6%) of the patients (polysplenism, situs viscerum inversus, malrotation and others). In 3/24 (12.5%) concurrentlyCMV infection was detected.Results: In the Czech Republic at present some 5 -⁠ 6 children with biliary atresia are born every year. At thepresent birth rate an incidence is of 1 : 15 652 liveborn infants. By June 30, 2002 17/24 children survive (70.8%). Of these 17 two are after a successful transplantation of the liver (performed at the ages of 7 and 11 months resp.).Nine children are in a stabilized condition (bilirubin

Key words:
incidence of biliary atresia, cholestatic jaundice, portoenteroanastomosis, transplantation of theliver