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 < 17
mol/l, the growth parameters are satisfactory. Now atthe age of 2 - 49 months (mean age 29.4 months) one of them is treated for epilepsy. Two children are at presentindicated for transplantation of the liver on account of advanced hepatic failure (age 24, 26months). Another fourchildren have serious problems (cholestasis, cholangitis, ascites, not thriving) - at present they do not meetindication criteris for transplantation of the liver. So far 7/24 (29.2%) children age 5.5 - 18 months died (mean age13 months), two patients died after transplantation of the liver performed abroad.Conclusion: The incidence of BA in the CR is among the lowest incidences of the disease reported in Europeand the USA. BA has an adverse prognosis and is in childhood the most frequent indication for transplantation ofthe liver and only 37.5% children of the authors’ group will not require this therapeutic procedure in the nearfuture. In the CR it is necessary to promote knowledge, in particular of practical paediatricians on this disease toachieve early diagnosis in infants with suspicion of BA and infants with conjugated hyperbilirubinaemia. Thesechildren should be referred to the mentioned departments in the Motol hospital already during the first 4 weeksof life.

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