Biliary Atresia - Incidence and Therapeutic Results in Czech Republic
Kotalová R.1, Bláhová K.1, Janda J.1, Nevoral J.1, Rygl M.2, Kalousová J.2,Šnajdauf J.2, Ryska M.3
I. dětská klinika 2. LF UK a FN Motol, Praha1 přednosta doc. MUDr. J. Janda, CSc.Klinika dětské chirurgie 2. LF UK a FN Motol, Praha2přednosta prof. MUDr. J. Šnajdauf, DrSc.Klinika transplantační chirurgie IKEM, Praha3přednosta doc. MUDr. M. Ryska, CSc. |
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Summary:
Introduction: Biliary atresia (BA) is the result of a destructive idiopathic inflammatory process which affects
intra- and extrahepatic biliary pathways. It leads to their obliteration, to the development of fibrosis or even biliary
cirrhosis. The incidence of the disease is 1 : 10 - 15 000 liveborn neonates. In ca 10 - 20% it is associated with further
extrahepatic anomalies.
Objective: The investigation is focused on the epidemiology and subsequent course of the disease in children
with 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 Motol
admitted during that period a total of 24 children with BA (16 girls and 8 boys). In all patients at the Clinic of
Paediatric Surgery in the above department portoenteroanastomosis according to Kasai was performed at the age
of 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%) concurrently
CMV infection was detected.
Results: In the Czech Republic at present some 5 - 6 children with biliary atresia are born every year. At the
present 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 at
the age of 2 - 49 months (mean age 29.4 months) one of them is treated for epilepsy. Two children are at present
indicated for transplantation of the liver on account of advanced hepatic failure (age 24, 26months). Another four
children have serious problems (cholestasis, cholangitis, ascites, not thriving) - at present they do not meet
indication criteris for transplantation of the liver. So far 7/24 (29.2%) children age 5.5 - 18 months died (mean age
13 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 Europe
and the USA. BA has an adverse prognosis and is in childhood the most frequent indication for transplantation of
the liver and only 37.5% children of the authors’ group will not require this therapeutic procedure in the near
future. In the CR it is necessary to promote knowledge, in particular of practical paediatricians on this disease to
achieve early diagnosis in infants with suspicion of BA and infants with conjugated hyperbilirubinaemia. These
children should be referred to the mentioned departments in the Motol hospital already during the first 4 weeks
of life.
Key words:
incidence of biliary atresia, cholestatic jaundice, portoenteroanastomosis, transplantation of the
liver
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