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Annals of Diagnostic Paediatric Pathology 2006, 10(1–2):37–42
© Copyright by Polish Paediatric Pathology Society Annals of
Comparison of histological changes in liver biopsy specimens
in patients with biliary atresia of poor and good prognosis
Joanna Cielecka-Kuszyk 1 , Piotr Czubkowski 2 , Ludmi³a Bacewicz 3 ,
Joanna Paw³owska 2 , Irena Jankowska 2 , Tamara Szymañska-Dêbiñska 1
Diagnostic
Paediatric
Pathology
1
Department of Pathology
2
Department of Gastroenterology, Hepatology and Immunology
3
Department of Pediatric Surgery and Organ Transplantation
The Children’s Memorial Health Institute, Warsaw, Poland
Warsaw, Poland
Abstract
In order to compare intrahepatic histological changes in patients with biliary atresia of poor and good
prognosis, we have examined retrospectively 29 liver biopsy specimens taken during hepatoportoenterostomy
modo Kasai. We divided the material into two groups: 12 biopsy specimens were qualified to
the first – unfavorable group with poor prognosis, because patients died or needed liver transplantation
within 2 years after Kasai procedure and 17 biopsy specimens were qualified to the second – favorable
group with good prognosis, because children have survived over 5 years with native liver. Presence of
prominent giant cell transformation, lobular inflammation and features of ductal plate malformation differed
in these two groups. Lobular cholestasis in zone 2 and 3 was of statistical significance in patients with poor
prognosis.
Key words: biliary atresia, hepatoportoenterostomy, ductal plate malformation, giant cells transformation,
lobular inflammation, cholestasis
Introduction
Biliary atresia (BA) is the commonest cause of infantile cholestasis
and leading indication for liver transplantation in children.
[1]. Progressive fibro-inflammatory changes lead to irreversible
cholangiopathy with complete obliteration of extra
and intrahepatic bile ducts, though basic pathomechnisms of
disease remain unclear. BA could also result from a developmental
aberration occurring between 11 and 13 weeks gestation
[13]. Destructive character of BA leads to death in the
first 2 years of life if untreated [4]. The only chance to limit
disease progression is to establish bile flow by Kasai hepatoportoenterostomy
with best results if procedure is performed
before 8 weeks of age. [11]. Introducing liver transplantation
(LTx) in children opened new era in outcome of BA treatment,
reaching overall 5 year survival in almost 80% of patients.
LTx timing is an important factor influencing outcome
so it is crucial to distinguish early prognostic factors. Characteristic
histopathological changes in liver include cholestasis,
inflammation, fibrosis and ductular proliferation. Prognostic
value of liver histopathology is believed to be crucial but so
far no firm criteria were established [4].
The present study is based on a histological analysis
of the intrahepatic histopathological changes in patients with
poor and good prognosis of the disease. The aim of the study
was to compare the stage of liver fibrosis, inflammation,
ductular proliferation, giant cell transformation and cholestasis
in surgical specimens taken during hepatoportoenterostomy
modo Kasai from patients who had good and poor prognosis.
Address for correspondence
Jaonna Cielecka-Kuszyk Phone: +48 22 815 19 60
Department of Pathology
E-mail: j.kuszyk@czd.pl
The Children’s Memorial Health Institute
Al. Dzieci Polskich 20
04-730 Warsaw, Poland