Literature DB >> 34618182

The favorable prognosis of cystic biliary atresia may be related to early surgery and mild liver pathological changes.

Zheng Qipeng1,2, Yang Fang1,2, Zhao Yilin1,2, Liu Gengxin1,2, Li Mengdi1,2, Hu Xiaoli3, Zhan Jianghua4.   

Abstract

OBJECTIVE: The objectives of this study is to compare the prognostic differences between cystic biliary atresia (CBA) and non-CBA, analyze the clinical and liver pathological differences between the two groups, and explore the possible factors that affect the native liver survival of infants with CBA after Kasai portoenterostomy (KPE).
METHODS: From 2013 to 2020, 131 infants with BA were admitted to Tianjin Children's Hospital. A total of 108 infants with BA were included after excluding those who did not undergo surgery after diagnosis (n = 23), including 12 cases of CBA and 96 cases of non-CBA. The clinical data, native liver survival and liver pathology, including liver fibrosis, bile ductular proliferation (BDP), bile plug, and portal area inflammation infiltration of the two study groups were compared.
RESULTS: CBA accounts for 9.16% (12/131) and type I CBA accounts for 6.87% (9/131) of all types of BA. 16.7% (2/12) of CBA were detected prenatally with diagnosis of choledochal cyst (CC). The age at KPE, total bilirubin, direct bilirubin, and total bile acid levels of CBA were significantly lower than those of non-CBA (P = 0.047, P = 0.013, P = 0.009, P = 0.010, respectively). The long and wide diameters of the gallbladder were significantly larger than those of non-CBA (both P < 0.001). The 1-, 3-, and 5-year survival rates of CBA were 83.3%, 71.4%, and 71.4%, respectively, and 56.5%, 32.5%, and 29.8%, respectively, in non-CBA. The difference between the two groups was statistically significant (P = 0.031). The degree of liver fibrosis and bile plug in non-CBA was higher than that of CBA (P = 0.004, P < 0.001, respectively). There was no difference of BDP and inflammation infiltration between the two groups (P = 0.285, P = 0.243, respectively).
CONCLUSION: CBA is a distinct type different from non-CBA, with different pathological processes, pathological manifestations, and clinical prognosis. The favorable prognosis of CBA may be derived from the early diagnosis, early operation, and mild pathological changes.
© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.

Entities:  

Keywords:  Biliary atresia; Cyst; Hepatic fibrosis; Native liver survival

Mesh:

Year:  2021        PMID: 34618182     DOI: 10.1007/s00383-021-05030-w

Source DB:  PubMed          Journal:  Pediatr Surg Int        ISSN: 0179-0358            Impact factor:   1.827


  27 in total

1.  Objective differential characteristics of cystic biliary atresia and choledochal cysts in neonates and young infants: sonographic findings.

Authors:  Lu-Yao Zhou; Bu-Yun Guan; Le Li; Zuo-Feng Xu; Chang-Ping Dai; Wei Wang; Hui-Min Xia; Xiao-Yan Xie
Journal:  J Ultrasound Med       Date:  2012-06       Impact factor: 2.153

2.  An algorithm for the grading of activity in chronic hepatitis C. The METAVIR Cooperative Study Group.

Authors:  P Bedossa; T Poynard
Journal:  Hepatology       Date:  1996-08       Impact factor: 17.425

Review 3.  Biliary atresia with choledochal cyst: implications for classification.

Authors:  Aleixo M Muise; Dan Turner; Eytan Wine; Peter Kim; Margaret Marcon; Simon C Ling
Journal:  Clin Gastroenterol Hepatol       Date:  2006-09-18       Impact factor: 11.382

4.  Morphologic studies of the liver and bile ducts in biliary atresia.

Authors:  R Ohi; T Chiba; N Endo
Journal:  Acta Paediatr Jpn       Date:  1987-08

5.  Usefulness of a scoring system in the interpretation of histology in neonatal cholestasis.

Authors:  Way Seah Lee; Lai Meng Looi
Journal:  World J Gastroenterol       Date:  2009-11-14       Impact factor: 5.742

6.  Practical approach to imaging diagnosis of biliary atresia, Part 1: prenatal ultrasound and magnetic resonance imaging, and postnatal ultrasound.

Authors:  Marcello Napolitano; Stéphanie Franchi-Abella; Maria Beatrice Damasio; Thomas A Augdal; Fred Efraim Avni; Costanza Bruno; Kassa Darge; Damjana Ključevšek; Annemieke S Littooij; Luisa Lobo; Hans-Joachim Mentzel; Michael Riccabona; Samuel Stafrace; Seema Toso; Magdalena Maria Woźniak; Gianni Di Leo; Francesco Sardanelli; Lil-Sofie Ording Müller; Philippe Petit
Journal:  Pediatr Radiol       Date:  2020-11-17

7.  A pathological scoring system in the diagnosis and judgment of prognosis of biliary atresia.

Authors:  Gong Chen; Ping Xue; Shan Zheng; Lian Chen; Yangyang Ma
Journal:  J Pediatr Surg       Date:  2015-08-28       Impact factor: 2.545

8.  Clinical and pathological characteristics of cystic lesions of extrahepatic bile duct in neonates.

Authors:  F Jiexiong; L Minju; T Hongfeng; G Weizhong; Y Shaoyong
Journal:  Acta Paediatr       Date:  2003-10       Impact factor: 2.299

9.  Temporal histopathological changes in biliary atresia: A perspective for rapid fibrosis progression.

Authors:  Hanaa A El-Araby; Magdy A Saber; Noha M Radwan; Doha M Taie; Nermin M Adawy; Ahmad M Sira
Journal:  Ann Hepatol       Date:  2020-09-29       Impact factor: 2.400

10.  Cystic biliary atresia: an etiologic and prognostic subgroup.

Authors:  Enrica Caponcelli; Alex S Knisely; Mark Davenport
Journal:  J Pediatr Surg       Date:  2008-09       Impact factor: 2.545

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