Pediatric Surgery International

, Volume 34, Issue 4, pp 381–386 | Cite as

Clinical and pathological features of patients with biliary atresia who survived for more than 5 years with native liver

  • Song Sun
  • Shan Zheng
  • Xuexin Lu
  • Gong Chen
  • Yangyang Ma
  • Lian Chen
  • Kuiran Dong
Original Article



The objective of this study was to determine the predictive index for prognosis in patients with biliary atresia (BA).


A total of 71 patients were divided into two groups. Group A included 39 postoperative BA patients who survived for more than 5 years with normal liver function and did not present cirrhosis, and group B included 32 patients who died from liver failure within 1 year after surgery. The clinical data of the two study groups were compared, and liver pathology was evaluated using a scoring system.


The average age and weight were similar in the two groups (64.1 ± 16.8 days vs. 60.7 ± 19.3 days, p > 0.05; 4.9 ± 0.9 kg vs. 4.7 ± 0.8 kg, p > 0.05). There were no significant intergroup differences in preoperative total bilirubin (TB), direct bilirubin (DB), alanine transaminase, aspartate transaminase, and international normalized ratio. The preoperative levels of gamma-glutamyl transpeptidase (γ-GT) and albumin in group A were significantly higher than those in group B (γ-GT: 956.8 ± 503.8 IU/L vs. 620.2 ± 437.1 IU/L, p = 0.00; ALB: 40.8 ± 2.5 g/L vs. 36.8 ± 3.6 g/L, p = 0.04), whereas alkaline phosphatase was significantly lower in group A compared to group B (512.2 ± 224.6 IU/L vs. 631.7 ± 254.7 IU/L, p = 0.02). The postoperative TB and DB after 2 weeks of the Kasai procedure decreased significantly more in group A than in group B (TB: 53.9 vs. 21.4%, p = 0.00; DB: 51.0 vs. 22.7%, p = 0.00), whereas γ-GT increased significantly less in group A than in group B (48.3 vs. 142.1%, p = 0.00). Cystic structures were observed at the porta hepatis on ultrasound in more patients from group A (28.2 vs. 3.2%, p < 0.00). There was no significant difference in the total pathological score between the two groups (p = 0.38) whereas the score of bile plugs was significantly higher in group A (0.95 vs. 0.38, p = 0.03).


The cystic structures observed at the porta hepatis on ultrasound preoperatively and the rapid decrease in TB and DB within 2 weeks postoperatively predict good long-term prognosis, whereas a significant increase in γ-GT with a lower preoperative level predicts poor long-term prognosis. The development of bile plugs may be an indicator of favorable prognosis.


Biliary atresia Prognosis Pathology Liver function Ultrasound 


Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Research involving human and animal participants

This article does not contain any studies with human participants or animals performed by any of the authors.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Song Sun
    • 1
  • Shan Zheng
    • 1
  • Xuexin Lu
    • 1
  • Gong Chen
    • 1
  • Yangyang Ma
    • 2
  • Lian Chen
    • 2
  • Kuiran Dong
    • 1
  1. 1.Surgical DepartmentChildren’s Hospital of Fudan UniversityShanghaiChina
  2. 2.Pathology DepartmentChildren’s Hospital of Fudan UniversityShanghaiChina

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