Literature DB >> 29427257

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

Song Sun1, Shan Zheng2, Xuexin Lu1, Gong Chen1, Yangyang Ma3, Lian Chen3, Kuiran Dong1.   

Abstract

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

Entities:  

Keywords:  Biliary atresia; Liver function; Pathology; Prognosis; Ultrasound

Mesh:

Year:  2018        PMID: 29427257     DOI: 10.1007/s00383-018-4231-7

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


  34 in total

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4.  The most reliable early predictors of outcome in patients with biliary atresia after Kasai's operation.

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

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6.  Hepatic arterial diameter measured with US: adjunct for US diagnosis of biliary atresia.

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7.  Impact of age at Kasai operation on its results in late childhood and adolescence: a rational basis for biliary atresia screening.

Authors:  Marie-Odile Serinet; Barbara E Wildhaber; Pierre Broué; Alain Lachaux; Jacques Sarles; Emmanuel Jacquemin; Frédéric Gauthier; Christophe Chardot
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Review 8.  Screening and outcomes in biliary atresia: summary of a National Institutes of Health workshop.

Authors:  Ronald J Sokol; Ross W Shepherd; Riccardo Superina; Jorge A Bezerra; Patricia Robuck; Jay H Hoofnagle
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Review 9.  International incidence and outcomes of biliary atresia.

Authors:  Carolina Jimenez-Rivera; Kheira S Jolin-Dahel; Kyle J Fortinsky; Peter Gozdyra; Eric I Benchimol
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10.  Correlation of pre- and post-operative liver function, duct diameter at porta hepatis, and portal fibrosis with surgical outcomes in biliary atresia.

Authors:  Rajib Ray Baruah; Veereshwar Bhatnagar; Sandeep Agarwala; Siddhartha Datta Gupta
Journal:  J Indian Assoc Pediatr Surg       Date:  2015 Oct-Dec
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2.  A Novel Model for Predicting the Clearance of Jaundice in Patients With Biliary Atresia After Kasai Procedure.

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3.  Low gamma-glutamyl transpeptidase levels at presentation are associated with severity of liver illness and poor outcome in biliary atresia.

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