Peri-Operative Liver Fibrosis and Native Liver Survival in Pediatric Patients with Biliary Atresia: A Systematic Review and Meta-Analysis

Ashkan Jahangirnia; Irina Oltean; Youssef Nasr; Nayaar Islam; Arielle Weir; Joseph de Nanassy; Ahmed Nasr; Dina El Demellawy

doi:10.5223/pghn.2022.25.5.353

Peri-Operative Liver Fibrosis and Native Liver Survival in Pediatric Patients with Biliary Atresia: A Systematic Review and Meta-Analysis

Pediatr Gastroenterol Hepatol Nutr. 2022 Sep;25(5):353-375. doi: 10.5223/pghn.2022.25.5.353. Epub 2022 Sep 5.

Authors

Ashkan Jahangirnia¹, Irina Oltean^{2

3

4}, Youssef Nasr³, Nayaar Islam³, Arielle Weir², Joseph de Nanassy^{1

3}, Ahmed Nasr^{1

2

4}, Dina El Demellawy^{1

2

3}

Affiliations

¹ Faculty of Medicine, University of Ottawa, Ottawa, ON, Canada.
² Clinical Research Unit, Children's Hospital of Eastern Ontario, Ottawa, ON, Canada.
³ Department of Pathology, Children's Hospital of Eastern Ontario, Ottawa, ON, Canada.
⁴ Department of Surgery, Children's Hospital of Eastern Ontario, Ottawa, ON, Canada.

Abstract

No systematic review to date has examined histopathological parameters in relation to native liver survival in children who undergo the Kasai operation for biliary atresia (BA). A systematic review and meta-analysis is presented, comparing the frequency of native liver survival in peri-operative severe vs. non-severe liver fibrosis cases, in addition to other reported histopathology parameters. Records were sourced from MEDLINE, Embase, and CENTRAL databases. Studies followed the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines and compared native liver survival frequencies in pediatric patients with evidence of severe vs. non-severe liver fibrosis, bile duct proliferation, cholestasis, lobular inflammation, portal inflammation, and giant cell transformation on peri-operative biopsies. The primary outcome was the frequency of native liver survival. A random effects meta-analysis was used. Twenty-eight observational studies were included, 1,171 pediatric patients with BA of whom 631 survived with their native liver. Lower odds of native liver survival in the severe liver fibrosis vs. non-severe liver fibrosis groups were reported (odds ratio [OR], 0.16; 95% confidence interval [CI], 0.08-0.33; I² =46%). No difference in the odds of native liver survival in the severe bile duct destruction vs. non-severe bile duct destruction groups were reported (OR, 0.17; 95% CI, 0.00-63.63; I² =96%). Lower odds of native liver survival were documented in the severe cholestasis vs. non-severe cholestasis (OR, 0.10; 95% CI, 0.01-0.73; I² =80%) and severe lobular inflammation vs. non-severe lobular inflammation groups (OR, 0.02; 95% CI, 0.00-0.62; I² =69%). There was no difference in the odds of native liver survival in the severe portal inflammation vs. non-severe portal inflammation groups (OR, 0.03; 95% CI, 0.00-3.22; I² =86%) or between the severe giant cell transformation vs. non-severe giant cell transformation groups (OR, 0.15; 95% CI, 0.00-175.21; I² =94%). The meta-analysis loosely suggests that the presence of severe liver fibrosis, cholestasis, and lobular inflammation are associated with lower odds of native liver survival in pediatric patients after Kasai.

Keywords: Biliary atresia; Cholestasis; Inflammation; Liver fibrosis; Liver transplantation; Native liver; Pathology; Pediatric patients.

Publication types

Review