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Comparison of the outcomes of biliary atresia with cystic degeneration and isolated biliary atresia: A matched-pair analysis

Authors
 Kyong Ihn  ;  In Geol Ho  ;  Ji Hee Lee  ;  Younghyun Na  ;  Dongeun Lee  ;  Seok Joo Han 
Citation
 JOURNAL OF PEDIATRIC SURGERY, Vol.50(10) : 2177-2182, 2020-10 
Journal Title
 JOURNAL OF PEDIATRIC SURGERY 
ISSN
 0022-3468 
Issue Date
2020-10
Keywords
Biliary atresia ; Cystic degeneration ; Kasai portoenterostomy ; Liver fibrosis ; Matching ; Prognosis
Abstract
Background: Patients with biliary atresia (BA) with extrahepatic cystic degeneration (BACD) have a unique pathophysiology; however, clinical outcomes and progression of perinatal degeneration are not well-defined. We aimed to investigate the differences in clinical characteristics and outcomes between BACD and isolated BA (IBA). Methods: We performed a retrospective analysis of patients with BA who underwent Kasai portoenterostomy (KPE) from August 1997 to January 2018 and compared the clinical features and outcomes between BACD (n = 21) and IBA (n = 237). Matched-pair analysis for age and sex was performed between BACD and IBA groups to reduce confounding. Results: Before matched-pair analysis, we found that BACD patients were younger at KPE (45 vs. 64 days, p = 0.008), showed lower total bilirubin at the 3-month follow-up (0.5 vs. 1.4 mg/dL, p = 0.002), and higher 5-year native liver survival rate (95.2% vs. 61.4%, p = 0.006) than IBA patients. After matching, the BACD group showed significantly lower total bilirubin levels at the 3-month follow-up (0.5 vs. 1.5 mg/dL, p = 0.036) and higher 5-year native liver survival rate (95.2% vs. 57.5%, p = 0.006) than the IBA group. Conclusion: BACD demonstrated higher bilirubin clearance and native liver survival rates than IBA. Levels of evidence: Treatment Study, Level III.
Full Text
https://www.sciencedirect.com/science/article/pii/S0022346820301858
DOI
10.1016/j.jpedsurg.2020.02.054
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Surgery (외과학교실) > 1. Journal Papers
Yonsei Authors
Ihn, Kyong(인경) ORCID logo https://orcid.org/0000-0002-6161-0078
Han, Seok Joo(한석주) ORCID logo https://orcid.org/0000-0001-5224-1437
Ho, In Geol(호인걸)
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/180497
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