Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2022 Dec 1;39(1):24.
doi: 10.1007/s00383-022-05313-w.

Native liver survivors of portoenterostomy for biliary atresia with excellent outcome: redefining "successful" portoenterostomy

Koichi Tsuboi  1 , Hiroko Watayo  2 , Takafumi Tsukui  2 , Kazuto Suda  2 , Eri Abe  3 , Takamori Fujimoto  4 , Takanori Ochi  2 , Geoffrey J Lane  2 , Hiroyuki Koga  2 , Atsuyuki Yamataka  2
Affiliations

Native liver survivors of portoenterostomy for biliary atresia with excellent outcome: redefining "successful" portoenterostomy

Koichi Tsuboi et al. Pediatr Surg Int. .

Abstract

Purpose: Native liver survivors (NLS) after portoenterostomy (PE) for biliary atresia (BA) with normal biomarkers defined as total bilirubin (T-Bil), aspartate aminotransferase (AST)/alanine aminotransferase (ALT) for liver function (LF), cholinesterase (ChE), platelet count (PC), and absence of portal hypertension (PHT) were reviewed to redefine "successful" PE.

Methods: 92 post-PE BA patients were classified as NLS-1: normal biomarkers, PHT (-); NLS-2: at least one abnormal biomarker, PHT (-); NLS-3: normal biomarkers, PHT ( +); NLS-4: abnormal biomarkers, PHT ( +) and reviewed for a maximum 32 years.

Results: As of June 2022, 55/92 (59.8%) had received liver transplants and 37/92 (40.2%) were NLS. NLS patients were classified as excellent outcome (EO): NLS-1 (n = 10; 27.0%) or non-EO: NLS-2: (n = 8; 21.6%), NLS-3: (n = 6; 16.2%), and NLS-4: (n = 13; 35.1%). Compared with non-EO, EO had PE earlier (50.5 versus 65 days; not significant; p = 0.08), significantly earlier onset of symptoms (13 days versus 32 days; p = 0.01) and significantly shorter jaundice-clearance (JC; 34.5 days versus 56.0 days; p < 0.001). Durations of follow-up were similar: 13 years in EO, 18.5 years in NLS-2, 20 years in NLS-3, and 15 years in NLS-4.

Conclusion: Incidence of "successful" PE or EO is low and correlated with early onset of symptoms and quicker JC.

Keywords: Biliary atresia; Classification; Excellent outcome; Liver transplantation; Native liver; Portal hypertension; Portoenterostomy; Survival.

PubMed Disclaimer

References

    1. Nio M (2017) Japanese biliary atresia registry. Pediatr Surg Int 33(12):1319–1325. https://doi.org/10.1007/s00383-017-4160-x - DOI - PubMed
    1. Hopkins PC, Yazigi N, Nylund CM (2017) Incidence of biliary atresia and timing of hepatoportoenterostomy in the United States. J Pediatr 187:253–257. https://doi.org/10.1016/j.jpeds.2017.05.006 - DOI - PubMed
    1. Kasai M (1959) A new operation for non-correctable biliary atresia: hepatic portoenterostomy. Shujutsu 13:733
    1. Kasai M, Kimura S, Asakura Y, Suzuki H, Taira Y et al (1968) Surgical treatment of biliary atresia. J Pediatr Surg 3(6):665–675. https://doi.org/10.1016/0022-3468(68)90897-x - DOI
    1. Sharma S, Gupta DK (2017) Surgical modifications, additions, and alternatives to Kasai hepato-portoenterostomy to improve the outcome in biliary atresia. Pediatr Surg Int 33(12):1275–1282. https://doi.org/10.1007/s00383-017-4162-8 - DOI - PubMed

Substances

LinkOut - more resources