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

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..

Medienart:

E-Artikel

Erscheinungsjahr:

2022

Erschienen:

2022

Enthalten in:

Zur Gesamtaufnahme - volume:39

Enthalten in:

Pediatric surgery international - 39(2022), 1 vom: 01. Dez.

Sprache:

Englisch

Beteiligte Personen:

Tsuboi, Koichi [VerfasserIn]
Watayo, Hiroko [VerfasserIn]
Tsukui, Takafumi [VerfasserIn]
Suda, Kazuto [VerfasserIn]
Abe, Eri [VerfasserIn]
Fujimoto, Takamori [VerfasserIn]
Ochi, Takanori [VerfasserIn]
Lane, Geoffrey J. [VerfasserIn]
Koga, Hiroyuki [VerfasserIn]
Yamataka, Atsuyuki [VerfasserIn]

Links:

Volltext [lizenzpflichtig]

Themen:

Biliary atresia
Classification
Excellent outcome
Liver transplantation
Native liver
Portal hypertension
Portoenterostomy
Survival

RVK:

RVK Klassifikation

Anmerkungen:

© The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature 2022. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

doi:

10.1007/s00383-022-05313-w

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

OLC2132932026

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