Life long follow up and management strategies of patients living with native livers after Kasai portoenterostomy

Abstract We present a 37 years’ experience in the management of biliary atresia (BA) and discuss long-term complications after Kasai portoenterostomy (KPE). A retrospective territory-wide study from 1980 to 2017 on 231 patients with open KPE from three tertiary paediatric surgical centres was perfor...

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Autores principales:	Patrick Ho Yu Chung, Edwin Kin Wai Chan, Fanny Yeung, Albert Chi Yan Chan, Jennifer Wai Cheung Mou, Kim Hung Lee, Judy Wing Suet Hung, Michael Wai Yip Leung, Paul Kwong Hang Tam, Kenneth Kak Yuen Wong
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2021
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/cd43f546b5924971a20dca65ede4e12b
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spelling	oai:doaj.org-article:cd43f546b5924971a20dca65ede4e12b2021-12-02T15:00:20ZLife long follow up and management strategies of patients living with native livers after Kasai portoenterostomy10.1038/s41598-021-90860-w2045-2322https://doaj.org/article/cd43f546b5924971a20dca65ede4e12b2021-05-01T00:00:00Zhttps://doi.org/10.1038/s41598-021-90860-whttps://doaj.org/toc/2045-2322Abstract We present a 37 years’ experience in the management of biliary atresia (BA) and discuss long-term complications after Kasai portoenterostomy (KPE). A retrospective territory-wide study from 1980 to 2017 on 231 patients with open KPE from three tertiary paediatric surgical centres was performed. Outcome parameters were clearance of jaundice (COJ), native liver survival (NLS) and long-term complications. Factors affecting the operative outcomes were analyzed. The median duration of follow up was 17.5 (IQR: 13.5–22) years. Over 66% of patients became jaundice-freed at 1 year after KPE. Seventy patients (30.3%) received liver transplant (LT) at a median age of 6.2 (IQR: 4.3–8.4) years. The NLS rates at 10 and 20 years were 70.7% and 61.5% respectively with no significant change over the study period. The median age at KPE was 59 (IQR: 49–67) days. KPE performed before 70 days was associated with higher odd ratios for successful drainage but the age of KPE did not have an impact on the long-term NLS. Among all native liver survivors (n = 153), the median bilirubin level was 24 (IQR: 16–36) µmol/L. Portal hypertension (PHT) and recurrent cholangitis were found in 51.6% and 27.5% of them respectively. With a vigilant follow up program, more than 60% of BA patients could remain stable with the disease and achieve long-term survival without LT. Although cholestasis, portal hypertension and recurrent cholangitis are common in long-term NLS, with a comprehensive follow management strategy, they do not always necessitate LT. Our study serves as an example for countries where deceased donor organs are scarce due to very low donation rate.Patrick Ho Yu ChungEdwin Kin Wai ChanFanny YeungAlbert Chi Yan ChanJennifer Wai Cheung MouKim Hung LeeJudy Wing Suet HungMichael Wai Yip LeungPaul Kwong Hang TamKenneth Kak Yuen WongNature PortfolioarticleMedicineRScienceQENScientific Reports, Vol 11, Iss 1, Pp 1-9 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Patrick Ho Yu Chung Edwin Kin Wai Chan Fanny Yeung Albert Chi Yan Chan Jennifer Wai Cheung Mou Kim Hung Lee Judy Wing Suet Hung Michael Wai Yip Leung Paul Kwong Hang Tam Kenneth Kak Yuen Wong Life long follow up and management strategies of patients living with native livers after Kasai portoenterostomy
description	Abstract We present a 37 years’ experience in the management of biliary atresia (BA) and discuss long-term complications after Kasai portoenterostomy (KPE). A retrospective territory-wide study from 1980 to 2017 on 231 patients with open KPE from three tertiary paediatric surgical centres was performed. Outcome parameters were clearance of jaundice (COJ), native liver survival (NLS) and long-term complications. Factors affecting the operative outcomes were analyzed. The median duration of follow up was 17.5 (IQR: 13.5–22) years. Over 66% of patients became jaundice-freed at 1 year after KPE. Seventy patients (30.3%) received liver transplant (LT) at a median age of 6.2 (IQR: 4.3–8.4) years. The NLS rates at 10 and 20 years were 70.7% and 61.5% respectively with no significant change over the study period. The median age at KPE was 59 (IQR: 49–67) days. KPE performed before 70 days was associated with higher odd ratios for successful drainage but the age of KPE did not have an impact on the long-term NLS. Among all native liver survivors (n = 153), the median bilirubin level was 24 (IQR: 16–36) µmol/L. Portal hypertension (PHT) and recurrent cholangitis were found in 51.6% and 27.5% of them respectively. With a vigilant follow up program, more than 60% of BA patients could remain stable with the disease and achieve long-term survival without LT. Although cholestasis, portal hypertension and recurrent cholangitis are common in long-term NLS, with a comprehensive follow management strategy, they do not always necessitate LT. Our study serves as an example for countries where deceased donor organs are scarce due to very low donation rate.
format	article
author	Patrick Ho Yu Chung Edwin Kin Wai Chan Fanny Yeung Albert Chi Yan Chan Jennifer Wai Cheung Mou Kim Hung Lee Judy Wing Suet Hung Michael Wai Yip Leung Paul Kwong Hang Tam Kenneth Kak Yuen Wong
author_facet	Patrick Ho Yu Chung Edwin Kin Wai Chan Fanny Yeung Albert Chi Yan Chan Jennifer Wai Cheung Mou Kim Hung Lee Judy Wing Suet Hung Michael Wai Yip Leung Paul Kwong Hang Tam Kenneth Kak Yuen Wong
author_sort	Patrick Ho Yu Chung
title	Life long follow up and management strategies of patients living with native livers after Kasai portoenterostomy
title_short	Life long follow up and management strategies of patients living with native livers after Kasai portoenterostomy
title_full	Life long follow up and management strategies of patients living with native livers after Kasai portoenterostomy
title_fullStr	Life long follow up and management strategies of patients living with native livers after Kasai portoenterostomy
title_full_unstemmed	Life long follow up and management strategies of patients living with native livers after Kasai portoenterostomy
title_sort	life long follow up and management strategies of patients living with native livers after kasai portoenterostomy
publisher	Nature Portfolio
publishDate	2021
url	https://doaj.org/article/cd43f546b5924971a20dca65ede4e12b
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Life long follow up and management strategies of patients living with native livers after Kasai portoenterostomy

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