Clearance of jaundice after the modified Kasai`s operation predicts survival outcomes in patients with biliary atresia

The aim of this study was to assess the probability of survival with native liver (SNL) and the rate of esophageal variceal bleeding (EVB) as well as their potential risk factors, in patients diagnosed with Biliary Atresia (BA), who underwent the hepaticoportoenterostomy (HPE) by retrospectively re...

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Main Authors: Jinchanya Tamgal, Alisara Damrongmanee, Jiraporn Khorana, Kanokkan Tepmalai, Nuthapong Ukarapol
Format: Article
Language:English
Published: Hacettepe University Institute of Child Health 2019-02-01
Series:The Turkish Journal of Pediatrics
Subjects:
Online Access:https://turkjpediatr.org/article/view/644
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author Jinchanya Tamgal
Alisara Damrongmanee
Jiraporn Khorana
Kanokkan Tepmalai
Nuthapong Ukarapol
author_facet Jinchanya Tamgal
Alisara Damrongmanee
Jiraporn Khorana
Kanokkan Tepmalai
Nuthapong Ukarapol
author_sort Jinchanya Tamgal
collection DOAJ
description The aim of this study was to assess the probability of survival with native liver (SNL) and the rate of esophageal variceal bleeding (EVB) as well as their potential risk factors, in patients diagnosed with Biliary Atresia (BA), who underwent the hepaticoportoenterostomy (HPE) by retrospectively reviewing medical records between 2007 and 2016. The subjects were classified as poor outcomes if they died or a liver transplant (LT) was performed. A total of 73 cases were enrolled. The average age at HPE was 106.2 +/- 58.5 days. Poor outcome was observed in 27.4%, 54.8% survived with native liver and 17.8% were lost to follow-up. The principal cause of death was sepsis, followed by massive upper GI hemorrhage. The overall 10-year SNL was 66.8%. Only total bilirubin (TB) > 3 mg/dL at 3, 6 months after HPE and presence of associated anomalies negatively affected SNL (p=0.0155, 0.0042 and 0.001, respectively). Most of the patients experienced EVB within 3 years of age, in which TB > 9 mg/dL at 12 months after HPE was significantly associated with probability of the EVB outcome. Any interventions to improve jaundice clearance after HPE should be strongly pursued in order to improve outcomes in BA patients, particularly in centers where liver transplantation (LT) is not available. Surveillance esophagogastroduodenoscopy around the age of 1.5 years in patients having TB > 9 mg/dL may be beneficial to identify large varices having potential fatal bleeding.
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spelling doaj-art-bbf4098e1bd24206a8b6c99dfe032d472025-08-20T02:01:47ZengHacettepe University Institute of Child HealthThe Turkish Journal of Pediatrics0041-43012791-64212019-02-0161110.24953/turkjped.2019.01.002Clearance of jaundice after the modified Kasai`s operation predicts survival outcomes in patients with biliary atresiaJinchanya Tamgal0Alisara Damrongmanee1Jiraporn Khorana2Kanokkan Tepmalai3Nuthapong Ukarapol4Departments of Pediatrics, Chiang Mai University, Faculty of Medicine, Thailand.Departments of Pediatrics, Chiang Mai University, Faculty of Medicine, Thailand.Departments of Surgery, Chiang Mai University, Faculty of Medicine, Thailand.Departments of Surgery, Chiang Mai University, Faculty of Medicine, Thailand.Departments of Pediatrics, Chiang Mai University, Faculty of Medicine, Thailand. The aim of this study was to assess the probability of survival with native liver (SNL) and the rate of esophageal variceal bleeding (EVB) as well as their potential risk factors, in patients diagnosed with Biliary Atresia (BA), who underwent the hepaticoportoenterostomy (HPE) by retrospectively reviewing medical records between 2007 and 2016. The subjects were classified as poor outcomes if they died or a liver transplant (LT) was performed. A total of 73 cases were enrolled. The average age at HPE was 106.2 +/- 58.5 days. Poor outcome was observed in 27.4%, 54.8% survived with native liver and 17.8% were lost to follow-up. The principal cause of death was sepsis, followed by massive upper GI hemorrhage. The overall 10-year SNL was 66.8%. Only total bilirubin (TB) > 3 mg/dL at 3, 6 months after HPE and presence of associated anomalies negatively affected SNL (p=0.0155, 0.0042 and 0.001, respectively). Most of the patients experienced EVB within 3 years of age, in which TB > 9 mg/dL at 12 months after HPE was significantly associated with probability of the EVB outcome. Any interventions to improve jaundice clearance after HPE should be strongly pursued in order to improve outcomes in BA patients, particularly in centers where liver transplantation (LT) is not available. Surveillance esophagogastroduodenoscopy around the age of 1.5 years in patients having TB > 9 mg/dL may be beneficial to identify large varices having potential fatal bleeding. https://turkjpediatr.org/article/view/644biliary atresiaesophageal variceal bleedingprognosissurvival
spellingShingle Jinchanya Tamgal
Alisara Damrongmanee
Jiraporn Khorana
Kanokkan Tepmalai
Nuthapong Ukarapol
Clearance of jaundice after the modified Kasai`s operation predicts survival outcomes in patients with biliary atresia
The Turkish Journal of Pediatrics
biliary atresia
esophageal variceal bleeding
prognosis
survival
title Clearance of jaundice after the modified Kasai`s operation predicts survival outcomes in patients with biliary atresia
title_full Clearance of jaundice after the modified Kasai`s operation predicts survival outcomes in patients with biliary atresia
title_fullStr Clearance of jaundice after the modified Kasai`s operation predicts survival outcomes in patients with biliary atresia
title_full_unstemmed Clearance of jaundice after the modified Kasai`s operation predicts survival outcomes in patients with biliary atresia
title_short Clearance of jaundice after the modified Kasai`s operation predicts survival outcomes in patients with biliary atresia
title_sort clearance of jaundice after the modified kasai s operation predicts survival outcomes in patients with biliary atresia
topic biliary atresia
esophageal variceal bleeding
prognosis
survival
url https://turkjpediatr.org/article/view/644
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AT alisaradamrongmanee clearanceofjaundiceafterthemodifiedkasaisoperationpredictssurvivaloutcomesinpatientswithbiliaryatresia
AT jirapornkhorana clearanceofjaundiceafterthemodifiedkasaisoperationpredictssurvivaloutcomesinpatientswithbiliaryatresia
AT kanokkantepmalai clearanceofjaundiceafterthemodifiedkasaisoperationpredictssurvivaloutcomesinpatientswithbiliaryatresia
AT nuthapongukarapol clearanceofjaundiceafterthemodifiedkasaisoperationpredictssurvivaloutcomesinpatientswithbiliaryatresia