Outcomes of biliary atresia in the Nordic countries - a multicenter study of 158 patients during 2005-2016

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http://hdl.handle.net/10138/304168

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Nordic Pediat Surg Study Consortiu 2018 , ' Outcomes of biliary atresia in the Nordic countries - a multicenter study of 158 patients during 2005-2016 ' , Journal of Pediatric Surgery , vol. 53 , no. 8 , pp. 1509-1515 . https://doi.org/10.1016/j.jpedsurg.2017.08.048

Title: Outcomes of biliary atresia in the Nordic countries - a multicenter study of 158 patients during 2005-2016
Author: Nordic Pediat Surg Study Consortiu
Date: 2018-08
Language: eng
Number of pages: 7
Belongs to series: Journal of Pediatric Surgery
ISSN: 0022-3468
URI: http://hdl.handle.net/10138/304168
Abstract: Background/purpose: Biliary atresia is the most common reason for newborn cholestasis and pediatric liver transplantation. Even after normalization of serum bilirubin after portoenterostomy, most patients require liver transplantation by adulthood due to expanding fibrosis. We addressed contemporary outcomes of biliary atresia in the Nordic countries. Methods: Data on center and patients characteristics, diagnostic practices, surgical treatment, adjuvant medical therapy after portoenterostomy, follow-up and outcomes were collected from all the Nordic centers involved with biliary atresia care during 2005-2016. Results: Of the 154 patients, 148 underwent portoenterostomy mostly by assigned surgical teams at median age of 64 (interquartile range 37-79) days, and 95 patients (64%) normalized their serum bilirubin concentration while living with native liver. Postoperative adjuvant medical therapy, including steroids, ursodeoxycholic acid and antibiotics was given to 137 (93%) patients. Clearance of jaundice associated with young age at surgery and favorable anatomic type of biliary atresia, whereas annual center caseload >3 patients and diagnostic protocol without routine liver biopsy predicted early performance of portoenterostomy. The cumulative 5-year native liver and overall survival estimate was 53% (95% CI 45-62) and 88% (95% CI 83-94), respectively. Portoenterostomy age 3 patients were predictive for long-term native liver survival, while normalization of serum bilirubin after portoenterostomy was the major predictor of both native liver and overall 5-year survival. Conclusions: The outcomes of biliary atresia in the Nordic countries compared well with previous European studies. Further improvement should be pursued by active measures to reduce patient age at portoenterostomy. Retrospective prognosis study: Level II. (C) 2017 Elsevier Inc. All rights reserved.
Subject: Clearance of jaundice
Liver transplantation
Portoenterostomy
Steroids
Survival
Ursodeoxycholic acid
TRANSPLANT-FREE SURVIVAL
KASAI PORTOENTEROSTOMY
LIVER-TRANSPLANTATION
URSODEOXYCHOLIC ACID
SINGLE-CENTER
NATIVE LIVER
LONG-TERM
INFANTS
CHILDREN
HEPATOPORTOENTEROSTOMY
3123 Gynaecology and paediatrics
3126 Surgery, anesthesiology, intensive care, radiology
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