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

Pakarinen, Mikko P; Johansen, Lars Søndergaard; Svensson, Jan F.; Bjørnland, Kristin; Gatzinsky, Vladimir; Stenström, Pernilla LU ; Koivusalo, Antti; Kvist, Nina; Almström, Markus and Emblem, Ragnhild, et al. (2017) In Journal of Pediatric Surgery
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... (More)

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 <. 65. days and annual center caseload >. 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.

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Clearance of jaundice, Liver transplantation, Portoenterostomy, Steroids, Survival, Ursodeoxycholic acid
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Journal of Pediatric Surgery
publisher
Elsevier
external identifiers
  • scopus:85029738879
ISSN
0022-3468
DOI
10.1016/j.jpedsurg.2017.08.048
language
English
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yes
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0dec242d-3956-413d-b3e1-f48074073d70
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2017-10-10 11:30:14
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2018-05-06 04:37:25
@article{0dec242d-3956-413d-b3e1-f48074073d70,
  abstract     = {<p>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 &gt;. 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 &lt;. 65. days and annual center caseload &gt;. 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.</p>},
  author       = {Pakarinen, Mikko P and Johansen, Lars Søndergaard and Svensson, Jan F. and Bjørnland, Kristin and Gatzinsky, Vladimir and Stenström, Pernilla and Koivusalo, Antti and Kvist, Nina and Almström, Markus and Emblem, Ragnhild and Björnsson, Sigurdur and Backman, Torbjörn and Almaas, Runar and Jalanko, Hannu and Fischler, Björn and Thorup, Jørgen and , },
  issn         = {0022-3468},
  keyword      = {Clearance of jaundice,Liver transplantation,Portoenterostomy,Steroids,Survival,Ursodeoxycholic acid},
  language     = {eng},
  publisher    = {Elsevier},
  series       = {Journal of Pediatric Surgery},
  title        = {Outcomes of biliary atresia in the Nordic countries - a multicenter study of 158 patients during 2005-2016},
  url          = {http://dx.doi.org/10.1016/j.jpedsurg.2017.08.048},
  year         = {2017},
}