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

Mikko P Pakarinen; Lars Søndergaard Johansen; Jan F Svensson; Kristin Bjørnland; Vladimir Gatzinsky; Pernilla Stenström; Antti Koivusalo; Nina Kvist; Markus Almström; Ragnhild Emblem; Sigurdur Björnsson; Torbjörn Backman; Runar Almaas; Hannu Jalanko; Björn Fischler; Jørgen Thorup; Nordic Pediatric Surgery Study Consortium

doi:10.1016/j.jpedsurg.2017.08.048

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

Mikko P Pakarinen, Lars Søndergaard Johansen, Jan F Svensson, Kristin Bjørnland, Vladimir Gatzinsky, Pernilla Stenström, Antti Koivusalo, Nina Kvist, Markus Almström, Ragnhild Emblem, Sigurdur Björnsson, Torbjörn Backman, Runar Almaas, Hannu Jalanko, Björn Fischler, Jørgen Thorup, Nordic Pediatric Surgery Study Consortium

Department of Clinical Medicine

18 Citations (Scopus)

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 <65days 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.

Original language	English
Journal	Journal of Pediatric Surgery
Volume	53
Issue number	8
Pages (from-to)	1509-1515
ISSN	0022-3468
DOIs	https://doi.org/10.1016/j.jpedsurg.2017.08.048
Publication status	Published - Aug 2018

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Journal Article

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10.1016/j.jpedsurg.2017.08.048

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Pakarinen, M. P., Johansen, L. S., Svensson, J. F., Bjørnland, K., Gatzinsky, V., Stenström, P., Koivusalo, A., Kvist, N., Almström, M., Emblem, R., Björnsson, S., Backman, T., Almaas, R., Jalanko, H., Fischler, B., Thorup, J., & Nordic Pediatric Surgery Study Consortium (2018). Outcomes of biliary atresia in the Nordic countries - a multicenter study of 158 patients during 2005-2016. Journal of Pediatric Surgery, 53(8), 1509-1515. https://doi.org/10.1016/j.jpedsurg.2017.08.048

Pakarinen, MP, Johansen, LS, Svensson, JF, Bjørnland, K, Gatzinsky, V, Stenström, P, Koivusalo, A, Kvist, N, Almström, M, Emblem, R, Björnsson, S, Backman, T, Almaas, R, Jalanko, H, Fischler, B, Thorup, J & Nordic Pediatric Surgery Study Consortium 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

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

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 <65days 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.",

keywords = "Journal Article",

author = "Pakarinen, {Mikko P} and Johansen, {Lars S{\o}ndergaard} and Svensson, {Jan F} and Kristin Bj{\o}rnland and Vladimir Gatzinsky and Pernilla Stenstr{\"o}m and Antti Koivusalo and Nina Kvist and Markus Almstr{\"o}m and Ragnhild Emblem and Sigurdur Bj{\"o}rnsson and Torbj{\"o}rn Backman and Runar Almaas and Hannu Jalanko and Bj{\"o}rn Fischler and J{\o}rgen Thorup and {Nordic Pediatric Surgery Study Consortium}",

year = "2018",

month = aug,

doi = "10.1016/j.jpedsurg.2017.08.048",

language = "English",

volume = "53",

pages = "1509--1515",

journal = "Journal of Pediatric Surgery",

issn = "0022-3468",

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TY - JOUR

T1 - Outcomes of biliary atresia in the Nordic countries - a multicenter study of 158 patients during 2005-2016

AU - Pakarinen, Mikko P

AU - Johansen, Lars Søndergaard

AU - Svensson, Jan F

AU - Bjørnland, Kristin

AU - Gatzinsky, Vladimir

AU - Stenström, Pernilla

AU - Koivusalo, Antti

AU - Kvist, Nina

AU - Almström, Markus

AU - Emblem, Ragnhild

AU - Björnsson, Sigurdur

AU - Backman, Torbjörn

AU - Almaas, Runar

AU - Jalanko, Hannu

AU - Fischler, Björn

AU - Thorup, Jørgen

AU - Nordic Pediatric Surgery Study Consortium

PY - 2018/8

Y1 - 2018/8

N2 - 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 <65days 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.

AB - 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 <65days 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.

KW - Journal Article

U2 - 10.1016/j.jpedsurg.2017.08.048

DO - 10.1016/j.jpedsurg.2017.08.048

M3 - Journal article

C2 - 28947328

SN - 0022-3468

VL - 53

SP - 1509

EP - 1515

JO - Journal of Pediatric Surgery

JF - Journal of Pediatric Surgery

IS - 8

ER -

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

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