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

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

Pakarinen, Mikko P (author): Helsinki University Central Hospital

Johansen, Lars Søndergaard (author): Copenhagen University Hospital

Svensson, Jan F. (author): Karolinska Institutet,Karolinska University Hospital

Bjørnland, Kristin (author): Oslo university hospital

Gatzinsky, Vladimir (author): Queen Silvia Children’s Hospital

Stenström, Pernilla (author): Lund University,Lunds universitet,Pediatrik, Lund,Sektion V,Institutionen för kliniska vetenskaper, Lund,Medicinska fakulteten,Paediatrics (Lund),Section V,Department of Clinical Sciences, Lund,Faculty of Medicine,Skåne University Hospital

Koivusalo, Antti (author): Helsinki University Central Hospital

Kvist, Nina (author): Copenhagen University Hospital

Almström, Markus (author): Karolinska Institutet,Karolinska University Hospital

Emblem, Ragnhild (author): Oslo university hospital

Björnsson, Sigurdur (author): Queen Silvia Children’s Hospital

Backman, Torbjörn (author): Lund University,Lunds universitet,Pediatrik, Lund,Sektion V,Institutionen för kliniska vetenskaper, Lund,Medicinska fakulteten,Paediatrics (Lund),Section V,Department of Clinical Sciences, Lund,Faculty of Medicine,Skåne University Hospital

Almaas, Runar (author): Oslo university hospital

Jalanko, Hannu (author): Helsinki University Central Hospital

Fischler, Björn (author): Karolinska Institutet,Karolinska University Hospital

Thorup, Jørgen (author): Copenhagen University Hospital

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Elsevier BV, 2018
2018
English.
In: Journal of Pediatric Surgery. - : Elsevier BV. - 0022-3468 .- 1531-5037. ; 53:8, s. 1509-1515

Related links:: http://dx.doi.org/10...; show more...; https://helda.helsin...; https://lup.lub.lu.s...; https://doi.org/10.1...; http://kipublication...; show less...

Journal article (peer-reviewed)

Abstract Subject headings

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.

Subject headings

MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Kirurgi (hsv//swe)
MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Surgery (hsv//eng)

Keyword

Clearance of jaundice
Liver transplantation
Portoenterostomy
Steroids
Survival
Ursodeoxycholic acid

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By the author/editor: Pakarinen, Mikko ...; Johansen, Lars S ...; Svensson, Jan F.; Bjørnland, Krist ...; Gatzinsky, Vladi ...; Stenström, Perni ...; show more...; Koivusalo, Antti; Kvist, Nina; Almström, Markus; Emblem, Ragnhild; Björnsson, Sigur ...; Backman, Torbjör ...; Almaas, Runar; Jalanko, Hannu; Fischler, Björn; Thorup, Jørgen; show less...

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MEDICAL AND HEALTH SCIENCES: MEDICAL AND HEAL ...; and Clinical Medicin ...; and Surgery

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By the university: Lund University; Karolinska Institutet

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