Literature DB >> 22171974

National centralization of biliary atresia care to an assigned multidisciplinary team provides high-quality outcomes.

Hanna Lampela1, Annukka Ritvanen, Silja Kosola, Antti Koivusalo, Risto Rintala, Hannu Jalanko, Mikko Pakarinen.   

Abstract

BACKGROUND AND AIMS: Effects of caseload and organization of care on outcomes of biliary atresia (BA) remain unclear. We compared outcomes before and after national centralization of BA treatment in Finland with a population of 5.4 million people and 60,000 live births/year.
METHODS: All children born in Finland from 1987 to 2010 with BA were included. Complete patient identification was ascertained from the national Register of Congenital Malformations. Hospital records were reviewed for confirmation of the diagnosis, treatment, and follow-up data. Clearance of jaundice (serum bilirubin ≤ 20 μmol/l) and survival modalities were compared before and after centralization from five centers to Helsinki.
RESULTS: The incidence of BA was 1 in 20,100 live births. A total of 72 BA patients of whom 64 had undergone surgery for BA were identified. After centralization, the median caseload per center increased from 0 (range, 0-3) to 4 (2-5) patients/year (p < 0.001), clearance of jaundice rate increased from 27% to 75% (p = 0.001), 2-year jaundice-free native liver survival from 25% to 75% (p = 0.002), transplant-free survival from 27% to 75% (p = 0.005), and overall survival from 64% to 92% (p = 0.082). Baseline patient characteristics including type of BA and age at portoenterostomy remained unaltered. In a logistic regression analysis including treatment era, operating center, BA splenic malformation syndrome, and age at portoenterostomy as variables, only treatment in Helsinki after centralization predicted clearance of jaundice (odds ratio 4.2; 95% confidence interval 1.05-16.5; p = 0.043).
CONCLUSIONS: In small countries, BA treatment should be centralized to appointed multidisciplinary teams allowing high quality results with a median of four cases/year.

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Year:  2012        PMID: 22171974     DOI: 10.3109/00365521.2011.627446

Source DB:  PubMed          Journal:  Scand J Gastroenterol        ISSN: 0036-5521            Impact factor:   2.423


  17 in total

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Authors:  Alison E Butler; Richard A Schreiber; Natalie Yanchar; Sherif Emil; Jean-Martin Laberge
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4.  Surgical treatment of childhood hepatoblastoma in the Netherlands (1990-2013).

Authors:  Linde A D Busweiler; Marc H W A Wijnen; Jim C H Wilde; Egbert Sieders; Sheila E J Terwisscha van Scheltinga; L W Ernest van Heurn; Joseph Ziros; Roel Bakx; Hugo A Heij
Journal:  Pediatr Surg Int       Date:  2016-10-11       Impact factor: 1.827

5.  Total Serum Bilirubin within 3 Months of Hepatoportoenterostomy Predicts Short-Term Outcomes in Biliary Atresia.

Authors:  Benjamin L Shneider; John C Magee; Saul J Karpen; Elizabeth B Rand; Michael R Narkewicz; Lee M Bass; Kathleen Schwarz; Peter F Whitington; Jorge A Bezerra; Nanda Kerkar; Barbara Haber; Philip Rosenthal; Yumirle P Turmelle; Jean P Molleston; Karen F Murray; Vicky L Ng; Kasper S Wang; Rene Romero; Robert H Squires; Ronen Arnon; Averell H Sherker; Jeffrey Moore; Wen Ye; Ronald J Sokol
Journal:  J Pediatr       Date:  2015-12-24       Impact factor: 4.406

6.  Use of corticosteroids after hepatoportoenterostomy for bile drainage in infants with biliary atresia: the START randomized clinical trial.

Authors:  Jorge A Bezerra; Cathie Spino; John C Magee; Benjamin L Shneider; Philip Rosenthal; Kasper S Wang; Jessi Erlichman; Barbara Haber; Paula M Hertel; Saul J Karpen; Nanda Kerkar; Kathleen M Loomes; Jean P Molleston; Karen F Murray; Rene Romero; Kathleen B Schwarz; Ross Shepherd; Frederick J Suchy; Yumirle P Turmelle; Peter F Whitington; Jeffrey Moore; Averell H Sherker; Patricia R Robuck; Ronald J Sokol
Journal:  JAMA       Date:  2014-05-07       Impact factor: 56.272

7.  An Analysis of the Care Structure for Congenital Malformations in Germany.

Authors:  Julia Elrod; Michael Boettcher; Christoph Mohr; Konrad Reinshagen
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8.  Myofibroblastic cell activation and neovascularization predict native liver survival and development of esophageal varices in biliary atresia.

Authors:  Janne S Suominen; Hanna Lampela; Päivi Heikkilä; Jouko Lohi; Hannu Jalanko; Mikko P Pakarinen
Journal:  World J Gastroenterol       Date:  2014-03-28       Impact factor: 5.742

Review 9.  Biliary atresia: Where do we stand now?

Authors:  Krishna Kumar Govindarajan
Journal:  World J Hepatol       Date:  2016-12-28

10.  Biliary atresia: 50 years after the first kasai.

Authors:  Barbara E Wildhaber
Journal:  ISRN Surg       Date:  2012-12-06
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