| Biliary atresia in England and Wales: results of centralization and new benchmark. | |
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MedLine Citation:
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PMID: 21929975 Owner: NLM Status: In-Data-Review |
Abstract/OtherAbstract:
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INTRODUCTION: Biliary atresia (BA) is a rare, potentially life-threatening condition of the newborn presenting with conjugated jaundice. Typically, it is treated by an initial attempt to restore bile flow (the Kasai portoenterostomy [KP]) as soon as possible after diagnosis and, if this fails, liver transplantation. Since 1999, the treatment of BA has been centralized to 3 centers in England and Wales able to offer both treatment options. The aim of this study was to review the outcome of this policy change and provide a national benchmark. METHODS: The management of all infants born within England and Wales during the period January 1999 to December 2009 was assessed using 3 key performance indicators such as median time to KP, percentage clearance of jaundice (≤20 mol/L) post-KP, and 5- and 10-year native liver and true survival estimates. Data are quoted as median (range), and P < .05 was considered significant. RESULTS: A total of 443 infants had confirmed BA; and of these, most were isolated BA (n = 359), with 84 having other significant anomalies (but predominantly BA splenic malformation syndrome). Four infants died before any biliary intervention. Kasai portoenterostomy was performed in 424 infants (median age, 54 [range 7-209] days), and a primary liver transplant was performed in 15. Clearance of jaundice post-KP was achieved in 232 (55%). There were 41 deaths, including 4 (10%) without any intervention, 24 (58%) post-KP usually because of end-stage liver disease and mostly on a transplant waiting list, and 13 (32%) post-LT usually because of multiorgan failure. Overall, the 5- and 10-year native liver survival estimates were 46% (95% confidence interval [CI], 41-51) and 40% (95% CI, 34-46), respectively. The 5- and 10-year true patient survival estimates were 90% (95% CI, 88-93) and 89% (95% CI, 86-93), respectively. Outcome was worse for those with other anomalies (lower clearance of jaundice post-KP [43% vs 57%; odds ratio, 1.7; 95% CI, 1.04-2.8]; P = .02) and an increased mortality overall (eg, at 5 years, 72 [95% CI, 64-83] vs 94 [95% CI, 91-96]; χ(2) = 33; P < .0001). CONCLUSIONS: National outcome measures in BA appear better than those from previously published series from comparable countries and may be attributed to centralization of surgical and medical resources. |
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Authors:
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Mark Davenport; Evelyn Ong; Khalid Sharif; Naved Alizai; Patricia McClean; Nedim Hadzic; Deirdre A Kelly |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Journal of pediatric surgery Volume: 46 ISSN: 1531-5037 ISO Abbreviation: J. Pediatr. Surg. Publication Date: 2011 Sep |
Date Detail:
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Created Date: 2011-09-20 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0052631 Medline TA: J Pediatr Surg Country: United States |
Other Details:
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Languages: eng Pagination: 1689-94 Citation Subset: IM |
Copyright Information:
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Copyright © 2011 Elsevier Inc. All rights reserved. |
Affiliation:
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Paediatric Liver Centre, Kings College Hospital, SE5 9RS London, UK. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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