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Epidemiology of Biliary Atresia in England and Wales (1999 - 2006)
  1. Emily Livesey (emilylivesey{at}doctors.org.uk)
  1. Kings College Hospital, United Kingdom
    1. Mario Cortina Borja (m.cortina{at}ich.ucl.ac.uk)
    1. Institute of Child Health, London, United Kingdom
      1. Khalid Sharif (ksharif{at}hotmail.com)
      1. Birmingham Children's Hospital, United Kingdom
        1. Naved Kamal Alizai (nkalizai{at}hotmail.com)
        1. St James' Hospital, Leeds, United Kingdom
          1. Patricia McClean (patricia.mcclean{at}leedsth.nhs.uk)
          1. St James' Hospital, Leeds, United Kingdom
            1. Deidre A Kelly (d.a.kelly{at}bham.ac.uk)
            1. Birmingham Children's Hospital, United Kingdom
              1. Nedim Hadzic (nedim.hadzic{at}kcl.ac.uk)
              1. Kings College Hospital, United Kingdom
                1. Mark Davenport (markdav2{at}ntlworld.com)
                1. Kings College Hospital, United Kingdom

                  Abstract

                  Objective: To identify the epidemiological characteristics of infants with biliary atresia in England and Wales, since centralisation of its management in 1999.

                  Methods: The care of infants with biliary atresia (BA) in England and Wales is centralized to only three centres. All infants (treated from Jan. 1999 to Dec. 2006) were identified from a prospective national database; demographic details were ascertained from medical records and compared between two groups based on presumed aetiology [isolated biliary atresia (IBA) and developmental biliary atresia (DBA) (e.g. syndromic infants, BASM, cystic biliary atresia)].

                  Results: There were 302 (133 male (44%)) infants with BA that could be divided into IBA (n = 219, 73%) and DBA (n = 76, 25%). The overall incidence was 0.58 / 10,000 (i.e. 1 in 17,049) live births with marked regional differences along a north-west / south –east axis varying from 0.38 (North-West England) to 0.78 (South-East England) /10,000 live births (OR 2.05 (95%CI 1.26 – 3.41); P =0.002). The commonest month of birth was September with December being the least common, although there was no evidence for significant seasonal variation (P = 0.2). Infants with DBA were more likely to be female (P < 0.001), of Caucasian background (P = 0.01), first-born (P = 0.04) and to be formula-fed (P = 0.07). Infants of South Asian origin came to surgery at an older age [59 (IQ 45-75) versus 52 (IQ 42-65); P = 0.03] days.

                  Conclusions: There is a remarkable variation of incidence of biliary atresia within England and Wales, some of which may have been caused by factors related to a different aetiological and racial background.

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