Epidemiology of biliary atresia in England and Wales (1999-2006)
E Livesey1, M Cortina Borja, K Sharif
1Paediatric Liver Centre, King's College Hospital, Denmark Hill, London SE5 9RS, UK.
Insights
Biliary atresia (BA) incidence varies significantly across England and Wales, with developmental BA (DBA) more common in females and white infants. These epidemiological patterns suggest potential links to aetiological and racial factors.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Epidemiology
Background:
- Biliary atresia (BA) is a rare neonatal liver disease requiring prompt diagnosis and treatment.
- Centralization of BA management in England and Wales occurred in 1999, necessitating updated epidemiological data.
- Understanding BA's characteristics is crucial for improving patient outcomes and public health strategies.
Purpose of the Study:
- To determine the epidemiological features of biliary atresia in infants in England and Wales post-1999.
- To compare characteristics between isolated biliary atresia (IBA) and developmental biliary atresia (DBA) groups.
- To investigate regional and demographic variations in BA incidence.
Main Methods:
- A prospective national database identified 302 infants with BA treated between January 1999 and December 2006.
- Infants were categorized into IBA (n=219) and DBA (n=76) based on presumed aetiology.
- Demographic data were collected and compared between groups, with statistical analysis of incidence and risk factors.
Main Results:
- The overall incidence of BA was 0.58 per 10,000 live births, with significant regional variation (0.38 in NW England to 0.78 in SE England).
- Infants with DBA were more likely to be female, of white background, first-born, and formula-fed.
- South Asian infants with BA underwent surgery at a later age compared to other groups.
Conclusions:
- Significant geographical disparities in BA incidence exist within England and Wales.
- Aetiological and racial background may contribute to observed variations in BA incidence and presentation.
- Further research into these factors is warranted to understand and potentially mitigate BA prevalence.
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 centralised to only three centres. All infants (treated from January 1999 to December 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) (for example, syndromic infants, biliary atresia splenic malformation, 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 (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 white 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) days; p = 0.03).
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.
