Henry Gowen¹, Sue Beath¹, Girish Gupte¹, Deirdre Kelly¹, Ian Booth¹, Ian Sugarman², John Puntis²

¹Birmingham Children’s Hospital, Birmingham, United Kingdom; ²The General Infirmary Leeds, Leeds, United Kingdom

The prevalence of short-term intestinal failure (IF) in children necessitating PN in the UK is estimated to be around 5000 (420 per million) of which 136 (14 per million) are on home PN.   In order to plan services, BIFS began in 2006 with the aim of determining the incidence and outcome of paediatric IF in/outside hospital, and to quantify the number of children who may require small bowel transplantation (SBTx).

Methods: Children who were on PN > 28 days were eligible for inclusion.  Children were recruited via paediatric gastroenterologists who are part of the UK wide professional network linked via membership of the British Society of Paediatric Gastroenterology Hepatology and Nutrition (www.bspghan.org.uk).  Written consent was required and registered patients were followed up by the BIFS administrator.

Results: 353 patients were recruited with numbers rising to 120 in 2009/10, which represents a national recruitment rate of 15%.  The main reason for the poor recruitment rate was the need for written consent.  However, professional support for the registry was evident from the response to requests for outcome data, which was 91.4% 86.5 % and 79% at 6, 12 and 24 months post registration.  Most children came off PN 203/353(57.5%) by 6 months and 235/279 (84.2%) at 2yrs.  During 2yrs follow up 1/3 of patients suffered complications: 91(31%) children had a septic episode, 94(33%), required further surgery and 69(22%) had been jaundiced.  34 children died (9.6%), 63 children (18%) were referred for SBTx and 27 were transplanted.  Jaundice was a significant risk factor for death (16/34 v.s. 53/319).

Conclusion: The comprehensive nature of outcome data collection has supplied useful information on duration of PN dependency, mortality risk factors and rates of small bowel transplantation which provides a platform for future health care planning and incentive for improved registration of patients.