title: Improved outcome of referrals for intestinal transplantation in the UK.
abstract: OBJECTIVE
  To describe the outcome of children with intestinal failure referred to Birmingham Children's Hospital (BCH) for consideration of intestinal transplantation (ITx), to determine factors for an adverse outcome and to analyse the impact of post-1998 strategies on survival.
  
  
  METHODS
  A retrospective analysis was performed of children referred for ITx assessment from January 1989 to December 2003. Children were assessed by a multidisciplinary team and categorised into: (a) stable on parenteral nutrition; (b) unsuitable for transplantation (Tx); and (c) recommended for Tx. To analyse the impact of the post-1998 strategies on survival, a comparison was made between the two eras (pre-1998 and post-1998).
  
  
  RESULTS
  152 children with chronic intestinal failure were identified (63M:89F, median age 10 months (range 1-170)). After assessment, 69 children were considered stable on parenteral nutrition (5-year survival 95%); 28 children were unsuitable for Tx (5-year survival 4%); and 55 children were recommended for Tx (5-year survival 35%, which includes 14 children who died waiting for size-matched organs). Twenty three ITx and nine isolated liver transplants (iLTx) were performed. In a multivariate analysis, the following factors in combination had an adverse effect on survival: the presence of a primary mucosal disorder (p = 0.007, OR ratio 3.16, 95% CI 1.37 to 7.31); absence of involvement of a nutritional care team at the referring hospital (p = 0.001, OR ratio 2.55, 95% CI 1.44 to 4.52); and a serum bilirubin>100 micromol/l (p = 0.001, OR ratio 3.70, 95% CI 1.84 to 7.47). Earlier referral (median serum bilirubin 78 micromol/l in the post-1998 era compared with 237 micromol/l in the pre-1998 era, p = 0.001) may be a contributory factor to improved survival. The strategies of combined en bloc reduced liver/small bowel transplantation and iLTx resulted in fewer deaths on the waiting list in the post-1998 era (2 deaths in post-1998 era v 12 deaths in pre-1998 era). The overall 3-year survival in the post-1998 era (69%) has improved compared with the pre-1998 era (31%; p<0.001) CONCLUSION: The changing characteristics at the time of referral, including earlier referral and innovative surgical strategies have resulted in improved long-term survival of children referred for ITx.
authors.affiliation: Liver Unit, Birmingham Children's Hospital, Birmingham, UK. girish.gupte@bch.nhs.uk
authors.forename: Girish L
authors.initials: GL
authors.lastname: Gupte
authors.forename: Susan V
authors.initials: SV
authors.lastname: Beath
authors.forename: Sue
authors.initials: S
authors.lastname: Protheroe
authors.forename: M Stephen
authors.initials: MS
authors.lastname: Murphy
authors.forename: Paul
authors.initials: P
authors.lastname: Davies
authors.forename: Khalid
authors.initials: K
authors.lastname: Sharif
authors.forename: Patrick J
authors.initials: PJ
authors.lastname: McKiernan
authors.forename: Jean
authors.initials: J
authors.lastname: de Ville de Goyet
authors.forename: Ian W
authors.initials: IW
authors.lastname: Booth
authors.forename: Deirdre A
authors.initials: DA
authors.lastname: Kelly
mesh_terms: D000293:Adolescent; D001663:Bilirubin / Q000097:blood; D015415:Biomarkers / Q000097:blood; D002648:Child; D002664:Child Nutritional Physiological Phenomena; D002675:Child, Preschool; D004812:Epidemiologic Methods; D005260:Female; D006627:Hirschsprung Disease / Q000601:surgery / Q000628:therapy; D006801:Humans; D007223:Infant; D007410:Intestinal Diseases / Q000601:surgery* / Q000628:therapy; D007421:Intestine, Small / Q000637:transplantation*; D016031:Liver Transplantation; D008297:Male; D010288:Parenteral Nutrition; D018579:Patient Selection; D011379:Prognosis; D012017:Referral and Consultation; D012778:Short Bowel Syndrome / Q000601:surgery / Q000628:therapy; D016896:Treatment Outcome
chemical_list: D015415:Biomarkers; D001663:Bilirubin