{
  "abstract": "Background Paediatric patients awaiting combined liver and intestinal transplant (LITx) face a high risk of deterioration. We aimed to evaluate whether perceived long wait times for these patients would increase waitlist mortality or necessitate life-saving isolated liver transplantation.Methods A retrospective case review of LITx candidates (n=50), evaluated waitlist mortality as the primary outcome. Subgroup analyses compared patients requiring urgent isolated liver transplantation due to deterioration with those receiving other grafts. Statistical analyses included survival analysis, correlation between wait times and mortality, and multivariate regression to identify predictors of waitlist death.Results Median transplant wait-time was 209 days (20–1248), longer than recent UK adult registry data of median 54 days. Waitlist mortality was 20%. Intestinal Failure Associated Liver Disease (IFALD) was the key risk-factor, with 27.6% mortality among IFALD patients compared to 0% in non-IFALD (p=0.035). In IFALD, extended wait-time (r=0.663, p<0.001) and higher referral bilirubin levels (r=-0.424, p=0.024) predicted poorer outcomes. Nine patients (24%) initially listed for small bowel and liver containing grafts, underwent isolated liver transplantation due to clinical deterioration. These patients had shorter median wait-times (379.5 vs 411.7 days) and higher serum bilirubin levels (179.3 vs 116.9µmol/L) than those receiving other transplants.Conclusions These findings highlight the need for timely transplantation, particularly for IFALD. Early consideration of isolated liver transplantation as a life-saving bridge for rapidly deteriorating patients is required. Further measures, such as increased use of living-related liver bowel transplant and machine perfusion, may be needed to reduce long wait times.",
  "authors": [
    {
      "affiliations": [
        "Paediatric Liver, GI and Nutrition Centre, King’s College Hospital"
      ],
      "name": "Sinead Cunningham"
    },
    {
      "affiliations": [
        "Paediatric Liver, GI and Nutrition Centre, King’s College Hospital"
      ],
      "name": "Carly Bambridge"
    },
    {
      "affiliations": [
        "Paediatric Liver, GI and Nutrition Centre, King’s College Hospital"
      ],
      "name": "Sergio Assia-Zamora"
    },
    {
      "affiliations": [
        "Paediatric Liver, GI and Nutrition Centre, King’s College Hospital"
      ],
      "name": "Miriam Cortes-Cerisuelo"
    },
    {
      "affiliations": [
        "Paediatric Liver, GI and Nutrition Centre, King’s College Hospital"
      ],
      "name": "Nigel Heaton"
    },
    {
      "affiliations": [
        "Paediatric Liver, GI and Nutrition Centre, King’s College Hospital"
      ],
      "name": "Anil Dhawan"
    },
    {
      "affiliations": [
        "Paediatric Liver, GI and Nutrition Centre, King’s College Hospital"
      ],
      "name": "Jonathan Hind"
    }
  ],
  "title": "LBA18 The mortality burden of paediatric intestinal failure associated liver disease (IFALD)- a single centre experience",
  "uid": "21305132-0a2c-5f2e-9131-7e3108b2ae5b"
}