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Presenter: Khalid, Sharif, Birmingham, United Kingdom
Authors: Taha Ahmed1, Khalid Sharif1, Tracey Johnson2,3, Sara Clarke1,3, Stephen Murphy2, Girish Gupte1
Taha Ahmed1, Khalid Sharif1, Tracey Johnson2,3, Sara Clarke1,3, Stephen Murphy2, Girish Gupte1
1Liver Unit (including small bowel transplantation), Birmingham Children's Hospital, Birmingham, United Kingdom; 2Department of Gastroenterology, Birmingham Children's Hospital, Birmingham, United Kingdom; 3Department of Dietetics, Birmingham Children's Hospital, Birmingham, United Kingdom
A select group of children with short bowel syndrome (SBS) and intestinal failure associated liver disease (IFALD) fulfil the criteria for isolated liver transplantation (iLTx). We report on the long-term outcomes of children undergoing iLTx for the above indication, managed by a multidisciplinary intestinal rehabilitation team. Of the 14 transplants performed, 4 died in the immediate post-operative period. Two further children died at 12.3 and 46 months after transplantation. Eight children survived in the long-term with a median follow-up of 107.5 months (range 89-155 months). 5/8 children were weaned from parenteral nutrition (PN) into enteral nutrition (EN) in a median of 10 months after iLTx (range 3-32 months). Three of those 5 children were subsequently weaned from EN into full oral feeding in 13, 24 and 53 months after stopping PN while the other 2 are still on EN 118 and 74 months after stopping PN. The 5 children maintain their weight median z-score with a median increase of 1.59 (range 1.24-1.79) compared with the pre-transplant z-scores, whereas the height z-sores show fluctuations over the years with a median change of 0.12 (range -0.29 to 0.36). The other 3/8 children developed irreversible intestinal failure; 2 undergone isolated small bowel transplantation 112 and 84 months after iLTx and the third is on PN.
Conclusion: Children with SBS and IFALD need close follow-up with an experienced multidisciplinary team to monitor long term nutritional outcome and may need consideration for non-transplant/transplant surgery in the follow-up period.
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