P4.16 (original) (raw)
Transplantation, 2019
Abstract
Introduction: Patients requiring long term parenteral nutrition for intestinal failure are at risk of developing associated liver disease (IFALD) which can progress to cirrhosis that requires liver transplantation along with the intestine. There is evidence to suggest that pre-emptive intestinal transplantation in this setting can prevent progression of liver disease and may improve fibrosis. Methods: A retrospective review of liver biopsies, liver blood tests (at the time of biopsy) intestinal anatomy and outcomes in patients referred to Addenbrooke’s Hospital Cambridge UK between 2008 and 2018 for consideration of transplantation for IFALD. Fibrosis was scored subjectively by pathologists as mild, moderate, severe or cirrhosis. Results: 21 patients (out of a total of 82) underwent transplantation for IFALD. In 13 cases, liver fibrosis was considered too advanced for intestine alone and they required liver containing grafts, the others received intestine without liver. Post transplant survival is shown in fig 1. In addition, 13 patients have been referred and/or listed and 9 died on the waiting list, 5 of which from advanced liver disease. Liver biopsies which showed at least moderate fibrosis (in the absence of additional pathology) from patients with >20cms intestine to stoma were performed at a mean of 11.6 years (SD7yrs) after starting PN (n=15), those with <20cms to stoma were performed at a mean of 2.2 years (SD 0.6yrs) (n=10). LFTs in patients (excluding end stage disease and severe cholestasis) were calculated. Mean bilirubin and ALT in patients with cirrhosis or severe fibrosis were 30 umols/l (+/-11) and 52 U/l (+/34). Only 10 out of 24 patients with at least moderate fibrosis on biopsy showed bilirubin and/or ALT >1.5x ULN. Conclusions: Patients with ultrashort intestine are at risk of early progression to significant degrees of IFALD. Liver blood tests are not predictive of underlying IFALD. Post-transplant survival in patients with IFALD requiring liver transplantation is inferior to that of those receiving intestine alone Earlier detection of IFALD and referral for pre-emptive intestinal transplantation is indicated P4.16
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