2013 - ISBTS 2013 Symposium
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Posters and Exhibition
15.36 - Late referral of Intestinal Failure Associated Liver disease; an ongoing issue?
Presenter: Vandana, Jain, , United Kingdom
Authors: Vandana Jain1, Jonathan Hind1
Late referral of Intestinal Failure Associated Liver disease; an ongoing issue?
Vandana Jain1, Jonathan Hind1
1Paediatric Liver, GI and Nutrition centre, Kings College Hospital, London, United Kingdom
Introduction
Cases of intestinal failure associated liver disease (IFALD) requiring combined liver bowel transplantation have decreased with earlier referral to intestinal rehabilitation (IR) units. We describe our recent experience of children with complex intestinal failure referred to a paediatric hepatology unit with end-stage liver disease having missed opportunity for IR management.
Case Studies
2 patients (A and B) were ex-premature (30+5 week gestation female and 24 week male) infants who developed necrotizing enterocolitis in the first week of life; patient A managed conservatively and B surgically. Both required parenteral-nutrition (PN), being unable to tolerate nutritive volumes of enteral feeding. Both had recurrent sepsis with deterioration of liver function. Both were managed in tertiary neonatal units, neither was discussed with or referred to an IR unit
Hepatology referral was made after finding an abnormal gallbladder on ultrasound of patient A and stomal variceal bleeding in B, at 4 and 5 months of age respectively. At referral, both had severe cholestasis, portal hypertension (splenomegaly/ascites), and coagulopathy. Liver biopsy was not performed due to coagulopathy.
Results (A): total bilirubin [TB] 429µmol/l, direct bilirubin [DB] 359µmol/l, ALT 108IU/l albumin 40,
platelets 66, INR 1.9.
(B): [TB]580µmol/l, [DB]461µmol/l, ALT 279IU/l, albumin 33, platelets 64, INR 2.1
The IR team was subsequently involved and IF management instituted with further GI investigation, re-commencement of enteral feeding and optimization of PN. Neither patient established enteral feeding successfully. Transplant was not possible due to lack of venous access and severe multi-organ dysfunction. Both patients died at 3 months after referral.
Conclusion
Late recognition of complex IF and IFALD with consequent delayed appropriate referral and management remains a problem outside of IR and transplant centres. We have reported 2 such cases to one unit within the last year - is this the tip of an iceberg?
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