401.4 Late allograft fibrosis in protocol post-liver transplant biopsies in children can be associated with persistent splenomegaly and sub-clinical portal hypertension- A long-term follow-up study
Tuesday May 07, 2019 from 08:00 to 09:30
Bayshore E
Presenter

Venkatesh Karthik, Singapore

Consultant

Division of Paediatric Gastroenterology, Hepatology, Nutrition and Liver Transplantation Services

National University Health System

Abstract

Late allograft fibrosis in protocol post-liver transplant biopsies in children can be associated with persistent splenomegaly and sub-clinical portal hypertension- A long-term follow-up study

Venkatesh Karthik1, Nicholas Ng1, Michelle Tan1, James Huang1, Seng Hock Quak1, Marion Aw1.

1Department of Paediatrics, National University Hospital, Singapore, Singapore

Objectives: Persistent or increasing splenomegaly after liver transplantation (LT), despite good graft function and normal graft vasculature on imaging, may represent sub-clinical portal hypertension (PHT). In the absence of extra-hepatic causes, graft pathology is likely to be the underlying cause. We describe our experience with splenomegaly in children with significant graft fibrosis detected on post-LT protocol liver biopsies (PLB).

Methods: Retrospective chart review including ultrasonography (US) records.

Results: Thirty-three children (17 girls) with a median age at LT of 1.7 years (range: 0.8-17.9) underwent 45 PLB (12 had two) over the study period. The median interval between LT and first PLB was 9.8 years (range: 3-17) and between LT and second PLB 14.3 years (range: 7-21). Median post-LT follow-up was 13.8 years (range: 3-22.5). 85% had undergone living donor LT and 94% were on Tacrolimus-based immunosuppression regimens. Two patients (1 moved overseas and 1 with portal vein thrombosis that necessitated shunt surgery), were excluded. 26/33 (79%) were anti-nuclear antibody positive. None tested positive for any other auto-antibody. 23 of 31 (74%) had splenomegaly on US (Spleen size assessed and quantified in centimetres). 22 of these 23 (96%) had abnormal PLB (graft fibrosis and/or graft inflammation and/or steatosis) and 19 (83%) had fibrosis. Splenomegaly on US was associated with abnormal PLB (p-0.012)  as well as allograft fibrosis (p- 0.027). Ten of these 23 had increasing splenomegaly on follow-up US with 9 having graft fibrosis. However, progressive splenomegaly was not associated with either abnormal PLB (p-0.6) or graft fibrosis (p-0.21). None had clinical concerns  with gastrointestinal bleeding. Thrombocytopenia secondary to hypersplenism was seen in a minority (26%) and  was not  associated  with either abnormal PLB (p-0.3) or graft fibrosis (p-0.64).

Conclusions:  Several studies have highlighted the increasing prevalence of graft hepatitis and fibrosis and this is thought to be immune-mediated or represent an atypical form of graft rejection. Our study has shown a correlation between late allograft fibrosis and sub-clinical post-LT PHT. Although its clinical significance is uncertain, it would be important to determine long-term outcomes for this group of patients, especially as they transition to adulthood. Multi-center longitudinal studies are necessary to explore this further.


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