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P299 Ulcerative colitis and paediatric liver transplantation for primary sclerosis cholangitis - multi-case report

P. Czubkowski*, J. Pawlowska, M. Wozniak, K. Zieniewska-Pingwara, I. Jankowska, J. Kierkus

The Children's Memorial Health Institute, Department of Gastroenterology, Hepatology, Feeding Disorders and Pediatrics, Warsaw, Poland

Background

Primary sclerosing cholangitis (PSC) is strongly associated with ulcerative colitis (UC) and may enhance the risk of colorectal dysplasia and cancer. Liver transplantation (LTx) for PSC and immunosupressive regiments, especially those including steroids, may alter the outcome of UC but up-to-date evidence in children is scarce. The aim of the study was to analyze the influence of pediatric LTx on the course of UC.

Methods

We retrospectively analyzed the data of children with PSC and UC who underwent LTx in our institution between 2000 and 2013. In all patients PSC was confirmed by endoscopic retrograde cholangiopancreatography (ERCP) and/or magnetic resonance cholangiopancreatography (MRCP) and liver biopsy. UC diagnosis was based on clinical presentation, endoscopy and histology.

Results

Seven patients (4 male/3 female) with PSC and UC underwent LTx from deceased donors at the median age of 15,6 years (11,5-17,5). In 6 patients UC was diagnosed before LTx at the median age of 11 years (5-12), and one patient developed UC six months after LTx. Six patients (85%) had pancolitis at presentation and 1 proctosigmoiditis. Severe macroscopic inflammation was present in 5 (71%) cases. Initial treatment consisted of mesalazyne or sulfasalazine in combination with steroids and azathioprine. All patients received ursodeoxycholic acid once PSC was diagnosed and it was continued after LTx. None of the patient received biological treatment.

At the moment of transplantation (median 4 year after onset of UC, range 5-11) clinical activity according to PUCAI was remission in 4, mild in 2 and moderate in 1 case. All patients received deceased donor, AB0 compatible, whole liver grafts. Biliary anastomosis was hepaticojejunostomy in 6 and duct-to-duct anastomosis in 1 patient. Post transplant immunosuppressive regiment consisted of tacrolimus (n=4), cyclosporine (n=3), mycofenolate mofetil (n=5), azathioprine (n=1) and steroids (n=6). Neither of the patients developed colorectal neoplasia/cancer, nor underwent colectomy within median post-transplant follow-up of 7,3 years (1,3-13). Currently full remission is maintained in 4 (57%). One patient has moderate UC activity and another one with severe endoscopic inflammation was recently switched from tacrolimus to cyclosporine. Recurrence of PSC after LTx was not observed. One patient developed antiphospholipid syndrome with severe thrombosis requiring re-transplantation 7 years after the first LTx. One patient was deceased due to chronic rejection as a result of non-compliance. Up-to date overall patient and graft survival is 85% and 71% respectively.

Conclusion

The course of UC is not worsened by liver transplantation for PSC and likewise UC does not adversely affect patient or graft survival.