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Endocrine Abstracts (2015) 39 OC8.1 | DOI: 10.1530/endoabs.39.OC8.1

BSPED2015 ORAL COMMUNICATIONS Oral Communications 8 (5 abstracts)

4 year outcome of combined ‘en bloc’ liver-pancreas transplant in two adolescents with cystic fibrosis

Moira Cheung , Fiona Bartlett , Hilary Wyatt , Charles Buchanan & Ritika Kapoor


Kings College Hospital, London, UK.


Background: Cystic fibrosis related diabetes (CFRD), a common complication of CF, contributes to increased morbidity and mortality and is a poor prognostic indicator. Whilst liver transplant is a well-established treatment for end stage liver disease (ESLD) in CF, there are few reports of simultaneous pancreatic transplant in the paediatric population. We report the nutritional and endocrine outcomes of two adolescent CF patients who underwent combined liver and pancreas transplantation.

Case Presentation: A 14 year male and 16 year old female CF patient presented with end stage liver disease and CFRD and became eligible for liver transplantation. Insulin requirements were 0.5 and 2.1 units/kg per day respectively. Both required pancreatic enzyme replacement therapy (PERT) with requirements of 9200 and 7600 IU lip/kg per day and supplemental overnight nutritional support. During combined liver-pancreas transplantation, intravenous insulin infusions were required to maintain euglycaemia and were able to be discontinued soon after surgery. Both patients remain off insulin beyond 4 years post-transplant, OGTT 120 min glucose levels (3.3 and 3.6 mmol/l respectively) and HbA1c (DCCT – 5.1 and 4.6%) were within normal range. PERT requirements decreased to <1000 and 0 IU lip kg/day. Previous mid-upper arm circumferences increased from below 5th Centile prior to transplant to the 25 and 75 centile post-transplant. BMI (initially confounded by massive hepatosplenomegaly) prior to transplantation was 18.1 (z-score −0.4) and 20.2 (z-score −0.1). Post-transplant BMI peaked to 26.91 (z-score 2.09) and 23.4 (z-score 0.6).

Conclusion: These cases demonstrate that endocrine and exocrine functions were restored with subsequent improvement in nutritional status in both patients through combined liver-pancreas transplant. This was sustained beyond 4 years suggesting good prognosis for these patients. This procedure has technical advantages over isolated liver and pancreas transplantation, making it an appealing option to treat ESLD in the presence of CFRD.

Volume 39

43rd Meeting of the British Society for Paediatric Endocrinology and Diabetes

British Society for Paediatric Endocrinology and Diabetes 

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