Crull, M.H. (2012) Do children with cystic fibrosis related end stage liver disease benefit from combinedliver-pancreas transplant? thesis, Medicine.
Full text available on request.Abstract
Introduction: Liver disease is the second most important cause of death in cystic fibrosis (CF) with liver transplantation being the only effective treatment. Over 85% of CF patients develop exocrine pancreatic insufficiency of which more than 40% develop CF related diabetes (CFRD). Exocrine and endocrine pancreas insufficiency is associated with poor growth, pulmonary decline and increased mortality. Only seven cases of CF patients receiving combined liver pancreas transplantation (cLPTx) have been described in literature so far. Our aim was to evaluate the clinical outcome of cLPTx compared to isolated liver transplantation (iLTx) in children with CF related liver disease. Material and methods: Through a multinational survey across North-America, Europe and Oceania, 4 centers were identified with experience in performing cLPTx who participated in our study. We developed our own homemade questionnaire to obtain clinical data on patients who underwent iLTx or cLPTx in those centers. Pre transplant and post transplant information about height, weight and specific organ function of lung, kidney, liver and pancreas were obtained. Results: A total of 8 patients with iLTx (75% male, median age at time of iLTx 13 years with a range of 0-16 years) and 8 patients with cLPTx (25% male, median age at time of cLPTx 15 years with a range of 12-22 years) were included. Pre transplant parameters in terms of lung and kidney function, growth and body mass index (BMI) were similar in both groups. Endocrine pancreas insufficiency was diagnosed in 1 out of 8 patients pre iLTx and 8 out of 8 patients pre cLPTx. Median post transplantation follow up was 19 months (range 3-114 months). cLPTx restored exocrine and endocrine pancreatic function in all patients. At least one episode of hepatic acute cellular rejection developed in 4 out of 8 patients with iLTx compared to 2 out of 8 patients with cLPTx. Two patients developed CFRD post iLTx. Other than a significant difference in Z-score BMI one year post transplantation between patients receiving iLTx and cLPTx, no other clear post transplant differences were found in anthropometry, lung, liver or kidney function between the two groups. Conclusions: Combined liver pancreas transplantation appears to be effective in restoring pancreatic function in children with CF, is associated with increase in BMI in these patients and does not seem to be associated with increased post transplant morbidity. This procedure should be considered in patients with CF related liver disease.
| Item Type: | Thesis (Thesis) |
|---|---|
| Supervisor name: | Bandsma, R.H.J. Md. Phd. and Division of Hepatology, Gastroenterology and Nutrition UMCG |
| Faculty: | Medical Sciences |
| Date Deposited: | 25 Jun 2020 10:58 |
| Last Modified: | 25 Jun 2020 10:58 |
| URI: | https://umcg.studenttheses.ub.rug.nl/id/eprint/1847 |
Actions (login required)
 |
View Item |