Abstract
Purpose
To expand our knowledge on liver transplantation for cirrhosis associated with cystic fibrosis in adults.
Methods
Five patients who underwent a liver transplantation due to cystic fibrosis were reviewed. The outcome of the patients in terms of age, immunosuppression regimen, patient and graft survival, and pre- and post-transplant complications were investigated.
Results
Five adult liver transplant patients had cystic fibrosis (0.2%). These included 4 men and 1 woman with a mean age of 31 ± 10, ranging from 22 to 52 years old at the time of transplantation. All patients had lung problems. Four patients had exocrine and two had endocrine pancreatic insufficiency. Two are currently alive with a follow-up of 5.8 years and 4 months after transplantation, respectively. There were three deaths from pulmonary embolism at 4.5 years, myocardial infarction with cyclosporine nephrotoxicity at 10.7 years, and lymphoproliferative disorder at 5 months after transplantation. No deaths occurred from lung infection. Only one patient had postoperative pulmonary infectious complications, which were successfully treated with antibiotics and did not result in mortality.
Conclusion
Adult liver transplantation for end-stage liver disease associated with cystic fibrosis offers encouraging results with a rapid general improvement after surgery and it is now considered to be a safe and acceptable treatment for this disease population.
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References
EP Molmenti RH Squires D Nagata JS Roden H Molmenti CG Fasola et al. (2003) ArticleTitleLiver transplantation for cholestasis associated with cystic fibrosis in the pediatric population Pediatr Transplant 7 93–7 Occurrence Handle12654048 Occurrence Handle10.1034/j.1399-3046.2003.00021.x
E Pfister A Strassburg B Nashan T Becker M Ballmann A Arning et al. (2002) ArticleTitleLiver transplantation for liver cirrhosis in cystic fibrosis Transplant Proc 34 2281–2 Occurrence Handle12270399 Occurrence Handle10.1016/S0041-1345(02)03236-0 Occurrence Handle1:STN:280:DC%2BD38votlentA%3D%3D
JA Fridell GJ Bond GV Mazariegos DM Orenstein A Jain R Sindhi et al. (2003) ArticleTitleLiver transplantation in children with cystic fibrosis: a long-term longitudinal review of a single center's experience J Pediatr Surg 38 1152–6 Occurrence Handle12891484 Occurrence Handle10.1016/S0022-3468(03)00260-4
R Chavez-Cartaya M Drage NV Jamieson P Friend G Noble-Jamieson R Levy et al. (1999) ArticleTitleBiliary complications in liver transplantation for cystic fibrosis Transplant Proc 31 2411–2 Occurrence Handle10500646 Occurrence Handle10.1016/S0041-1345(99)00407-8 Occurrence Handle1:STN:280:DyaK1MvjtVCgsQ%3D%3D
MD Pescovitz JG Puente RM Jindal J Fitzgerald SK Chong ML Milgrom et al. (1996) ArticleTitleImproved absorption of cyclosporine for microemulsion in a pediatric liver transplant recipient with cystic fibrosis Transplantation 61 331–3 Occurrence Handle8600647 Occurrence Handle10.1097/00007890-199601270-00030 Occurrence Handle1:CAS:528:DyaK28XhsV2msb8%3D
T Lamireau S Monnereau S Martin JE Marcotte M Winnock F Alvarez (2004) ArticleTitleEpidemiology of liver disease in cystic fibrosis: a longitudinal study J Hepatol 41 920–5 Occurrence Handle15582124 Occurrence Handle10.1016/j.jhep.2004.08.006
DL Waters SF Dorney MA Gruca HC Martin R Howman-Giles AE Kan et al. (1995) ArticleTitleHepatobiliary disease in cystic fibrosis patients with pancreatic sufficiency Hepatology 21 963–9 Occurrence Handle7535738 Occurrence Handle1:STN:280:ByqB38%2FovVQ%3D
JR Yankaskas BC Marshall B Sufian RH Simon D Rodman (2004) ArticleTitleCystic fibrosis adult care: consensus conference report Chest 125 1S–39S Occurrence Handle14734689 Occurrence Handle10.1378/chest.125.1_suppl.1S
M Kulich M Rosenfeld CH Goss R Wilmott (2003) ArticleTitleImproved survival among young patients with cystic fibrosis J Pediatr 142 631–6 Occurrence Handle12838190 Occurrence Handle10.1067/mpd.2003.197
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Ikegami, T., Sanchez, E., Uemura, T. et al. Liver Transplantation for Cystic Fibrosis in Adults. Surg Today 38, 26–29 (2008). https://doi.org/10.1007/s00595-007-3576-7
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DOI: https://doi.org/10.1007/s00595-007-3576-7