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Cystic Fibrosis Associated with Worse Survival After Liver Transplantation

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Abstract

Background

Survival in cystic fibrosis patients after liver transplantation and liver–lung transplantation is not well studied.

Aims

To discern survival rates after liver transplantation and liver–lung transplantation in patients with and without cystic fibrosis.

Methods

The United Network for Organ Sharing database was queried from 1987 to 2013. Univariate Cox proportional hazards, multivariate Cox models, and propensity score matching were performed.

Results

Liver transplant and liver–lung transplant were performed in 212 and 53 patients with cystic fibrosis, respectively. Univariate Cox proportional hazards regression identified lower survival in cystic fibrosis after liver transplant compared to a reference non-cystic fibrosis liver transplant cohort (HR 1.248; 95 % CI 1.012, 1.541; p = 0.039). Supplementary analysis found graft survival was similar across the 3 recipient categories (log-rank test: χ2 2.68; p = 0.262). Multivariate Cox models identified increased mortality hazard among cystic fibrosis patients undergoing liver transplantation (HR 2.439; 95 % CI 1.709, 3.482; p < 0.001) and liver–lung transplantation (HR 2.753; 95 % CI 1.560, 4.861; p < 0.001). Propensity score matching of cystic fibrosis patients undergoing liver transplantation to non-cystic fibrosis controls identified a greater mortality hazard in the cystic fibrosis cohort using a Cox proportional hazards model stratified on matched pairs (HR 3.167; 95 % CI 1.265, 7.929, p = 0.014).

Conclusions

Liver transplantation in cystic fibrosis is associated with poorer long-term patient survival compared to non-cystic fibrosis patients, although the difference is not due to graft survival.

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References

  1. O’Sullivan BP, Freedman SD. Cystic fibrosis. Lancet. 2009;373:1891–1904.

    Article  PubMed  Google Scholar 

  2. Cystic Fibrosis Foundation. Cystic Fibrosis Foundation Patient Registry Annual Data Report 2012. Available at: http://www.cff.org/livingwithcf/qualityimprovement/patientregistryreport/, Accessed October 24, 2014.

  3. Lindblad A, Glaumann H, Strandvik B. Natural history of liver disease in cystic fibrosis. Hepatology. 1999;30:1151–1158.

    Article  CAS  PubMed  Google Scholar 

  4. Bartlett JR, Friedman KJ, Ling SC, et al. Genetic modifiers of liver disease in cystic fibrosis. JAMA. 2009;302:1076–1083.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  5. Cox KL, Ward RE, Furgiuele TL, et al. Orthotopic liver transplantation in patients with cystic fibrosis. Pediatrics. 1987;80:571–574.

    CAS  PubMed  Google Scholar 

  6. Murray KF, Carithers RL. Jr; AASLD. AASLD practice guidelines: evaluation of the patient for liver transplantation. Hepatology. 2005;41:1407–1432.

    Article  PubMed  Google Scholar 

  7. Couetil JP, Houssin DP, Soubrane O, et al. Combined lung and liver transplantation in patients with cystic fibrosis. A 4 1/2-year experience. J Thorac Cardiovasc Surg. 1995;110:1415–1422. (discussion 1422–1423).

    Article  CAS  PubMed  Google Scholar 

  8. Yi SG, Burroughs SG, Loebe M, et al. Combined lung and liver transplantation: analysis of a single-center experience. Liver Transpl. 2014;20:46–53.

    Article  PubMed  Google Scholar 

  9. Henn C, Kapellen T, Prenzel F, et al. Combined heterotopic liver–pancreas transplantation as a curative treatment for liver cirrhosis and diabetes mellitus in cystic fibrosis. Pediatr Transpl. 2014;18:E6–E9.

    Article  Google Scholar 

  10. Bandsma RH, Bozic MA, Fridell JA, et al. Simultaneous liver–pancreas transplantation for cystic fibrosis-related liver disease: a multicenter experience. J Cyst Fibros. 2014;13:471–477.

    Article  CAS  PubMed  Google Scholar 

  11. Dowman JK, Watson D, Loganathan S, et al. Long-term impact of liver transplantation on respiratory function and nutritional status in children and adults with cystic fibrosis. Am J Transplant. 2012;12:954–964.

    Article  CAS  PubMed  Google Scholar 

  12. Harring TR, Nguyen NT, Liu H, et al. Liver transplantation in cystic fibrosis: a report from Baylor College of Medicine and the Texas Children’s Hospital. Pediatr Transplant. 2013;17:271–277.

    Article  CAS  PubMed  Google Scholar 

  13. Desai CS, Gruessner A, Habib S, et al. Survival of cystic fibrosis patients undergoing liver and liver–lung transplantations. Transplant Proc. 2013;45:290–292.

    Article  CAS  PubMed  Google Scholar 

  14. Milkiewicz P, Skiba G, Kelly D, et al. Transplantation for cystic fibrosis: outcome following early liver transplantation. J Gastroenterol Hepatol. 2002;17:208–213.

    Article  PubMed  Google Scholar 

  15. United Network for Organ Sharing/Organ Procurement and Transplantation Network Standard Transplant Analysis and Research Database. Available at: http://optn.transplant.hrsa.gov/data/about/OPTNDatabase.asp, Accessed September 6, 2013.

  16. Mendizabal M, Reddy KR, Cassuto J, et al. Liver transplantation in patients with cystic fibrosis: analysis of United Network for Organ Sharing data. Liver Transpl. 2011;17:243–250.

    Article  PubMed  Google Scholar 

  17. Debray D, Kelly D, Houwen R, et al. Best practice guidance for the diagnosis and management of cystic fibrosis-associated liver disease. J Cyst Fibros. 2011;10:S29–S36.

    Article  PubMed  Google Scholar 

  18. Noble-Jamieson G, Barnes N, Jamieson N, et al. Liver transplantation for hepatic cirrhosis in cystic fibrosis. J R Soc Med. 1996;89:31–37.

    PubMed  PubMed Central  Google Scholar 

  19. Milkiewicz P, Skiba G, Kelly D, et al. Transplantation for cystic fibrosis: outcome following early liver transplantation. J Gastroenterol Hepatol. 2002;17:208–213.

    Article  PubMed  Google Scholar 

  20. Fridell JA, Bond GJ, Mazariegos GV, et al. Liver transplantation in children with cystic fibrosis: a long-term longitudinal review of a single center’s experience. J Pediatr Surg. 2003;38:1152–1156.

    Article  PubMed  Google Scholar 

  21. Miller MR, Sokol RJ, Narkewicz MR, et al. Pulmonary function in individuals who underwent liver transplantation: from the US cystic fibrosis foundation registry. Liver Transpl. 2012;18:585–593.

    Article  PubMed  Google Scholar 

  22. Colombo C, Costantini D, Rocchi A, et al. Effects of liver transplantation on the nutritional status of patients with cystic fibrosis. Transpl Int. 2005;18:246–255.

    Article  PubMed  Google Scholar 

  23. Weill D, Benden C, Corris PA, et al. A consensus document for the selection of lung transplant candidates: 2014–an update from the Pulmonary Transplantation Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant. 2015;34:1–15.

    Article  PubMed  Google Scholar 

  24. Hook JL, Lederer DJ. Selecting lung transplant candidates: where do current guidelines fall short? Expert Rev Respir Med. 2012;6:51–61.

    Article  PubMed  PubMed Central  Google Scholar 

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Authors and Affiliations

  1. Department of Surgery, The Ohio State University College of Medicine, Columbus, OH, USA

    Sylvester M. Black, Bryan A. Whitson & Don Hayes Jr.

  2. Department of Pediatrics, The Ohio State University College of Medicine, Columbus, OH, USA

    Frederick W. Woodley, Dmitry Tumin & Don Hayes Jr.

  3. Department of Anesthesiology, The Ohio State University College of Medicine, Columbus, OH, USA

    Joseph D. Tobias

  4. Department of Internal Medicine, The Ohio State University College of Medicine, Columbus, OH, USA

    Khalid Mumtaz & Don Hayes Jr.

  5. Center for Epidemiology of Organ Failure and Transplantation, Nationwide Children’s Hospital, The Ohio State University, 700 Children’s Drive, Columbus, OH, 43205, USA

    Sylvester M. Black, Frederick W. Woodley, Dmitry Tumin, Khalid Mumtaz, Bryan A. Whitson, Joseph D. Tobias & Don Hayes Jr.

  6. Section of Pulmonary Medicine, Nationwide Children’s Hospital, The Ohio State University, Columbus, OH, 43205, USA

    Don Hayes Jr.

  7. Section of Gastroenterology, Hepatology, and Nutrition, Nationwide Children’s Hospital, The Ohio State University, Columbus, OH, 43205, USA

    Frederick W. Woodley

  8. Department of Anesthesiology and Pain Medicine, Nationwide Children’s Hospital, The Ohio State University, 700 Children’s Drive, Columbus, OH, 43205, USA

    Dmitry Tumin & Joseph D. Tobias

Authors
  1. Sylvester M. Black
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  2. Frederick W. Woodley
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  3. Dmitry Tumin
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  4. Khalid Mumtaz
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Correspondence to Don Hayes Jr..

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Black, S.M., Woodley, F.W., Tumin, D. et al. Cystic Fibrosis Associated with Worse Survival After Liver Transplantation. Dig Dis Sci 61, 1178–1185 (2016). https://doi.org/10.1007/s10620-015-3968-2

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  • DOI: https://doi.org/10.1007/s10620-015-3968-2

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