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Published ahead of print on December 30, 2005, doi:10.1164/rccm.200410-1369OC

Am. J. Respir. Crit. Care Med., Volume 173, Number 6, March 2006, 659-666

A more recent version of this article appeared on March 15, 2006
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Submitted on October 13, 2004
Accepted on December 16, 2005

Risk Factors for Death in Cystic Fibrosis Patients Awaiting Lung Transplantation

Richard A Belkin1*, Noreen R Henig2, Lianne G Singer3, Cecilia Chaparro3, Ronald C Rubenstein4, Sharon X Xie5, Justin Y Yee6, Robert M Kotloff6, David A Lipson6, and Greta R Bunin7

1 Division of Pulmonary, Allergy and Critical Care Medicine, University of Pennsylvania, Philadelphia, PA, USA; Division of Biostatistics, University of Pennsylvania Medical Center, Center for Clinical Epidemiology and Biostatistics, Philadelphia, PA, USA, 2 Division of Pulmonary and Critical Care Medicine, Stanford University Medical Center, Stanford, CA, USA, 3 Division of Respirology, University Health Network and University of Toronto, Toronto, ON, Canada, 4 Division of Pulmonary Medicine, Children's Hospital of Philadelphia, Philadelphia, PA, USA; Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, PA, USA, 5 Division of Biostatistics, University of Pennsylvania Medical Center, Center for Clinical Epidemiology and Biostatistics, Philadelphia, PA, USA, 6 Division of Pulmonary, Allergy and Critical Care Medicine, University of Pennsylvania, Philadelphia, PA, USA, 7 Division of Oncology, University of Pennsylvania Medical Center, Children's Hospital of Philadelphia and Center for Clinical Epidemiology and Biostatistics, Philadelphia, PA, USA

* To whom correspondence should be addressed. E-mail: rbelkin{at}sansumclinic.org.

Rationale: Controversy exists over optimal timing for listing of cystic fibrosis patients for lung transplantation. Objectives: We conducted a retrospective cohort study of 343 patients listed for lung transplantation at four academic medical centers to identify risk factors for patient death while awaiting transplantation. Methods: Data on possible risk factors were abstracted from medical records. Measurements: Time to death, patient demographic characteristics, biomedical/biological risk factors for patient death while awaiting transplantation were assessed. Univariate and multivariate survival analyses were performed using Cox regression. Results: In the univariate analyses, FEV1≤30% predicted (HR 3.8; 95%CI 2.0-7.5), PaCO2≥50mmHg (HR 1.85; 95%CI 1.1-3.0) and shorter height (HR 1.8; 95%CI 1.1-3.0) were associated with a higher risk of death. Referral from an accredited cystic fibrosis center was associated with a lower risk of death (HR 0.53; 95%CI 0.30-0.92). The final multivariate model included FEV1≤30% predicted (HR 6.8, 95% CI 2.4-19.3), PaCO2≥50mmg (HR 6.9, 95% CI 1.5-32.1), referral from an accredited cystic fibrosis center (HR 0.5; 95% CI 0.3-0.9), and listing year after 1996 (HR 0.4 95% CI 0.2-0.7) They were all associated with a lower risk of death. Use of a nutritional intervention was associated with a higher risk of death (HR 2.3 95% CI 1.3-4.1). Interestingly, patients with an FEV1>30% predicted had a significantly higher risk of death only when their PaCO2 was ≥50mmHg (HR 9.6; 95%CI 2.20-41.0). Conclusions: We identified risk factors for waiting list mortality that could impact upon transplant referral, listing and allocation guidelines.
Key words: Cystic Fibrosis, Lung transplantation, Survival




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