Survival Comparison of Patients With Cystic Fibrosis in Canada and the United States: A Population-Based Cohort Study
Open accessAbstract
BACKGROUND: In 2011, the median age of survival of patients with cystic fibrosis reported in the United States was 36.8 years, compared with 48.5 years in Canada. Direct comparison of survival estimates between national registries is challenging because of inherent differences in methodologies used, data processing techniques, and ascertainment bias. OBJECTIVE: To use a standardized approach to calculate cystic fibrosis survival estimates and to explore differences between Canada and the United States. DESIGN: Population-based study. SETTING: 42 Canadian cystic fibrosis clinics and 110 U.S. cystic fibrosis care centers. PATIENTS: Patients followed in the Canadian Cystic Fibrosis Registry (CCFR) and U.S. Cystic Fibrosis Foundation Patient Registry (CFFPR) between 1990 and 2013. MEASUREMENTS: Cox proportional hazards models were used to compare survival between patients followed in the CCFR (n = 5941) and those in the CFFPR (n = 45 448). Multivariable models were used to adjust for factors known to be associated with survival. RESULTS: Median age of survival in patients with cystic fibrosis increased in both countries between 1990 and 2013; however, in 1995 and 2005, survival in Canada increased at a faster rate than in the United States (P < 0.001). On the basis of contemporary data from 2009 to 2013, the median age of survival in Canada was 10 years greater than in the United States (50.9 vs. 40.6 years, respectively). The adjusted risk for death was 34% lower in Canada than the United States (hazard ratio, 0.66 [95% CI, 0.54 to 0.81]). A greater proportion of patients in Canada received transplants (10.3% vs. 6.5%, respectively [standardized difference, 13.7]). Differences in survival between U.S. and Canadian patients varied according to U.S. patients' insurance status. LIMITATION: Ascertainment bias due to missing data or nonrandom loss to follow-up might affect the results. CONCLUSION: Differences in cystic fibrosis survival between Canada and the United States persisted after adjustment for risk factors associated with survival, except for private-insurance status among U.S. patients. Differential access to transplantation, increased posttransplant survival, and differences in health care systems may, in part, explain the Canadian survival advantage. PRIMARY FUNDING SOURCE: U.S. Cystic Fibrosis Foundation.
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Cites
Establishing a standard definition for child overweight and obesity worldwide: international survey
Cole, T. J. (2000) · BMJ · doi:10.1136/bmj.320.7244.1240 · Cited by 16,500 · see citing works
<b>mice</b>: Multivariate Imputation by Chained Equations in<i>R</i>
Buuren, S. V., & Groothuis-Oudshoorn, K. (2011) · Journal of Statistical Software · doi:10.18637/jss.v045.i03 · Cited by 13,870 · see citing works
CDC growth charts: United States.
Kuczmarski, R. J., Ogden, C. L., Grummer‐Strawn, L. M., Flegal, K. M., Guo, S. S., Wei, R. X., Mei, Z., Curtin, L. R., Roche, A. F., & Johnson, C. L. (2000) · PubMed · Cited by 5,298 · see citing works
Estimating regression models with unknown break‐points
Muggeo, V. M. R. (2003) · Statistics in Medicine · doi:10.1002/sim.1545 · Cited by 2,408 · see citing works
Normand, S., Landrum, M., Guadagnoli, E., Ayanian, J. Z., Ryan, T. J., Cleary, P., & McNeil, B. J. (2001) · Journal of Clinical Epidemiology · doi:10.1016/s0895-4356(00)00321-8 · Cited by 1,256 · see citing works
Farrell, P. M., Rosenstein, B. J., White, T. B., Accurso, F. J., Castellani, C., Cutting, G. R., Durie, P. R., LeGrys, V. A., Massie, J., Parad, R. B., et al. (2008) · The Journal of Pediatrics · doi:10.1016/j.jpeds.2008.05.005 · Cited by 1,077 · see citing works
Corey, M. L., McLaughlin, F. J., Williams, M., & Levison, H. (1988) · Journal of Clinical Epidemiology · doi:10.1016/0895-4356(88)90063-7 · Cited by 812 · see citing works
Development of the New Lung Allocation System in the United States
Egan, T. M., Murray, S., Bustami, R. T., Shearon, T. H., McCullough, K. P., Edwards, L. B., Coke, M. A., Garrity, E. R., Sweet, S. C., Heiney, D. A., et al. (2006) · American Journal of Transplantation · doi:10.1111/j.1600-6143.2006.01276.x · Cited by 767 · see citing works
Predictive 5-year survivorship model of cystic fibrosis
Liou, T. G., Adler, F. R., FitzSimmons, S. C., Cahill, B. C., Hibbs, J. R., & Marshall, B. C. (2001) · American Journal of Epidemiology · doi:10.1093/aje/153.4.345 · Cited by 749 · see citing works
Self-citationReference Ranges for Spirometry Across All Ages
Stanojevic, S., Wade, A. M., Stocks, J., Hankinson, J., Coates, A. L., Pan, H., Rosenthal, M., Corey, M. L., Lebecque, P., & Cole, T. J. (2007) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.200708-1248oc · Cited by 699 · see citing works
Self-citationDeterminants of Mortality from Cystic Fibrosis in Canada, 1970-1989
Corey, M., & Farewell, V. T. (1996) · American Journal of Epidemiology · doi:10.1093/oxfordjournals.aje.a008664 · Cited by 361 · see citing works
MacKenzie, T. A., Gifford, A. H., Sabadosa, K. A., Quinton, H. B., Knapp, E. A., Goss, C. H., & Marshall, B. C. (2014) · Annals of Internal Medicine · doi:10.7326/m13-0636 · Cited by 352 · see citing works
Self-citationSchechter, M. S., Shelton, B. J., Margolis, P. A., & FitzSimmons, S. C. (2001) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/ajrccm.163.6.9912100 · Cited by 347 · see citing works
Yen, E. H., Quinton, H., & Borowitz, D. (2013) · The Journal of Pediatrics · doi:10.1016/j.jpeds.2012.08.040 · Cited by 306 · see citing works
Diagnosis, screening and management of cystic fibrosis related diabetes mellitus
Moran, A., Hardin, D. S., Rodman, D. M., Allen, H. F., Beall, R. J., Borowitz, D., Brunzell, C., Campbell, P., Chesrown, S. E., Duchow, C., et al. (1999) · Diabetes Research and Clinical Practice · doi:10.1016/s0168-8227(99)00058-3 · Cited by 290 · see citing works
Impact of U.S. Lung Allocation Score on Survival After Lung Transplantation
Merlo, C. A., Weiss, E. S., Orens, J. B., Borja, M. C., Diener-West, M., Conte, J. V., & Shah, A. S. (2009) · The Journal of Heart and Lung Transplantation · doi:10.1016/j.healun.2009.04.024 · Cited by 144 · see citing works
Improved survival at low lung function in cystic fibrosis: cohort study from 1990 to 2007
George, P. M., BANYA, W. A. S., Pareek, N., Bilton, D., Cullinan, P., Hodson, M. E., & Simmonds, N. J. (2011) · BMJ · doi:10.1136/bmj.d1008 · Cited by 137 · see citing works
Lai, H. J. (2004) · American Journal of Epidemiology · doi:10.1093/aje/kwh083 · Cited by 133 · see citing works
McCormick, J., Mehta, G., Olesen, H. V., Viviani, L., Maçek, M., & Mehta, A. (2010) · The Lancet · doi:10.1016/s0140-6736(09)62161-9 · Cited by 128 · see citing works
Nick, J. A., Chacon, C. S., Brayshaw, S. J., Jones, M. C., Barboa, C., Clair, C. G. S., Young, R. L., Nichols, D. P., Janssen, J. S., Huitt, G. A., et al. (2010) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201001-0092oc · Cited by 123 · see citing works
Stephenson, A. L., Sykes, J., Berthiaume, Y., Singer, L. G., Aaron, S. D., Whitmore, G. A., & Stanojevic, S. (2015) · The Journal of Heart and Lung Transplantation · doi:10.1016/j.healun.2015.05.003 · Cited by 102 · see citing works
Self-citationQuon, B. S., Psoter, K. J., Mayer-Hamblett, N., Aitken, M. L., Li, C. I., & Goss, C. H. (2012) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201205-0949oc · Cited by 100 · see citing works
Self-citationInternational Comparison of Median Age at Death From Cystic Fibrosis
Fogarty, A., Hubbard, R., & Britton, J. R. (2000) · CHEST Journal · doi:10.1378/chest.117.6.1656 · Cited by 91 · see citing works
Lung Allocation Score for Lung Transplantation
Gries, C., Mulligan, M. S., Edelman, J. D., Raghu, G., Curtis, J. R., & Goss, C. H. (2007) · CHEST Journal · doi:10.1378/chest.07-1160 · Cited by 81 · see citing works
Self-citationA statistical model to predict one-year risk of death in patients with cystic fibrosis
Aaron, S. D., Stephenson, A. L., Cameron, D. W., & Whitmore, G. A. (2014) · Journal of Clinical Epidemiology · doi:10.1016/j.jclinepi.2014.12.010 · Cited by 72 · see citing works
Self-citationRamos, K. J., Quon, B. S., Psoter, K. J., Lease, E. D., Mayer-Hamblett, N., Aitken, M. L., & Goss, C. H. (2015) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2015.11.005 · Cited by 67 · see citing works
Self-citationChildren and young adults with CF in the USA have better lung function compared with the UK
Goss, C. H., MacNeill, S., Quinton, H. B., Marshall, B. C., Elbert, A., Knapp, E. A., Petren, K., Gunn, E., Osmond, J., & Bilton, D. (2014) · Thorax · doi:10.1136/thoraxjnl-2014-205718 · Cited by 58 · see citing works
Self-citationComparative analysis of Cystic Fibrosis Registry data from the UK with USA, France and Australasia
McCormick, J., Sims, E. J., Green, M. W., Mehta, G., Culross, F., & Mehta, A. (2005) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2005.01.001 · Cited by 55 · see citing works
Comparison of the US and Australian Cystic Fibrosis Registries: The Impact of Newborn Screening
Martin, B., Schechter, M. S., Jaffé, A., Cooper, P., Bell, S. C., & Ranganathan, S. (2012) · PEDIATRICS · doi:10.1542/peds.2011-0567 · Cited by 46 · see citing works
Sykes, J. R., Stanojevic, S., Goss, C. H., Quon, B. S., Marshall, B. C., Petren, K., Ostrenga, J., Fink, A. K., Elbert, A., & Stephenson, A. L. (2015) · Journal of Clinical Epidemiology · doi:10.1016/j.jclinepi.2015.08.026 · Cited by 39 · see citing works
Self-citationINCIDENCE AND SURVIVAL CURVES OF CYSTIC FIBROSIS IN SWEDEN
Kollberg, H. (1982) · Acta Paediatrica · doi:10.1111/j.1651-2227.1982.tb09399.x · Cited by 32 · see citing works
National Healthcare Delivery Systems Influence Lung Transplant Outcomes for Cystic Fibrosis
Merlo, C. A., Clark, S. C., Arnaoutakis, G. J., Yonan, N. A., Thomas, D., Simon, A. R., Thompson, R. D., Thomas, H., Orens, J. B., & Shah, A. S. (2015) · American Journal of Transplantation · doi:10.1111/ajt.13226 · Cited by 28 · see citing works
Jackson, A. D., Daly, L., Kelleher, C., Marshall, B. C., Quinton, H. B., Foley, L., & Fitzpatrick, P. (2010) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2010.08.021 · Cited by 23 · see citing works
Self-citationComparison of sputum processing techniques in cystic fibrosis
Cai, Y., Carty, K., Gibson, P. G., & Henry, R. L. (1996) · Pediatric Pulmonology · doi:10.1002/(sici)1099-0496(199612)22:6 · Cited by 18 · see citing works
Flagged first, then by citations
Cited by
Sorted by citations
The future of cystic fibrosis care: a global perspective
Bell, S. C., Mall, M. A., Gutierrez, H., Macek, M., Madge, S. L., Davies, J. C., Burgel, P., Tullis, E., Castaños, C., Castellani, C., et al. (2019) · The Lancet Respiratory Medicine · doi:10.1016/s2213-2600(19)30337-6 · Cited by 1,042 · see citing works
Self-citationCFTR Modulators: The Changing Face of Cystic Fibrosis in the Era of Precision Medicine
Lopes‐Pacheco, M. (2020) · Frontiers in Pharmacology · doi:10.3389/fphar.2019.01662 · Cited by 571 · see citing works
Grasemann, H., & Ratjen, F. A. (2023) · New England Journal of Medicine · doi:10.1056/nejmra2216474 · Cited by 338 · see citing works
Epidemiology, Biology, and Impact of Clonal Pseudomonas aeruginosa Infections in Cystic Fibrosis
Parkins, M. D., Somayaji, R., & Waters, V. J. (2018) · Clinical Microbiology Reviews · doi:10.1128/cmr.00019-18 · Cited by 252 · see citing works
The Relationship of Health Insurance and Mortality: Is Lack of Insurance Deadly?
Woolhandler, S. J., & Himmelstein, D. U. (2017) · Annals of Internal Medicine · doi:10.7326/m17-1403 · Cited by 240 · see citing works
Farooq, F., Mogayzel, P. J., Lanzkron, S., Haywood, C., & Strouse, J. J. (2020) · JAMA Network Open · doi:10.1001/jamanetworkopen.2020.1737 · Cited by 187 · see citing works
Cystic Fibrosis-Related Diabetes
Kayani, K., Mohammed, R., & Mohiaddin, H. (2018) · Frontiers in Endocrinology · doi:10.3389/fendo.2018.00020 · Cited by 103 · see citing works
Sputum microbiota is predictive of long-term clinical outcomes in young adults with cystic fibrosis
Acosta, N., Heirali, A., Somayaji, R., Surette, M. G., Workentine, M. L., Sibley, C. D., Rabin, H. R., & Parkins, M. D. (2018) · Thorax · doi:10.1136/thoraxjnl-2018-211510 · Cited by 93 · see citing works
Epidemiology of CF: How registries can be used to advance our understanding of the CF population
Jackson, A. D., & Goss, C. H. (2017) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2017.11.013 · Cited by 92 · see citing works
Self-citationRayment, J. H., Couch, M. J., McDonald, N., Kanhere, N., Manson, D. E., Santyr, G. E., & Ratjen, F. A. (2019) · European Respiratory Journal · doi:10.1183/13993003.02188-2018 · Cited by 86 · see citing works
The changing epidemiology and demography of cystic fibrosis
Stephenson, A. L., Stanojevic, S., Sykes, J. R., & Burgel, P. (2017) · La Presse Médicale · doi:10.1016/j.lpm.2017.04.012 · Cited by 85 · see citing works
Self-citationde Macedo, A. N., Mathiaparanam, S., Brick, L., Keenan, K., Gonska, T. Y., Pedder, L., Hill, S., & Britz‐McKibbin, P. (2017) · ACS Central Science · doi:10.1021/acscentsci.7b00299 · Cited by 80 · see citing works
Coronavirus Disease 2019 (COVID-19) Pandemic and Pregnancy Outcomes in a U.S. Population
Son, M., Gallagher, K., Lo, J. Y., Lindgren, E., Burris, H. H., Dysart, K., Greenspan, J. S., Culhane, J. F., & Handley, S. C. (2021) · Obstetrics and Gynecology · doi:10.1097/aog.0000000000004547 · Cited by 73 · see citing works
Rho, J., Ahn, C., Gao, A., Sawicki, G., Keller, A., & Jain, R. (2018) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201711-2357oc · Cited by 62 · see citing works
Corriveau, S., Sykes, J. R., & Stephenson, A. L. (2018) · Current Opinion in Pulmonary Medicine · doi:10.1097/mcp.0000000000000520 · Cited by 66 · see citing works
Self-citationThe Changing Epidemiology of Cystic Fibrosis
Burgel, P., Burnet, E., Regard, L., & Martin, C. (2022) · CHEST Journal · doi:10.1016/j.chest.2022.07.004 · Cited by 67 · see citing works
Goss, C. H., Sykes, J. R., Stanojevic, S., Marshall, B. C., Petren, K., Ostrenga, J., Fink, A. K., Elbert, A., Quon, B. S., & Stephenson, A. L. (2017) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201707-1541oc · Cited by 63 · see citing works
Self-citationHyperpolarized Gas Magnetic Resonance Imaging of Pediatric Cystic Fibrosis Lung Disease
Santyr, G. E., Kanhere, N., Morgado, F., Rayment, J. H., Ratjen, F. A., & Couch, M. J. (2018) · Academic Radiology · doi:10.1016/j.acra.2018.04.024 · Cited by 61 · see citing works
Neighborhood-Level Disadvantage Impacts on Patients with Fibrotic Interstitial Lung Disease
Goobie, G. C., Ryerson, C. J., Johannson, K. A., Schikowski, E., Zou, R. H., Khalil, N., Marcoux, V., Assayag, D., Manganas, H., Fisher, J. H., et al. (2021) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.202109-2065oc · Cited by 58 · see citing works
McKone, E. F., Ariti, C. A., Jackson, A., Zolin, A., Carr, S. B., Orenti, A., van Rens, J. G., Lemonnier, L., Maçek, M., Keogh, R. H., et al. (2021) · European Respiratory Journal · doi:10.1183/13993003.02288-2020 · Cited by 57 · see citing works
The Clinical Biology of Cystic Fibrosis Transmembrane Regulator Protein
Liou, T. G. (2018) · CHEST Journal · doi:10.1016/j.chest.2018.10.006 · Cited by 55 · see citing works
TGFβ as a therapeutic target in cystic fibrosis
Kramer, E. L., & Clancy, J. P. (2017) · Expert Opinion on Therapeutic Targets · doi:10.1080/14728222.2018.1406922 · Cited by 54 · see citing works
Gilmour, J. A., Sykes, J., Etchells, E., & Tullis, E. (2019) · Canadian Journal of Diabetes · doi:10.1016/j.jcjd.2018.04.008 · Cited by 51 · see citing works
Self-citationCystic Fibrosis: Emerging Understanding and Therapies
Rey, M. M., Bonk, M. P., & Hadjiliadis, D. (2018) · Annual Review of Medicine · doi:10.1146/annurev-med-112717-094536 · Cited by 50 · see citing works
Improving the quality of health care in Canada
Dhalla, I. A., & Tepper, J. D. (2018) · Canadian Medical Association Journal · doi:10.1503/cmaj.171045 · Cited by 49 · see citing works
Earnest, A., Salimi, F., Wainwright, C. E., Bell, S. C., Ruseckaite, R., Ranger, T. A., Kotsimbos, T. C., & Ahern, S. (2020) · Scientific Reports · doi:10.1038/s41598-020-74502-1 · Cited by 45 · see citing works
DiBattista, A., McIntosh, N., Lamoureux, M., Al-Dirbashi, O. Y., Chakraborty, P. K., & Britz‐McKibbin, P. (2018) · Journal of Proteome Research · doi:10.1021/acs.jproteome.8b00351 · Cited by 41 · see citing works
The Potential Causes of Cystic Fibrosis-Related Diabetes
Coderre, L., Debieche, L., Plourde, J., RABASA-LHORET, R. P. R., & Lesage, S. (2021) · Frontiers in Endocrinology · doi:10.3389/fendo.2021.702823 · Cited by 39 · see citing works
Precision Medicine In Action: The Impact Of Ivacaftor On Cystic Fibrosis–Related Hospitalizations
Feng, L. B., Grosse, S. D., Green, R. F., Fink, A. K., & Sawicki, G. S. (2018) · Health Affairs · doi:10.1377/hlthaff.2017.1554 · Cited by 38 · see citing works
Self-citationCystic fibrosis – an example of personalized and precision medicine
Skov, M., Hansen, C. R., & Pressler, T. (2019) · Apmis · doi:10.1111/apm.12915 · Cited by 36 · see citing works
Cystic fibrosis patient registries: A valuable source for clinical research
Dasenbrook, E. C., & Sawicki, G. S. (2018) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2018.03.001 · Cited by 35 · see citing works
Tai, W., Hu, L., & Wen, J. (2022) · Frontiers in Medicine · doi:10.3389/fmed.2022.837762 · Cited by 33 · see citing works
Inhaled Drug Therapy 2016: The Year in Review
Dhand, R. (2017) · Respiratory Care · doi:10.4187/respcare.05624 · Cited by 32 · see citing works
Data that empower: The success and promise of CF patient registries
Fink, A. K., Loeffler, D. R., Marshall, B. C., Goss, C. H., & Morgan, W. J. (2017) · Pediatric Pulmonology · doi:10.1002/ppul.23790 · Cited by 31 · see citing works
Self-citationLehr, C. J., Fink, A. K., Skeans, M. A., Faro, A., Fernandez, G. S., Dasenbrook, E. C., & Valapour, M. (2020) · Annals of the American Thoracic Society · doi:10.1513/annalsats.202001-030oc · Cited by 31 · see citing works
Self-citationBirth Hospital Length of Stay and Rehospitalization During COVID-19
Handley, S. C., Gallagher, K., Breden, A., Lindgren, E., Lo, J. Y., Son, M., Murosko, D. C., Dysart, K., Lorch, S. A., Greenspan, J. S., et al. (2021) · PEDIATRICS · doi:10.1542/peds.2021-053498 · Cited by 31 · see citing works
Singh, H., Jani, C., Marshall, D. C., Franco, R., Bhatt, P. S., Podder, S., Shalhoub, J., Kurman, J. S., Nanchal, R. S., Uluer, A., et al. (2023) · Scientific Reports · doi:10.1038/s41598-023-41868-x · Cited by 33 · see citing works
Stephenson, A. L., Ramos, K. J., Sykes, J., Ma, X., Stanojevic, S., Quon, B. S., Marshall, B. C., Petren, K., Ostrenga, J. S., Fink, A. K., et al. (2021) · The Journal of Heart and Lung Transplantation · doi:10.1016/j.healun.2020.12.001 · Cited by 30 · see citing works
Self-citationAn update on new and emerging therapies for cystic fibrosis
Hudock, K. M., & Clancy, J. P. (2017) · Expert Opinion on Emerging Drugs · doi:10.1080/14728214.2017.1418324 · Cited by 28 · see citing works
Jarvis, S. W., Roberts, D., Flemming, K., Richardson, G. A., & Fraser, L. (2021) · Pediatric Research · doi:10.1038/s41390-021-01396-8 · Cited by 28 · see citing works
Beyond borders: cystic fibrosis survival between Australia, Canada, France and New Zealand
Coriati, A., Ma, X., Sykes, J. R., Stanojevic, S., Ruseckaite, R., Lemonnier, L., Dehillotte, C., Tate, J., Byrnes, C. A., Bell, S. C., et al. (2022) · Thorax · doi:10.1136/thorax-2022-219086 · Cited by 28 · see citing works
Self-citationDesai, S., Wong, H., Sykes, J. R., Stephenson, A. L., Singer, J., & Quon, B. S. (2018) · Annals of the American Thoracic Society · doi:10.1513/annalsats.201801-037oc · Cited by 27 · see citing works
Self-citationBanjar, H. H., Tuleimat, L. M., Seoudi, A. A. A. E., Mogarri, I., Alhaider, S. A., Nizami, I. Y., Almaghamsi, T., AlKaf, S., & Moghrabi, N. N. (2020) · Annals of Saudi Medicine · doi:10.5144/0256-4947.2020.15 · Cited by 27 · see citing works
Ramos, K. J., Sykes, J. R., Stanojevic, S., Ma, X., Ostrenga, J. S., Fink, A. K., Quon, B. S., Marshall, B. C., Faro, A., Petren, K., et al. (2021) · CHEST Journal · doi:10.1016/j.chest.2021.04.010 · Cited by 24 · see citing works
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Thorat, T., McGarry, L. J., Bonafede, M., Limone, B. L., Rubin, J. L., Jariwala‐Parikh, K., & Konstan, M. W. (2021) · Pediatric Pulmonology · doi:10.1002/ppul.25535 · Cited by 24 · see citing works
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Ruseckaite, R., Salimi, F., Earnest, A., Bell, S. C., Douglas, T. A., Frayman, K. B., Keatley, L., King, S., Kotsimbos, T. C., Middleton, P. G., et al. (2022) · Scientific Reports · doi:10.1038/s41598-022-24374-4 · Cited by 24 · see citing works
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Perrem, L., Stanojevic, S., Solomon, M. P., Carpenter, S., & Ratjen, F. A. (2019) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2019.04.015 · Cited by 24 · see citing works
Self-citationGaffney, A. W., Steffie, W., Himmelstein, D. U., & McCormick, D. (2018) · BMC Health Services Research · doi:10.1186/s12913-018-3407-0 · Cited by 23 · see citing works
A guide to interpreting estimated median age of survival in cystic fibrosis patient registry reports
Keogh, R. H., & Stanojevic, S. (2018) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2017.11.014 · Cited by 22 · see citing works
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Advances in the Diagnosis and Management of Cystic Fibrosis in the Genomic Era
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Evaluation of a five-year predicted survival model for cystic fibrosis in later time periods
Liou, T. G., Kartsonaki, C., Keogh, R. H., & Adler, F. R. (2020) · Scientific Reports · doi:10.1038/s41598-020-63590-8 · Cited by 20 · see citing works
Zlosnik, J. E. A., Henry, D. A., Hird, T. J., Hickman, R., Campbell, M. E., Cabrera, A., Chiavegatti, G. L., Chilvers, M. A., & Sadarangani, M. (2020) · Annals of the American Thoracic Society · doi:10.1513/annalsats.201906-443oc · Cited by 20 · see citing works
Sanders, D. B., Khan, U., Heltshe, S. L., Skalland, M., West, N. E., VanDevanter, D. R., Goss, C. H., & Flume, P. A. (2021) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2021.11.009 · Cited by 19 · see citing works
Self-citationHobler, M. R., Engelberg, R. A., Curtis, J. R., Ramos, K. J., Zander, M. I., Howard, S. S., Goss, C. H., & Aitken, M. L. (2018) · Journal of Palliative Medicine · doi:10.1089/jpm.2017.0259 · Cited by 19 · see citing works
Self-citationUnmet needs in cystic fibrosis: the next steps in improving outcomes
West, N., & Flume, P. (2018) · Expert Review of Respiratory Medicine · doi:10.1080/17476348.2018.1483723 · Cited by 19 · see citing works
Jackson, A. D., Kirwan, L., Gibney, S., Jeleniewska, P., Fletcher, G., & Doyle, G. (2019) · European Journal of Public Health · doi:10.1093/eurpub/ckz148 · Cited by 19 · see citing works
Cheng, T. Z., Choi, K. J., Honeybrook, A. L., Zakare-Fagbamila, R. T., Gray, A., Snyder, L. D., Palmer, S. M., Abi-Hachem, R. N., & Jang, D. W. (2019) · American Journal of Rhinology and Allergy · doi:10.1177/1945892419830624 · Cited by 18 · see citing works
Dugger, D. T., Fung, M., Zlock, L. T., Caldera, S., Sharp, L. Z., Hays, S. R., Singer, J. P., Leard, L. E., Golden, J. A., Shah, R., et al. (2020) · Cell Reports Medicine · doi:10.1016/j.xcrm.2020.100055 · Cited by 18 · see citing works
Rubin, J. L., McKinnon, C. J., Pedra, G., Morgan, D. A., Zweig, K., & Liou, T. G. (2025) · Pulmonary Therapy · doi:10.1007/s41030-025-00303-4 · Cited by 19 · see citing works
Optimism, opportunities, outcomes: the Australian Cystic Fibrosis Data Registry
Ahern, S., Sims, G. C., Earnest, A., & Bell, S. C. (2018) · Internal Medicine Journal · doi:10.1111/imj.13807 · Cited by 16 · see citing works
Elborn, J. S. (2019) · Seminars in Respiratory and Critical Care Medicine · doi:10.1055/s-0039-3400289 · Cited by 16 · see citing works
Zampoli, M., Sykes, J. R., Verstraete, J., Cheng, S. Y., Morrow, B., Pepper, M. S., Stewart, C., Zar, H. J., & Stephenson, A. L. (2023) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2023.09.003 · Cited by 15 · see citing works
Self-citationDisease caused by non-tuberculous mycobacteria in children with cystic fibrosis
Lu, M., Saddi, V., Britton, P. N., Selvadurai, H. C., Robinson, P. D., Pandit, C., Marais, B. J., & Fitzgerald, D. A. (2018) · Paediatric Respiratory Reviews · doi:10.1016/j.prrv.2018.05.001 · Cited by 15 · see citing works
Using registry data to improve quality of care
McIntyre, K., Bertrand, D. P., & Rault, G. (2018) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2018.06.006 · Cited by 14 · see citing works
Screening strategies for glucose tolerance abnormalities and diabetes in people with cystic fibrosis
Weiss, L. M., Reix, P., Mosnier-Pudar, H., Ronsin, O., Beltrand, J., Reynaud, Q., Mély, L., Burgel, P., Stremler, N., Rakotoarisoa, L., et al. (2023) · Diabetes & Metabolism · doi:10.1016/j.diabet.2023.101444 · Cited by 14 · see citing works
Jackson, A. D., Jackson, A. L., Fletcher, G., Doyle, G., Harrington, M., Zhou, S., Cullinane, F., Gallagher, C. G., & McKone, E. F. (2017) · PharmacoEconomics · doi:10.1007/s40273-017-0530-4 · Cited by 14 · see citing works
Managing Cystic Fibrosis in Polish Healthcare
Rachel, M., Topolewicz, S., Śliwczyński, A. M., & Galiniak, S. (2020) · International Journal of Environmental Research and Public Health · doi:10.3390/ijerph17207630 · Cited by 13 · see citing works
Mainbourg, S., Reynaud, Q., & Durieu, I. (2018) · Clinical Transplantation · doi:10.1111/ctr.13269 · Cited by 13 · see citing works
McBennett, K., & Davis, P. B. (2022) · Clinics in Chest Medicine · doi:10.1016/j.ccm.2022.06.002 · Cited by 13 · see citing works
Mosquera, F. E. C., Perlaza, C. L., Rojas, A. N., Rios, S. M., Gallego, A. C., Fischersworring, S. I., Rodríguez, J. S., & Liscano, Y. (2025) · Medicina · doi:10.3390/medicina61030489 · Cited by 12 · see citing works
Quon, B. S., Sykes, J. R., Stanojevic, S., Marshall, B. C., Petren, K., Ostrenga, J., Fink, A. K., Elbert, A., Faro, A., Goss, C. H., et al. (2018) · Clinical Transplantation · doi:10.1111/ctr.13188 · Cited by 12 · see citing works
Self-citationCoriati, A., Sykes, J. R., Lemonnier, L., Ma, X., Stanojevic, S., Dehillotte, C., Carlier, N., Stephenson, A. L., & Burgel, P. (2021) · European Respiratory Journal · doi:10.1183/13993003.00014-2021 · Cited by 12 · see citing works
Self-citationLee, D. J., Sykes, J. R., Griffin, K., Noel, C. W., Hyung, B., Chokar, K., Yao, C. M. K. L., Tullis, E. D., & Lee, J. M. (2022) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2022.05.008 · Cited by 12 · see citing works
Self-citationKim, S., Corey, M. L., Stephenson, A. L., & Strug, L. J. (2018) · Thorax · doi:10.1136/thoraxjnl-2017-210899 · Cited by 12 · see citing works
Self-citationEndocrine complications after solid-organ transplant in cystic fibrosis
Sidhaye, A. R., Goldswieg, B., Kaminski, B. A., Blackman, S. M., & Kelly, A. T. (2019) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2019.08.019 · Cited by 12 · see citing works
Self-management and health care transition: Trials, tribulations and triumphs
BETZ, C. L. (2017) · Journal of Pediatric Rehabilitation Medicine · doi:10.3233/prm-170446 · Cited by 12 · see citing works
Ruseckaite, R., Pekin, N. P., King, S., Carr, E., Ahern, S., Oldroyd, J. C., Earnest, A., Wainwright, C. E., & Armstrong, D. (2018) · Respiratory Medicine · doi:10.1016/j.rmed.2018.07.007 · Cited by 12 · see citing works
Measuring the burden of cystic fibrosis: A scoping review
Cheema, Z. M., Gomez, L. C., Johnson, N., Laflamme, O. D., Rabin, H. R., Steele, K., Wallenburg, J., Leong, J., Cheng, S. Y., Quon, B. S., et al. (2023) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2023.11.014 · Cited by 11 · see citing works
Self-citationSears, E. H., Hinton, A. C., López‐Pintado, S., Lary, C. W., & Zuckerman, J. B. (2023) · Annals of the American Thoracic Society · doi:10.1513/annalsats.202211-951oc · Cited by 11 · see citing works
Trial Refresh: A Case for an Adaptive Platform Trial for Pulmonary Exacerbations of Cystic Fibrosis
Schultz, A., Marsh, J. A., Saville, B. R., Norman, R., Middleton, P. G., Greville, H. W., Bellgard, M., Berry, S. M., & Snelling, T. (2019) · Frontiers in Pharmacology · doi:10.3389/fphar.2019.00301 · Cited by 11 · see citing works
Schieppati, D., Germon, R., Galli, F., Rigamonti, M. G., Stucchi, M., & Boffito, D. C. (2019) · Respiratory Medicine · doi:10.1016/j.rmed.2019.04.011 · Cited by 11 · see citing works
Cystic Fibrosis: Proteostatic correctors of CFTR trafficking and alternative therapeutic targets.
Hanrahan, J. W., Sato, Y., Carlile, G. W., Jansen, G., Young, J. C., & Thomas, D. Y. (2019) · Expert Opinion on Therapeutic Targets · doi:10.1080/14728222.2019.1628948 · Cited by 11 · see citing works
Leung, G. J., Cho, T. J., Kovesi, T. A., Hamid, J. S., & Radhakrishnan, D. (2019) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2019.06.007 · Cited by 11 · see citing works
Coriati, A., Bouvet, G. F., Massé, C., Ducruet, T., & Berthiaume, Y. (2020) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2020.11.011 · Cited by 11 · see citing works
Impact of COVID-19 on social determinants of health for adults with cystic fibrosis
Albon, D. P., Bruschwein, H. M., Soper, M., List, R., Jennings, D., Gettle, L. S., Compton, M. H., Bailey, M., Starheim, E., Murray, R., et al. (2021) · Therapeutic Advances in Respiratory Disease · doi:10.1177/17534666211037459 · Cited by 10 · see citing works
Development and external validation of 1- and 2-year mortality prediction models in cystic fibrosis
Stanojevic, S., Sykes, J. R., Stephenson, A. L., Aaron, S. D., & Whitmore, G. A. (2019) · European Respiratory Journal · doi:10.1183/13993003.00224-2019 · Cited by 10 · see citing works
Self-citationCystic fibrosis survival outcomes following second lung transplant: The north American experience
Alshehri, M., Ramos, K. J., Sykes, J., Ma, X., Stanojevic, S., Quon, B. S., Marshall, B. C., Cromwell, E., Ostrenga, J. S., Faro, A., et al. (2023) · Clinical Transplantation · doi:10.1111/ctr.15097 · Cited by 9 · see citing works
Self-citationMacDougall, A. M., Jarvis, D., Keogh, R. H., Bowerman, C., Bilton, D., Davies, G., Carr, S. B., & Stanojevic, S. (2022) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2022.09.001 · Cited by 9 · see citing works
Self-citationInnovating cystic fibrosis clinical trial designs in an era of successful standard of care therapies
VanDevanter, D. R., & Mayer-Hamblett, N. (2017) · Current Opinion in Pulmonary Medicine · doi:10.1097/mcp.0000000000000418 · Cited by 9 · see citing works
Wright, K. D., Power, H. A., & Shivak, S. M. (2021) · Comprehensive Clinical Psychology · doi:10.1016/b978-0-12-818697-8.00145-x · Cited by 9 · see citing works
Family health and primary prevention
Tulchinsky, T. H., Varavikova, E. A., & Cohen, M. J. (2023) · Elsevier eBooks · doi:10.1016/b978-0-12-822957-6.00007-7 · Cited by 9 · see citing works
Antibiotic Cycling Reverts Extensive Drug Resistance in Burkholderia multivorans
Kavanaugh, L. G., Harrison, S. K., Flanagan, J. N., & Steck, T. R. (2021) · Antimicrobial Agents and Chemotherapy · doi:10.1128/aac.00611-21 · Cited by 9 · see citing works
Looking toward the future: Approaching care of the aging CF patient
Sala, M. A., Vitale, K. M., & Prickett, M. H. (2021) · Pediatric Pulmonology · doi:10.1002/ppul.25745 · Cited by 8 · see citing works
Marmor, M., Jonas, A. M., Mirza, A. A., Rad, E., Wong, H.-N., Aslakson, R. A., Aslakson, R. A., Ast, K., Carroll, T., Dzeng, E. W., et al. (2019) · Journal of Pain and Symptom Management · doi:10.1016/j.jpainsymman.2019.08.017 · Cited by 8 · see citing works
Cystic fibrosis-related diabetes: The unmet need
Pozo, L., Bello, F. O., Mendez, Y. H., & Surani, S. (2020) · World Journal of Diabetes · doi:10.4239/wjd.v11.i6.213 · Cited by 8 · see citing works
Endoscopic Sinus Surgery for Cystic Fibrosis: Variables Influencing Sinonasal and Pulmonary Outcomes
Ji, K. S. Y., Frank-Ito, D. O., Hachem, R. A., Issa, K., Johnson, C. A., Mohamedaly, O. H., Goldstein, B. J., & Jang, D. W. (2021) · American Journal of Rhinology and Allergy · doi:10.1177/19458924211059606 · Cited by 8 · see citing works
Alternative Facts Have No Place in Science
Laine, C. M., & Taichman, D. B. (2017) · Annals of Internal Medicine · doi:10.7326/m17-0878 · Cited by 8 · see citing works
Diagnosing Cystic Fibrosis in Adults: Better Late Than Never
Jain, R. (2018) · Annals of the American Thoracic Society · doi:10.1513/annalsats.201806-432ed · Cited by 8 · see citing works
- Authors
- Stephenson, A. L., Sykes, J., Stanojevic, S., Quon, B. S., Marshall, B. C., Petren, K., Ostrenga, J., Fink, A. K., Elbert, A., & Goss, C. H.
- Affiliations
- St. Michael's Hospital (CA)
- ORCID
- Venue
- Annals of Internal Medicine
- Publisher
- American College of Physicians
- Published
- 2017-04-18
- Volume
- 166
- Issue
- 8
- Pages
- 537-546
- ISSN
- 0003-4819 (ISSN-L)
- 1539-3704
- PMC
- PMC5467971
- OpenAlex
- W2595084745
- Cited by
- 192
- References
- 34
- Citations by year
- 2026: 7 · 2025: 12 · 2024: 8 · 2023: 15 · 2022: 17 · 2021: 25 · 2020: 23 · 2019: 25 · 2018: 38 · 2017: 22
- FWCI
- 24.43
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Annals of Internal Medicine (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- Funders
- Cystic Fibrosis Foundation (STEPHE14A0)
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Cystic Fibrosis Research Advances
- Health Sciences › Medicine › Surgery › Transplantation: Methods and Outcomes
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Neonatal Respiratory Health Research
- Keywords
- cystic fibrosis, survival, cross-national comparison, Canada, United States, lung transplantation, access to transplantation, insurance status, health insurance, mortality risk, ascertainment bias, health care disparities, quality of care
- MeSH terms
- Canada, Cystic Fibrosis, Humans, Insurance, Health, Risk Factors, United States, Survival Rate, Proportional Hazards Models, Lung Transplantation, Single-Payer System, Insurance Coverage
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