Lung function decline from adolescence to young adulthood in cystic fibrosis
Open accessAbstract
BACKGROUND: Despite improving survival in cystic fibrosis (CF) patients, there is a mortality peak in early adulthood. Defining risk factors that predict significant worsening of lung disease in young adulthood may identify opportunities to improve outcomes in adults. METHODS: We identified 4,680 patients in the Epidemiologic Study of Cystic Fibrosis 1994-2005 with data in both adolescence (age 14.0-17.4 years) and young adulthood (age 18.5-22.0 years) and analyzed 2,267 who had ≥5 encounters and ≥5 measurements of forced expiratory volume in 1 second (FEV(1) ) spanning ≥1 year during both adolescence and young adulthood, and ≥1 encounter with weight and height and ≥1 FEV(1) measurement age 17.5-18.5 years. We compared the annualized rates of decline in FEV(1) during adolescence and young adulthood stratified by best FEV(1) around age 18. Logistic regression was used to identify risk factors associated with substantial decline (>20 points) in FEV(1) % predicted in young adulthood. RESULTS: Annual rate of decline was greater in young adulthood than in adolescence. Risk factors for substantial decline included slower rate of FEV(1) decline, greater FEV(1) variability, faster body mass index (BMI) decline, male sex, chronic inhaled antibiotics, Haemophilus influenzae detection, and absence of multidrug-resistant Pseudomonas aeruginosa in adolescence, and lower than expected FEV(1) and BMI around age 18. CONCLUSIONS: Decline in lung function accelerates in young adults with CF, especially in those with early stage lung disease. Adolescents at risk for substantial decline in lung function in young adulthood have higher FEV(1) and worse nutritional status, among other identifiable risk factors.
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Integrity signals
Registry
Cites
Statistics
Claims the paper makes
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
It is likely that an intervention will work in slightly different ways for children and adolescents compared with adults with CF.
“It is therefore likely that an intervention will work in slightly different ways for children and adolescents (who may have high parent/ caregiver involvement), compared with adults with CF.”
From Psychological interventions for improving adherence to inhaled therapies in people with cystic fibrosis (2023) · cites this paper as VandenBranden 2012
The Introduction says that many people with CF take over responsibility for managing their disease around age 18, and the Discussion recommends strategies tailored to adolescents transitioning to adulthood. These passages support an age-related difference in care context, but the paper does not establish that an intervention works differently in children and adolescents than in adults, or assess parent/caregiver involvement as the reason. (LLM, conf=0.78)
Experimental — accuracy not yet measured
Self-check
Percentages, sums and reported differences re-computed from the paper’s own numbers.
The self-check needs the paper’s full text.
Cites
Spirometric Reference Values from a Sample of the General U.S. Population
Hankinson, J., Odencrantz, J. R., & Fedan, K. B. (1999) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/ajrccm.159.1.9712108 · Cited by 4,220 · see citing works
Emerson, J., Rosenfeld, M., McNamara, S., Ramsey, B. W., & Gibson, R. L. (2002) · Pediatric Pulmonology · doi:10.1002/ppul.10127 · Cited by 1,009 · 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
Cystic Fibrosis–Related Diabetes: Current Trends in Prevalence, Incidence, and Mortality
Moran, A., Dunitz, J., Nathan, B. M., Saeed, A., Holme, B., & Thomas, W. T. B. (2009) · Diabetes Care · doi:10.2337/dc09-0586 · Cited by 648 · see citing works
Konstan, M. W., Morgan, W. J., Butler, S. M., Pasta, D. J., Craib, M. L., Silva, S. J., Stokes, D. C., Wohl, M. E. B., Wagener, J. S., Regelmann, W. E., et al. (2007) · The Journal of Pediatrics · doi:10.1016/j.jpeds.2007.03.006 · Cited by 436 · see citing works
Self-citationMucoid Pseudomonas aeruginosa is a marker of poor survival in cystic fibrosis
Henry, R. L., Mellis, C. M., & Petrovic, L. (1992) · Pediatric Pulmonology · doi:10.1002/ppul.1950120306 · Cited by 372 · see citing works
Gender Gap in Cystic Fibrosis Mortality
Rosenfeld, M., Davis, R. L., FitzSimmons, S., Pepe, M. S., & Ramsey, B. W. (1997) · American Journal of Epidemiology · doi:10.1093/oxfordjournals.aje.a009172 · Cited by 366 · see citing works
Wasting as an independent predictor of mortality in patients with cystic fibrosis
Sharma, R. K. (2001) · Thorax · doi:10.1136/thorax.56.10.746 · Cited by 266 · see citing works
Factors Influencing Outcomes in Cystic Fibrosis
Johnson, C. A., Butler, S. M., Konstan, M. W., Morgan, W. J., & Wohl, M. E. B. (2003) · CHEST Journal · doi:10.1378/chest.123.1.20 · Cited by 226 · see citing works
Self-citationHaemophilus influenzae Forms Biofilms on Airway Epithelia
Starner, T. D., Zhang, N., Kim, G., Apicella, M. A., & McCray, P. B. (2006) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.200509-1459oc · Cited by 207 · see citing works
Predictors of mortality in adults with cystic fibrosis
Courtney, J. M., Bradley, J. M., McCaughan, J., et al. (2007) · Pediatric Pulmonology · doi:10.1002/ppul.20619 · Cited by 163 · see citing works
Risky Behavior in Teens With Cystic Fibrosis or Sickle Cell Disease: A Multicenter Study
Britto, M. T., Garrett, J. M., Dugliss, M. A. J., Daeschner, C. W., Johnson, C. A., Leigh, M. W., Majure, J. M., Schultz, W. H., & Konrad, T. R. (1998) · PEDIATRICS · doi:10.1542/peds.101.2.250 · Cited by 137 · see citing works
Year-to-year changes in lung function in individuals with cystic fibrosis
Liou, T. G., Elkin, E. P., Pasta, D. J., Jacobs, J. R., Konstan, M. W., Morgan, W. J., & Wagener, J. S. (2010) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2010.04.002 · Cited by 117 · see citing works
Self-citationClinical use of dornase alfa is associated with a slower rate of FEV1 decline in cystic fibrosis
Konstan, M. W., Wagener, J. S., Pasta, D. J., et al. (2011) · Pediatric Pulmonology · doi:10.1002/ppul.21388 · Cited by 82 · see citing works
Self-citationRen, C. L., Pasta, D. J., Rasouliyan, L., et al. (2008) · The Journal of Pediatrics · doi:10.1016/j.jpeds.2008.07.010 · Cited by 73 · see citing works
Self-citationThe self-image of adolescents with cystic fibrosis
Sawyer, S. M., Rosier, M. J., Phelan, P. D., & Bowes, G. (1995) · Journal of Adolescent Health · doi:10.1016/1054-139x(94)00071-l · Cited by 69 · see citing works
Is There Still a Gender Gap in Cystic Fibrosis?
Verma, N., Bush, A., & Buchdahl, R. (2005) · CHEST Journal · doi:10.1016/s0012-3692(15)52709-8 · Cited by 64 · see citing works
Berge, J. M., Patterson, J. M., Goetz, D. R., et al. (2007) · Families Systems & Health · doi:10.1037/1091-7527.25.2.190 · Cited by 40 · see citing works
Verma, V., Singh, J., Chand, N., et al. (2005) · CHEST Journal · doi:10.1378/chest.128.4.2824 · Cited by 34 · see citing works
Cultural barriers to asthma management
Enarson, D. A., & Ait-Khaled, N. (1999) · Pediatric Pulmonology · doi:10.1002/(sici)1099-0496(199910)28:4 · Cited by 31 · see citing works
Packe, G. E., & Hodson, M. E. (1992) · Respiratory Medicine · doi:10.1016/s0954-6111(06)80147-2 · Cited by 21 · see citing works
Transition and Transfer of Patients Who Have Cystic Fibrosis to Adult Care
Parker, H. W. (2007) · Clinics in Chest Medicine · doi:10.1016/j.ccm.2007.02.007 · Cited by 14 · see citing works
Flagged first, then by citations
Research works printed but not linked: 1
- [8]Konstan MW Butler SM Schidlow DV Morgan WJ Julius JR Johnson CA Patterns of medical practice in cystic fibrosis: part II. Use of therapies. Investigators and Coordinators of the Epidemiologic Study of Cystic Fibrosis Pediatr Pulmonol 1999 28 248 254 10497373
Cited by
Sorted by citations
Sawicki, G. S., Ostrenga, J., Petren, K., Fink, A. K., D’Agostino, E., Strassle, C., Schechter, M. S., & Rosenfeld, M. (2018) · Annals of the American Thoracic Society · doi:10.1513/annalsats.201705-357oc · Cited by 53 · see citing works
Self-citationFidika, A., Herle, M., & Goldbeck, L. (2014) · BMC Pulmonary Medicine · doi:10.1186/1471-2466-14-205 · Cited by 46 · see citing works
Hayes, D., Higgins, R. S., Kirkby, S., McCoy, K. S., Wehr, A. M., Lehman, A. M., & Whitson, B. A. (2014) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2013.12.004 · Cited by 35 · see citing works
Dynamic predictive probabilities to monitor rapid cystic fibrosis disease progression
Szczesniak, R. D., Su, W., Brokamp, C., Keogh, R. H., Pestian, J. P., Seid, M., Diggle, P. J., & Clancy, J. P. (2020) · Statistics in Medicine · doi:10.1002/sim.8443 · Cited by 30 · see citing works
The feasibility of home monitoring of young people with cystic fibrosis: results from CLIMB-CF
Edmondson, C., Westrupp, N., Seddon, P., Olden, C., Wallis, C., Dawson, C., Brodlie, M., Baxter, F., McCormick, J., MacFarlane, S., et al. (2022) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2021.09.018 · Cited by 26 · see citing works
Szczesniak, R. D., Brokamp, C., Su, W., Mcphail, G. L., Pestian, J., & Clancy, J. P. (2019) · IEEE Journal of Translational Engineering in Health and Medicine · doi:10.1109/jtehm.2018.2878534 · Cited by 15 · see citing works
Dawson, S., Girling, C.-J., Cowap, L., & Clark-Carter, D. (2023) · Cochrane Database of Systematic Reviews · doi:10.1002/14651858.cd013766.pub2 · Cited by 14 · see citing works
- Authors
- VandenBranden, S. L., McMullen, A., Schechter, M. S., Pasta, D. J., Michaelis, R. L., Konstan, M. W., Wagener, J. S., Morgan, W. J., McColley, S. A., & for the Investigators and Coordinators of the Epidemiologic Study of Cystic Fibrosis
- Affiliations
- Lurie Children's Hospital (US)
- University of Rochester Medicine (US)
- Emory University (US)
- ICON PLC (Ireland) (IE)
- Rainbow Babies & Children's Hospital (US)
- University of Colorado Anschutz (US)
- University of Arizona (US)
- Northwestern University (US)
- Venue
- Pediatric Pulmonology
- Publisher
- Wiley
- Published
- 2011-08-24
- Volume
- 47
- Issue
- 2
- Pages
- 135-143
- ISSN
- 1099-0496 (ISSN-L)
- 8755-6863
- PMC
- PMC4219484
- OpenAlex
- W1966052078
- Cited by
- 128
- References
- 22
- Citations by year
- 2026: 2 · 2025: 6 · 2024: 11 · 2023: 13 · 2022: 6 · 2021: 15 · 2020: 13 · 2019: 7 · 2018: 9 · 2017: 8 · 2016: 7 · 2015: 13 · 2014: 5 · 2013: 8 · 2012: 5
- FWCI
- 4.89
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Pediatric Pulmonology (publishedVersion)
- PubMed (publishedVersion)
- PubMed Central (acceptedVersion)
- Funders
- Cystic Fibrosis Foundation
- Genentech
- National Institute of Diabetes and Digestive and Kidney Diseases (P30DK027651)
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Cystic Fibrosis Research Advances
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Chronic Obstructive Pulmonary Disease (COPD) Research
- Health Sciences › Medicine › Surgery › Transplantation: Methods and Outcomes
- Keywords
- cystic fibrosis, lung function decline, FEV1, adolescence, early adulthood, risk factors, body mass index, FEV1 variability, inhaled antibiotics, Haemophilus influenzae, multidrug-resistant Pseudomonas aeruginosa, nutritional status
- MeSH terms
- Administration, Inhalation, Adolescent, Anti-Bacterial Agents, Cystic Fibrosis, Female, Haemophilus Infections, Haemophilus influenzae, Humans, Longitudinal Studies, Lung, Male, Nutritional Status, Pseudomonas aeruginosa, Pseudomonas Infections, Respiratory Function Tests, Risk Factors, Sex Factors, Body Mass Index, Young Adult
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
