Cystic fibrosis colorectal cancer screening consensus recommendations
Open accessAbstract
BACKGROUND & AIMS: Improved therapy has substantially increased survival of persons with cystic fibrosis (CF). But the risk of colorectal cancer (CRC) in adults with CF is 5–10 times greater compared to the general population, and 25–30 times greater in CF patients after an organ transplantation. To address this risk, the CF Foundation convened a multistakeholder task force to develop CRC screening recommendations. METHODS: The 18-member task force consisted of experts including pulmonologists, gastroenterologists, a social worker, nurse coordinator, surgeon, epidemiologist, statistician, CF adult, and a parent. The committee comprised 3 workgroups: Cancer Risk, Transplant, and Procedure and Preparation. A guidelines specialist at the CF Foundation conducted an evidence synthesis February–March 2016 based on PubMed literature searches. Task force members conducted additional independent searches. A total of 1159 articles were retrieved. After initial screening, the committee read 198 articles in full and analyzed 123 articles to develop recommendation statements. An independent decision analysis evaluating the benefits of screening relative to harms and resources required was conducted by the Department of Public Health at Erasmus Medical Center, Netherlands using the Microsimulation Screening Analysis model from the Cancer Innervation and Surveillance Modeling Network. The task force included recommendation statements in the final guideline only if they reached an 80% acceptance threshold. RESULTS: The task force makes 10 CRC screening recommendations that emphasize shared, individualized decision-making and familiarity with CF-specific gastrointestinal challenges. We recommend colonoscopy as the preferred screening method, initiation of screening at age 40 years, 5-year re-screening and 3-year surveillance intervals (unless shorter interval is indicated by individual findings), and a CF-specific intensive bowel preparation. Organ transplant recipients with CF should initiate CRC screening at age 30 years within 2 years of the transplantation because of the additional risk for colon cancer associated with immunosuppression. CONCLUSIONS: These recommendations aim to help CF adults, families, primary care physicians, gastroenterologists, and CF and transplantation centers address the issue of CRC screening. They differ from guidelines developed for the general population with respect to the recommended age of screening initiation, screening method, preparation, and the interval for repeat screening and surveillance.
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?
Colorectal cancer risk is increased in cystic fibrosis compared with age-matched controls.
“Finally, the risk of colorectal cancer is increased in CF compared to age-matched controls, and transplant programs should screen CF candidates with colonoscopy beginning at age 40 years based on the 2017 CF Foundation Guidelines.(271)”
From Consensus document for the selection of lung transplant candidates: An update from the International Society for Heart and Lung Transplantation. (2021) · cites this paper as [271]
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
Lieberman, D. A., Rex, D. K., Winawer, S. J., Giardiello, F. M., Johnson, D. A., & Levin, T. R. (2012) · Gastroenterology · doi:10.1053/j.gastro.2012.06.001 · Cited by 1,889 · see citing works
Self-citationSyngal, S., Brand, R. E., Church, J. M., Giardiello, F. M., Hampel, H. L., & Burt, R. W. (2015) · American Journal of Gastroenterology · doi:10.1038/ajg.2014.435 · Cited by 1,662 · see citing works
Cairns, S. R., Scholefield, J. H., Steele, R. J., Dunlop, M. G., Thomas, H. J. W., Evans, D. G., Eaden, J., Rutter, M. D., Atkin, W. S., Saunders, B. P., et al. (2010) · Gut · doi:10.1136/gut.2009.179804 · Cited by 1,139 · see citing works
Yusen, R. D., Edwards, L. B., Kucheryavaya, A. Y., Benden, C., Dipchand, A. I., Goldfarb, S., Levvey, B. J., Lund, L. H., Meiser, B., Rossano, J. W., et al. (2015) · The Journal of Heart and Lung Transplantation · doi:10.1016/j.healun.2015.08.014 · Cited by 595 · see citing works
Estimation of Benefits, Burden, and Harms of Colorectal Cancer Screening Strategies
Knudsen, A. B., Zauber, A. G., Rutter, C. M., Naber, S. K., Doria-Rose, V. P., Pabiniak, C. J., Johanson, C., Fischer, S. E., Lansdorp‐Vogelaar, I., & Kuntz, K. M. (2016) · JAMA · doi:10.1001/jama.2016.6828 · Cited by 476 · see citing works
Self-citationBowel preparation for colonoscopy: European Society of Gastrointestinal Endoscopy (ESGE) Guideline
Hassan, C., Bretthauer, M., Kaminski, M. F., et al. (2013) · Endoscopy · doi:10.1055/s-0032-1326186 · Cited by 459 · see citing works
Dominitz, J. A., Eisen, G. M., Baron, T. H., Goldstein, J. L., Hirota, W. K., Jacobson, B. C., Johanson, J. F., Leighton, J. A., Mallery, J. S., Raddawi, H. M., et al. (2003) · Gastrointestinal Endoscopy · doi:10.1016/s0016-5107(03)80005-6 · Cited by 451 · see citing works
Prevalence of missed adenomas in patients with inadequate bowel preparation on screening colonoscopy
Chokshi, R. V., Hovis, C. E., Hollander, T. G., et al. (2012) · Gastrointestinal Endoscopy · doi:10.1016/j.gie.2012.01.005 · Cited by 362 · see citing works
The Risk of Cancer among Patients with Cystic Fibrosis
Neglia, J. P., FitzSimmons, S. C., Maisonneuve, P., et al. (1995) · New England Journal of Medicine · doi:10.1056/nejm199502233320803 · Cited by 352 · see citing works
Self-citationGustafsson, J. K., Ermund, A., Ambort, D., Johansson, M., Nilsson, H. E., Thorell, K., Hebert, H., Sjövall, H., & Hansson, G. C. (2012) · The Journal of Experimental Medicine · doi:10.1084/jem.20120562 · Cited by 345 · see citing works
A Transposon-Based Genetic Screen in Mice Identifies Genes Altered in Colorectal Cancer
Starr, T. K., Allaei, R., Silverstein, K. A. T., Staggs, R. A., Sarver, A. L., Bergemann, T. L., Gupta, M., O’Sullivan, M. G., Matise, I., Dupuy, A. J., et al. (2009) · Science · doi:10.1126/science.1163040 · Cited by 344 · see citing works
Fisher, D. A., Maple, J. T., Ben‐Menachem, T., et al. (2011) · Gastrointestinal Endoscopy · doi:10.1016/j.gie.2011.07.025 · Cited by 325 · see citing works
Cancer Risk in Cystic Fibrosis: A 20-Year Nationwide Study From the United States
Maisonneuve, P., Marshall, B. C., Knapp, E. A., & Lowenfels, A. B. (2012) · JNCI Journal of the National Cancer Institute · doi:10.1093/jnci/djs481 · Cited by 313 · see citing works
Self-citationTHE RISK OF CANCER AMONG PATIENTS WITH CYSTIC FIBROSIS
Eglia, J. O. P. N., Itz, S. T. C. F., Aisonneuve, P. A. M., et al. (1995) · Cited by 301 · see citing works
CFTR, Mucins, and Mucus Obstruction in Cystic FibrosisPreprint
Kreda, S. M., Davis, C. W., & Rose, M. C. (2012) · Cold Spring Harbor Perspectives in Medicine · doi:10.1101/cshperspect.a009589 · Cited by 220 · see citing works
Atkin, W. S., Valori, R., Kuipers, E. J., Hoff, G. S., Senore, C., Segnan, N., Jover, R., Schmiegel, W., Lambert, R. G. W., & Pox, C. P. (2012) · Endoscopy · doi:10.1055/s-0032-1309821 · Cited by 220 · see citing works
CFTR is a tumor suppressor gene in murine and human intestinal cancer
Than, B. L. N., Linnekamp, J. F., Starr, T. K., Largaespada, D. A., Rod, A., Zhang, Y., Bruner, V., Abrahante, J. E., Schumann, A. C., Luczak, T. S., et al. (2016) · Oncogene · doi:10.1038/onc.2015.483 · Cited by 180 · see citing works
Norkina, O., Burnett, T. G., & Lisle, R. C. D. (2004) · Infection and Immunity · doi:10.1128/iai.72.10.6040-6049.2004 · Cited by 175 · see citing works
Cancer Risk in Nontransplanted and Transplanted Cystic Fibrosis Patients: A 10-Year Study
Maisonneuve, P., FitzSimmons, S. C., Neglia, J. P., et al. (2003) · JNCI Journal of the National Cancer Institute · doi:10.1093/jnci/95.5.381 · Cited by 140 · see citing works
Self-citationBa, O., & Bs, M. (1975) · PubMed · Cited by 116 · see citing works
Menees, S. B., Kim, H. M., Elliott, E. E., et al. (2013) · Gastrointestinal Endoscopy · doi:10.1016/j.gie.2013.03.1334 · Cited by 105 · see citing works
Lochhead, P. J., & Chan, A. T. (2012) · Clinical Gastroenterology and Hepatology · doi:10.1016/j.cgh.2012.08.037 · Cited by 97 · see citing works
CFTR, bicarbonate, and the pathophysiology of cystic fibrosis
Borowitz, D. (2015) · Pediatric Pulmonology · doi:10.1002/ppul.23247 · Cited by 79 · see citing works
Niccum, D. E., Billings, J. L., Dunitz, J., & Khoruts, A. (2016) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2016.01.002 · Cited by 79 · see citing works
Self-citationCancer risk among lung transplant recipients with cystic fibrosis
Fink, A. K., Yanik, E. L., Marshall, B. C., Wilschanski, M. A., Lynch, C. F., Austin, A. A., Copeland, G. E., Safaeian, M., & Engels, E. A. (2016) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2016.07.011 · Cited by 63 · see citing works
Self-citationSamarasena, J., Muthusamy, R. V., & Jamal, M. M. (2012) · The American Journal of Gastroenterology · doi:10.1038/ajg.2012.115 · Cited by 62 · see citing works
MiraLAX Is Not as Effective as GoLytely in Bowel Cleansing Before Screening Colonoscopies
Hjelkrem, M., Stengel, J. Z., Liu, M., et al. (2010) · Clinical Gastroenterology and Hepatology · doi:10.1016/j.cgh.2010.11.007 · Cited by 59 · see citing works
Cystic fibrosis: Evidence for gut inflammation
Munck, À. (2014) · The International Journal of Biochemistry & Cell Biology · doi:10.1016/j.biocel.2014.02.005 · Cited by 59 · see citing works
Optimal bowel preparation—a practical guide for clinicians
Rex, D. K. (2014) · Nature Reviews Gastroenterology & Hepatology · doi:10.1038/nrgastro.2014.35 · Cited by 57 · see citing works
Billings, J. L., Dunitz, J., McAllister, S., Herzog, T. L., Bobr, A., & Khoruts, A. (2013) · Journal of Clinical Gastroenterology · doi:10.1097/mcg.0000000000000034 · Cited by 52 · see citing works
Self-citationNagler, J., Poppers, D. M., & Turetz, M. L. (2006) · Journal of Clinical Gastroenterology · doi:10.1097/00004836-200607000-00017 · Cited by 51 · see citing works
Colon cancer in lung transplant recipients with CF: Increased risk and results of screening
Meyer, K. C., Francois, M. L., Thomas, H. K., et al. (2011) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2011.05.003 · Cited by 50 · see citing works
Self-citationOncologic Issues and Kidney Transplantation: A Review of Frequency, Mortality, and Screening
Asch, W. S., & BIA, M. J. (2013) · Advances in Chronic Kidney Disease · doi:10.1053/j.ackd.2013.07.003 · Cited by 49 · see citing works
Atassi, T., & Thuluvath, P. J. (2003) · Journal of Clinical Gastroenterology · doi:10.1097/00004836-200307000-00018 · Cited by 48 · see citing works
Bowel Preparation for Colonoscopy: Entering an Era of Increased Expectations for Efficacy
Rex, D. K. (2013) · Clinical Gastroenterology and Hepatology · doi:10.1016/j.cgh.2013.11.003 · Cited by 44 · see citing works
Validation of a predictive survival model in Italian patients with cystic fibrosis
Buzzetti, R., Alicandro, G., Minicucci, L., Notarnicola, S., Furnari, M. L., Giordano, G., Lucidi, V., Montemitro, E., Raia, V., Magazzù, G., et al. (2011) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2011.08.007 · Cited by 34 · see citing works
Cost Effectiveness of Screening Individuals With Cystic Fibrosis for Colorectal Cancer
Gini, A., Zauber, A. G., Cenin, D. R., Omidvari, A., Hempstead, S. E., Fink, A. K., Lowenfels, A. B., & Lansdorp‐Vogelaar, I. (2017) · Gastroenterology · doi:10.1053/j.gastro.2017.10.036 · Cited by 30 · see citing works
Self-citationMiralax With Gatorade for Bowel Preparation: A Meta-Analysis of Randomized Controlled Trials
Siddique, S., Lopez, K. T., Hinds, A. M., et al. (2014) · The American Journal of Gastroenterology · doi:10.1038/ajg.2014.238 · Cited by 27 · see citing works
Gory, I., Brown, G. J., Wilson, J. W., et al. (2014) · Scandinavian Journal of Gastroenterology · doi:10.3109/00365521.2014.936035 · Cited by 26 · see citing works
CYSTIC FIBROSIS AND ILEAL CARCINOMA
Siraganian, P. A., Miller, R., & Swender, P. (1987) · The Lancet · doi:10.1016/s0140-6736(87)91598-4 · Cited by 25 · see citing works
The risk of gastrointestinal malignancies in cystic fibrosis
Alexander, C. L., Urbanski, S. J., Hilsden, R. J., et al. (2007) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2007.07.005 · Cited by 23 · see citing works
Adenocarcinoma of the ileum presenting as non-traumatic clostridial myonecrosis in cystic fibrosis.
Redington, A. N., Spring, R., & Batten, J. C. (1985) · BMJ · doi:10.1136/bmj.290.6485.1871 · Cited by 22 · see citing works
Colon Cancer in Cystic Fibrosis patients: Is this a growing problem?
Hernandez-Jimenez, I., Fischman, D., & Cheriyath, P. (2008) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2008.02.006 · Cited by 19 · see citing works
Colonic adenocarcinoma in a 13-year-old with cystic fibrosis
Ibele, A. R., Koplin, S. A., Slaughenhoupt, B. L., et al. (2007) · Journal of Pediatric Surgery · doi:10.1016/j.jpedsurg.2007.07.024 · Cited by 14 · see citing works
Soriano, E., Fischman, D., & Cheriyath, P. (2008) · Southern Medical Journal · doi:10.1097/smj.0b013e318172f697 · Cited by 10 · see citing works
Early onset of colonic adenocarcinoma associated with cystic fibrosis--a case report.
McKenna, P. B., Mulcahy, E. M., & Waldron, D. J. (2007) · PubMed · Cited by 8 · see citing works
Delayed Diagnosis of Colon Carcinoma in Cystic Fibrosis
Brown, J. A., Chaun, H., & MASON, A. C. (2000) · Clinical Radiology · doi:10.1053/crad.1999.0287 · Cited by 7 · see citing works
Adenocarcinoma of the ileum in a patient with cystic fibrosis
Samuels, L. E., Amrom, G., & Kerstein, M. D. (1995) · Digestive Diseases and Sciences · doi:10.1007/bf02063951 · Cited by 4 · see citing works
Intussusception and metastatic colon cancer in an adult with cystic fibrosis
Gilchrist, F. J., Jones, A. M., & Bright-Thomas, R. J. (2012) · Journal of the Royal Society of Medicine · doi:10.1258/jrsm.2012.12s008 · Cited by 4 · see citing works
Lees, A. N., & Reid, D. W. (2008) · Journal of Medical Case Reports · doi:10.1186/1752-1947-2-384 · Cited by 2 · see citing works
Flagged first, then by citations
Cited by
Sorted by citations
Leard, L. E., Holm, A. M., Valapour, M., Glanville, A. R., Attawar, S., Aversa, M., Campos, S. V., Christon, L. M., Cypel, M., Dellgren, G., et al. (2021) · The Journal of Heart and Lung Transplantation · doi:10.1016/j.healun.2021.07.005 · Cited by 821 · see citing works
Shteinberg, M., Haq, I. J., Polineni, D., & Davies, J. C. (2021) · The Lancet · doi:10.1016/s0140-6736(20)32542-3 · Cited by 682 · see citing works
Lung transplantation for cystic fibrosis
Morrell, M. R., & Pilewski, J. M. (2016) · Clinics in Chest Medicine · doi:10.1016/j.ccm.2015.11.008 · Cited by 56 · see citing works
- Authors
- Hadjiliadis, D., Khoruts, A., Zauber, A. G., Hempstead, S. E., Maisonneuve, P., Lowenfels, A. B., Braid, A. L., Cullina, J., Daggett, A., Fink, A., Gini, A., Hadjiliadis, D., Harron, P. F., Hempstead, S., Khoruts, A., Lansdorp-Vogelaar, I., Lieberman, D., Liou, T., Lomas, P., Lowenfels, A., Maisonneuve, P., Marshall, B., Meyer, K., Rustgi, A., Shaukat, A., Zauber, A., & Sabadosa, K.
- Venue
- Gastroenterology
- Publisher
- Elsevier BV
- Published
- 2018-02
- Volume
- 154
- Issue
- 3
- Pages
- 736-745.e14
- ISSN
- 0016-5085 (ISSN-L)
- PMC
- PMC9675422
- OpenAlex
- W2778185093
- Semantic Scholar
- 79cb039f1b8a2be9837badc5e833b8edaa66bb04
- Cited by
- 195
- References
- 50
- Citations by year
- 2026: 15 · 2025: 25 · 2024: 23 · 2023: 24 · 2022: 30 · 2021: 24 · 2020: 18 · 2019: 20 · 2018: 13 · 2017: 2 · 2016: 1
- FWCI
- 6.39
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Gastroenterology (publishedVersion)
- PubMed (publishedVersion)
- EUR Research Repository (Erasmus University Rotterdam) (publishedVersion)
- PubMed Central (acceptedVersion)
- Touro Scholar (Touro College) (submittedVersion)
- EUR Research Repository (Erasmus University Rotterdam) (submittedVersion)
- Funders
- National Heart, Lung, and Blood Institute (R01HL125520)
- Topics
- Health Sciences › Medicine › Oncology › Colorectal Cancer Screening and Detection
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Cystic Fibrosis Research Advances
- Health Sciences › Medicine › Pathology and Forensic Medicine › Genetic factors in colorectal cancer
- Keywords
- cystic fibrosis, colorectal cancer screening, colonoscopy, bowel preparation, organ transplant recipients, immunosuppression, cancer risk, screening frequency, shared decision-making, personalized screening, decision analysis
- MeSH terms
- Clinical Decision-Making, Adult, Aged, Colonoscopy, Cystic Fibrosis, Decision Support Techniques, Female, Gastroenterology, Humans, Male, Middle Aged, Predictive Value of Tests, Prognosis, Risk Factors, Colorectal Neoplasms, Organ Transplantation, Risk Assessment, Consensus, Early Detection of Cancer
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
