caa a22 4500 445330686 CHVBK 20180317142736.0 cr unu---uuuuu 170323e20110301xx s 000 0 eng 10.1007/s10689-010-9394-x doi (NATIONALLICENCE)springer-10.1007/s10689-010-9394-x Colorectal cancer: no longer the issue in familial adenomatous polyposis? [Elektronische Daten] [Daniel Gibbons, Ashish Sinha, Robin Phillips, Susan Clark] Familial adenomatous polyposis (FAP) is a dominantly inherited colorectal cancer (CRC) syndrome with an untreated lifetime prevalence of CRC close to 100% and extracolonic manifestations (ECM) of increasing clinical significance. This study examined the effect of systematic callup and prophylactic colectomy on FAP survival. Patients diagnosed, treated and followed-up at our institution were analysed. ‘Callups' were those identified via the callup system; ‘probands' were those identified by other means. Proportions were analysed by Chi-squared or Fischer's exact test. Mortality rates were indirectly standardised to the UK population. Survival curves from birth were estimated by Kaplan-Meier. A total of 439 patients (293 callups, 146 probands) were analysed. Crude mortality rates (CMRs) of callups and probands were 4.85 per 1,000 person years (PY) and 9.71 per 1,000 PY, respectively—a rate ratio of 0.50 (95% CI 0.34-0.72, P=0.0001). The standardised mortality ratio (SMR) of callups was non-significantly lower than probands (4.12 vs. 4.70). Callups experienced non-significantly lower age-band specific SMR up to 45years. More probands died of CRC (42.4 vs. 22.5%, P=0.025), whereas more callups died of ECM (30.6 vs. 13.4%, P=0.027). Median survival was 64years for callups and 60years for probands; survival curves did not differ significantly (P=0.253). The crude mortality rate of callups is approximately half that of probands. As fewer callups die of CRC, a greater proportion die of ECMs. Callups experienced non-significantly reduced mortality up to 45years. Whilst the FAP callup system reduces CRC risk, mortality attributable to ECMs needs to be addressed. Springer Science+Business Media B.V., 2010 Cause of death nationallicence Colorectal cancer nationallicence FAP nationallicence Extracolonic manifestations nationallicence Survival nationallicence APC : Adenomatous polyposis coli (gene) nationallicence CI : Confidence interval nationallicence CMR : Crude mortality ration nationallicence CRC : Colorectal cancer nationallicence ECM : Extracolonic manifestations nationallicence FAP : Familial adenomatous polyposis nationallicence NHS : National health service (UK) nationallicence OR : Odds ratio nationallicence PY : Person-years nationallicence RR : Relative risk nationallicence SMR : Standardised mortality ratio nationallicence Gibbons Daniel Academic Clinical Fellow, Department of Primary Care, School of Public Health, Imperial College London, Harrow, UK aut Sinha Ashish Clinical Research Fellow, Polyposis Registry, St Mark's Hospital and Imperial College London, Harrow, UK aut Phillips Robin Consultant Colorectal Surgeon and Assistant Director, The Polyposis Registry, St Mark's Hospital, Imperial College London, HA1 3UJ, Harrow, UK aut Clark Susan Consultant Colorectal Surgeon and Assistant Director, The Polyposis Registry, St Mark's Hospital, Imperial College London, HA1 3UJ, Harrow, UK aut Familial Cancer Springer Netherlands 10/1(2011-03-01), 11-20 1389-9600 10:1<11 2011 10 10689 https://doi.org/10.1007/s10689-010-9394-x text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s10689-010-9394-x text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Gibbons Daniel Academic Clinical Fellow, Department of Primary Care, School of Public Health, Imperial College London, Harrow, UK aut NATIONALLICENCE 700 1- Sinha Ashish Clinical Research Fellow, Polyposis Registry, St Mark's Hospital and Imperial College London, Harrow, UK aut NATIONALLICENCE 700 1- Phillips Robin Consultant Colorectal Surgeon and Assistant Director, The Polyposis Registry, St Mark's Hospital, Imperial College London, HA1 3UJ, Harrow, UK aut NATIONALLICENCE 700 1- Clark Susan Consultant Colorectal Surgeon and Assistant Director, The Polyposis Registry, St Mark's Hospital, Imperial College London, HA1 3UJ, Harrow, UK aut NATIONALLICENCE 773 0- Familial Cancer Springer Netherlands 10/1(2011-03-01), 11-20 1389-9600 10:1<11 2011 10 10689 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer