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Region Hovedstaden - en del af Københavns Universitetshospital
Udgivet

Colorectal Cancer in Individuals With Familial Adenomatous Polyposis, Based on Analysis of the Danish Polyposis Registry

Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

DOI

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Vis graf over relationer

Background & Aims: Familial adenomatous polyposis (FAP) is an autosomal dominant disorder that increases risk for colorectal cancer (CRC). We assessed changes in the incidence and prevalence of CRC, and survival times, of patients with FAP participating in the Danish follow-up study. Methods: We collected data from the Danish Polyposis Registry, a nationwide, complete registry of patients with FAP that includes clinical information, surgical procedures, follow-up findings, and pathology reports. We compared data between the periods of 1990–1999 and 2000–2017. In 2017, the registry contained 226 families with 721 individuals with FAP. Probands were defined as patients diagnosed based on bowel symptoms, without any knowledge of hereditary bowel disease. Call-up patients were defined as those found to have FAP during screening and due to a diagnosis of FAP in first-degree relatives. Results: Although the mean incidence rate of FAP was stable from 1990–1999 (0.19/100,000/year) to 2000–2017 (0.32/100,000/year) (P = .91), the point prevalence increased from 4.86/100,000 in 1999 to 6.11/100,000 by the end of 2017 (P = .005). During 2000–2017, 25 of 72,218 CRC cases were associated with FAP (0.03%)—this was a significant decrease from 1990–1999 (26/30,005 cases; 0.09%) (P = .001). The risk of CRC was significantly higher for probands (n = 191; 61.6%) than call-up cases (n = 5; 1.9%) (P < .001). All CRCs in call-up patients were detected at the diagnosis of FAP (no cases were identified in the follow-up program). The median life expectancy for call-up patients was 72.0 years (95% CI, 63.3–80.7), compared to 55.0 years for probands (95% CI, 51.2–58.8) (P < .001). Therefore, the tracing and follow-up program increased life expectancy by 17.0 years for first-degree family members. Conclusion: The Danish Polyposis Registry enables close monitoring of patients with FAP, reducing risk of CRC and prolonging life.

OriginalsprogEngelsk
TidsskriftClinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association
Vol/bind17
Udgave nummer11
Sider (fra-til)2294-2300.e1
ISSN1542-3565
DOI
StatusUdgivet - okt. 2019

ID: 56519407