title: [Hereditary colorectal cancer].
abstract: HNPCC and FAP are inherited diseases with a lifetime risk of colorectal cancer (CRC) of 80-100% in gene carriers. Disease-causing mutations have been identified in the APC gene at FAP and in MMR genes at HNPCC. In FAP-patients, screening has reduced the prevalence of CRC by 55%, and the survival rate has improved considerably. For HNPCC-patients, 77% of CRCs found by screening were Duke' A or B, and survival after CRC has improved significantly since 1990. Continuous central registration in the HNPCC and Polyposis registers is recommended to ensure identification of high-risk families and evaluate the effect of screening.
authors.affiliation: H:S Hvidovre Hospital, Gastroenheden 435, HNPCC-registret og Polyposeregistret, Hvidovre. inge.bernstein@hh.hosp.dk
authors.forename: Inge T
authors.initials: IT
authors.lastname: Bernstein
authors.forename: Steffen
authors.initials: S
authors.lastname: Bülow
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D003113:Colonoscopy; D015179:Colorectal Neoplasms / Q000175:diagnosis / Q000235:genetics* / Q000401:mortality / Q000517:prevention & control; D003123:Colorectal Neoplasms, Hereditary Nonpolyposis / Q000175:diagnosis / Q000235:genetics* / Q000401:mortality / Q000517:prevention & control; D005260:Female; D017491:Genes, APC; D020022:Genetic Predisposition to Disease; D006801:Humans; D008297:Male; D008403:Mass Screening; D008875:Middle Aged; D010375:Pedigree; D011379:Prognosis; D012042:Registries; D012307:Risk Factors