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Show Cancer Risk Profile of HNPCC Cancers Linked to Utah Population Database JartdCox (Richard Kerber,Wad« Samowitz, Randy Burtr Deborah Nek la son) Huntsman Cancer Institute University Utah The objective of this study u to evaluate cancer ifch in ped^reps of H NPCC piob?r>ds usir>3 the Utah Pdpul?iH?n Datable -IUPPES1. The UPDB include? genealogies linked to cancer records from the Utah Cancer Regisi nv lUCfll back to l%5, ?ndUt?hde?ih ?r>d birth ceri"rfic?tes Resulting cancer nskdatn was then tested arjamst a set oF fancies containing one or noreWSI colon ca ncer to tfelemime tfie predVtrve value of this data wases?rmnedm J9£hqh risk colon cancersdefined nsa diagnosis^ 50 yearsor Iwofirjt decj ree relatives with colon cancer. Fiftptwo of thewc?n^er& demonstrate WSU13.6%l. The WSIc^n^ers appear to z luster In iwo nroups, thow v^"d h a ^ur^ ane of onwt KS^and likelylo be HNF^Cl. nnd thowwithar oW a^e of onset l^ 70 and hkely dup to hypern^eth^ritKiri of MLH1 ^ Family h ^tnry of tv^ or more generations wa* avni^b|plnUPO0on J7oFihflM&kancercwes>"etonh/7fulnll Arn&iflrdarnlkriteria. UPDB v*as uwd to evalu?1e 1he obwr"^d verses expelled rate of the c?r>raf5 In four large HMPCC kindreds iTable U.The expecied number of ¦ *r*:w5 v^s determined f\orn nqe nd| usted pcpu Uitinn rate? in Utah uslrq IJ PO0 da-1w Hre p va lue was c^kulated u nder the null hypdhe&is analyzing cn^e? under? Poison distribution: and the FSIRi? the ratio of observed to m r^cted ^ance^. We observed a signirVant eK^H oF^obn, uterine. blacUer, krdnerand ureter, mouth and prostate fesed on Hrese increased cancer rate^iwo criteria v^ere tested for their ability to idenilFy prospective HNPCC faml Bes. The fi rst enters rdenfrfied U PO0 Famllres v^"d h hqh rates of colon cancer and a p value of ^0.0-S. fifteen of the 37 MSI car>rer ca5e? werernembersoFthese Familfcgs There wasercelLgnt con<^rdance between 1 hew IS ewe? and erases v^iere HNKC is^u^Hcted based on age of onset, im rnunohisioc hem^try ar>d sequenclncj of MLH1 and WSH2. The serond arteri? Vlentified UPDB Fami Hes v^th high rales of uterine and urina ny tract cancers com bined. This arwlpis "dentined 3 oF 1 he J ? case? but e*c lud?d nenetiralfy defined HNPCC families m this set. a sjfl mfiranity ir>rrewed ane adjusied rate of colon cancer Is a senjiti-i-e predVtor of HNPCC In a population analysis Although uterine and urinary in>rt cancers are Irvraasedm HNPCC fam "he?, ttay ?kme a re nd flood predictors of HNKC ¦incei P value Observed a KEted F5ln EJaddei <O01 S O-.37 11*4 ""o*?n <O01 n D.76 17i>| Uterine <O01 OvM I5.&3 Breasi o.co 1^j IB :er*w 0.07 1 0^3 2.91 Ktfnev <ftO1 0.21 I44fi Luna [>.{]^ 2 o.s^ J.37 Wo nth 0.17 17-78 Prosiate 0.01 S 1^2 TesHcuLar 0.01 1 0.1 S 6r4* RrvTcW 0.01 1 0-.15 9.JS Uieter <O01 1 0.Q1 76-11 i/wiiru <O.Ul 1 Jarb$ Cox Bsbosch ^feWoscw 1 |