{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,2]],"date-time":"2026-09-02T21:55:18Z","timestamp":1788386118372,"version":"build-2803163510"},"reference-count":39,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2011,9,1]],"date-time":"2011-09-01T00:00:00Z","timestamp":1314835200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. pediatr. gastroenterol. nutr."],"published-print":{"date-parts":[[2011,9]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Objective:<\/jats:title><jats:p>To retrospectively assess, in a pediatric multicenter cohort, guidelines for the management of familial adenomatous polyposis (FAP).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods:<\/jats:title><jats:p>Ten centers from the French\u2010speaking Pediatric Gastroenterology Hepatology and Nutrition Group provided follow\u2010up data on patients up to 18 years of age. Clinical records, genetic test results, endoscopy with histopathology examination, and therapeutic modalities were reviewed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results:<\/jats:title><jats:p>A total of 70 children from 47 families were included. When initial consultation resulted from a surveillance program because of an affected family member, 12 of 59 children were already symptomatic. Among 11 patients whose initial consultation was based only on symptoms, families were unaware at the time of a familial FAP history for 7 children, whereas only 4 cases were sporadic. A panel of 27 different pathogenic adenomatous polyposis coli (<jats:italic>APC<\/jats:italic>) germ\u2010line mutations and large genomic deletions were identified in 43 families. Extracolonic manifestations were found in half of the patients. As part of the standard practice for initial screening, the entire cohort underwent colonoscopy, which revealed adenoma above an intact rectosigmoid in 8 cases. Prophylactic colectomy was performed in 42 cases; high\u2010grade dysplastic adenoma and 1 invasive carcinoma were detected in 6 children. For timing of surgery, indications were in accordance with recent international guidelines.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions:<\/jats:title><jats:p>Defining optimal screening and therapeutic modalities in pediatric FAP cohorts is a challenge. Specific advice for genetic screening, endoscopy surveillance, and type of surgery based on recent guidelines is recommended.<\/jats:p><\/jats:sec>","DOI":"10.1097\/mpg.0b013e3182198f4d","type":"journal-article","created":{"date-parts":[[2011,10,4]],"date-time":"2011-10-04T20:46:18Z","timestamp":1317761178000},"page":"296-302","source":"Crossref","is-referenced-by-count":23,"title":["Evaluation of Guidelines for Management of Familial Adenomatous Polyposis in a Multicenter Pediatric Cohort"],"prefix":"10.1002","volume":"53","author":[{"given":"Anne","family":"Munck","sequence":"first","affiliation":[{"name":"Pediatric Gastroenterology, Nutrition, and Cystic Fibrosis Department Universit\u00e9 Paris 7 H\u00f4pital Robert Debr\u00e9 Paris"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lamia","family":"Gargouri","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology, Nutrition, and Cystic Fibrosis Department Universit\u00e9 Paris 7 H\u00f4pital Robert 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