{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,13]],"date-time":"2026-07-13T10:10:21Z","timestamp":1783937421527,"version":"3.55.0"},"reference-count":28,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,7,13]],"date-time":"2026-07-13T00:00:00Z","timestamp":1783900800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2026,7,13]],"date-time":"2026-07-13T00:00:00Z","timestamp":1783900800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. pediatr. gastroenterol. nutr."],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>Hereditary polyposis syndromes in children are rare, heterogeneous disorders associated with significant morbidity and long\u2010term risk of malignancy. Current data on their presentation, management, and outcomes in pediatric populations remain limited.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We conducted a nationwide, multicenter, retrospective study that included children diagnosed with polyposis syndromes in eight pediatric gastroenterology centers in Israel. Demographic, genetic, clinical, endoscopic, and outcome data were collected.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>A total of 105 children were included: 30 with juvenile polyposis syndrome (JPS), 26 with familial adenomatous polyposis (FAP), 19 with Peutz\u2013Jeghers syndrome, 16 with phosphatase and tensin homolog\u2010hamartoma tumor syndrome, 8 with constitutional mismatch repair deficiency syndrome, 5 with hereditary mixed polyposis syndrome, and 1 with Lynch syndrome. The median age at diagnosis was 8 years (interquartile range 4.1\u201311.5). Overall, 43 (41%) were of Ashkenazi Jewish origin, and a family history of polyposis was present in 54 (51%). Genetic testing was performed in 91 (87%), identifying pathogenic variants in 77 (73%) of them. Gastrointestinal symptoms were reported in 60 (57%) and extra\u2010intestinal manifestations in 48 (46%). Polyposis\u2010related surgery was required in 22 (21%) children. Malignancy in eight children included two cases of colorectal carcinoma and glioblastoma multiforme, T cell lymphoma with Wilm's tumor, medulloblastoma, ganglioneuroblastoma, and abdominal adenocarcinoma. Three mortality events were recorded.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Israeli children with hereditary polyposis syndromes present with diverse clinical and genetic features. Despite increasing use of genetic testing, morbidity and malignancy risks remain substantial, highlighting the need for early recognition, surveillance, and multidisciplinary care.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/jpn3.70500","type":"journal-article","created":{"date-parts":[[2026,7,13]],"date-time":"2026-07-13T09:23:57Z","timestamp":1783934637000},"source":"Crossref","is-referenced-by-count":0,"title":["Clinical and genetic features of pediatric hereditary polyposis syndromes in Israel: A nationwide multicenter cohort"],"prefix":"10.1002","author":[{"given":"Shlomi","family":"Cohen","sequence":"first","affiliation":[{"name":"Pediatric Gastroenterology Institute, \u201cDana\u2010Dwek\u201d Children's Hospital Tel Aviv Sourasky Medical Center Tel Aviv Israel"},{"name":"Gray Faculty of Medical and Health Sciences Tel Aviv University Tel Aviv Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tal","family":"Cozacov","sequence":"additional","affiliation":[{"name":"Gray Faculty of Medical and Health Sciences Tel Aviv University Tel Aviv Israel"},{"name":"Institute of Gastroenterology, Nutrition and Liver Diseases Schneider Children's Medical Center of Israel Petach Tikva Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ilana","family":"Weintraub","sequence":"additional","affiliation":[{"name":"Gray Faculty of Medical and Health Sciences Tel Aviv University Tel Aviv Israel"},{"name":"Pediatric Gastroenterology Unit, Edmond &amp; Lily Safra Children's Hospital Sheba Medical Center Ramat\u2010Gan Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ron","family":"Shaoul","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology &amp; Nutrition Institute, Ruth Rappaport Children's Hospital, Rambam Health Care Campus and the Faculty of Medicine, Technion Israel Institute of Technology Haifa Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8109-814X","authenticated-orcid":false,"given":"Oren","family":"Ledder","sequence":"additional","affiliation":[{"name":"The Juliet Keidan Institute of Paediatric Gastroenterology and Nutrition, The Eisenberg R&amp;D Authority, Shaare Zedek Medical Center The Hebrew University of Jerusalem Jerusalem Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dotan","family":"Yogev","sequence":"additional","affiliation":[{"name":"The Juliet Keidan Institute of Paediatric Gastroenterology and Nutrition, The Eisenberg R&amp;D Authority, Shaare Zedek Medical Center The Hebrew University of Jerusalem Jerusalem Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mordechai","family":"Slae","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Hadassah \u2010 Hebrew University Medical Center Jerusalem Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ranya","family":"Egbarieh","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology Unit HaEmek Medical Center Afula Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Galina","family":"Ling","sequence":"additional","affiliation":[{"name":"The Pediatric Day\u2010Care Unit, Soroka University Medical Center, and Faculty of Health Sciences Ben\u2010Gurion University of the Negev Beer\u2010Sheva Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anat","family":"Yerushalmy\u2010Feler","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology Institute, \u201cDana\u2010Dwek\u201d Children's Hospital Tel Aviv Sourasky Medical Center Tel Aviv Israel"},{"name":"Gray Faculty of Medical and Health Sciences Tel Aviv University Tel Aviv 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