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Peutz-Jeghers syndrome: a systematic review and recommendations for management

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dc.creator Beggs, A. D.
dc.creator Latchford, A. R.
dc.creator Vasen, Hans
dc.creator Möslein, Gabriela
dc.creator Alonso, A.
dc.creator Aretz, S.
dc.creator Bertario, Lucio
dc.creator Blanco Guillermo, Ignacio
dc.creator Bülow, S.
dc.creator Burn, John
dc.creator Capellá, G. (Gabriel)
dc.creator Colas, Chrystelle
dc.creator Friedl, W.
dc.creator Møller, Pål
dc.creator Hes, F. J.
dc.creator Järvinen, Heikki
dc.creator Mecklin, Jukka-Pekka
dc.creator Nagengast, F. M.
dc.creator Parc, Yann
dc.creator Phillips, R. K. S.
dc.creator Hyer, W.
dc.creator Ponz de Leon, Maurizio
dc.creator Renkonen Sinisalo, Laura
dc.creator Sampson, J. R.
dc.creator Stormorken, A.
dc.creator Tejpar, Sabine
dc.creator Thomas, H. J. W.
dc.creator Wijnen, Juul
dc.creator Clark, Susan K.
dc.creator Hodgson, Shirley V.
dc.date 2011-07-07T11:33:20Z
dc.date 2011-07-07T11:33:20Z
dc.date 2010
dc.date.accessioned 2024-12-16T10:27:14Z
dc.date.available 2024-12-16T10:27:14Z
dc.identifier 0017-5749
dc.identifier http://hdl.handle.net/2445/18638
dc.identifier 578379
dc.identifier.uri http://fima-docencia.ub.edu:8080/xmlui/handle/123456789/22101
dc.description Peutz¿Jeghers syndrome (PJS, MIM175200) is an autosomal dominant condition defined by the development of characteristic polyps throughout the gastrointestinal tract and mucocutaneous pigmentation. The majority of patients that meet the clinical diagnostic criteria have a causative mutation in the STK11 gene, which is located at 19p13.3. The cancer risks in this condition are substantial, particularly for breast and gastrointestinal cancer, although ascertainment and publication bias may have led to overestimates in some publications. Current surveillance protocols are controversial and not evidence-based, due to the relative rarity of the condition. Initially, endoscopies are more likely to be done to detect polyps that may be a risk for future intussusception or obstruction rather than cancers, but surveillance for the various cancers for which these patients are susceptible is an important part of their later management. This review assesses the current literature on the clinical features and management of the condition, genotype¿phenotype studies, and suggested guidelines for surveillance and management of individuals with PJS. The proposed guidelines contained in this article have been produced as a consensus statement on behalf of a group of European experts who met in Mallorca in 2007 and who have produced guidelines on the clinical management of Lynch syndrome and familial adenomatous polyposis.
dc.format 12 p.
dc.format application/pdf
dc.language eng
dc.publisher BMJ Group
dc.relation Reproducció digital del document publicat a: http://dx.doi.org/10.1136/gut.2009.198499
dc.relation Gut, 2010, vol. 59, núm. 7, p. 975-986
dc.relation http://dx.doi.org/10.1136/gut.2009.198499
dc.rights (c) BMJ Publishing Group Ltd and British Society of Gastroenterology, 2010
dc.rights info:eu-repo/semantics/openAccess
dc.source Articles publicats en revistes (Patologia i Terapèutica Experimental)
dc.subject Malalties intestinals
dc.subject Pòlips (Patologia)
dc.subject Intestinal diseases
dc.subject Polyps (Pathology)
dc.title Peutz-Jeghers syndrome: a systematic review and recommendations for management
dc.type info:eu-repo/semantics/article
dc.type info:eu-repo/semantics/publishedVersion


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