{"id":1094,"date":"2018-02-10T19:28:10","date_gmt":"2018-02-10T19:28:10","guid":{"rendered":"http:\/\/stg-blogs.bmj.com\/jmg\/?p=1094"},"modified":"2018-02-10T19:28:10","modified_gmt":"2018-02-10T19:28:10","slug":"evidence-for-genetic-anticipation-in-von-hippel-lindau-syndrome","status":"publish","type":"post","link":"https:\/\/stg-blogs.bmj.com\/jmg\/2018\/02\/10\/evidence-for-genetic-anticipation-in-von-hippel-lindau-syndrome\/","title":{"rendered":"Evidence for genetic anticipation in von Hippel-Lindau syndrome"},"content":{"rendered":"<p style=\"margin: 0in;margin-bottom: .0001pt;background: white\"><span style=\"font-family: 'Calibri',sans-serif;color: black\">Von Hippel-Lindau syndrome (vHL) is a hereditary cancer syndrome that predisposes to developing tumours in\u00a0multiple organs. Initiating tumour surveillance before tumours are likely to occur is\u00a0critical\u00a0for optimal patient outcomes. In our study, we found significantly earlier ages of tumour onset in children with vHL compared to their vHL parents, indicating vHL\u00a0may exhibit genetic anticipation (where\u00a0symptoms present at younger ages in subsequent generations). Unlike dyskeratosis congenita, another cancer syndrome, anticipation in vHL was not associated with telomere shortening. Our observations support\u00a0modifying surveillance guidelines to account for earlier ages of onset in younger generations with vHL. (By Kalene van Engelen, <a href=\"http:\/\/jmg.bmj.com\/content\/early\/2018\/02\/06\/jmedgenet-2017-104882\">http:\/\/jmg.bmj.com\/content\/early\/2018\/02\/06\/jmedgenet-2017-104882<\/a> )<\/span><\/p>\n<p><!--TrendMD v2.4.8--><\/p>\n","protected":false},"excerpt":{"rendered":"<p>Von Hippel-Lindau syndrome (vHL) is a hereditary cancer syndrome that predisposes to developing tumours in\u00a0multiple organs. Initiating tumour surveillance before tumours are likely to occur is\u00a0critical\u00a0for optimal patient outcomes. In our study, we found significantly earlier ages of tumour onset in children with vHL compared to their vHL parents, indicating vHL\u00a0may exhibit genetic anticipation (where\u00a0symptoms [&#8230;]<\/p>\n<p><a class=\"btn btn-secondary understrap-read-more-link\" href=\"https:\/\/stg-blogs.bmj.com\/jmg\/2018\/02\/10\/evidence-for-genetic-anticipation-in-von-hippel-lindau-syndrome\/\">Read More&#8230;<\/a><\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"open","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"_jetpack_memberships_contains_paid_content":false,"footnotes":""},"categories":[1],"tags":[],"class_list":["post-1094","post","type-post","status-publish","format-standard","hentry","category-uncategorized"],"jetpack_featured_media_url":"","jetpack_sharing_enabled":true,"_links":{"self":[{"href":"https:\/\/stg-blogs.bmj.com\/jmg\/wp-json\/wp\/v2\/posts\/1094","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/stg-blogs.bmj.com\/jmg\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/stg-blogs.bmj.com\/jmg\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/stg-blogs.bmj.com\/jmg\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/stg-blogs.bmj.com\/jmg\/wp-json\/wp\/v2\/comments?post=1094"}],"version-history":[{"count":0,"href":"https:\/\/stg-blogs.bmj.com\/jmg\/wp-json\/wp\/v2\/posts\/1094\/revisions"}],"wp:attachment":[{"href":"https:\/\/stg-blogs.bmj.com\/jmg\/wp-json\/wp\/v2\/media?parent=1094"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/stg-blogs.bmj.com\/jmg\/wp-json\/wp\/v2\/categories?post=1094"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/stg-blogs.bmj.com\/jmg\/wp-json\/wp\/v2\/tags?post=1094"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}