{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,12]],"date-time":"2026-01-12T22:39:32Z","timestamp":1768257572209,"version":"3.49.0"},"reference-count":16,"publisher":"S. Karger AG","issue":"1","license":[{"start":{"date-parts":[[2020,1,1]],"date-time":"2020-01-01T00:00:00Z","timestamp":1577836800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"},{"start":{"date-parts":[[2020,1,1]],"date-time":"2020-01-01T00:00:00Z","timestamp":1577836800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Horm Res Paediatr"],"published-print":{"date-parts":[[2020]]},"abstract":"<b><i>Background and Objective:<\/i><\/b> Gastrointestinal (GI) polyps with unknown malignant potential and a platelet storage pool deficiency that increases the risk of severe intraoperative and other types of bleeding have been identified in McCune-Albright syndrome (MAS). The natural course of these disorders has not been well characterized. The aim of this study was to report the follow-up of GI polyps and platelet dysfunction (PD) in a cohort of 28 patients with MAS. <b><i>Methods:<\/i><\/b> Twenty-eight patients with MAS (15 females) were included. Endoscopic screening for GI polyps was undertaken in 14 subjects and 19 were tested for PD. <b><i>Results:<\/i><\/b> Six subjects (5 males) were diagnosed with GI polyps at a median age of 23 (range 15\u201343) years, and were monitored for a median period of 8 (range 4.5\u201311.5) years. At endoscopic follow-up, the 4 patients with hamartomatous polyps at first endoscopy had either normal findings (<i>n<\/i> = 2), or duodenal gastric metaplasia (<i>n<\/i> = 2). Two patients with caecal polyps were identified. Of 8 subjects with a platelet storage pool deficiency, 5 required transfusions during surgery, and subsequent platelet cover in 2 markedly reduced intraoperative blood loss. <b><i>Conclusions:<\/i><\/b> New polyps with uncertain malignant potential are diagnosed after long term follow-up in MAS. Platelet cover reduces the need for red blood cell transfusion during orthopaedic surgery and may be useful to reduce non-operative bleeding events. We recommend regular upper and lower endoscopy and screening for PD in all MAS patients. <\/jats:p>","DOI":"10.1159\/000507442","type":"journal-article","created":{"date-parts":[[2020,5,8]],"date-time":"2020-05-08T21:00:26Z","timestamp":1588971626000},"page":"40-45","source":"Crossref","is-referenced-by-count":1,"title":["McCune Albright Syndrome: Gastrointestinal Polyps and Platelet Dysfunction over 12 Years"],"prefix":"10.1159","volume":"93","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7685-9311","authenticated-orcid":false,"given":"Francisca","family":"Grob","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Margaret","family":"Zacharin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"127","published-online":{"date-parts":[[2020,5,8]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","DOI":"10.1186\/s12885-018-4133-z"},{"key":"ref2","doi-asserted-by":"publisher","DOI":"10.1038\/onc.2010.202"},{"key":"ref3","doi-asserted-by":"publisher","DOI":"10.1126\/scisignal.2004088"},{"key":"ref4","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2010.086330"},{"key":"ref5","doi-asserted-by":"publisher","DOI":"10.1007\/s00428-017-2086-2"},{"key":"ref6","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2005.01338.x"},{"key":"ref7","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2008.02.045"},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.1111\/cen.13419"},{"key":"ref9","doi-asserted-by":"publisher","DOI":"10.1111\/cen.13722"},{"key":"ref10","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2018-01022"},{"key":"ref11","doi-asserted-by":"publisher","DOI":"10.1038\/bjc.2015.104"},{"key":"ref12","doi-asserted-by":"publisher","DOI":"10.1016\/j.trecan.2016.05.005"},{"key":"ref13","doi-asserted-by":"publisher","DOI":"10.1111\/pin.12070"},{"key":"ref14","doi-asserted-by":"publisher","DOI":"10.1053\/j.gastro.2010.03.002"},{"key":"ref15","doi-asserted-by":"publisher","DOI":"10.1002\/jbmr.2267"},{"key":"ref16","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-11-395418"}],"container-title":["Hormone Research in Paediatrics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/507442","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/507442","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,23]],"date-time":"2025-04-23T16:00:32Z","timestamp":1745424032000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000507442"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020]]},"references-count":16,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2020,7,6]]},"published-print":{"date-parts":[[2020,7,3]]}},"URL":"https:\/\/doi.org\/10.1159\/000507442","archive":["Portico"],"relation":{},"ISSN":["1663-2818","1663-2826"],"issn-type":[{"value":"1663-2818","type":"print"},{"value":"1663-2826","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020]]}}}