Blue Rubber Bleb Nevus Syndrome: Successful Treatment With Sirolimus

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dc.contributor.authorYuksekkaya, Hasan
dc.contributor.authorOzbek, Orhan
dc.contributor.authorKeser, Melike
dc.contributor.authorToy, Hatice
dc.date.accessioned2020-03-26T18:24:09Z
dc.date.available2020-03-26T18:24:09Z
dc.date.issued2012
dc.departmentSelçuk Üniversitesien_US
dc.description.abstractBlue rubber bleb nevus syndrome (BRBNS) is a rare disorder with characteristic vascular malformations of the skin, gastrointestinal system, and, less often, other organ systems. The characteristic cutaneous lesions consist of deep-blue, soft, rubbery blebs, which are easily compressible. The most serious complication is abundant gastrointestinal bleeding. We describe the case of an 8-year-old girl with diagnosed BRBNS who had multiple venous malformations all over her body, importantly, throughout the gastrointestinal tract, mouth, esophagus, stomach, small bowel, and colon. She presented with recurrent massive gastrointestinal bleeding and soft tissue hematoma despite prednisolone and a-interferon therapy. We started low-dose sirolimus as an antiangiogenic agent. The vascular masses were reduced rapidly and there was no gastrointestinal bleeding and muscular hematoma after sirolimus therapy. There was no drug adverse reaction at 20-month follow-up. To the best of our knowledge, this is the first report related to the use of sirolimus in a patient with BRBNS. Pediatrics 2012;129:e1080-e1084en_US
dc.identifier.doi10.1542/peds.2010-3611en_US
dc.identifier.endpageE1084en_US
dc.identifier.issn0031-4005en_US
dc.identifier.issue4en_US
dc.identifier.pmid22392180en_US
dc.identifier.scopusqualityQ1en_US
dc.identifier.startpageE1080en_US
dc.identifier.urihttps://dx.doi.org/10.1542/peds.2010-3611
dc.identifier.urihttps://hdl.handle.net/20.500.12395/27796
dc.identifier.volume129en_US
dc.identifier.wosWOS:000302541700032en_US
dc.identifier.wosqualityQ1en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherAMER ACAD PEDIATRICSen_US
dc.relation.ispartofPEDIATRICSen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.selcuk20240510_oaigen_US
dc.subjectgastrointestinal bleedingen_US
dc.subjecthemangiomatosisen_US
dc.subjectsirolimusen_US
dc.subjectblue rubber bleb nevus syndromeen_US
dc.titleBlue Rubber Bleb Nevus Syndrome: Successful Treatment With Sirolimusen_US
dc.typeArticleen_US

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