西罗莫司治疗蓝色橡皮疱痣综合征：一项系统评价。
Sirolimus in blue rubber bleb naevus syndrome: A systematic review.
作者信息
Wong Xin Lin, Phan Kevin, Rodríguez Bandera Ana I, Sebaratnam Deshan F
机构信息
University of New South Wales, Sydney, New South Wales, Australia.
Department of Dermatology, Liverpool Hospital, Sydney, New South Wales, Australia.
出版信息
J Paediatr Child Health. 2019 Feb;55(2):152-155. doi: 10.1111/jpc.14345. Epub 2018 Dec 18.
The aim of this study is to review sirolimus as a treatment for blue rubber bleb naevus syndrome (BRBNS). A literature search of Medline, Embase, CINAHL, SCOPUS and Google Scholar was conducted for publications reporting treatment of patients with BRBNS with sirolimus. Of 46 articles identified, 17 studies reporting 23 patients met inclusion criteria. Sirolimus was well tolerated in all but one patient who required treatment cessation; 17/18 patients noted an improvement in gastrointestinal disease where this was reported; 21/22 patients noted an improvement in cutaneous disease where this was reported. Based on these results, sirolimus may be considered a first-line treatment of BRBNS depending on patient morbidity.
本研究的目的是回顾西罗莫司作为蓝色橡皮疱痣综合征（BRBNS）的一种治疗方法。我们在Medline、Embase、CINAHL、SCOPUS和谷歌学术上进行了文献检索，以查找报告用西罗莫司治疗BRBNS患者的出版物。在检索到的46篇文章中，17项研究报告的23例患者符合纳入标准。除1例需要停药的患者外，所有患者对西罗莫司耐受性良好；在报告有胃肠道疾病的患者中，17/18例患者病情有所改善；在报告有皮肤疾病的患者中，21/22例患者病情有所改善。基于这些结果，根据患者的发病率，西罗莫司可被视为BRBNS的一线治疗方法。
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