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血管畸形的晚期后遗症及长期预后

Late sequelae and long-term outcomes of vascular anomalies.

作者信息

Adams Denise M, Fishman Steven J

机构信息

Department of Medicine, Vascular Anomalies Center, Boston Children's Hospital, Harvard Medical School, Boston, MA; Division of Hematology, Boston Children's Hospital, Harvard Medical School, Boston, MA.

Department of Medicine, Vascular Anomalies Center, Boston Children's Hospital, Harvard Medical School, Boston, MA; Department of Surgery, Boston Children's Hospital, Harvard Medical School, Boston, MA.

出版信息

Semin Pediatr Surg. 2017 Oct;26(5):317-321. doi: 10.1053/j.sempedsurg.2017.09.007. Epub 2017 Sep 9.

DOI:10.1053/j.sempedsurg.2017.09.007
PMID:29110828
Abstract

Vascular anomalies are classified as tumors and malformations depending on their clinical characteristics, pathological diagnosis and recent genomic information. Diagnosis can still be challenging because of the heterogeneity of clinical presentation. Thus, the best care is provided by an interdisciplinary team of specialists. An updated classification system has helped provide more consistent terminology with the addition of new diagnoses and genomic discovery. Historically, treatment of vascular anomalies was primarily surgical and or interventional with limited medical therapies. The field of vascular anomalies lacked prospective clinical trials in both medical treatments as well as surgical and interventional therapeutic options. Recent interdisciplinary collaboration has led to collaborative studies in which short- and long-term outcomes are being prospectively evaluated. Specifically, discoveries of pharmacologic agents effective in treating vascular anomalies have broadened our medical therapeutic options, which has led to innovative approaches in combined treatment of vascular anomalies and has stressed the need to prospectively assess long-term outcomes and sequelae for these patients which has been lacking in this field.

摘要

血管异常根据其临床特征、病理诊断和最新的基因组信息分为肿瘤和畸形。由于临床表现的异质性,诊断仍然具有挑战性。因此,由多学科专家团队提供最佳治疗。更新的分类系统通过增加新的诊断和基因组发现,有助于提供更一致的术语。历史上,血管异常的治疗主要是手术和/或介入治疗,药物治疗有限。血管异常领域在药物治疗以及手术和介入治疗选择方面都缺乏前瞻性临床试验。最近的跨学科合作导致了合作研究,其中正在对短期和长期结果进行前瞻性评估。具体而言,有效治疗血管异常的药物的发现拓宽了我们的药物治疗选择,这导致了血管异常联合治疗的创新方法,并强调需要前瞻性评估这些患者的长期结果和后遗症,而这在该领域一直缺乏。

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