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关于戈勒姆-斯托特病中血管与骨质消失的观点。

Viewpoints on vessels and vanishing bones in Gorham-Stout disease.

作者信息

Dellinger Michael T, Garg Nupur, Olsen Bjorn R

机构信息

Division of Surgical Oncology, Department of Surgery, University of Texas Southwestern Medical Center, Dallas, TX, USA; The Lymphatic Malformation Institute, Bethesda, MD, USA.

The Lymphatic Malformation Institute, Bethesda, MD, USA.

出版信息

Bone. 2014 Jun;63:47-52. doi: 10.1016/j.bone.2014.02.011. Epub 2014 Feb 26.

DOI:10.1016/j.bone.2014.02.011
PMID:24583233
Abstract

Gorham-Stout disease (GSD) is a rare disorder characterized by the proliferation of endothelial-lined vessels in bone and the progressive destruction of bone. Although Jackson described the first case of GSD in 1838, the clinical and histological features of GSD were not defined until Gorham and Stout published their report on massive osteolysis in 1955. In the years since Gorham and Stout's groundbreaking publication, more than 300 cases of GSD have been described in the literature. These reports have revealed that the progressive resorption of bone in GSD causes severe physical deformities, disabilities, and life-threatening complications. Unfortunately, the underlying cause of GSD remains unknown and, as a result, the therapeutic options for individuals with GSD are limited. Here we review the latest advances in GSD research and present strategies to address basic and clinical research questions related to GSD.

摘要

戈勒姆-斯托特病(GSD)是一种罕见的疾病,其特征是骨内内皮细胞衬里的血管增生以及骨的进行性破坏。尽管杰克逊在1838年描述了第一例GSD,但直到1955年戈勒姆和斯托特发表关于大量骨质溶解的报告后,GSD的临床和组织学特征才得以明确。自戈勒姆和斯托特具有开创性的发表以来的这些年里,文献中已描述了300多例GSD病例。这些报告表明,GSD中骨的进行性吸收会导致严重的身体畸形、残疾和危及生命的并发症。不幸的是,GSD的根本原因仍然未知,因此,GSD患者的治疗选择有限。在此,我们回顾GSD研究的最新进展,并提出解决与GSD相关的基础和临床研究问题的策略。

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