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戈谢-斯托特综合征合并乳糜胸一例

Gorham-Stout syndrome with chylothorax in a six-year-old boy.

机构信息

Department of Pediatric Cardiology, Ege University Faculty of Medicine, 225 sokak, No: 10/25, 35040 Bornova-Izmir, Turkey.

出版信息

Indian J Pediatr. 2011 Jun;78(6):737-9. doi: 10.1007/s12098-010-0328-2. Epub 2010 Dec 25.

Abstract

Gorham-Stout syndrome, also called "disappearing bone disease, diffuse cystic angiomatosis of bone, disseminated lymphangiomatosis, Gorham's vanishing bone disease, phantom bone disease or idiopathic massive osteolysis, is a rare disease of unknown etiology and pathogenesis. It is characterized by rapidly progressive localized massive osteolysis associated with proliferation of vascular structures of benign origin in which the absence of new bone formation is representative. When it is complicated by chylothorax, the prognosis is poor. The authors report a 6-year-old boy with Gorham-Stout syndrome who presented with pleural effusion showing features of chylothorax, who responsed poorly to currently available therapeutic modalities.

摘要

戈勒姆-斯托特综合征,又称“消失性骨病”、“弥漫性囊状血管瘤病、骨播散性淋巴管瘤病、戈勒姆氏消失性骨病、幻影骨病或特发性骨溶解症”,是一种病因和发病机制不明的罕见疾病。其特征为迅速进展的局限性骨溶解,伴有良性起源的血管结构增生,其中无新骨形成是其代表性特征。当合并乳糜胸时,预后较差。作者报告了一例 6 岁男孩患戈勒姆-斯托特综合征,表现为胸腔积液,具有乳糜胸的特征,对现有治疗方法反应不佳。

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