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4 岁患儿多系统受累的 Erdheim-Chester 病。

Erdheim-Chester disease with multisystem involvement in a 4-year-old.

机构信息

Department of Radiology, Ewha Womans University School of Medicine, Seoul, South Korea.

出版信息

Pediatr Radiol. 2012 May;42(5):632-5. doi: 10.1007/s00247-011-2235-8. Epub 2011 Aug 31.

Abstract

Erdheim-Chester disease is a rare form of non-Langerhans histiocytosis of unknown origin occurring mainly in adults. It is extremely rare in children. We report a case of a 4-year-old boy with Erdheim-Chester disease that initially presented as hemifacial palsy and bone pain with multisystem involvement. We describe radiographic findings of bones that show characteristic bilateral symmetrical osteosclerosis with atypical osteolytic lesions in addition to CT findings for pulmonary involvement and MR findings for intracranial lesions.

摘要

厄尔-道伊姆-切斯特病是一种罕见的原因不明的非朗格汉斯组织细胞增生症,主要发生于成人,在儿童中极为罕见。我们报告了一例 4 岁男孩患厄尔-道伊姆-切斯特病的病例,其最初表现为单侧面瘫和骨痛,并伴有多系统受累。我们描述了骨骼的放射学表现,除了肺部受累的 CT 表现和颅内病变的磁共振表现外,还显示出特征性的双侧对称性骨硬化伴非典型溶骨性病变。

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