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朗格汉斯细胞组织细胞增多症的颅面及颅内表现:100例患者的研究结果报告

Craniofacial and intracranial manifestations of langerhans cell histiocytosis: report of findings in 100 patients.

作者信息

D'Ambrosio Nicholas, Soohoo Stephanie, Warshall Craig, Johnson Alan, Karimi Sasan

机构信息

Department of Radiology, Schneider Children's Hospital, Long Island Jewish Medical Center, New Hyde Park, NY 11040, USA.

出版信息

AJR Am J Roentgenol. 2008 Aug;191(2):589-97. doi: 10.2214/AJR.07.3573.

Abstract

OBJECTIVE

The purpose of this study was to review the craniofacial and intracranial clinical and radiologic manifestations of patients diagnosed with Langerhans cell histiocytosis (LCH). This report will compare the frequency of the various manifestations found in our series with those reported in the medical literature.

CONCLUSION

In LCH, involvement of the calvaria, skull base, maxillofacial bones, and hypothalamic-pituitary axis is fairly common. The precise location of these lesions contributes to the variety of clinical manifestations of LCH, which includes scalp and/or facial swelling, seizures, hearing loss, recurrent otitis media, gingival bleeding, proptosis, diabetes insipidus, and cranial nerve palsies.

摘要

目的

本研究的目的是回顾诊断为朗格汉斯细胞组织细胞增多症(LCH)患者的颅面及颅内临床和影像学表现。本报告将比较我们系列中发现的各种表现的频率与医学文献中报道的频率。

结论

在LCH中,颅盖、颅底、颌面骨及下丘脑-垂体轴受累相当常见。这些病变的确切位置导致了LCH临床表现的多样性,包括头皮和/或面部肿胀、癫痫发作、听力丧失、复发性中耳炎、牙龈出血、眼球突出、尿崩症及颅神经麻痹。

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