Behandlungskonzepte ossärer Manifestationen der Langerhanszellhistiozytose
Behandlungskonzepte ossärer Manifestationen der Langerhanszellhistiozytose
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朗格汉氏组织糖化病表现的相关问题
DOI：
10.1055/s-2008-1037400
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发表时间：
2008
期刊：
影响因子：
--
通讯作者：
U. Mittler
中科院分区：
文献类型：
--
作者：
W. Nebelung;M. Röpke;U. Kluba;V. Aumann;K. Radig;U. Mittler
从骨科的角度评估朗格汉斯细胞组织细胞增多症（LCH或嗜酸性肉芽肿）的患者，为疾病的治疗提供建议。材料与方法：回顾性分析48例经组织学证实的36例骨性表现。回顾分析1970年至1995年间我们治疗的LCH骨表现病例。结果：22例有孤立性骨表现，18例为单骨，4例为多骨。我们治疗了包括LCH骨表现在内的多器官疾病14例。在排他性骨表现的情况下，再激活是罕见的，通常发生在其他骨骼。结论：保证局部稳定控制是骨科治疗的总目标。在孤立的病变中，可以通过拔牙和填充松质骨或注射强的松来控制病变。多发病变应主要通过全身药物治疗，只有在发生严重局部问题时才需要手术治疗。此外，我们建议骨科医生，儿科医生和病理学家之间的跨学科合作。
INTRODUCTION: Patients with Langerhans Cell Histiocytosis (LCH or Eosinophilic granuloma) were assessed from the orthopaedic point of view to give recommendations for the management of the disease. MATERIAL AND METHODS: The results of 36 cases of histologically proven bony manifestations out of 48 treated cases were reviewed. A retrospective analysis of our treated cases with bony manifestations of LCH between 1970 and 1995 was performed. RESULTS: Twenty-two cases exhibited isolated bony manifestations, 18 were monoostotic and 4 were polyostotic. We treated 14 cases with multi-organ disease including bony manifestations of LCH. In the cases of exclusive bony manifestations reactivations were rare and usually occurred in other bones. CONCLUSIONS: In order to assure stability local control is the general goal of orthopaedic treatment. In isolated lesions control can be achieved by excochleation and filling with cancellous bone or prednisolon instillation. Multiple lesions should be treated primarily by systemic drugs and operative procedures are only necessary if severe local problems occur. Additionally, we recommend interdisciplinary cooperation between ortopedic surgeon, pediatrist and pathologist.