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以早发和快速进展为特征的家族性颌骨多囊病的诊断与治疗

Diagnosis and treatment of familial cherubism characterized by early onset and rapid development.

作者信息

Mortellaro Carmen, Bello Lucilla, Lucchina Alberta Greco, Pucci Angela

机构信息

Department of Medical Science, Faculty of Medicine, University of Eastern Piedmont, Novara, Italy.

出版信息

J Craniofac Surg. 2009 Jan;20(1):116-20. doi: 10.1097/SCS.0b013e318190e23c.

Abstract

Cherubism is a benign maxillary bone dysplasia of childhood, usually showing an autosomically dominant inheritance with variable penetrance and spontaneously resolving after puberty. Only maxillary bones are affected and develop pseudocystic osteolytic lesions. This article presents an early and rapidly evolving familial case of cherubism. The 3-year-old child underwent conservative curettage of lesions, with a conservative approach that allowed a normal permanent dentition in adolescence. Family history revealed that the father had been treated for similar lesions between 14 and 21 years of age, but the late treatment caused edentulism. In conclusion, although cherubism represents a benign and localized maxillary dysplasia, it requires prompt surgical but conservative treatment and careful follow-up to avoid permanent lesions, that is, malocclusion and/or edentulism.

摘要

颌骨肥大症是一种儿童期的良性上颌骨发育异常,通常呈常染色体显性遗传,具有可变的外显率,青春期后可自发缓解。仅上颌骨受累并出现假性囊性溶骨性病变。本文介绍了一例早期且进展迅速的家族性颌骨肥大症病例。该3岁儿童接受了病变的保守刮除术,采用的保守方法使青春期恒牙列正常。家族史显示,父亲在14至21岁之间曾接受过类似病变的治疗,但治疗延迟导致了无牙症。总之,尽管颌骨肥大症是一种良性局限性上颌骨发育异常,但仍需要及时进行手术但保守的治疗,并进行仔细的随访,以避免永久性病变,即错牙合畸形和/或无牙症。

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