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侵袭性颌骨肥大症病例:17年随访

Aggressive case of cherubism: 17-year follow-up.

作者信息

Pontes Flávia S C, Ferreira Armando C, Kato Alberto M, Pontes Hélder A R, Almeida Diele S, Rodini Camila O, Pinto Décio S

机构信息

Department of Pathology, Dental School, Federal University of Pará, Pará, Brazil.

出版信息

Int J Pediatr Otorhinolaryngol. 2007 May;71(5):831-5. doi: 10.1016/j.ijporl.2007.01.017. Epub 2007 Mar 13.

Abstract

Cherubism is an autosomal dominant disorder in which the normal bone is replaced by cellular fibrous and immature bone, resulting in painless symmetrical enlargement of the jaws. An aggressive case of cherubism with extensive swelling on several facial bones in a 19-year-old boy is reported. The disorder was diagnosed 15 years ago, but the patient has not been submitted to any type of surgery so far. The highlights of this case are the great proportion of the lesions, the enormous functional and emotional disturbances brought about by these lesions, and the difficulty to choose the most appropriate age and form of treatment.

摘要

颌骨纤维异常增殖症是一种常染色体显性疾病,正常骨组织被细胞性纤维组织和未成熟骨取代,导致颌骨无痛性对称性肿大。本文报告了1例侵袭性颌骨纤维异常增殖症,患者为19岁男性,多块面骨广泛肿胀。该疾病于15年前确诊,但患者至今未接受任何手术治疗。该病例的突出特点是病变范围大、病变导致严重的功能和情感障碍,以及难以选择最合适的治疗年龄和治疗方式。

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