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家族性 florid 骨化性纤维瘤病——病例报告及文献综述。

Familial florid cemento-osseous dysplasia--a case report and review of the literature.

作者信息

Coleman H, Altini M, Kieser J, Nissenbaum M

机构信息

Department of Oral Pathology, University of the Witwatersrand, Johannesburg, Wits.

出版信息

J Dent Assoc S Afr. 1996 Dec;51(12):766-70.

PMID:9462035
Abstract

Cemento-osseous dysplasia is a fairly common lesion of the jaws originating from the elements of the periodontal ligament. Most lesions do not have a hereditary basis, however a few familial cases have been documented. This is the first report of the florid form of cemento-osseous dysplasia in an African family. The mother, son and one daughter exhibited multiple sclerotic cemental masses of the jaws with large tumour-like swellings in the symphyseal area of the mandible. In the case of the mother the lesions had become infected. The familial form of cemento-osseous dysplasia differs from the non-hereditary type in that the lesions present in children, all cases are florid in nature, there is no predilection for black females, the rate of growth appears to be more rapid and the symphyseal area of the mandible is most severely affected. The mode of transmission appears to be autosomal dominant with variable phenotypic expression.

摘要

骨化性纤维瘤是一种起源于牙周膜成分的颌骨常见病变。大多数病变没有遗传基础,不过已有少数家族性病例的记录。这是非洲家族中出现的 florid 型骨化性纤维瘤的首例报告。母亲、儿子和一个女儿均表现出颌骨多发硬化性牙骨质肿块,在下颌骨联合区有巨大肿瘤样肿胀。母亲的病变已发生感染。骨化性纤维瘤的家族型与非遗传型不同,在于病变出现在儿童中,所有病例本质上均为 florid 型,无黑人女性偏好,生长速度似乎更快,且下颌骨联合区受影响最严重。遗传方式似乎为常染色体显性遗传,具有可变的表型表达。

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