Ramos Filipa, Figueira Ricardo, Romeu José Carlos, Queiroz Mario Viana
Serviço de Reumatologia e Doenças Osseas Metabólicas Hospital Santa Maria, Lisboa.
Acta Reumatol Port. 2007 Oct-Dec;32(4):387-91.
Fibrous Dysplasia FD of bone is a rare non-inheritable congenital disease characterized by a focal proliferation of fibrous tissue in the bone marrow leading to osteolytic lesions deformities and fractures. FD can be presented in a monostotic or polyostotic form this last one is usually more severe. FD primarily affects adolescents and young adults and in some cases in an asymptomatic way. We describe the case of a male patient with long evolution form of polyostotic fibrous dysplasia of bone with delayed clinical presentation. It is also highlighted the importance of an activity and involvement form evaluation as well as possible endocrine abnormalities associated in order to establish the correct treatment and prognosis.
骨纤维发育不良（FD）是一种罕见的非遗传性先天性疾病，其特征是骨髓中纤维组织局灶性增生，导致溶骨性病变、畸形和骨折。FD可表现为单骨型或多骨型，后者通常更严重。FD主要影响青少年和年轻人，在某些情况下无明显症状。我们描述了一名患有长期演进型多骨型骨纤维发育不良且临床表现延迟的男性患者病例。还强调了评估活动和受累形式以及相关可能的内分泌异常对于确立正确治疗和预后的重要性。
