PDF(Pubmed)
Abstract：
Fibrous dysplasia (FD) is a rare, non-hereditary skeletal disorder characterized by its chronic course of non-neoplastic fibrous tissue buildup in place of healthy bone. A myriad of factors have been associated with its onset and progression. Perturbation of cell-cell signaling networks and response outputs leading to disrupted building blocks, incoherent multi-level organization, and loss of rigid structural motifs in mineralized tissues are factors that have been identified to participate in FD induction. In more recent years, novel insights into the unique biology of FD are transforming our understandings of its pathology, natural discourse of the disease, and treatment prospects. Herein, we built upon existing knowledge with recent findings to review clinical, etiologic, and histological features of FD and discussed known and potential mechanisms underlying FD manifestations. Subsequently, we ended on a note of optimism by highlighting emerging therapeutic approaches aimed at either halting or ameliorating disease progression.
摘要：
纤维发育不良（FD）是一种罕见的，非遗传性骨骼疾病，其特征是慢性非肿瘤性纤维组织积聚代替健康骨骼。无数因素与其发病和进展有关。细胞-细胞信号网络和响应输出的扰动导致构建块中断，不连贯的多层次组织，矿化组织中刚性结构基序的丢失是已确定参与FD诱导的因素。近年来,对FD独特生物学的新见解正在改变我们对其病理学的理解，疾病的自然话语，和治疗前景。在这里，我们基于现有的知识和最近的发现来回顾临床，病因学，和FD的组织学特征，并讨论了FD表现的已知和潜在机制。随后，我们以乐观的态度结束,我们强调了旨在阻止或改善疾病进展的新兴治疗方法.
