Department of Orthodontics, Affiliated Stomatological Hospital, Zhejiang University School of Medicine, Hangzhou, China.
J Periodontol. 2011 Jul;82(7):1089-95. doi: 10.1902/jop.2010.100599. Epub 2010 Dec 13.
Hereditary gingival fibromatosis (HGF) is a rare benign disorder characterized by progressive overgrowth of gingiva. Although the clinical and histopathologic characteristics of HGF are explicit, the pathogenic mechanism remains unclear. The goal of this article is to describe a three-generation HGF case and discuss the diagnosis, treatment, and inheritance of the disease. The known cellular and molecular features of HGF are also emphasized.
Family and medical histories of the patients were recorded, and a series of preliminary examinations, including clinical, histologic, radiographic, and gene examination, were performed to make a diagnosis and learn about the genetic characteristics. An all-quadrant flap surgery was performed to remove excess gingiva, and orthodontic treatment was undertaken to help tooth eruption. Recent advances were reviewed for further knowledge of genetic, cellular, and molecular features of HGF.
The patient's manifestations and examinations showed a typical HGF characteristic. There was no recurrence after surgery, and the premolars and molars erupted to bite plane. Genetic studies have found several gene mutations involved in HGF. Only the son-of-sevenless-1 gene is identified. Multiple molecular factors, such as transforming growth factor-β and matrix metalloproteinases, participate in HGF, regulating the extracellular matrix.
Surgical intervention is the usual treatment of HGF, but patients still have to deal with the risk of recurrence. Once the correlations between gene mutations, molecular changes, histology, and clinical situation are clear, they can be applied to clinical application, providing novel methods for disease prognosis and diagnosis and targets for disease prevention and treatment.
遗传性牙龈纤维瘤病（Hereditary gingival fibromatosis，HGF）是一种罕见的良性疾病，其特征为牙龈进行性过度生长。尽管 HGF 的临床和组织病理学特征明确，但发病机制仍不清楚。本文旨在描述一个三代 HGF 病例，并讨论该病的诊断、治疗和遗传。还强调了 HGF 的已知细胞和分子特征。
记录患者的家族和病史，并进行一系列初步检查，包括临床、组织学、影像学和基因检查，以做出诊断并了解遗传特征。进行全象限瓣手术以切除多余的牙龈，并进行正畸治疗以帮助牙齿萌出。回顾了最新进展，以进一步了解 HGF 的遗传、细胞和分子特征。
患者的表现和检查显示出典型的 HGF 特征。手术后无复发，前磨牙和磨牙萌出至咬合平面。基因研究发现了几个与 HGF 相关的基因突变。仅鉴定出 Son-of-sevenless-1 基因。多种分子因素，如转化生长因子-β和基质金属蛋白酶，参与 HGF，调节细胞外基质。
手术干预是 HGF 的常用治疗方法，但患者仍需应对复发的风险。一旦明确基因突变、分子变化、组织学和临床情况之间的相关性，就可以将其应用于临床应用，为疾病的预后和诊断提供新的方法，并为疾病的预防和治疗提供靶点。
