Gummy smile: could it be genetic? Hereditary gingival fibromatosis.

Rania Livada, Jacob Shiloah
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Abstract

Gingival enlargement is common among patients and can be caused by a variety of etiological factors. The most common reason is poor oral hygiene and high bacterial load that leads to gingival inflammation and enlargement. Other implicated factors include systemic drugs, such as phenytoin (Dilantin) taken by epileptic patients, calcium channel blockers such as nifedipine (Procardia) and verapamil (Calan) for the treatment of hypertension, arrhythmia and angina. Another class of medication associated with gingival enlargement is immunosuppressive agents given to organ-transplant patients to prevent rejection of the new element such as cyclosporine. Some enlargements could be associated with other conditions such as puberty, pregnancy or diabetes or be a symptom of a systemic disease (leukemia, Wegener's granulomatosis or sarcoidosis). In rare cases the cause for the enlargement is genetic and termed hereditary gingival fibromatosis (HGF). HGF is a genetic disorder characterized by a progressive enlargement of the gingiva. Histologically, the gingiva is characterized by an accumulation of dense fibrous connective tissue. This is believed to be due to an imbalance between synthesis and degradation of extracellular matrix composed mainly of collagen molecules or due to an alteration in fibroblast proliferation. Different pathogenic mechanisms have been proposed and examined over the years but no precise process has been identified. The main objective of this paper is to discuss this genetic anomaly and support it with clinical cases of a mother and her two children. It will focus on the clinical and histologic characteristics of HGF as well as known biologic and genetic features and treatment modalities.

软绵绵的微笑:这是遗传的吗?遗传性牙龈纤维瘤病。
牙龈肿大在患者中很常见,可由多种病因引起。最常见的原因是口腔卫生不良和细菌负荷高,导致牙龈炎症和扩大。其他相关因素包括全身性药物,如癫痫患者服用的苯妥英(苯妥英),钙通道阻滞剂,如硝苯地平(Procardia)和维拉帕米(Calan),用于治疗高血压、心律失常和心绞痛。另一类与牙龈扩大有关的药物是给予器官移植患者的免疫抑制剂,以防止对新元素(如环孢素)的排斥反应。一些肿大可能与其他疾病有关,如青春期、怀孕或糖尿病,也可能是全身性疾病(白血病、韦格纳肉芽肿病或结节病)的症状。在极少数情况下,增大的原因是遗传性的,称为遗传性牙龈纤维瘤病(HGF)。HGF是一种遗传性疾病,其特征是牙龈进行性扩大。在组织学上,牙龈的特点是密集的纤维结缔组织的积累。这被认为是由于主要由胶原分子组成的细胞外基质的合成和降解之间的不平衡或由于成纤维细胞增殖的改变。多年来,人们提出并研究了不同的致病机制,但没有确定确切的过程。本文的主要目的是讨论这种遗传异常,并支持它与一个母亲和她的两个孩子的临床病例。它将侧重于HGF的临床和组织学特征,以及已知的生物学和遗传学特征和治疗方式。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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