Zimmerman-Laband Syndrome Oral Manifestations A Case Report

A. Mathew, M. James, H. Maria
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Abstract

Zimmerman-Laband syndrome was reported by Zimmerman in the year 1928 which is a rare inherited autosomal dominant disease characterized by generalized enlargement of the attached and marginal gingiva, abnormalities of nose, ear, deformities of nails, joint hyperextensibility, hepatosplenomegaly, skeletal abnormalities and occasional mental retardation. Idiopathic gingival enlargement is usually evident after the eruption of the permanent teeth. Both sexes are equally affected. Genetic loci for autosomal dominant modes of gingival fibromatosis is localized to chromosome 2p21p22 (HGF-1) and chromosome 5q12-q22 (HGF-2). This syndrome is not a life threatening disorder. Hereditary gingival enlargement is associated with syndromes like Rutherford syndrome, Zimmerman-Laband syndrome, Murray-Puretic-Drescher syndrome, Cross syndrome and Ramon’s syndrome. The most important feature of this syndrome is gingival enlargement appearing early in childhood. Idiopathic gingival enlargement is usually evident after the eruption of the permanent teeth. Surgical correction of gingival fibromatosis is recommended, although there is no information on the permanence of the results of this treatment. We present a case of a 14 year old female patient with Zimmerman-Laband syndrome. Gingivectomy was carried out in the upper and lower anterior region there by exposing the impacted teeth.
Zimmerman-Laband综合征口腔表现1例报告
Zimmerman- laband综合征由Zimmerman于1928年报道,是一种罕见的遗传常染色体显性遗传病,其特征是附着龈和边缘龈的广泛性扩大,鼻、耳异常,指甲畸形,关节过伸,肝脾肿大,骨骼异常和偶尔的智力迟钝。特发性牙龈肿大通常在恒牙长出后很明显。男女都受到同样的影响。牙龈纤维瘤病常染色体显性遗传位点定位于染色体2p21p22 (HGF-1)和染色体5q12-q22 (HGF-2)。这种综合症并不会危及生命。遗传性牙龈肿大与卢瑟福综合征、Zimmerman-Laband综合征、Murray-Puretic-Drescher综合征、Cross综合征和Ramon综合征等综合征有关。该综合征最重要的特征是在儿童早期出现牙龈肿大。特发性牙龈肿大通常在恒牙长出后很明显。手术矫正牙龈纤维瘤病是推荐的,虽然没有关于这种治疗结果的持久性的信息。我们提出一个病例14岁的女性患者与齐默曼-拉邦综合征。通过暴露阻生牙,在上、下前牙区进行牙龈切除术。
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