Katz Joseph, Guelmann Marcio, Barak Shlomo
Department of Oral and Maxillofacial Surgery, University of Florida, Gainesville, USA.
Pediatr Dent. 2002 May-Jun;24(3):253-6.
A case of a 9-year-old child with hereditary gingival fibromatosis, supernumerary tooth, chest deformities, auricular cartilage deformation, joint laxity and undescended testes is described. The exact mode of inheritance is unclear; a new mutation pattern is possible. These features resemble but differ from the previously reported Laband syndrome. The dental treatment consisted of surgical removal of the fibrous tissue and conservative restorative treatment under general anesthesia. The dental practitioner should be alert for developmental abnormalities such as supernumerary teeth and delayed tooth eruption. A comprehensive medical history and physical systemic evaluation is essential to rule out other systemic abnormalities. Genetic consultation is mandatory for future family planing.
本文描述了一例9岁儿童,患有遗传性牙龈纤维瘤病、多生牙、胸部畸形、耳廓软骨变形、关节松弛和隐睾。确切的遗传模式尚不清楚;可能存在新的突变模式。这些特征与先前报道的拉班德综合征相似但又有所不同。牙科治疗包括在全身麻醉下手术切除纤维组织和进行保守性修复治疗。牙科医生应警惕多生牙和牙齿萌出延迟等发育异常情况。全面的病史和身体系统评估对于排除其他全身异常至关重要。对于未来的计划生育,遗传咨询是必不可少的。