1型神经纤维瘤病口腔康复6年随访病例报告

Somnath Pralhad Pachrupe, M. Phadnaik, Pooja Gupta
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摘要

神经纤维瘤病是一种常染色体显性遗传病,影响骨骼、神经系统、软组织和皮肤。该疾病的表现非常多变,表现范围从轻度病变到几种并发症和功能损害。否则,外显率为100%。大约72%的1型神经纤维瘤病患者表现为口腔表现。据估计,这些患者一生中患恶性疾病的风险增加3-15%。下面的报告提出了一个32岁的妇女诊断为1型神经纤维瘤病在儿童谁提出的部门抱怨牙龈出血。x线片显示左侧乙状切口浅,下颌骨左角钝角变薄,这是一些附加特征。这里采用牙科和医学专业的多学科方法进行口腔康复,管理和治疗牙周表现和其他口腔疾患。病例随访6年以上。神经纤维瘤病无法治愈,因此,当这些患者向牙科专业人员报告时,他们需要作为初级护理提供者,并灌输积极的心态,以帮助减少压力生活事件。
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Oral Rehabilitation in Neurofibromatosis Type 1- A Six Year Follow-Up Case Report
Neurofibromatosis is an autosomal dominant disorder that affects the bone, the nervous system, soft-tissue, and the skin. The expressivity of the disease is extremely variable, with manifestations ranging from mild lesions to several complications and functional impairment. The penetrance, otherwise, is 100%. Approximately 72% of Neurofibromatosis Type1 patients present with oral manifestations. These patients have an estimated 3-15% additional risk of malignant disease in their life-time. The following report presents a 32 year old lady diagnosed with Neurofibromatosis Type 1 in childhood who presented to department with complain of bleeding gums. Radiograph showed shallow sigmoid notch on left side, obtuse and thinned out left angle of mandible which are some additional features. A multidisciplinary approach of dental and medical specialties was taken here for oral rehabilitation and to manage and treat periodontal manifestations and other oral complaints. Case was followed for more than 6 years. Neurofibromatosis has no cure hence when such patients report to dental professionals it is up to them to act as primary care giver and instill positive frame of mind to help reduce stressful life events.
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