Somnath Pralhad Pachrupe, M. Phadnaik, Pooja Gupta
{"title":"1型神经纤维瘤病口腔康复6年随访病例报告","authors":"Somnath Pralhad Pachrupe, M. Phadnaik, Pooja Gupta","doi":"10.18311/IJMDS/2021/26284","DOIUrl":null,"url":null,"abstract":"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.","PeriodicalId":14010,"journal":{"name":"International Journal of Medical and Dental Sciences","volume":"12 1","pages":"1956-1960"},"PeriodicalIF":0.0000,"publicationDate":"2021-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Oral Rehabilitation in Neurofibromatosis Type 1- A Six Year Follow-Up Case Report\",\"authors\":\"Somnath Pralhad Pachrupe, M. Phadnaik, Pooja Gupta\",\"doi\":\"10.18311/IJMDS/2021/26284\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"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.\",\"PeriodicalId\":14010,\"journal\":{\"name\":\"International Journal of Medical and Dental Sciences\",\"volume\":\"12 1\",\"pages\":\"1956-1960\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2021-01-29\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"International Journal of Medical and Dental Sciences\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.18311/IJMDS/2021/26284\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"International Journal of Medical and Dental Sciences","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.18311/IJMDS/2021/26284","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
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.