Dental outcomes in Fibrous Dysplasia/McCune Albright Syndrome

纤维性发育不良/麦库恩奥尔布赖特综合征的牙科结果

基本信息

批准号：
8705613
负责人：
Sunday O Akintoye
金额：
$ 20万
依托单位：
UNIVERSITY OF PENNSYLVANIA
依托单位国家：
美国
项目类别：
财政年份：
2013
资助国家：
美国
起止时间：
2013-09-25 至 2015-07-31
项目状态：
已结题

来源：
https://reporter.nih.gov/project-details/8705613
关键词：
Accounting Address Adenylate Cyclase Affect Bone Diseases Bone Pain Bone necrosis Case Study Case-Control Studies Clinical Practice Guideline Cohort Analysis Conflict (Psychology)Cyclic AMP Data Data Set Deformity Dental Dental Care Dental Enamel Dental Enamel Hypoplasia Dental Pulp Dental caries Dentin Dysplasia Development Disabled Persons Disease Dysplasia Effectiveness Endocrine Endocrine System Diseases Endodontics Enrollment Enzymes Extramural Activities Failure GNAS gene Gene Mutation Goals Growth Guidelines Health Personnel Hyperpigmentation Impaired wound healing Implant Intravenous Jaw Jaw Diseases Knowledge Lesion Malocclusion Mandible McCune-Albright Syndrome Mutation National Institute of Dental and Craniofacial Research Natural History Odontoma Oral health Orthodontic Osteitis Fibrosa Disseminata Outcome Patients Polyostotic fibrous dysplasia Publishing Pulp Chambers Quality of life Rare Diseases Reaction Reporting Research Risk Rotation Skin Skin Pigmentation Skin Tissue Tooth Attrition Tooth Diseases Tooth structure Treatment outcome United States National Institutes of Health bisphosphonate bone bone healing burden of illness cohort craniofacial craniofacial complex deciduous tooth dental surgery evidence base follow-up handicapping condition improved indexing mandible/maxilla orofacial outcome forecast patient population prospective protein complex response skeletal disorder success treatment planning

项目摘要

DESCRIPTION (provided by applicant): McCune-Albright syndrome (MAS), a rare multisystem disorder caused by GNAS1 gene mutation is characterized by polyostotic fibrous dysplasia of bone (FD), endocrine disorders and caf¿-au-lait skin hyperpigmentation. FD affects craniofacial bones including the jaws in 90% of cases. FD/MAS patients are also associated with dental disorders that include tooth rotation, tooth displacement, missing teeth, enamel hypoplasia, enamel hypomineralization, abnormally large pulp chamber, retained deciduous teeth, attrition and severe malocclusion Dental management of FD/MAS is medically compromised by endocrine disorders and handicapping bone pain. Furthermore, bisphosphonates often used to control FD and associated bone pain poses a risk for jaw bone necrosis. Earlier subjective reports indicate dental surgery could exacerbate jaw FD lesions but there is still limited qualitative data on dental outcomes and effectiveness of dental treatments i maxillo-mandibular FD/MAS patients. Our collaborative group has access to the largest well-described and well-characterized cohort of 140 FD/MAS patients enrolled in an ongoing NIDCR/NIH natural history study of MAS. This patient population represents an appropriate cohort for intramural-extramural collaborative research that will assess outcomes of dental treatment in FD/MAS. Our objective is to determine the outcomes of dental therapies on maxillo-mandibular FD lesions in MAS patients and also the impact of FD on the prognosis of dental therapies. Using a combination of retrospective and prospective longitudinal case-cohort analysis we will assess in Aim 1 the outcomes and effectiveness of endodontic and orthodontic therapies in FD/MAS patients with maxillo-mandibular FD. In Aim 2, we will determine whether or not dental surgery within FD is associated with delayed healing, bone necrosis and exacerbation of FD disease burden. New knowledge gained from this unique patient population with a rare disease will provide evidence-based data to support 'best clinical practice' guidelines for dental care that will improve quality of life of FD/MAS patients.

描述（申请人提供）：McCune-Albright 综合征（MAS）是一种由 GNAS1 基因突变引起的罕见多系统疾病，其特征是骨多发性纤维性发育不良（FD）、内分泌紊乱和咖啡因。 FD 皮肤色素沉着过度影响 90% 的 FD/MAS 患者的颅面骨，还与牙齿疾病有关，包括牙齿旋转、牙齿移位、牙齿缺失、牙釉质发育不全、牙釉质矿化不足、牙髓异常大。 FD/MAS 的牙科治疗在医学上因内分泌失调和骨痛而受到损害。通常用于控制 FD 和相关骨痛的双磷酸盐会带来颌骨坏死的风险，早期的主观报告表明牙科手术可能会加剧颌骨 FD 病变，但有关上下颌 FD/牙科治疗效果的定性数据仍然有限。 MAS 患者。我们的合作小组拥有 140 名 FD/MAS 患者的最大队列，该患者参与了正在进行的 NIDCR/NIH 自然史研究。代表了一个适合壁内-壁外合作研究的队列，该研究将评估 FD/MAS 牙科治疗的结果，我们的目标是确定牙科治疗对 MAS 患者上颌-下颌 FD 病变的结果以及 FD 对预后的影响。通过结合回顾性和前瞻性纵向病例队列分析，我们将在目标 1 中评估 FD/MAS 患者的牙髓和正畸治疗的结果和有效性。在目标 2 中，我们将确定 FD 内的牙科手术是否与愈合延迟、骨坏死和 FD 疾病负担加重有关，这将提供证据——基于数据支持“最佳临床实践”指南牙科护理将改善 FD/MAS 患者的生活质量。