Dental outcomes in Fibrous Dysplasia/McCune Albright Syndrome

Summary

Principal Investigator: Sunday O Akintoye
Abstract: 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[unreadable]-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.
Funding Period: 2013-09-25 - 2014-07-31
more information: NIH RePORT

Top Publications

  1. pmc Recurrent aphthous stomatitis
    Sunday O Akintoye
    Department of Oral Medicine, School of Dental Medicine, University of Pennsylvania, 240 South 40th Street, Philadelphia, PA 19104, USA Electronic address
    Dent Clin North Am 58:281-97. 2014

Research Grants

Detail Information

Publications1

  1. pmc Recurrent aphthous stomatitis
    Sunday O Akintoye
    Department of Oral Medicine, School of Dental Medicine, University of Pennsylvania, 240 South 40th Street, Philadelphia, PA 19104, USA Electronic address
    Dent Clin North Am 58:281-97. 2014
    ..The goals of therapy are to decrease pain and ulcer size, promote healing, and decrease the frequency of recurrence. ..

Research Grants30

  1. The Shelf Live Evaluation of Investigational Dosage Forms
    Jonathan White; Fiscal Year: 2013
    ..This contract is essential for continued assurance of the quality of drugs undergoing clinical investigation for different types of cancer by Cancer Therapeutics Evaluation Program. ..
  2. Musculoskeletal Heritable Influences on Malocclusion
    James J Sciote; Fiscal Year: 2013
    ....
  3. North American Mitochondrial Disease Consortium (NAMDC)
    JOHN L THOMPSON; Fiscal Year: 2013
    ....
  4. Hereditary Causes of Nephrolithaisis and Kidney Failure
    DAWN SCHMAUTZ MILLINER; Fiscal Year: 2013
    ..This work will address barriers of rare diseases research through vigorous multidisciplinary cooperation. ..
  5. M. D. Anderson Cancer Center SPORE in Multiple Myeloma
    ROBERT ZYGMUNT ORLOWSKI; Fiscal Year: 2013
    ..abstract_text> ..
  6. Growth Control in Multiple Myeloma
    Bart Barlogie; Fiscal Year: 2013
    ..This work will be accomplished with access to 5 shared resource cores. ..
  7. Lysosomal Disease Network
    Chester B Whitley; Fiscal Year: 2013
    ..Solutions to these problems will have direct impact on patients suffering from lysosomal diseases, and important implications for medical practice. ..
  8. Clinical Registry of Dental Outcomes in Head and Neck Cancer Patients
    Rajesh V Lalla; Fiscal Year: 2013
    ....
  9. Early Life Environmental Toxicant Exposure and Oral Health
    Manish Arora; Fiscal Year: 2013
    ..The proposed training and career development will enable Dr. Arora to become an independent investigator and position him to play a leadership role in the field of environmental dentistry. ..
  10. Patient-Reported Oral Health Outcomes Measurement Information System
    Honghu Liu; Fiscal Year: 2013
    ..1 ..
  11. Developmental Disabilities Dentistry Online
    Richard K Fleming; Fiscal Year: 2013
    ....
  12. Caregiver Illness Perception and Childs Early Childhood Caries Status
    Mary Beth Slusar; Fiscal Year: 2013
    ....
  13. Caloric Restricted Rodent Colony
    RICK MORIN; Fiscal Year: 2013
    ..The purpose of this project is to develop, maintain and distribute a standing colony ofaged, calorically restricted rodents ofdefined strains for use by investigators in studies of aging. ..
  14. Modeling Dentin by G Protein Coupled Receptor Signaling
    Orapin V Horst; Fiscal Year: 2013
    ..Horst's natural progression from current research experiences to the mentored clinical scientist development phase and then to independence. ..
  15. Stem Cell Based Therapy for Regenerative Endodontics
    George T J Huang; Fiscal Year: 2013
    ..If successful, a subsequent human trial may be carried out in the future and its success will promise a reduced need of traditional aggressive root canal therapies. ..
  16. Functional Tooth Mobility
    Zi Jun Liu; Fiscal Year: 2013
    ..The deliverables of the proposed study will be the first in vivo determinations of how chewing displaces teeth, and the potential translational payoffs will be to develop better clinical strategies to preserve compromised teeth. ..
  17. Signaling in Inflammation, Stress, and Tumorigenesis
    GEORGE ROBERT STARK; Fiscal Year: 2013
    ..abstract_text> ..
  18. INFLUENCES ON STABILITY FOLLOWING ORTHOGNATHIC SURGERY
    Ceib Phillips; Fiscal Year: 2013
    ....
  19. An Innovative Approach to Disseminate Dental Research
    WILLIAM ADAMS RUSH; Fiscal Year: 2013
    ..While conducting this project, we will also plan the distribution of the simulation tool to the broader dental community. ..
  20. Genetic Risk of Osteonecrosis of the Jaw (ONJ) in Patients with Metastatic Cancer
    Catherine H Van Poznak; Fiscal Year: 2013
    ..Patients, dentists and medical oncologists will benefit from an instrument that will provide a personalized ONJ risk assessment. ..
  21. Center to Address Disparities in Children's Oral Health
    Stuart A Gansky; Fiscal Year: 2013
    ..These endeavors, with other education and career development activities, will foster new investigators to increase oral-health-disparity research capacity. ..
  22. A Novel Approach for Biomaterials Assisted Regeneration of Pulp-Dentin Complex
    Satish Alapati; Fiscal Year: 2013
    ....
  23. Core Center for Musculoskeletal Disorders
    Louis J Soslowsky; Fiscal Year: 2013
    ..abstract_text> ..
  24. Salivary Biomarkers in the Diagnosis of BRONJ
    Tara L Aghaloo; Fiscal Year: 2013
    ..Aghaloo to design future clinical studies, whether for ONJ or other oral diseases. ..
  25. Proteomics and Genetics of Enamel and Dentin
    James P Simmer; Fiscal Year: 2013
    ..Future genetic analyses may link genetic changes in these genes to susceptibility for dental caries and provide insights into mechanisms of tooth formation. ..
  26. Intraoral Ultrasound Probe for Dental Hard Tissue Imaging
    Rahul Singh; Fiscal Year: 2013
    ..The focus of this Phase I SBIR effort is to design and integrate a customized ultrasound probe for imaging of tooth fractures, and to demonstrate technical feasibility through in vitro and ex vivo studies. ..
  27. Assessment of outcomes and cost-effectiveness in early orthodontic treatment
    James J Chen; Fiscal Year: 2013
    ..Towards the end of this K23 award Dr. Chen will prepare and apply for a R34 award to develop the intervention trial followed by an application for an U01 clinical trial award. ..