Pain in Fibrous Dysplasia
- Conditions
- Fibrous Dysplasia/McCune-Albright Syndrome
- Interventions
- Diagnostic Test: MRI-based NeuroimagingDiagnostic Test: Non-contrast MRIDiagnostic Test: 18F-FDG-PET/CTDiagnostic Test: 18F-NaF-PET/CT
- Registration Number
- NCT04125862
- Lead Sponsor
- Boston Children's Hospital
- Brief Summary
Pain remains a common and frequently debilitating symptom, particularly during adulthood of Fibrous Dysplasia/McCune-Albright Syndrome (FD/MAS). For many FD/MAS patients, the amount of pain perceived is not commensurate with the level of detectable musculoskeletal pathology. Using a combination of clinical and biological assessments, this investigation aims to understand what drives pain in FD/MAS.
- Detailed Description
In Fibrous Dysplasia/McCune-Albright Syndrome (FD/MAS), healthy bone tissue and marrow is replaced with pre-osteoblastic, fibrous tissue, yielding skeletal deformities and an increased propensity towards fracture, musculoskeletal weakness and bone pain. Despite the frequent use of pharmacological and non-pharmacological analgesic strategies, pain in FD remains common and frequently debilitating, particularly during adulthood. Moreover, for many patients there is a discordance between perceived pain levels and detectable musculoskeletal pathology. To elucidate the mechanism underlying pain in FD/MAS patients, investigators at the National Institute of Dental and Craniofacial Research (NIDCR), National Center for Complementary and Integrative Heath (NCCIH) and Boston Children's Hospital (BCH) aim to probe three inter-related domains that are projected to underlie pain experience(s) in FD/MAS patients. These include (i.) the presence of maladaptive central nervous system processes that amplify afferent pain or somatosensory signals, and also facilitate persistent pain; (ii.) aberrant interplay between neurological and musculoskeletal systems; (iii.) a mental health status shaped by the overall burden of living with FD and (iv.) the influence of childhood, FD-related complications on adulthood pain phenotypes. To investigate these four domains hypothesized to underlie FD pain as well as inform on the disconnect between pain and FD disease burden, the investigators will employ methods that complement routine clinical evaluation and diagnostic tests (i.e., 18F-NaF PET/CT or 18F-FDG PET/CT) such as neuroimaging, musculoskeletal MRI and such as neuroimaging, musculoskeletal MRI and assessment of inflammatory and pain mediator expression in blood samples.
Recruitment & Eligibility
- Status
- ENROLLING_BY_INVITATION
- Sex
- All
- Target Recruitment
- 40
Not provided
Not provided
Study & Design
- Study Type
- INTERVENTIONAL
- Study Design
- PARALLEL
- Arm && Interventions
Group Intervention Description Fibrous Dysplasia/McCune-Albright Syndrome Non-contrast MRI 20, Fibrous Dysplasia/McCune-Albright Syndrome Patients with or without pain Fibrous Dysplasia/McCune-Albright Syndrome MRI-based Neuroimaging 20, Fibrous Dysplasia/McCune-Albright Syndrome Patients with or without pain Fibrous Dysplasia/McCune-Albright Syndrome 18F-FDG-PET/CT 20, Fibrous Dysplasia/McCune-Albright Syndrome Patients with or without pain Fibrous Dysplasia/McCune-Albright Syndrome 18F-NaF-PET/CT 20, Fibrous Dysplasia/McCune-Albright Syndrome Patients with or without pain Healthy Controls MRI-based Neuroimaging 20, matched healthy controls
- Primary Outcome Measures
Name Time Method % signal difference in BOLD signal 50-60 Minutes • % signal difference within striatal and limbic network structures during evoked-heat pain fMRI between FD/MAS patients with pain, FD/MAS patients without pain and matched, healthy volunteers.
- Secondary Outcome Measures
Name Time Method Numerical clinical pain rating score 8 weeks • Numerical clinical pain rating score (NPRS, 0-10 scale) at weeks 0, 1, 4 and 8.
Trial Locations
- Locations (1)
Boston Children's Hospital
🇺🇸Boston, Massachusetts, United States