Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

Familial expansile osteolysis.

R G Wallace1, R J Barr, P H Osterberg

  • 1Musgrave Park Hospital Belfast, Northern Ireland.

Clinical Orthopaedics and Related Research
|November 1, 1989
PubMed
Summary
This summary is machine-generated.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Serum OPG/TRAIL ratio predicts the presence of cardiovascular disease in people with type 2 diabetes mellitus.

Diabetes research and clinical practice·2022
Same author

Significant morphological change in osteoarthritic hips identified over 6-12 months using statistical shape modelling.

Osteoarthritis and cartilage·2018
Same author

DXA-derived hip shape is related to osteoarthritis: findings from in the MrOS cohort.

Osteoarthritis and cartilage·2017
Same author

Blowback: new formal perspectives on agriculturally driven pathogen evolution and spread.

Epidemiology and infection·2015
Same author

Can we improve the prediction of hip fracture by assessing bone structure using shape and appearance modelling?

Bone·2012
Same author

Population screening for osteoporosis risk: a randomised control trial of medication use and fracture risk.

Osteoporosis international : a journal established as result of cooperation between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA·2009

Familial expansile osteolysis (FEO) is a rare autosomal dominant bone dysplasia causing progressive bone destruction, deformities, and fractures. Early-onset deafness and dental loss are also characteristic features.

Area of Science:

  • Genetics
  • Orthopedics
  • Pathology

Background:

  • Familial expansile osteolysis (FEO) is a rare, inherited bone disorder.
  • It follows an autosomal dominant inheritance pattern over multiple generations.
  • Characterized by progressive osteolysis and skeletal deformities.

Purpose of the Study:

  • To describe the clinical and natural history of a unique bone dysplasia, Familial Expansile Osteolysis (FEO).
  • To identify the inheritance pattern and associated symptoms of FEO.
  • To evaluate the therapeutic response to dichloro-methylene-diphosphonate (dichloro-MDP).

Main Methods:

  • Clinical observation and pedigree analysis over five generations.
  • Radiographic and biochemical assessments of affected individuals.

Related Experiment Videos

  • Evaluation of therapeutic trial with dichloro-MDP.

    • Main Results:

    • FEO identified as a distinct autosomal dominant bone dysplasia in a Northern Ireland family.
    • Progressive osteoclastic resorption leads to deformities, pathologic fractures, early-onset deafness, and tooth loss.
    • Initial biochemical improvement with dichloro-MDP was not sustained.

    Conclusions:

    • Familial expansile osteolysis is a unique, severe bone dysplasia with distinct clinical features.
    • The condition involves significant skeletal, auditory, and dental abnormalities.
    • Current therapeutic interventions show limited long-term efficacy.