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familial expansile osteolysis
Epidemiology: rare
Pathology:
- bone disorder characterized by focal areas of increased bone remodeling
Genetics:
- autosomal dominant
- associated with defects in TNFRSF11A are the cause of
Clinical manifestations:
- often associated with early onset deafness & loss of dentition
Radiology:
- osteolytic lesions develop usually in the long bones during early adulthood
General
inherited disorder of osteolysis; vanishing bone syndrome; osteolysis syndrome
Database Correlations
OMIM 174810
References
OMIM :accession 174810