Fibrous dysplasia is a disorder where normal bone and marrow is replaced with fibrous tissue, resulting in formation of bone that is weak and prone to expansion. As a result, most complications result from fracture, deformity, functional impairment, and pain.[2] Disease occurs along a broad clinical spectrum ranging from asymptomatic, incidental lesions, to severe disabling disease. Disease can affect one bone (monostotic), multiple (polyostotic), or all bones (panostotic)[3][4] and may occur in isolation or in combination with café au lait skin macules and hyperfunctioning endocrinopathies, termed McCune–Albright syndrome.[2] More rarely, fibrous dysplasia may be associated with intramuscular myxomas, termed Mazabraud's syndrome.[5] Fibrous dysplasia is very rare, and there is no known cure. Fibrous dysplasia is not a form of cancer.
Micrograph showing fibrous dysplasia with the characteristic thin, irregular (Chinese character-like) bony trabeculae and fibrotic marrow space. H&E stain.SpecialtyMedical genetics SymptomsBone pain, bone deformities, local swellingComplicationsBone fracturesUsual onsetAdolescence or early adulthood (monostotic), before age 10 (polyostotic)TypesMonostotic (75–80% of cases),[1]polyostotic, panostoticCausesMutations of GNAS locusFrequency1 in 5,000 to 10,000[1]https://en.wikipedia.org/wiki/Fibrous_dysplasia_of_bone
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