What is Fibrous Dysplasia (Inflammatory fibrous hyperplasia; Idiopathic fibrous hyperplasia )

Fibrous dysplasia is a chronic disorder of the skeleton that causes the expansion of one or more bones due to the abnormal development of the fibrous, or connective tissue within the bone.

Statistics on Fibrous Dysplasia (Inflammatory fibrous hyperplasia; Idiopathic fibrous hyperplasia )

Fibrous dysplasia is a very rare disorder, and the total number of reported cases worldwide is unknown. It is usually diagnosed in children and young adults. If the disease involves more than one bone, it is more likely to produce problems before age 10.

The disease is found equally in males and females and does not appear to vary in incidence among races.

Risk Factors for Fibrous Dysplasia (Inflammatory fibrous hyperplasia; Idiopathic fibrous hyperplasia )

The disorder is believed to be congenital but it is classfied as a nonhereditary disorder of unknown cause.

Progression of Fibrous Dysplasia (Inflammatory fibrous hyperplasia; Idiopathic fibrous hyperplasia )

The natural history of the disease has two phases; an active phase until puberty and a later quiescent phase.

How is Fibrous Dysplasia (Inflammatory fibrous hyperplasia; Idiopathic fibrous hyperplasia ) Diagnosed?

 

  • A full physical examination
  • X-rays of bones

    Prognosis of Fibrous Dysplasia (Inflammatory fibrous hyperplasia; Idiopathic fibrous hyperplasia )

    The outlook depends on the severity of the condition and associated problems.

    How is Fibrous Dysplasia (Inflammatory fibrous hyperplasia; Idiopathic fibrous hyperplasia ) Treated?

    There is no specific treatment for these disorders. Observation and treatment of bone fractures or deformities should take place as indicated. There should also be observation and screening for the development of any endocrine disorders.

    Fibrous Dysplasia (Inflammatory fibrous hyperplasia; Idiopathic fibrous hyperplasia ) References

    [1] American National Institutes Health Oseoporosis and Related Bone Disorders
    [2] eMEDICINE
    [3] Lichenstein L, Jaffe HL: Fibrous dysplasia of bone: a condition affecting one, several or many bones, the graver cases of which may present abnormal pigmentation of skin, premature sexual development, hyperthyroidism or still other extraskeletal abnormalities. Arch Pathol 1942; 33: 777.
    [4] MEDLINE Plus

 

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