Fibrous dysplasia of bone

Fibrous dysplasia of bone

DiseaseDisorder infobox
Name = Fibrous dysplasia
ICD10 = ICD10|K|10|8|k|00, ICD10|M|85|0|m|80, ICD10|Q|78|1|q|65
ICD9 = ICD9|526.89, ICD9|733.29, ICD9|756.54
ICDO =


Caption =
OMIM =
MedlinePlus = 001234
eMedicineSubj = radio
eMedicineTopic = 284
DiseasesDB =
MeshID = D005357

Fibrous dysplasia is a disease that causes bone thinning [DorlandsDict|three/000033238|fibrous dysplasia of bone] and growths or lesions in one or more bones of the human body.

Presentation

These lesions are tumor-like growths that consist of replacement of the medullary bone with fibrous tissue, causing the expansion and weakening of the areas of bone involved. Especially when involving the skull or facial bones, the lesions can cause externally visible deformities. The skull is often, but not necessarily, affected, and any other bone(s) can be involved.
* Many patients have lesions localized in only one bone (monostotic fibrous dysplasia), affecting 70-80%.
* Others have them in many bones (polyostotic fibrous dysplasia).

In 3% of cases, people suffering from fibrous dysplasia also have endocrine diseases and skin pigmentation; the three together constitute McCune-Albright syndrome. These endocrine diseases include precocious (early) puberty, often occurring as early as 6 years old.

Causes

Fibrous dysplasia is very rare; not much is known about it, and there is no known cure. However, it is known that it is caused by a genetic mutation that occurs sometime during fetal development, and is not hereditary.Fact|date=May 2008There are two types of fibrous dysplasia: 1. Monostotic (Involving a single bone), and 2. Polystotic (Involving many bones). The most severe form of polystotic fibrous dysplasia is known as Albright Syndrome.

ee also

* Cherubism
* Dysplasia

References

External links

* [http://fdsol.org/public/faq.html Fibrous Dysplasia Support Online FAQ]
* [http://www.paget.org The Paget Foundation]
*


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Look at other dictionaries:

  • fibrous dysplasia of bone — noun a disturbance in which bone that is undergoing lysis is replaced by an abnormal proliferation of fibrous tissue resulting in bone lesions or skin lesions • Hypernyms: ↑dysplasia • Hyponyms: ↑Albright s disease, ↑polyostotic fibrous dysplasia …   Useful english dictionary

  • fibrous dysplasia of bone — a disease of bone marked by thinning of the cortex and replacement of bone marrow by gritty fibrous tissue containing bony spicules, producing pain, disability, and gradually increasing deformity. Two types or stages are distinguished: monostotic …   Medical dictionary

  • fibrous dysplasia — a developmental abnormality in which changes occur in bony tissue. Trabecular bone is replaced by fibrous tissue, resulting in aching and a tendency to pathological fracture. In monostotic fibrous dysplasia one bone is affected; polyostotic… …   Medical dictionary

  • fibrous dysplasia — a developmental abnormality in which changes occur in bony tissue. Trabecular bone is replaced by fibrous tissue, resulting in aching and a tendency to pathological fracture. In monostotic fibrous dysplasia one bone is affected; polyostotic… …   The new mediacal dictionary

  • Fibrous dysplasia, polyostotic — A genetic disorder of bones, skin pigmentation and hormonal problems with premature sexual development. Also called McCune Albright syndrome or the Albright syndrome. In the syndrome, there is bone disease with fractures and deformity of the legs …   Medical dictionary

  • fibrous dysplasia — ▪ pathology       rare congenital developmental disorder beginning in childhood and characterized by replacement of solid calcified bone with fibrous tissue, often only on one side of the body and primarily in the long bones and pelvis. One bone… …   Universalium

  • monostotic fibrous dysplasia — fibrous dysplasia of bone involving only one bone. Called also osteitis fibrosa localisata …   Medical dictionary

  • Polyostotic fibrous dysplasia — A genetic disorder of bones, skin pigmentation and hormonal problems with premature sexual development. Also called McCune Albright syndrome or the Albright syndrome. In the syndrome, there is bone disease with fractures and deformity of the legs …   Medical dictionary

  • monostotic fibrous dysplasia — noun fibrous dysplasia of bone confined to a single bone • Hypernyms: ↑fibrous dysplasia of bone …   Useful english dictionary

  • polyostotic fibrous dysplasia — noun fibrous dysplasia of bone affecting multiple bones • Syn: ↑Albright s disease • Hypernyms: ↑fibrous dysplasia of bone …   Useful english dictionary

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