2009
DOI: 10.1186/1746-160x-5-22
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The nature of fibrous dysplasia

Abstract: Fibrous dysplasia has been regarded as a developmental skeletal disorder characterized by replacement of normal bone with benign cellular fibrous connective tissue. It has now become evident that fibrous dysplasia is a genetic disease caused by somatic activating mutation of the Gsα subunit of G protein-coupled receptor resulting in upregulation of cAMP. This leads to defects in differentiation of osteoblasts with subsequent production of abnormal bone in an abundant fibrous stroma. In addition there is an inc… Show more

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Cited by 91 publications
(111 citation statements)
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“…Polyostotic fibrous dysplasia has its onset mainly in children younger than 10 years of age, and the lesions grow with the child and stabilize after puberty. 6,15 The ratio of occurrence of polyostotic to monostotic fibrous dysplasia is 3:7.…”
Section: Epidemiologymentioning
confidence: 99%
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“…Polyostotic fibrous dysplasia has its onset mainly in children younger than 10 years of age, and the lesions grow with the child and stabilize after puberty. 6,15 The ratio of occurrence of polyostotic to monostotic fibrous dysplasia is 3:7.…”
Section: Epidemiologymentioning
confidence: 99%
“…6 However, McCune-Albright syndrome has a clear female predilection. 3 Any bone may be affected by fibrous dysplasia.…”
Section: Epidemiologymentioning
confidence: 99%
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“…Malignant transformation is usually rare and is usually precipitated by radiation therapy. [3][4][5] Ichthyoses are a group of disorder of cornification which may be inherited or acquired. The inherited form of the disease usually presents in infancy and persists throughout the life.…”
Section: Imentioning
confidence: 99%