MCID: FBR009
MIFTS: 51

Fibrous Dysplasia malady

Bone category

Summaries for Fibrous Dysplasia

Sources:
8Disease Ontology, 43NIH Rare Diseases, 64Wikipedia, 33MalaCards
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NIH Rare Diseases:43 Fibrous dysplasia is a progressive bone disorder that is characterized by the replacement of normal bone with weaker tissues, such as fibrous tissue and woven bone.  it may involve one bone (monostotic) or multiple bones (polyostotic).  though many individuals with this condition do not have any symptoms, others may have bone pain, abnormally shaped bones, or an increased risk of breaking bones.  this condition can occur alone or as part of a genetic disorder, such as mccune-albright syndrome. last updated: 8/25/2011

MalaCards: Fibrous Dysplasia, also known as fibrous dysplasia of bone, is related to mccune albright syndrome and acromegaly. An important gene associated with Fibrous Dysplasia is GNAS (GNAS complex locus), and among its related pathways are Osteoblast Signaling and Endochondral Ossification. The compounds forskolin and ghrp have been mentioned in the context of this disorder. Affiliated tissues include lung, bone marrow and breast, and related mouse phenotypes are limbs/digits/tail and hearing/vestibular/ear.

Disease Ontology:8 A bone remodeling disease that results in the destruction of normal bone and replacing it with fibrous bone tissue.

Wikipedia:64 Fibrous dysplasia is an abnormal bone growth where normal bone is replaced with fibrous bone tissue.... more...

Aliases & Classifications for Fibrous Dysplasia

Sources:
43NIH Rare Diseases, 8Disease Ontology, 10DISEASES, 45Novoseek, 61UMLS
See all sources

Classifications:

Malacards categories (disease lists): (See all malacards categories)
Anatomical: Bone


Aliases & Descriptions:

fibrous dysplasia 8 43 10 45 61
fibrous dysplasia of bone 43


External Ids:

Disease Ontology8 DOID:0080031

Related Diseases for Fibrous Dysplasia

Sources:
17GeneCards, 18GeneDecks
See all sources

Diseases related to Fibrous Dysplasia via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 194)
idRelated DiseaseScoreTop Affiliating Genes
1mccune albright syndrome31.3GNAS, APC, GH1, PRL
2acromegaly30.6BGLAP, GNAS, APC, GHR, GHRH, GH1
3gigantism30.5PRL, GH1, GNAS
4osteofibrous dysplasia30.5BGLAP, GNAS, SPARC
5primary hyperparathyroidism30.3BGLAP, IBSP, FGF23
6hyperprolactinemia30.2GNAS, GHRH, GH1, PRL
7osteogenesis imperfecta30.2ALPPL2, SPARC, IBSP, BGLAP
8osteomalacia30.2ALPPL2, FGF23, GNAS, BGLAP
9precocious puberty30.2GNAS, APC, GHR, GH1, PRL
10empty sella syndrome29.9GH1, PRL
11hypophosphatemia29.9ALPPL2, FGF23, BGLAP
12rickets29.9ALPPL2, FGF23, BGLAP
13prolactinoma29.9ADCY10, PRL, GH1, GNAS
14arthritis29.9BGLAP, FOS, ALPPL2
15bone carcinoma29.9BGLAP, IBSP, RUNX2, SPARC
16hypercalcemia29.9BGLAP, FGF23, ALPPL2, ADCY10
17osteoporosis29.9BGLAP, IBSP, RUNX2, GHR, GH1, FGF23
18monostotic fibrous dysplasia11.0
19mccune–albright syndrome10.9
20ossifying fibroma10.6
21cherubism10.5
22mazabraud syndrome10.4
23panostotic fibrous dysplasia10.4
24fibrous meningioma10.3
25n syndrome10.2
26bone osteosarcoma10.2
27renal dysplasia10.2
28giant cell reparative granuloma10.1
29cleft palate10.1
30thoracic outlet syndrome10.1
31char syndrome10.1
32blindness10.1
33solitary bone cyst10.1
34multiple endocrine neoplasia10.0GNAS
35arthropathy10.0FGF23
36pancreatitis10.0ALPPL2
37amenorrhea10.0PRL
38carney complex10.0GNAS
39parkinson's disease10.0FOS
40paget's disease of bone10.0ALPPL2
41pituitary hypoplasia10.0GHRH
42fibrodysplasia ossificans progressiva10.0FOS
43pertussis10.0GNA11
44sarcoma10.0ADCY10
45meningioma10.0SPARC
46hepatitis10.0GHR
47prader-willi syndrome10.0GH1
48lipodystrophy10.0GHR
49thyroid adenoma10.0APC, GNAS
50goiter10.0BGLAP, GNAS

Graphical network of the top 20 diseases related to Fibrous Dysplasia:



Diseases related to fibrous dysplasia

Clinical Features for Fibrous Dysplasia

Drugs & Therapeutics for Fibrous Dysplasia

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 61UMLS, 41NDF-RT
See all sources

Approved drugs:

Search CenterWatch for Fibrous Dysplasia

Drug clinical trials:

Search ClinicalTrials for Fibrous Dysplasia

Search NIH Clinical Center for Fibrous Dysplasia

Search CenterWatch for Fibrous Dysplasia

Genetic Tests for Fibrous Dysplasia

Anatomical Context for Fibrous Dysplasia

Sources:
33MalaCards
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MalaCards organs/tissues related to Fibrous Dysplasia:

33
Lung, Bone marrow, Breast, Prostate, Myeloid

Animal Models for Fibrous Dysplasia or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

Publications for Fibrous Dysplasia

Sources:
51PubMed
See all sources

Articles related to Fibrous Dysplasia:

(show top 50)    (show all 650)
idTitleAuthorsYear
1
Fibrous dysplasia and central giant cell granuloma: a report of hybrid lesion with its review and hypotheticated pathogenesis. (23814754)
2013
2
Paediatric craniofacial fibrous dysplasia: the Hospital for Sick Children experience and treatment philosophy. (23829958)
2013
3
How can a Calcified fibroma mimicke a fibrous dysplasia? About one case report. (24343500)
2013
4
Fibrous dysplasia. (23858622)
2013
5
Raised CA19.9 and hepatic space occupying lesion after teriparatide therapy in a case of polyostotic fibrous dysplasia. (24083194)
2013
6
Frontonasal and fibrous dysplasia in a patient with unilateral cleft lip and palate. (23851889)
2013
7
CT and MRI of fibrous dysplasia of the spine. (22167510)
2012
8
An extensive hemimelic polyostotic fibrous dysplasia: a case report. (21331571)
2012
9
Denosumab treatment for fibrous dysplasia. (22431375)
2012
10
A dilemma of fibrous dysplasia versus chronic osteomyelitis of the clavicle. (22669928)
2012
11
GNAS1 mutations are hallmark expressions of fibrous dysplasia. (22793952)
2012
12
Polyostotic fibrous dysplasia of the thoracic spine: case report and review of the literature. (21358488)
2011
13
Computed tomographic features of fibrous dysplasia of maxillofacial region. (21977470)
2011
14
Craniofacial fibrous dysplasia. (20118604)
2010
15
Posterior semicircular canal dehiscence arising from temporal bone fibrous dysplasia. (19816226)
2010
16
Transfer, analysis, and reversion of the fibrous dysplasia cellular phenotype in human skeletal progenitors. (19874199)
2010
17
Malignant transformation of monostotic fibrous dysplasia in the mandible. (20489463)
2010
18
Polyostotic rib fibrous dysplasia resected by video-assisted thoracoscopic surgery with preservation of the overlying periosteum. (20416895)
2010
19
Isolated orbital fibrous dysplasia associated with ipsilateral keratoconus. (20497083)
2010
20
Periostin, a novel marker of intramembranous ossification, is expressed in fibrous dysplasia and in c-Fos-overexpressing bone lesions. (18799196)
2009
21
Monostotic fibrous dysplasia of a lumbar vertebral body with secondary aneurysmal bone cyst formation: a case report. (19830140)
2009
22
Secondary osteosarcoma arising in fibrous dysplasia, case report. (18560851)
2009
23
Craniofacial fibrous dysplasia: clinical characteristics and long-term outcomes. (19182771)
2009
24
Craniomaxillofacial fibrous dysplasia: a 10-year database 1996-2006. (19282072)
2009
25
Oral rehabilitation with endosseous implants in a patient with fibrous dysplasia (McCune-Albright syndrome): a case report. (19022142)
2008
26
Fine-needle aspiration of fibrous dysplasia of bone: a worthwhile endeavor or not? (18418882)
2008
27
Fibrous dysplasia-like tumor of the lacrimal sac. (18806675)
2008
28
Clonal status of fibrous dysplasia. (18446630)
2008
29
A giant cranial aneurysmal bone cyst associated with fibrous dysplasia. (18500019)
2008
30
Attention deficiency hyperactivity disorder and sphenoid bone fibrous dysplasia association in a boy: SPECT/SPECT fusion imaging. (18075425)
2007
31
Imaging diagnosis of monostotic fibrous dysplasia in thoracic and lumbar spine vertebrae. (18231743)
2007
32
Polyostotic fibrous dysplasia of the cervical spine: case report and review of the literature. (17434805)
2007
33
Cystic fibrous dysplasia in the long bone. (17990415)
2007
34
Maternal and infant outcome after pamidronate treatment of polyostotic fibrous dysplasia and osteogenesis imperfecta before conception: a report of four cases. (16551739)
2006
35
Pamidronate treatment of polyostotic fibrous dysplasia: failure to prevent expansion of dysplastic lesions during childhood. (16509531)
2006
36
Fibrous dysplasia. Pathophysiology, evaluation, and treatment. (16085630)
2005
37
Fibrous dysplasia. (15871496)
2005
38
Fibrous dysplasia with degenerative atypia: a benign lesion potentially mistaken for sarcoma. (15214819)
2004
39
Use of bone SPECT in the evaluation of fibrous dysplasia of the skull. (15311123)
2004
40
Treatment of monostotic fibrous dysplasia with pamidronate. (14729043)
2004
41
Osteoclastogenesis in fibrous dysplasia of bone: in situ and in vitro analysis of IL-6 expression. (13678786)
2003
42
FGF-23 in fibrous dysplasia of bone and its relationship to renal phosphate wasting. (12952917)
2003
43
Cryosurgery in fibrous dysplasia: good result of a multimodality protocol in 16 patients. (12358128)
2002
44
Renal phosphate wasting in fibrous dysplasia of bone is part of a generalized renal tubular dysfunction similar to that seen in tumor-induced osteomalacia. (11341325)
2001
45
Fibrous dysplasia of the temporal bone and cerebral occlusive vasculopathy: report of a case]. (1308396)
1992
46
Radiological case of the month. Monostotic fibrous dysplasia of the temporal bone. (2816865)
1989
47
Monostotic fibrous dysplasia of the temporal bone. (3681132)
1987
48
The solitary hand lesion: bone scintigraphy of monostotic fibrous dysplasia. (6237822)
1984
49
Monostotic fibrous dysplasia in the femoral neck. A clinicopathologic study. (6388940)
1984
50
Fibrous dysplasia of single bones (monostotic fibrous dysplasia). (20276798)
1946

Genetic Variations for Fibrous Dysplasia

Expression for genes affiliated with Fibrous Dysplasia

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
Expression patterns in normal tissues for genes affiliated with Fibrous Dysplasia

Search GEO for disease gene expression data for Fibrous Dysplasia.

Pathways for genes affiliated with Fibrous Dysplasia

Sources:
38NCBI BioSystems Database, 54Reactome, 12EMD Millipore, 52QIAGEN, 30KEGG
See all sources

Pathways related to Fibrous Dysplasia according to GeneCards/GeneDecks:

(show all 27)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
110.2FGF23, IBSP
210.1GH1, GHR, RUNX2
3
Hide members
10.1GHR, GH1, PRL
410.1RUNX2, IBSP, BGLAP
510.0GHR, RUNX2, BGLAP
6
Transcription Role of VDR in regulation of genes involved in osteoporosis
Hide members
10.0FOS, RUNX2, BGLAP
710.0BGLAP, FOS, PRL
8
Hide members
10.0GHR, GH1, PRL, FGF23
9
Hide members
9.9AREG, GHRH, GH1, FGF23
10
Hide members
9.9GH1, GHRH, FOS, GNA11
119.9BGLAP, RUNX2, FOS, FGF23
129.9IBSP, GHR, GH1, PRL, FGF23
13
Hide members
9.8PRL, GH1, GHRH, GHR, GNAS
14
Hide members
9.7FGF23, GH1, GHRH, AREG, GNA11
15
Hide members
9.7GNA11, AREG, GHRH, GH1, FGF23
16
Hide members
9.7GNA11, AREG, GHRH, GH1, FGF23
17
Hide members
9.7FOS, AREG, GHRH, GH1, FGF23
18
Hide members
9.7FOS, AREG, GHR, GH1, PRL
19
Hide members
9.6APC, FOS, AREG, GHRH, GH1, FGF23
20
Development Hedgehog and PTH signaling pathways in bone and cartilage development
Hide members
9.6FOS, BGLAP, IBSP, RUNX2, GNA11, GNAS
21
Hide members
9.6GNA11, FOS, AREG, GHRH, GH1, FGF23
22
Hide members
9.6GNA11, FOS, AREG, GHRH, GH1, FGF23
23
Hide members
9.6GNA11, FOS, AREG, GHRH, GH1, FGF23
24
Hide members
9.6GNA11, FOS, AREG, GHRH, GH1, FGF23
25
Hide members
9.4GNA11, APC, FOS, AREG, GHRH, GH1
26
Hide members
9.3IBSP, GNA11, GNAS, APC, GHR, GHRH
27
Hide members
9.3GNA11, FOS, AREG, GHR, GHRH, GH1

Compounds for genes affiliated with Fibrous Dysplasia

Sources:
45Novoseek, 50PharmGKB, 11DrugBank, 29IUPHAR, 24HMDB, 60Tocris Bioscience
See all sources

Compounds related to Fibrous Dysplasia according to GeneCards/GeneDecks:

(show top 50)    (show all 100)
idCompoundScoreTop Affiliating Genes
1forskolin45 50 1112.4GHRH, AREG, IBSP
2ghrp4510.4GHR, PRL, GH1, GHRH
3hexarelin45 2911.3PRL, GH1, GHRH, GHR
4bromocriptine29 45 1112.3PRL, ADCY10, GHR, GHRH
5lanreotide45 2911.3GH1, PRL, GHRH, GHR
6alizarin4510.3BGLAP, IBSP, RUNX2, SPARC
7estradiol45 11 2412.3PRL, IBSP, GHRH, GHR
8salbutamol45 50 29 11 2414.2ADCY10, APC, GH1, GHRH
9cycloheximide4510.2GHR, GHRH, IBSP, RUNX2, ADCY10
10pyridinoline4510.2GH1, SPARC, BGLAP, IBSP
11titanium4510.2RUNX2, IBSP, BGLAP, SPARC
12dopamine45 29 11 2413.2FOS, GHR, GHRH, APC, GNAS
13naloxone45 50 29 1113.2ADCY10, PRL, GH1, GHRH, FOS
141,25 dihydroxy vitamin d34510.1FGF23, GH1, RUNX2, IBSP, BGLAP
15pertussis4510.1GH1, FOS, APC, ADCY10
16leuprolide acetate4510.1PRL, GH1, GHR
17pyridostigmine45 1111.1GHRH, GH1, PRL
18acth4510.1GHRH, GHR, APC, GNAS, PRL, GH1
19clonidine45 29 1112.1GH1, BGLAP, GHR, GHRH, ADCY10, PRL
20glucose4510.1UMOD, GHRH, RUNX2, APC, SPARC
21hydroxyapatite4510.0FGF23, IBSP, BGLAP, SPARC, RUNX2, UMOD
22octreotide45 60 29 1113.0PRL, GNAS, GHR, GHRH, ADCY10, FGF23
23opiate4510.0ADCY10, GHRH, GH1, PRL
24pegvisomant45 1111.0GHRH, GH1, GHR
25isoproterenol45 1111.0APC, AREG, GH1, ADCY10, GNAS
26thyroxine45 2411.0GH1, FOS, GHR, BGLAP, PRL, GHRH
27ribonucleic acid459.9RUNX2, GHR, GHRH, FOS, GNAS, GH1
28dihydrotestosterone45 29 11 2412.9AREG, BGLAP, GHR, GH1, PRL
29norepinephrine45 11 2411.9ADCY10, PRL, GHRH, GNAS, APC, AREG
30gnrh459.9PRL, GHR, GHRH, FOS, GNAS, GH1
31dexamethasone45 50 29 1112.9GHRH, BGLAP, IBSP, FOS, AREG, GH1
32tamoxifen45 50 29 1112.8GH1, GHRH, GHR, FOS, PRL, BGLAP
33calcitriol45 60 11 2412.8SPARC, GH1, GHR, FOS, FGF23, AREG
34paraffin459.8BGLAP, IBSP, APC, GHR, GH1, SPARC
35serine459.8RUNX2, GNAS, APC, BGLAP, FGF23, FOS
36sb 20358045 6010.7SPARC, BGLAP, RUNX2, FOS, AREG, GH1
37steroid459.7IBSP, RUNX2, GNAS, GH1, PRL, MSX2
38progesterone45 60 29 11 2413.7GHRH, SPARC, PRL, GH1, GHR, AREG
39thymidine45 2410.7GHR, GNAS, IBSP, SPARC, AREG, GH1
40heparin45 29 11 2412.7SPARC, AREG, APC, BGLAP, IBSP, UMOD
41vitamin d459.7SPARC, IBSP, BGLAP, FGF23, RUNX2, APC
42arginine459.7GH1, FGF23, GHRH, GHR, GNAS, BGLAP
43testosterone45 60 11 2412.6GHR, AREG, APC, BGLAP, GHRH, GH1
44oligonucleotide459.5GHR, IBSP, RUNX2, GNAS, APC, FOS
45vegf459.5RUNX2, IBSP, FOS, AREG, GH1, POSTN
46fatty acid459.5GHR, APC, RUNX2, GHRH, SPARC
47cyclic amp45 2410.5AREG, ADCY10, ALPPL2, GHRH, GHR, GNAS
48retinoic acid45 2410.4GHR, FOS, APC, RUNX2, GH1, SPARC
49tyrosine459.3FOS, NPHP1, APC, GHR, IBSP, RUNX2
50calcium45 50 11 2411.9GNAS, RUNX2, IBSP, BGLAP, FOS, UMOD

GO Terms for genes affiliated with Fibrous Dysplasia

Sources:
16Gene Ontology
See all sources

Cellular components related to Fibrous Dysplasia according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular spaceGO:0056159.5UMOD, GHR, GHRH, GH1, FGF23, SPARC
2extracellular regionGO:0055769.0SPARC, BGLAP, IBSP, GNAS, GHR, GHRH

Biological processes related to Fibrous Dysplasia according to GeneCards/GeneDecks:

(show all 20)
idNameGO IDScoreTop Affiliating Genes
1growth hormone receptor signaling pathwayGO:06039610.4GH1, GHR
2regulation of osteoclast differentiationGO:04567010.4APC, BGLAP
3positive regulation of osteoblast differentiationGO:04566910.4RUNX2, GNAS, MSX2
4JAK-STAT cascade involved in growth hormone signaling pathwayGO:06039710.4PRL, GH1, GHR
5positive regulation of multicellular organism growthGO:04001810.4GHR, GHRH, GH1
6response to foodGO:03209410.4GHRH, GHR
7osteoblast developmentGO:00207610.4BGLAP, RUNX2, MSX2
8response to estradiol stimulusGO:03235510.4PRL, GH1, GHR
9osteoblast differentiationGO:00164910.3BGLAP, RUNX2, MSX2
10chondrocyte developmentGO:00206310.3MSX2, RUNX2
11response to gravityGO:00962910.3SPARC, FOS, BGLAP
12ossificationGO:00150310.3IBSP, RUNX2, SPARC
13cAMP biosynthetic processGO:00617110.2ADCY10, GNAS
14response to glucocorticoid stimulusGO:05138410.2BGLAP, GHR, SPARC
15positive regulation of JAK-STAT cascadeGO:04642710.2PRL, GH1
16cellular response to growth factor stimulusGO:07136310.2SPARC, MSX2, IBSP, BGLAP
17female pregnancyGO:00756510.2GNAS, FOS, PRL
18response to ethanolGO:04547110.1BGLAP, PRL, SPARC
19positive regulation of insulin-like growth factor receptor signaling pathwayGO:0435689.9GH1, GHRH
20response to drugGO:0424939.8BGLAP, GNAS, FOS, PRL

Molecular functions related to Fibrous Dysplasia according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1adenylate cyclase activityGO:00401610.2ADCY10, GNAS
2prolactin receptor bindingGO:0051489.9PRL, GH1

Products for genes affiliated with Fibrous Dysplasia

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Sources for Fibrous Dysplasia

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet