MCID: FBR009
MIFTS: 50

Fibrous Dysplasia malady

Rare diseases, Bone diseases categories
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Summaries for Fibrous Dysplasia

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8Disease Ontology, 43NIH Rare Diseases, 65Wikipedia, 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 gigantism. An important gene associated with Fibrous Dysplasia is GNAS (GNAS complex locus), and among its related pathways are Interleukin-11 Signaling Pathway and Osteoblast Signaling. The compounds pyridinoline and pphp have been mentioned in the context of this disorder. Affiliated tissues include bone, bone marrow and lung, and related mouse phenotypes are adipose tissue 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:65 Fibrous dysplasia is an abnormal bone growth where normal bone is replaced with fibrous bone tissue.... more...

Aliases & Classifications for Fibrous Dysplasia

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8Disease Ontology, 43NIH Rare Diseases, 10DISEASES, 45Novoseek, 62UMLS
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Classifications:

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


Aliases & Descriptions:

fibrous dysplasia 8 43 10 45 62
fibrous dysplasia of bone 43


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Disease Ontology8 DOID:0080031

Related Diseases for Fibrous Dysplasia

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17GeneCards, 18GeneDecks
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Diseases related to Fibrous Dysplasia via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 135)
idRelated DiseaseScoreTop Affiliating Genes
1mccune albright syndrome31.5FGF23, GNAS
2gigantism30.7GH1, GNAS
3osteofibrous dysplasia30.6BGLAP, GNAS
4acromegaly30.6BGLAP, GNAS, APC, GH1
5hyperparathyroidism30.5FGF23, BGLAP
6primary hyperparathyroidism30.3BGLAP, IBSP, FGF23
7hyperprolactinemia30.3GNAS, GH1
8osteogenesis imperfecta30.3IBSP, BGLAP
9precocious puberty30.3GH1, APC, GNAS
10osteomalacia30.2FGF23, GNAS, BGLAP
11prolactinoma30.1GH1, GNAS
12goiter30.1BGLAP, GNAS
13bone carcinoma30.0BGLAP, IBSP
14hypophosphatemia30.0FGF23, BGLAP
15giant cell tumor30.0BGLAP, FGF23
16rickets30.0FGF23, BGLAP
17hypercalcemia30.0BGLAP, FGF23
18hyperthyroidism29.9BGLAP, GNAS, GH1, FGF23
19prostate cancer29.9BGLAP, IBSP, GNAS, APC
20osteoporosis29.9FGF23, GH1, IBSP, BGLAP
21breast cancer29.8IBSP, APC, MSX2, GH1
22monostotic fibrous dysplasia11.0
23fibroma10.6
24ossifying fibroma10.6
25aneurysmal bone cysts10.6
26aneurysm10.6
27cherubism10.5
28cervicitis10.5
29osteosarcoma10.5
30panostotic fibrous dysplasia10.4
31sinusitis10.4
32sarcoma10.3
33meningioma10.3
34mazabraud syndrome10.2
35giant cell reparative granuloma10.1
36cleft lip10.1
37thoracic outlet syndrome10.1
38cholesteatoma10.1
39chondrosarcoma10.1
40hepatitis10.1
41hodgkin's lymphoma10.1
42osteomyelitis10.1
43blindness10.1
44acrodysostosis10.1GNAS
45pseudohypoparathyroidism type 1b10.1GNAS, BGLAP
46osteitis fibrosa10.1BGLAP, GNAS
47progressive osseous heteroplasia10.1BGLAP, GNAS
48idiopathic juvenile osteoporosis10.1GH1, BGLAP
49gastrinoma10.1APC, GH1
50thyroid adenoma10.1GNAS, APC

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



Diseases related to fibrous dysplasia

Symptoms for Fibrous Dysplasia

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Drugs & Therapeutics for Fibrous Dysplasia

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42NIH Clinical Center, 6ClinicalTrials, 62UMLS, 41NDF-RT
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Drug clinical trials:

Search ClinicalTrials for Fibrous Dysplasia

Search NIH Clinical Center for Fibrous Dysplasia

Genetic Tests for Fibrous Dysplasia

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Anatomical Context for Fibrous Dysplasia

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33MalaCards
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MalaCards organs/tissues related to Fibrous Dysplasia:

33
Bone, Bone marrow, Lung, Breast, Prostate, Testes, Myeloid

Animal Models for Fibrous Dysplasia or affiliated genes

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37MGI
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MGI Mouse Phenotypes related to Fibrous Dysplasia:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053759.2GH1, APC, GNAS
2MP:00053778.5MSX2, APC, GNAS
3MP:00053798.4GH1, MSX2, APC, GNAS
4MP:00107718.1GNAS, APC, MSX2, FGF23
5MP:00053717.8FGF23, MSX2, APC, GNAS, IBSP
6MP:00053907.7IBSP, GNAS, APC, MSX2, FGF23
7MP:00053787.4IBSP, GNAS, APC, MSX2, GH1, FGF23

Publications for Fibrous Dysplasia

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52PubMed
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Articles related to Fibrous Dysplasia:

(show top 50)    (show all 641)
idTitleAuthorsYear
1
Fibrous dysplasia of the craniofacial bones. (23918916)
2013
2
Low-grade central osteosarcoma of distal femur, resembling fibrous dysplasia. (24147271)
2013
3
Fibrous dysplasia and central giant cell granuloma: a report of hybrid lesion with its review and hypotheticated pathogenesis. (23814754)
2013
4
Isolated fibrous dysplasia of the inferior turbinate. (22698592)
2013
5
Real-time image-guided recontouring in the management of craniofacial fibrous dysplasia. (24113350)
2013
6
Clinical guidelines for the management of craniofacial fibrous dysplasia. (22640797)
2012
7
Peritrabecular clefting in fibrous dysplasia of the jaws: an important histopathologic feature for differentiating fibrous dysplasia from central ossifying fibroma. (22986246)
2012
8
CT and MRI of fibrous dysplasia of the spine. (22167510)
2012
9
An extensive hemimelic polyostotic fibrous dysplasia: a case report. (21331571)
2012
10
Resection of the entire first rib for fibrous dysplasia using a combined posterior-transmanubrial approach. (22614525)
2012
11
Long-term efficacy of oral alendronate therapy in an elderly patient with polyostotic fibrous dysplasia: A case report. (22848294)
2011
12
Dedifferentiated adamantinoma associated with fibrous dysplasia. (22000283)
2011
13
Fibrous dysplasia--a 13-year retrospective radiographic analysis in a south Indian population. (21697153)
2011
14
Fibrous dysplasia and aneurysmal bone cyst of the skull base presenting with blindness: a report of a rare locally aggressive example. (21396116)
2011
15
Monofocal maxillary fibrous dysplasia with orbital, nasal and oral obstruction. (19540186)
2010
16
Late diagnosed polyostotic fibrous dysplasia. Bone scan, radiography and magnetic resonance imaging findings. (20411176)
2010
17
Periostin, a novel marker of intramembranous ossification, is expressed in fibrous dysplasia and in c-Fos-overexpressing bone lesions. (18799196)
2009
18
Secondary aneurysmal bone cyst in fibrous dysplasia of the proximal femur. (19472953)
2009
19
Pain in fibrous dysplasia of bone: age-related changes and the anatomical distribution of skeletal lesions. (17622477)
2008
20
Oral rehabilitation with endosseous implants in a patient with fibrous dysplasia (McCune-Albright syndrome): a case report. (19022142)
2008
21
Fine-needle aspiration of fibrous dysplasia of bone: a worthwhile endeavor or not? (18418882)
2008
22
Attention deficiency hyperactivity disorder and sphenoid bone fibrous dysplasia association in a boy: SPECT/SPECT fusion imaging. (18075425)
2007
23
Concurrent Ewing sarcoma family of tumors and fibrous dysplasia: possible diagnostic pitfall. (17230061)
2007
24
Fibrous dysplasia of the clivus, a case report. (21503248)
2007
25
Craniofacial fibrous dysplasia and hypophysial microadenoma with Hodgkin's lymphoma: unusual involution of an extensive case. (16860225)
2006
26
Surgery for fibrous dysplasia. (17228997)
2006
27
Activating Gs a mutation rarely occurs in musculoskeletal tumors other than fibrous dysplasia. (16619580)
2006
28
Misleading clavicular osteolytic lesion revealing fibrous dysplasia. (16626990)
2006
29
Imaging of Paget's disease and fibrous dysplasia of bone. (17229004)
2006
30
Isolated fibrous dysplasia of the sphenoid sinus. (16405278)
2005
31
Oral pathology quiz # 43. Case number 1. Fibrous dysplasia. (15793915)
2004
32
Aneurysmal bone cyst arising from fibrous dysplasia of the frontal bone (2004:2b). (15162799)
2004
33
Chondroblastoma of the acromion mimicking fibrous dysplasia. (15669467)
2004
34
Fibrous dysplasia with degenerative atypia: a benign lesion potentially mistaken for sarcoma. (15214819)
2004
35
Polyostotic fibrous dysplasia with auditory impairment and delayed menarche in a Nigerian. (15298467)
2003
36
Natural history and treatment of fibrous dysplasia of bone: a multicenter clinicopathologic study promoted by the European Pediatric Orthopaedic Society. (12703030)
2003
37
Oral bisphosphonates in polyostotic fibrous dysplasia. (14530553)
2003
38
Magnetic resonance imaging and [11C]methyl-L-methionine positron emission tomography of fibrous dysplasia--two case reports. (12206488)
2002
39
Surgical treatment of fibrous dysplasia of the cranio-maxillo-facial area. Review of the literature and personal experience form 1984 to 1999. (12434124)
2002
40
Cellular and molecular biology of fibrous dysplasia]. (11910936)
2001
41
Polymerase chain reaction-based technique for the selective enrichment and analysis of mosaic arg201 mutations in G alpha s from patients with fibrous dysplasia of bone. (9267696)
1997
42
Fibrous dysplasia of bone in the McCune-Albright syndrome: abnormalities in bone formation. (9403710)
1997
43
Residents' corner. Answer to case of the month #12. McCune-Albright syndrome (polyostotic fibrous dysplasia). (1562893)
1992
44
Fibrous dysplasia of the mandible associated with large solitary bone cyst. (2294217)
1990
45
Empty sella syndrome occurring with polyostotic fibrous dysplasia of the midface: report of a case. (3507319)
1987
46
Ehlers-Danlos syndrome with monostotic fibrous dysplasia. (529175)
1979
47
Pseudopseudohypoparathyroidism with fibrous dysplasia. (1119829)
1975
48
Familial polyostotic fibrous dysplasia. (1060033)
1975
49
Effects of calcitonin in metastatic bone carcinoma, osteoporosis, polyostotic fibrous dysplasia and hypercalcemia. (4254451)
1971
50
MONOSTOTIC FIBROUS DYSPLASIA AFFECTING THE SKULL AND FACIAL BONES. (14290332)
1965

Variations for Fibrous Dysplasia

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Expression for genes affiliated with Fibrous Dysplasia

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2BioGPS, 15Gene Expression Omnibus DataSets
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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

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50PathCards, 38NCBI BioSystems Database, 60Thomson Reuters, 53QIAGEN, 30KEGG, 55Reactome
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Compounds for genes affiliated with Fibrous Dysplasia

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45Novoseek, 51PharmGKB, 11DrugBank, 29IUPHAR, 24HMDB, 61Tocris Bioscience
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Compounds related to Fibrous Dysplasia according to GeneCards/GeneDecks:

(show top 50)    (show all 60)
idCompoundScoreTop Affiliating Genes
1pyridinoline4510.5GH1
2pphp4510.1GNAS, APC
3anastrozole45 51 1111.9BGLAP, GH1
4salbutamol45 51 29 24 1113.9GH1, APC
5alendronate45 51 1111.8GH1, BGLAP
6ibandronate45 1110.8IBSP, BGLAP
7alizarin459.8BGLAP, IBSP
8pertussis459.8GH1, APC
9chitosan459.8IBSP, BGLAP
10sevelamer459.8FGF23, BGLAP
11titanium459.8IBSP, BGLAP
12fluoride459.7BGLAP, GH1
13zoledronic acid459.7IBSP, BGLAP
14raloxifene45 51 29 1112.7GH1, BGLAP
15isoproterenol45 1110.6GNAS, APC, GH1
16acth459.6GNAS, APC, GH1
17clonidine45 51 29 1112.6GH1, BGLAP
18guanine45 24 1111.6GNAS, APC, GH1
1925-hydroxyvitamin d459.6FGF23, BGLAP
20phosphorus459.6FGF23, BGLAP
21norepinephrine45 24 1111.5GNAS, APC, GH1
22gnrh459.5GH1, GNAS, BGLAP
23octreotide45 61 29 1112.5FGF23, GH1, GNAS
24deoxypyridinoline459.5GH1, IBSP, BGLAP
25procollagen459.4GH1, IBSP, BGLAP
26hydroxyproline45 24 1111.4BGLAP, IBSP
27dopamine45 29 24 1112.3GNAS, APC, GH1
28calcitriol45 61 24 1112.3FGF23, GH1, BGLAP
29estradiol45 24 1111.3BGLAP, IBSP, GH1
30hydroxyapatite459.2BGLAP, IBSP, FGF23
31phosphoserine45 2410.2APC, IBSP
32leucine459.2GH1, GNAS, IBSP
33forskolin45 51 1111.1IBSP, GNAS, APC, GH1
34thymidine45 2410.1GH1, GNAS, IBSP
35glutamine459.0GNAS, APC, GH1, FGF23
36pge2459.0APC, GNAS, IBSP, BGLAP
37paraffin459.0BGLAP, IBSP, APC, GH1
38cysteine459.0GH1, GNAS, IBSP, BGLAP
39lipid459.0IBSP, GNAS, APC, GH1
40arginine458.9FGF23, GH1, GNAS, BGLAP
41estrogen458.9BGLAP, IBSP, APC, GH1
421,25 dihydroxy vitamin d3458.9BGLAP, IBSP, GH1, FGF23
43creatinine458.8BGLAP, IBSP, GH1, FGF23
44steroid458.7GH1, MSX2, GNAS, IBSP
45tyrosine458.5FGF23, GH1, APC, GNAS, IBSP
46vitamin d458.4FGF23, GH1, APC, IBSP, BGLAP
47heparin45 29 24 1111.4BGLAP, IBSP, APC, GH1, FGF23
48oligonucleotide458.3GH1, MSX2, APC, GNAS, IBSP
49serine458.0BGLAP, IBSP, GNAS, APC, GH1, FGF23
50calcium45 51 24 1110.6BGLAP, IBSP, GNAS, APC, MSX2, FGF23

GO Terms for genes affiliated with Fibrous Dysplasia

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16Gene Ontology
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Cellular components related to Fibrous Dysplasia according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular regionGO:0055768.0FGF23, GH1, GNAS, IBSP, BGLAP

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

(show all 9)
idNameGO IDScoreTop Affiliating Genes
1regulation of osteoclast differentiationGO:0456709.7APC, BGLAP
2bone developmentGO:0603489.6BGLAP, GNAS
3embryonic hindlimb morphogenesisGO:0351169.4MSX2, GNAS
4osteoblast developmentGO:0020769.3BGLAP, MSX2
5anterior/posterior pattern specificationGO:0099529.2MSX2, APC
6osteoblast differentiationGO:0016499.0BGLAP, MSX2
7positive regulation of osteoblast differentiationGO:0456699.0MSX2, GNAS
8cell adhesionGO:0071559.0APC, IBSP, BGLAP
9cellular response to growth factor stimulusGO:0713638.8MSX2, IBSP, BGLAP

Products for genes affiliated with Fibrous Dysplasia

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Sources for Fibrous Dysplasia

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4CDC
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
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet