MCID: OST032
MIFTS: 47

Osteofibrous Dysplasia malady

Rare diseases, Bone diseases categories

Summaries for Osteofibrous Dysplasia

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66Wikipedia, 34MalaCards
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Wikipedia:66 Osteofibrous dysplasia (also known as ossifying fibroma) is a rare, benign non-neoplastic condition with... more...

MalaCards: Osteofibrous Dysplasia, also known as jaffe-campanacci syndrome, is related to fibroma and fibrous dysplasia. An important gene associated with Osteofibrous Dysplasia is BGLAP (bone gamma-carboxyglutamate (gla) protein), and among its related pathways are Platelet Aggregation Inhibitor Pathway, Pharmacodynamics and Estrogen signaling pathway. The compounds ti6al4v and chitosan have been mentioned in the context of this disorder. Affiliated tissues include bone, and related mouse phenotypes are liver/biliary system and normal.

Aliases & Classifications for Osteofibrous Dysplasia

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44NIH Rare Diseases, 63UMLS, 46Novoseek
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Classifications:

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


Aliases & Descriptions:

osteofibrous dysplasia 44 46 63
jaffe-campanacci syndrome 44 63
ossifying fibroma 44 63
intracortical fibrous dysplasia 44


Related Diseases for Osteofibrous Dysplasia

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18GeneCards, 19GeneDecks
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Diseases related to Osteofibrous Dysplasia via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 149)
idRelated DiseaseScoreTop Affiliating Genes
1fibroma30.9VIM, KRT1
2fibrous dysplasia30.7SPARC, BGLAP, GNAS, FOS
3hyperparathyroidism30.6NF1, BGLAP
4meningioma30.4KRT1, NF1, MUC1, VIM, SPARC
5osteoblastoma30.4SPARC
6fibromatosis30.4VIM
7chondrosarcoma30.4SPARC, VIM, MUC1, KRT19, CD36
8primary hyperparathyroidism30.3CD36, BGLAP
9giant cell tumor30.3BGLAP, VIM, MUC1
10osteosarcoma30.2SPP1, SPARC, BGLAP, FOS, CD36
11sarcoma30.1NF1, MUC1, VIM
12synovial sarcoma30.0VIM, MUC1, NF1, KRT1, KRT19
13sinusitis10.7
14fibromatosis multiple non ossifying10.6
15ossifying fibroma10.6
16pyogenic granuloma10.5
17neurofibromatosis10.3
18periodontitis10.3
19monostotic fibrous dysplasia10.3
20pseudoarthrosis10.3
21mccune albright syndrome10.3
22psammomatous meningioma10.2
23fanconi syndrome10.2
24exophthalmos10.2
25bilateral retinoblastoma10.2
26chronic apical periodontitis10.2
27orbital cellulitis10.2
28juxtacortical chondrosarcoma10.2
29adenoma10.2
30cellulitis10.2
31epulis10.2
32gingivitis10.2
33hyperthyroidism10.2
34parathyroid adenoma10.2
35periostitis10.2
36retinoblastoma10.2
37sphenoid sinusitis10.2
38florid cemento-osseous dysplasia10.2
39lipoblastoma10.2
40costello syndrome10.1
41long bone adamantinoma10.1
42aneurysmal bone cysts10.1
43aneurysm10.1
44idiopathic juvenile osteoporosis10.1CD36
45eccrine porocarcinoma10.1MUC1
46hidradenoma10.1MUC1
47glomus tumor10.1VIM
48cystadenocarcinoma10.1MUC1
49adenomatoid tumor10.1VIM
50pseudohypoparathyroidism type 1b10.1BGLAP, GNAS

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



Diseases related to osteofibrous dysplasia

Symptoms for Osteofibrous Dysplasia

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Drugs & Therapeutics for Osteofibrous Dysplasia

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Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials, 63UMLS, 42NDF-RT
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Approved drugs:

Search CenterWatch for Osteofibrous Dysplasia

Drug clinical trials:

Search ClinicalTrials for Osteofibrous Dysplasia

Search NIH Clinical Center for Osteofibrous Dysplasia

Search CenterWatch for Osteofibrous Dysplasia

Genetic Tests for Osteofibrous Dysplasia

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Anatomical Context for Osteofibrous Dysplasia

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34MalaCards
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MalaCards organs/tissues related to Osteofibrous Dysplasia:

34
Bone

Animal Models for Osteofibrous Dysplasia or affiliated genes

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38MGI
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MGI Mouse Phenotypes related to Osteofibrous Dysplasia:

38 (show all 17)
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053708.5SPP1, GNAS, MUC1, NF1, CD36
2MP:00028738.3KRT19, NF1, FOS, MUC1, GNAS
3MP:00053698.1SPP1, GNAS, VIM, NF1, CD36
4MP:00053798.0GNAS, MUC1, FOS, NF1, KRT19, CD36
5MP:00107717.9SPP1, GNAS, MUC1, FOS, NF1, KRT1
6MP:00020067.7SPP1, SPARC, MUC1, FOS, NF1, KRT19
7MP:00053857.7SPP1, GNAS, VIM, NF1, KRT1, KRT19
8MP:00053907.5SPP1, SPARC, GNAS, FOS, NF1, KRT19
9MP:00053917.5SPARC, VIM, MUC1, FOS, NF1, CD36
10MP:00053847.4SPP1, GNAS, VIM, FOS, NF1, KRT19
11MP:00036317.3SPP1, GNAS, VIM, FOS, NF1, KRT1
12MP:00053867.2SPP1, SPARC, GNAS, VIM, FOS, NF1
13MP:00053787.2SPP1, GNAS, VIM, FOS, NF1, KRT1
14MP:00107687.1SPP1, GNAS, VIM, FOS, NF1, KRT1
15MP:00053977.0SPP1, SPARC, GNAS, VIM, FOS, NF1
16MP:00053877.0SPP1, SPARC, GNAS, VIM, FOS, NF1
17MP:00053766.6CD36, SPP1, SPARC, GNAS, VIM, MUC1

Publications for Osteofibrous Dysplasia

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Sources:
53PubMed
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Articles related to Osteofibrous Dysplasia:

(show top 50)    (show all 74)
idTitleAuthorsYear
1
Imaging in osteofibrous dysplasia, osteofibrous dysplasia-like adamantinoma, and classic adamantinoma. (24199850)
2014
2
Congenital Osteofibrous Dysplasia Campanacci: Spontaneous Postbioptic Regression. (23774157)
2013
3
Analysis of stromal cells in osteofibrous dysplasia and adamantinoma of long bones. (21983933)
2012
4
Neonatal osteofibrous dysplasia associated with pathological tibia fracture: a case report and review of the literature. (21383639)
2012
5
Marginal excision and Ilizarov hemicallotasis for osteofibrous dysplasia of the tibia: a case report. (21164362)
2011
6
Osteofibrous dysplasia and adamantinoma. (20511441)
2010
7
Minimally invasive plate osteosynthesis for osteofibrous dysplasia of the tibia: a case report. (21187556)
2010
8
Congenital osteofibrous dysplasia of the tibia, associated with pseudoarthrosis of the ipsilateral fibula. (18946584)
2008
9
Treatment of osteofibrous dysplasia and associated lesions. (17594160)
2007
10
Frequent immunoexpression of TGF-beta1, FGF-2 and BMP-2 in fibroblast-like cells in osteofibrous dysplasia. (17273729)
2007
11
A classic adamantinoma arising from osteofibrous dysplasia-like adamantinoma in the lower leg: a case report and review of the literature. (16636523)
2006
12
Familial osteofibrous dysplasia. A case series. (16203897)
2005
13
Osteofibrous dysplasia and adamantinoma: correlation of proto-oncogene product and matrix protein expression. (14745727)
2004
14
Congenital (infantile) pseudarthrosis of the fibula associated with osteofibrous dysplasia. (15258702)
2004
15
Osteofibrous dysplasia treated with distraction osteogenesis: a report of two cases. (15449128)
2004
16
Classic adamantinoma with osteofibrous dysplasia-like foci and secondary aneurysmal bone cyst. (12574919)
2003
17
Adamantinoma, osteofibrous dysplasia and differentiated adamantinoma. (12679847)
2003
18
CD99 positive adamantinoma of the ulna with ipsilateral discrete osteofibrous dysplasia. (12616068)
2003
19
Comparative study of fibrous dysplasia and osteofibrous dysplasia: histopathological, immunohistochemical, argyrophilic nucleolar organizer region and DNA ploidy analysis. (11564214)
2001
20
Immunoexpression of neurofibromin, S-100 protein, and leu-7 and mutation analysis of the NF1 gene at codon 1423 in osteofibrous dysplasia. (11727265)
2001
21
Osteofibrous dysplasia of the ulna. (11793188)
2001
22
Osteofibrous dysplasia of the ulna. (11764436)
2001
23
A comparative study of fibrous dysplasia and osteofibrous dysplasia with regard to Gsalpha mutation at the Arg201 codon: polymerase chain reaction-restriction fragment length polymorphism analysis of paraffin-embedded tissues. (11272890)
2000
24
Osteofibrous dysplasia of the tibia: case report and review of the literature. (10709600)
1999
25
Treatment outcome of osteofibrous dysplasia. (9702669)
1998
26
Osteofibrous dysplasia-like adamantinoma of bone: a report of five cases with immunohistochemical and ultrastructural studies. (9712421)
1998
27
Cytogenetic analysis of adamantinoma of long bones: further indications for a common histogenesis with osteofibrous dysplasia. (9242211)
1997
28
Neonatal osteofibrous dysplasia: report of two cases. (8677152)
1996
29
Composition and posttranslational modification of individual collagen chains from osteosarcomas and osteofibrous dysplasias. (7635871)
1995
30
Clonal chromosomal abnormalities in osteofibrous dysplasia. Implications for histopathogenesis and its relationship with adamantinoma. (8156503)
1994
31
Relationship between osteofibrous dysplasia and adamantinoma. (7994967)
1994
32
Aggressive bone tumorous lesion in infancy: osteofibrous dysplasia of the tibia and fibula. (8376555)
1993
33
Osteofibrous dysplasia: clinicopathologic study of 80 cases. (8276381)
1993
34
Polyostotic lesions compatible with osteofibrous dysplasia. A case report. (8117510)
1993
35
Answer please. Osteofibrous dysplasia. (8290405)
1993
36
Osteofibrous dysplasia (ossifying fibroma of long bones). A report of four cases and review of the literature. (1563159)
1992
37
Osteofibrous dysplasia of the tibia treated by bracing. (1428320)
1992
38
Synovial sarcoma associated with osteofibrous dysplasia. A case report and review of the literature. (1510197)
1992
39
Osteofibrous dysplasia of long bones--a reactive process to adamantinomatous tissue. (1370955)
1992
40
Juvenile intracortical adamantinoma of the tibia with predominant osteofibrous dysplasia-like features. (1724319)
1991
41
Congenital ossifying fibroma (osteofibrous dysplasia) of the tibia--a case report. (1749686)
1991
42
Case report 587: Adamantinoma of the tibia mimicking osteofibrous dysplasia. (2326657)
1990
43
MRI findings in osteofibrous dysplasia. (2607907)
1989
44
Morphologic diversity of long bone adamantinoma. The concept of differentiated (regressing) adamantinoma and its relationship to osteofibrous dysplasia. (2804923)
1989
45
Osteofibrous dysplasia. A report of two cases. (3201274)
1988
46
Osteofibrous dysplasia of the tibia and fibula. (3205593)
1988
47
Radiologic case study. Osteofibrous dysplasia. (3615297)
1987
48
Osteofibrous dysplasia (ossifying fibroma) of the tibia and fibula and adamantinoma. A case report. (6475846)
1984
49
Osteofibrous dysplasia of the tibia and fibula. (7204433)
1981
50
Osteofibrous dysplasia of long bones a new clinical entity. (1024109)
1976

Variations for Osteofibrous Dysplasia

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Expression for genes affiliated with Osteofibrous Dysplasia

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Sources:
2BioGPS, 16Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Osteofibrous Dysplasia

Search GEO for disease gene expression data for Osteofibrous Dysplasia.

Pathways for genes affiliated with Osteofibrous Dysplasia

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Sources:
51PathCards, 52PharmGKB, 39NCBI BioSystems Database, 61Thomson Reuters, 31KEGG, 54QIAGEN
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Compounds for genes affiliated with Osteofibrous Dysplasia

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Sources:
46Novoseek, 3BitterDB, 62Tocris Bioscience, 12DrugBank, 25HMDB, 52PharmGKB, 30IUPHAR
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Compounds related to Osteofibrous Dysplasia according to GeneCards/GeneDecks:

(show top 50)    (show all 114)
idCompoundScoreTop Affiliating Genes
1ti6al4v4610.0BGLAP, CD36
2chitosan469.8CD36, BGLAP, SPP1
3tartrate469.6CD36, VIM, BGLAP
4alginate469.5SPP1, BGLAP, CD36
525-hydroxyvitamin d469.4CD36, BGLAP, SPP1
6pyridinoline469.3SPARC, BGLAP, CD36
7alizarin469.1SPP1, SPARC, BGLAP, CD36
8hydrocortisone46 3 62 1212.1BGLAP, VIM, MUC1, CD36
9b 723469.1KRT19, MUC1, VIM
10hematoxylin468.9KRT19, KRT1, MUC1, VIM
11adriamycin468.9VIM, MUC1, KRT19, CD36
12gnrh468.9BGLAP, GNAS, VIM, FOS, CD36
13ribonucleic acid468.8KRT19, FOS, MUC1, GNAS, SPP1
14chondroitin sulfate46 259.7CD36, VIM, SPARC, SPP1
15agar468.7FOS, MUC1, VIM, SPP1
16hyaluronic acid46 259.7SPP1, VIM, MUC1, KRT19, CD36
17calcitriol46 62 25 1211.6SPP1, SPARC, BGLAP, FOS, CD36
18matrigel468.6SPP1, VIM, MUC1, KRT19, CD36
19tgf beta1468.6CD36, VIM, SPARC, SPP1
20cytochalasin d46 629.6SPP1, VIM, FOS, NF1, CD36
21aspartate468.6KRT19, KRT1, VIM, BGLAP, SPP1
22retinoid468.6SPP1, VIM, FOS, KRT1, KRT19
23titanium468.5SPP1, SPARC, BGLAP, VIM, CD36
24hydroxyapatite468.5SPP1, SPARC, BGLAP, VIM, CD36
25procollagen468.5SPP1, SPARC, BGLAP, VIM, CD36
26forskolin46 52 1210.5NF1, FOS, MUC1, VIM, GNAS
27vitamin d468.4SPP1, SPARC, BGLAP, VIM, CD36
28arginine468.4SPP1, BGLAP, GNAS, VIM, NF1, KRT1
29creatinine468.4SPP1, BGLAP, VIM, MUC1, KRT19, CD36
30pge2468.3SPP1, SPARC, BGLAP, GNAS, FOS, CD36
31cyclic amp46 259.3GNAS, VIM, MUC1, FOS, NF1
32heparin46 30 25 1211.3SPP1, SPARC, BGLAP, VIM, CD36
33cycloheximide468.3SPP1, VIM, FOS, KRT19, CD36
34thymidine46 259.2SPARC, GNAS, MUC1, FOS, KRT19
35genistein46 30 62 3 25 1213.2CD36, FOS, VIM, SPARC, SPP1
36h2o2468.1SPP1, BGLAP, VIM, FOS, CD36
37sb 20358046 629.0SPP1, SPARC, BGLAP, VIM, FOS, CD36
38oligonucleotide467.8SPP1, SPARC, GNAS, VIM, FOS, NF1
39butyrate467.7SPP1, SPARC, VIM, MUC1, FOS, KRT19
40dexamethasone46 52 30 1210.6SPP1, SPARC, BGLAP, VIM, MUC1, FOS
41testosterone46 62 25 1210.5SPP1, SPARC, BGLAP, VIM, MUC1, KRT1
42cysteine467.3SPP1, SPARC, BGLAP, GNAS, VIM, MUC1
43tyrosine467.1SPP1, SPARC, GNAS, MUC1, FOS, NF1
44vegf467.1SPP1, SPARC, BGLAP, VIM, MUC1, FOS
45progesterone46 30 62 25 1211.0SPP1, SPARC, GNAS, VIM, MUC1, NF1
46serine466.8SPP1, BGLAP, GNAS, VIM, MUC1, FOS
47paraffin466.7CD36, SPP1, SPARC, BGLAP, VIM, MUC1
48calcium46 52 25 129.6SPP1, SPARC, BGLAP, GNAS, MUC1, FOS
49retinoic acid46 257.2KRT19, KRT1, NF1, FOS, MUC1, VIM
50estrogen466.2SPP1, CD36, KRT19, KRT1, NF1, FOS

GO Terms for genes affiliated with Osteofibrous Dysplasia

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17Gene Ontology
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Cellular components related to Osteofibrous Dysplasia according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1membrane-bounded vesicleGO:0319889.9BGLAP, SPP1
2dendriteGO:0304259.1BGLAP, GNAS, NF1
3membraneGO:0160209.0CD36, KRT1, FOS, GNAS
4extracellular vesicular exosomeGO:0700628.1KRT19, KRT1, MUC1, VIM, GNAS

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

(show all 16)
idNameGO IDScoreTop Affiliating Genes
1response to vitamin DGO:03328010.1BGLAP, SPP1
2positive regulation of osteoclast differentiationGO:0456729.9GNAS, FOS
3regulation of bone resorptionGO:0451249.9NF1, BGLAP
4DNA methylationGO:0063069.9GNAS, FOS
5negative regulation of transcription factor import into nucleusGO:0429929.9CD36, NF1
6positive regulation of Ras GTPase activityGO:0323209.8NF1, GNAS
7cognitionGO:0508909.8NF1, GNAS
8regulation of angiogenesisGO:0457659.7KRT1, NF1
9osteoblast differentiationGO:0016499.6SPP1, BGLAP, NF1
10response to drugGO:0424939.5BGLAP, GNAS, FOS
11cellular response to growth factor stimulusGO:0713639.4BGLAP, SPARC
12bone developmentGO:0603489.4GNAS, BGLAP, SPARC
13response to gravityGO:0096299.2FOS, BGLAP, SPARC
14response to cytokineGO:0340979.2FOS, SPARC
15response to cAMPGO:0515918.9FOS, SPARC
16extracellular matrix organizationGO:0301988.7SPP1, SPARC, NF1

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

idNameGO IDScoreTop Affiliating Genes
1extracellular matrix bindingGO:0508409.0SPARC, SPP1
2protein bindingGO:0055156.4GNAS, VIM, MUC1, FOS, NF1, KRT1

Products for genes affiliated with Osteofibrous Dysplasia

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  • Antibodies
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Sources for Osteofibrous Dysplasia

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet