MCID: OST032
MIFTS: 47

Osteofibrous Dysplasia malady

Rare diseases, Bone diseases categories
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Summaries for Osteofibrous Dysplasia

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65Wikipedia, 33MalaCards
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Wikipedia:65 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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43NIH Rare Diseases, 62UMLS, 45Novoseek
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Classifications:

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


Aliases & Descriptions:

osteofibrous dysplasia 43 45 62
jaffe-campanacci syndrome 43 62
ossifying fibroma 43 62
intracortical fibrous dysplasia 43


Related Diseases for Osteofibrous Dysplasia

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17GeneCards, 18GeneDecks
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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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42NIH Clinical Center, 6ClinicalTrials, 62UMLS, 41NDF-RT
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Drug clinical trials:

Search ClinicalTrials for Osteofibrous Dysplasia

Search NIH Clinical Center for Osteofibrous Dysplasia

Genetic Tests for Osteofibrous Dysplasia

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Anatomical Context for Osteofibrous Dysplasia

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33MalaCards
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MalaCards organs/tissues related to Osteofibrous Dysplasia:

33
Bone

Animal Models for Osteofibrous Dysplasia or affiliated genes

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37MGI
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MGI Mouse Phenotypes related to Osteofibrous Dysplasia:

37 (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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52PubMed
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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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2BioGPS, 15Gene 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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50PathCards, 51PharmGKB, 38NCBI BioSystems Database, 60Thomson Reuters, 30KEGG, 53QIAGEN
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Compounds for genes affiliated with Osteofibrous Dysplasia

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Sources:
45Novoseek, 3BitterDB, 61Tocris Bioscience, 11DrugBank, 24HMDB, 51PharmGKB, 29IUPHAR
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Compounds related to Osteofibrous Dysplasia according to GeneCards/GeneDecks:

(show top 50)    (show all 114)
idCompoundScoreTop Affiliating Genes
1ti6al4v4510.0BGLAP, CD36
2chitosan459.8CD36, BGLAP, SPP1
3tartrate459.6CD36, VIM, BGLAP
4alginate459.5SPP1, BGLAP, CD36
525-hydroxyvitamin d459.4CD36, BGLAP, SPP1
6pyridinoline459.3SPARC, BGLAP, CD36
7alizarin459.1SPP1, SPARC, BGLAP, CD36
8hydrocortisone45 3 61 1112.1BGLAP, VIM, MUC1, CD36
9b 723459.1KRT19, MUC1, VIM
10hematoxylin458.9KRT19, KRT1, MUC1, VIM
11adriamycin458.9VIM, MUC1, KRT19, CD36
12gnrh458.9BGLAP, GNAS, VIM, FOS, CD36
13ribonucleic acid458.8KRT19, FOS, MUC1, GNAS, SPP1
14chondroitin sulfate45 249.7CD36, VIM, SPARC, SPP1
15agar458.7FOS, MUC1, VIM, SPP1
16hyaluronic acid45 249.7SPP1, VIM, MUC1, KRT19, CD36
17calcitriol45 61 24 1111.6SPP1, SPARC, BGLAP, FOS, CD36
18matrigel458.6SPP1, VIM, MUC1, KRT19, CD36
19tgf beta1458.6CD36, VIM, SPARC, SPP1
20cytochalasin d45 619.6SPP1, VIM, FOS, NF1, CD36
21aspartate458.6KRT19, KRT1, VIM, BGLAP, SPP1
22retinoid458.6SPP1, VIM, FOS, KRT1, KRT19
23titanium458.5SPP1, SPARC, BGLAP, VIM, CD36
24hydroxyapatite458.5SPP1, SPARC, BGLAP, VIM, CD36
25procollagen458.5SPP1, SPARC, BGLAP, VIM, CD36
26forskolin45 51 1110.5NF1, FOS, MUC1, VIM, GNAS
27vitamin d458.4SPP1, SPARC, BGLAP, VIM, CD36
28arginine458.4SPP1, BGLAP, GNAS, VIM, NF1, KRT1
29creatinine458.4SPP1, BGLAP, VIM, MUC1, KRT19, CD36
30pge2458.3SPP1, SPARC, BGLAP, GNAS, FOS, CD36
31cyclic amp45 249.3GNAS, VIM, MUC1, FOS, NF1
32heparin45 29 24 1111.3SPP1, SPARC, BGLAP, VIM, CD36
33cycloheximide458.3SPP1, VIM, FOS, KRT19, CD36
34thymidine45 249.2SPARC, GNAS, MUC1, FOS, KRT19
35genistein45 29 61 3 24 1113.2CD36, FOS, VIM, SPARC, SPP1
36h2o2458.1SPP1, BGLAP, VIM, FOS, CD36
37sb 20358045 619.0SPP1, SPARC, BGLAP, VIM, FOS, CD36
38oligonucleotide457.8SPP1, SPARC, GNAS, VIM, FOS, NF1
39butyrate457.7SPP1, SPARC, VIM, MUC1, FOS, KRT19
40dexamethasone45 51 29 1110.6SPP1, SPARC, BGLAP, VIM, MUC1, FOS
41testosterone45 61 24 1110.5SPP1, SPARC, BGLAP, VIM, MUC1, KRT1
42cysteine457.3SPP1, SPARC, BGLAP, GNAS, VIM, MUC1
43tyrosine457.1SPP1, SPARC, GNAS, MUC1, FOS, NF1
44vegf457.1SPP1, SPARC, BGLAP, VIM, MUC1, FOS
45progesterone45 29 61 24 1111.0SPP1, SPARC, GNAS, VIM, MUC1, NF1
46serine456.8SPP1, BGLAP, GNAS, VIM, MUC1, FOS
47paraffin456.7CD36, SPP1, SPARC, BGLAP, VIM, MUC1
48calcium45 51 24 119.6SPP1, SPARC, BGLAP, GNAS, MUC1, FOS
49retinoic acid45 247.2KRT19, KRT1, NF1, FOS, MUC1, VIM
50estrogen456.2SPP1, CD36, KRT19, KRT1, NF1, FOS

GO Terms for genes affiliated with Osteofibrous Dysplasia

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16Gene 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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Sources for Osteofibrous 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