MCID: OST032
MIFTS: 47

Osteofibrous Dysplasia malady

Rare diseases, Bone diseases categories
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Summaries for Osteofibrous Dysplasia

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Wikipedia:65 Osteofibrous dysplasia (also known as ossifying fibroma) is a rare, benign non-neoplastic condition with... more...

MalaCards based summary: Osteofibrous Dysplasia, also known as jaffe-campanacci syndrome, is related to fibroma and osteoblastoma. 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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Sources:
42NIH Rare Diseases, 62UMLS, 44Novoseek
See all sources

Osteofibrous Dysplasia, Aliases & Descriptions:

Name: Osteofibrous Dysplasia 42 44 62
Jaffe-Campanacci Syndrome 42 62
 
Ossifying Fibroma 42 62
Intracortical Fibrous Dysplasia 42


Classifications:

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


Related Diseases for Osteofibrous Dysplasia

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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
1fibroma31.2KRT1, VIM
2osteoblastoma31.1SPARC
3fibromatosis30.9VIM
4primary hyperparathyroidism30.8CD36, BGLAP
5fibrous dysplasia30.7SPARC, GNAS, FOS, BGLAP
6hyperparathyroidism30.7BGLAP, NF1
7giant cell tumor30.5MUC1, VIM, BGLAP
8chondrosarcoma30.1VIM, CD36, KRT19, MUC1, SPARC
9osteosarcoma30.0BGLAP, SPP1, SPARC, FOS, CD36
10meningioma30.0SPARC, VIM, MUC1, NF1, KRT1
11sarcoma29.9NF1, VIM, MUC1
12synovial sarcoma29.5VIM, KRT1, NF1, KRT19, MUC1
13sinusitis10.8
14fibromatosis multiple non ossifying10.6
15ossifying fibroma10.6
16pyogenic granuloma10.5
17idiopathic juvenile osteoporosis10.5CD36
18eccrine porocarcinoma10.5MUC1
19hidradenoma10.5MUC1
20cystadenocarcinoma10.5MUC1
21paraganglioma10.5VIM
22adenomatoid tumor10.4VIM
23pseudohypoparathyroidism type 1b10.4GNAS, BGLAP
24gingivitis10.4
25osteitis fibrosa10.4GNAS, BGLAP
26osteogenesis imperfecta type i10.4BGLAP, CD36
27pseudohypoparathyroidism10.4BGLAP, GNAS
28aortic valve stenosis10.4SPP1, SPARC
29gray platelet syndrome10.4SPARC, CD36
30renal osteodystrophy10.4CD36, BGLAP
31calcinosis10.4SPARC, SPP1
32hyperphosphatemia10.3GNAS, SPP1
33dentinogenesis imperfecta10.3CD36, SPP1
34secondary hyperparathyroidism of renal origin10.3CD36, BGLAP
35neurofibromatosis10.3
36periodontitis10.3
37monostotic fibrous dysplasia10.3
38osteoporosis, postmenopausal10.3CD36, BGLAP
39pseudoarthrosis10.3
40mccune albright syndrome10.3
41neurilemmoma10.3NF1
42goiter10.3BGLAP, GNAS
43multinodular goiter10.3KRT19, GNAS
44progressive osseous heteroplasia10.3GNAS, BGLAP, SPARC
45rickets10.3CD36, BGLAP
46osteogenesis imperfecta10.3BGLAP, CD36, SPARC
47cataract10.3FOS, SPARC
48osteomalacia10.3CD36, GNAS, BGLAP
49malignant pleural mesothelioma10.3MUC1, SPP1
50clear cell ependymoma10.3MUC1, VIM

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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Drug clinical trials:

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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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MalaCards organs/tissues related to Osteofibrous Dysplasia:

32
Bone

Animal Models for Osteofibrous Dysplasia or affiliated genes

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MGI Mouse Phenotypes related to Osteofibrous Dysplasia:

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

Publications for Osteofibrous Dysplasia

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Articles related to Osteofibrous Dysplasia:

(show top 50)    (show all 75)
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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Expression patterns in normal tissues for genes affiliated with Osteofibrous Dysplasia

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Pathways for genes affiliated with Osteofibrous Dysplasia

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Compounds for genes affiliated with Osteofibrous Dysplasia

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Compounds related to Osteofibrous Dysplasia according to GeneCards/GeneDecks:

(show top 50)    (show all 114)
idCompoundScoreTop Affiliating Genes
1ti6al4v4410.0CD36, BGLAP
2chitosan449.8BGLAP, SPP1, CD36
3tartrate449.6BGLAP, VIM, CD36
4alginate449.5CD36, BGLAP, SPP1
525-hydroxyvitamin d449.4BGLAP, SPP1, CD36
6pyridinoline449.3SPARC, CD36, BGLAP
7alizarin449.1CD36, BGLAP, SPARC, SPP1
8hydrocortisone44 2 61 1112.1BGLAP, MUC1, CD36, VIM
9b 723449.1KRT19, VIM, MUC1
10hematoxylin448.9MUC1, KRT19, KRT1, VIM
11adriamycin448.9MUC1, CD36, KRT19, VIM
12gnrh448.9BGLAP, FOS, CD36, VIM, GNAS
13ribonucleic acid448.8KRT19, FOS, MUC1, GNAS, SPP1
14chondroitin sulfate44 249.7CD36, VIM, SPARC, SPP1
15agar448.7SPP1, FOS, MUC1, VIM
16hyaluronic acid44 249.7VIM, CD36, KRT19, MUC1, SPP1
17calcitriol44 61 24 1111.6SPP1, CD36, SPARC, BGLAP, FOS
18matrigel448.6VIM, CD36, KRT19, MUC1, SPP1
19tgf beta1448.6SPP1, SPARC, CD36, VIM
20cytochalasin d44 619.6VIM, FOS, NF1, CD36, SPP1
21aspartate448.6SPP1, BGLAP, VIM, KRT1, KRT19
22retinoid448.6SPP1, FOS, KRT1, KRT19, VIM
23titanium448.5CD36, VIM, BGLAP, SPARC, SPP1
24hydroxyapatite448.5SPP1, BGLAP, SPARC, CD36, VIM
25procollagen448.5SPP1, SPARC, BGLAP, VIM, CD36
26forskolin44 50 1110.5GNAS, VIM, MUC1, FOS, NF1
27vitamin d448.4VIM, CD36, BGLAP, SPARC, SPP1
28arginine448.4SPP1, KRT1, NF1, GNAS, BGLAP, VIM
29creatinine448.4KRT19, MUC1, VIM, BGLAP, SPP1, CD36
30pge2448.3CD36, FOS, GNAS, SPARC, BGLAP, SPP1
31cyclic amp44 249.3VIM, NF1, GNAS, FOS, MUC1
32heparin44 28 24 1111.3SPARC, SPP1, BGLAP, VIM, CD36
33cycloheximide448.3CD36, SPP1, VIM, KRT19, FOS
34thymidine44 249.2SPARC, GNAS, MUC1, FOS, KRT19
35genistein44 28 61 2 24 1113.2SPARC, CD36, VIM, SPP1, FOS
36h2o2448.1FOS, SPP1, CD36, BGLAP, VIM
37sb 20358044 619.0CD36, SPP1, SPARC, BGLAP, VIM, FOS
38oligonucleotide447.8GNAS, SPARC, SPP1, VIM, NF1, FOS
39butyrate447.7SPP1, FOS, KRT19, SPARC, VIM, MUC1
40dexamethasone44 50 28 1110.6CD36, FOS, MUC1, VIM, BGLAP, SPARC
41testosterone44 61 24 1110.5MUC1, VIM, KRT19, KRT1, BGLAP, SPARC
42cysteine447.3VIM, NF1, MUC1, GNAS, BGLAP, SPARC
43tyrosine447.1KRT1, NF1, FOS, MUC1, GNAS, SPP1
44vegf447.1FOS, CD36, SPP1, MUC1, VIM, SPARC
45progesterone44 28 61 24 1111.0MUC1, KRT19, NF1, VIM, GNAS, SPARC
46serine446.8NF1, FOS, CD36, VIM, GNAS, BGLAP
47paraffin446.7BGLAP, CD36, SPP1, SPARC, VIM, MUC1
48calcium44 50 24 119.6KRT19, KRT1, NF1, FOS, MUC1, GNAS
49retinoic acid44 247.2SPARC, FOS, MUC1, BGLAP, VIM, NF1
50estrogen446.2CD36, KRT19, KRT1, NF1, MUC1, VIM

GO Terms for genes affiliated with Osteofibrous Dysplasia

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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:0160209.0GNAS, FOS, KRT1, CD36
4extracellular vesicular exosomeGO:0700628.1VIM, MUC1, KRT1, KRT19, 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:0429929.9NF1, CD36
6positive regulation of Ras GTPase activityGO:0323209.8NF1, GNAS
7cognitionGO:0508909.8NF1, GNAS
8regulation of angiogenesisGO:0457659.7NF1, KRT1
9osteoblast differentiationGO:0016499.6NF1, SPP1, BGLAP
10response to drugGO:0424939.5BGLAP, GNAS, FOS
11cellular response to growth factor stimulusGO:0713639.4BGLAP, SPARC
12bone developmentGO:0603489.4SPARC, GNAS, BGLAP
13response to gravityGO:0096299.2SPARC, FOS, BGLAP
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:0508409.0SPP1, SPARC
2protein bindingGO:0055156.4SPARC, GNAS, VIM, MUC1, FOS, NF1

Products for genes affiliated with Osteofibrous Dysplasia

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Sources for Osteofibrous Dysplasia

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet