Fibrous Dysplasia of Bone

A disease of bone marked by thinning of the cortex and replacement of bone marrow by gritty fibrous tissue containing bony spicules, producing pain, disability, and gradually increasing deformity. Only one bone may be involved (FIBROUS DYSPLASIA, MONOSTOTIC) or several (FIBROUS DYSPLASIA, POLYOSTOTIC). (From Dorland, 28th ed)

Also Known As:

Networked: 53 relevant articles (1 outcomes, 2 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Pain (Aches)
2.	Polyostotic Fibrous Dysplasia (McCune Albright Syndrome)
3.	Neoplasms (Cancer)
4.	Precocious Puberty
5.	Osteoporosis

Experts

1.	Collins, Michael T: 4 articles (11/2014 - 08/2006)
2.	Lala, R: 4 articles (08/2002 - 02/2000)
3.	Matarazzo, P: 4 articles (08/2002 - 02/2000)
4.	Robey, Pamela G: 3 articles (11/2014 - 08/2006)
5.	Bastepe, Murat: 3 articles (11/2011 - 09/2007)
6.	de Sanctis, C: 3 articles (08/2002 - 02/2000)
7.	Chapurlat, Roland: 2 articles (12/2014 - 09/2008)
8.	Bianco, Paolo: 2 articles (11/2014 - 11/2014)
9.	Riminucci, Mara: 2 articles (11/2014 - 11/2014)
10.	Kelly, Marilyn H: 2 articles (11/2014 - 08/2006)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Fibrous Dysplasia of Bone:

1.	pegvisomantFDA LinkGeneric 08/01/2006 - "We examined efficacy of the GH receptor antagonist, pegvisomant, in controlling gsp oncogene-mediated GH excess and skeletal disease (fibrous dysplasia of bone) associated with MAS. "
2.	Alendronate (Alendronate Sodium)FDA LinkGeneric 11/01/2014 - "A randomized, double blind, placebo-controlled trial of alendronate treatment for fibrous dysplasia of bone." 12/01/2006 - "In open studies, bisphosphonate therapy (pamidronate, alendronate) reduced bone pain associated with fibrous dysplasia of bone and was associated to some radiological improvement. "
3.	pamidronate (APD)FDA LinkGeneric 09/01/2008 - "We report on a young boy initially diagnosed with fibrous dysplasia of bone, who underwent renal investigation because of treatment with pamidronate. " 07/01/2004 - "Treatment of fibrous dysplasia of bone with intravenous pamidronate: long-term effectiveness and evaluation of predictors of response to treatment." 10/01/1997 - "Long-term effects of intravenous pamidronate in fibrous dysplasia of bone." 04/16/1994 - "Long-term effects of intravenous pamidronate in fibrous dysplasia of bone." 01/01/2006 - "To examine outcomes of pamidronate treatment on fibrous dysplasia of bone in three children with McCune-Albright syndrome (MAS). "
4.	GTP Phosphohydrolases (GTPases)IBA 06/01/2002 - "Mutations of Gs(alpha) residues involved in the GTPase reaction that lead to constitutive activation are present in endocrine tumors, fibrous dysplasia of bone, and McCune-Albright syndrome. " 11/04/2011 - "Although isoproterenol-induced cAMP response was not prolonged by XLαs expression, a GTPase-deficient XLαs mutant found in certain tumors and patients with fibrous dysplasia of bone and McCune-Albright syndrome generated more basal cAMP accumulation in HEK293 cells and caused more severe impairment of osteoblastic differentiation of MC3T3-E1 cells than the cognate Gαs mutant (gsp oncogene). " 12/01/2004 - "Somatic activating G(s)alpha mutations, which alter key residues required for the GTPase turn-off reaction, are present in various endocrine tumors and fibrous dysplasia of bone, and in a more widespread distribution in patients with McCune- Albright syndrome. " 10/01/2001 - "Mutations of specific G(s)alpha residues (Arg(201) or Gln(227)) that are critical for the GTPase reaction lead to constitutive activation of G(s)-coupled signaling pathways, and such somatic mutations are found in endocrine tumors, fibrous dysplasia of bone, and the McCune-Albright syndrome. "
5.	DiphosphonatesIBA 11/01/1998 - "Bisphosphonates are a breakthrough in Paget's disease of bone, fibrous dysplasia of bone and osteoporosis." 01/01/2007 - "Bisphosphonates are pharmacological agents which are currently used both in osteoporosis than in other pathological conditions characterised by an increased bone resorption, such as Paget's disease of bone, malign hypocalcaemia during myeloma, osteolytic bone metastasis and fibrous dysplasia of bone. " 03/01/1999 - "The potential role of chronic growth hormone overproduction in the occurrence of malignant transformation and the possible value of bisphosphonates in the treatment of bone fibrous dysplasias are discussed." 12/01/2005 - "In this report we review the present state of knowledge regarding the use of bisphosphonates during childhood to ameliorate the skeletal abnormalities associated with osteogenesis imperfecta, idiopathic juvenile osteoporosis, fibrous dysplasia of bone and cerebral palsy. "
6.	Codon (Codons)IBA 04/01/2014 - "Fibrous dysplasia of bone was suspected, but there was no mutation in codon 201 of GNAS in samples from blood or tumor. " 01/01/2002 - "Activating missense mutations at the Arg 201 codon of the Gs alpha gene ultimately leading to increased levels of cyclic adenosine monophosphate have been implicated in McCune-Albright syndrome and sporadic fibrous dysplasia of bone. " 02/01/2002 - "To date, mutations at the codon encoding R201, typically underlying the McCune-Albright syndrome and isolated fibrous dysplasia of bone, have been demonstrated only in growth hormone secreting pituitary adenomas. " 08/01/2000 - "Activating missense mutations in the Arg 201 codon of the gene encoding the alpha subunit of Gs, the G protein that stimulates cAMP formation, have been recognized as the cause of many endocrine diseases, McCune-Albright syndrome and isolated fibrous dysplasia of bone. "
7.	Gs GTP-Binding Protein alpha Subunits (G(s)alpha)IBA 11/01/2014 - "Fibrous dysplasia of bone (FD) is a crippling skeletal disease associated with postzygotic mutations (R201C, R201H) of the gene encoding the α subunit of the stimulatory G protein, Gs. " 12/01/2004 - "Somatic activating G(s)alpha mutations, which alter key residues required for the GTPase turn-off reaction, are present in various endocrine tumors and fibrous dysplasia of bone, and in a more widespread distribution in patients with McCune- Albright syndrome. " 10/01/2001 - "Mutations of specific G(s)alpha residues (Arg(201) or Gln(227)) that are critical for the GTPase reaction lead to constitutive activation of G(s)-coupled signaling pathways, and such somatic mutations are found in endocrine tumors, fibrous dysplasia of bone, and the McCune-Albright syndrome. "
8.	GTP-Binding Proteins (G-Protein)IBA 01/01/2014 - "Mis-sense mutations in the α-subunit of the G-protein, Gsα, cause fibrous dysplasia of bone/McCune-Albright syndrome. " 08/01/2000 - "Activating missense mutations in the Arg 201 codon of the gene encoding the alpha subunit of Gs, the G protein that stimulates cAMP formation, have been recognized as the cause of many endocrine diseases, McCune-Albright syndrome and isolated fibrous dysplasia of bone. "
9.	Testosterone (Sustanon)FDA Link 06/01/1948 - "Testosterone therapy in a case of fibrous dysplasia of bone with hypogenitalism." 11/01/1947 - "Testosterone therapy in a case of fibrous dysplasia of bone with hypogenitalism."
10.	Growth Hormone (Somatotropin)IBA 03/01/1999 - "The potential role of chronic growth hormone overproduction in the occurrence of malignant transformation and the possible value of bisphosphonates in the treatment of bone fibrous dysplasias are discussed." 02/01/2002 - "To date, mutations at the codon encoding R201, typically underlying the McCune-Albright syndrome and isolated fibrous dysplasia of bone, have been demonstrated only in growth hormone secreting pituitary adenomas. "

Therapies and Procedures

1.	Transplants (Transplant) 09/01/1966 - "[Biological reconstruction of transplants following surgery in connection with fibrous dysplasia of bone]." 03/01/2000 - "Intralesional curettage and reconstruction with autogenous iliac crest graft in fibrous dysplasia of bone leads to a high recurrence rate. "
2.	Bone Transplantation (Bone Grafting) 03/01/1977 - "Pathologic subtrochanteric fracture in a femur rarefied by fibrous dysplasia of bone was successfully treated with condylocephalic nailing and bone grafting. " 01/01/2001 - "From January 1994 to June 1999, 148 cases of bone defect were repaired by embryonic bone transplantation following alcohol treatment, there were 63 cases with bone cyst, 42 cases with fibrous dysplasia of bone, 26 cases with giant cell tumor of bone, and 17 cases with enchondroma among them. "
3.	Curettage 03/01/2000 - "Intralesional curettage and reconstruction with autogenous iliac crest graft in fibrous dysplasia of bone leads to a high recurrence rate. " 04/01/2008 - "From January 2003 to December 2005, 61 cases of various bone defects following the curettage of the benign bone tumors were treated with interporous TCP, with 33 males and 28 females, including bone fibrous dysplasia in 8 cases, bone cyst in 23 cases, eosinophilic granuloma in 12 cases, enchondroma in 13 cases, non-ossifying fibroma in 2 cases, and osteoblastoma in 3 cases. "
4.	Ovariectomy (Oophorectomy) 08/01/2010 - "McCune-Albright syndrome (MAS): early and extensive bone fibrous dysplasia involvement and "mistaken identity" oophorectomy."
5.	Osteotomy 03/01/2009 - "Medial displacement valgus or valgus osteotomies were used to treat fibrous dysplasia of bone involving the proximal part of the femur with coax varus. "