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PubMed2000

Localization of the gene for fibrodysplasia ossificans progressiva (FOP) to chromosome 17q21-22.

Fibrodysplasia ossificans progressiva (FOP) is a very rare disease characterized by congenital malformation of the great toes and progressive heterotopic ossification of muscles. To identify the chromosomal localization of the FOP gene, we conducted a genomewide linkage analysis using seven affected families. The FOP phenotype is linked to markers located in the 17q21-22 region (LOD score of 3.41 at the recombination fraction theta = 0). Crossover events localize the putative FOP gene within a 12cM interval, bordered proximally by D17S809 and distally by D17S1838. Noggin (NOG) gene, located in 17q22, is an excellent candidate gene for FOP.

Chromosome Mapping↗
PubMed1993

Fibrodysplasia ossificans progressiva and synovial chondromatosis.

Two cases of an unusual association-fibrodysplasia ossificans progressiva and synovial chondromatosis-in non-related children are presented. This association does not seem coincidental and raises several questions about the pathogenesis. A genetic hypothesis related to G proteins is proposed. This is supported by the fact that such abnormalities have been demonstrated in pseudohypoparathyroidism and fibrous dysplasia; these diseases can also be associated with fibrodysplasia ossificans progressiva.

Adolescent↗
PubMed2002

Fibrodysplasia ossificans progressiva and corneal keloid.

PURPOSE: To report on the formation of a corneal keloid in a patient with fibrodysplasia ossificans progressiva after excision of a pterygium-like lesion. METHODS: Clinical and pathophysiological observations and hypothesis concerning pathophysiological mechanisms. RESULTS: An 11-year-old boy with fibrodysplasia ossificans progressiva presented with progressive development of a growing white scar in the central part of the left cornea after excision of a pterygium-like lesion. DISCUSSION: To the best of our knowledge, this is the first case report of a corneal keloid formation in a patient with fibrodysplasia ossificans progressiva. Pathophysiological mechanisms are considered. Therapeutic options are discussed.

Child↗
PubMed1998

Treatment of patients who have fibrodysplasia ossificans progressiva with isotretinoin.

Retinoids are a plausible family of therapeutic agents for fibrodysplasia ossificans progressiva due to their ability to inhibit differentiation of mesenchymal tissue into cartilage and bone. A prospective study was conducted to assess the efficacy of isotretinoin (13-cis-retinoic acid) in the prevention of heterotopic ossification in patients who had fibrodysplasia ossificans progressiva. Eleven anatomic regions were assessed in each of 21 patients by clinical examination, radiographs, and bone scans. An anatomic region was considered to be involved if there was clinical, radiographic, or radionuclide evidence of orthotopic or heterotopic ossification anywhere in the region. There were 143 involved anatomic regions and 88 uninvolved anatomic regions at the beginning of the study. Only one of the 88 anatomic regions that was completely uninvolved at the beginning of the study became involved during isotretinoin therapy. However, 16 of the 21 patients (76%) had major flare ups develop in 38 of 143 (27%) previously involved anatomic regions while administered isotretinoin therapy. Isotretinoin at steady state doses of 1 to 2 mg/kg per day decreased the incidence of heterotopic ossification at uninvolved anatomic regions compared with an external control group, as long as the medication was started before the appearance of any orthotopic or heterotopic ossification in that anatomic region. The data did not allow the determination of whether isotretinoin was effective or detrimental in preventing disease flareups in regions that had even minimal orthotopic or heterotopic ossification at the time the therapy began. Extreme caution should be exercised when using this medication in patients who have fibrodysplasia ossificans progressiva.

Adolescent↗
PubMed2003

Bone morphogenetic protein-4 regulation in fibrodysplasia ossificans progressiva.

Fibrodysplasia ossificans progressiva is a rare genetic disorder in which connective tissues are replaced with heterotopic bone through an endochondral process. Bone morphogenetic protein-4 messenger ribonucleic acid and protein levels are elevated in the cells of patients with fibrodysplasia ossificans progressiva, but the molecular mechanism of this steady-state elevation is unknown. Nuclear run-on assays and messenger ribonucleic acid stability assays were done to examine the molecular mechanisms of increased bone morphogenetic protein-4 messenger ribonucleic acid. The bone morphogenetic protein-4 transcription rate in patient cells was found to be enhanced fivefold to sevenfold over normal control cells, suggesting that elevated steady-state levels of this transcript were attributable at least in part to an enhancement in transcription initiation. The stability of bone morphogenetic protein-4 messenger ribonucleic acid was found to be similar for patient and control cells and to have an extremely brief half-life, with bone morphogenetic protein-4 messenger ribonucleic acid almost completely decayed (75%) by 40 minutes. This unusually brief half-life suggests that a high fidelity control over temporal expression of the bone morphogenetic protein 4-message can be maintained. The data document that enhanced transcription rather than increased messenger ribonucleic acid stability is responsible for the elevation in steady-state levels of bone morphogenetic protein-4 messenger ribonucleic acid, and suggest that an inappropriate enhancement of the rate of bone morphogenetic protein-4 transcription plays a critical role in the molecular pathophysiology of fibrodysplasia ossificans progressiva.

Adult↗
PubMed2006

Optimal treatment of fibrodysplasia ossificans progressiva with surgical excision of heterotopic bone, indomethacin, and irradiation.

A case report is presented of an 18-year-old boy with advanced fibrodysplasia ossificans progressiva. The patient's right hip was ankylosed in 35 degrees of flexion. Excision of the heterotopic bone from the right hip was followed by a combination of a single fraction irradiation (7Gy) given in the first postoperative day, and an 11-day treatment with indomethacin (25 mg, three times daily, orally). One year postoperatively, the patient ambulates nearly normally and he is able to sit and stand up without significant difficulty. Radiographs of the right hip showed a small amount of heterotopic bone formation at the operative site. In contrast with the results published in the literature, the clinical result of the operation is considered satisfactory. Further investigation of the combined use of indomethacin and single fraction irradiation as a preventive measure after surgical excision of heterotopic bone in patients with fibrodysplasia ossificans progressiva FOP should be performed.

Adolescent↗
PubMed1998

The genetics of fibrodysplasia ossificans progressiva.

Fibrodysplasia ossificans progressiva is an autosomal dominant disorder. Most cases are due to new gene mutations because people with fibrodysplasia ossificans progressiva have markedly reduced reproductive fitness. The gene or genes responsible for this disorder are unknown.

Child↗
PubMed1998

Human leukocyte antigen B27 allele is not correlated with fibrodysplasia ossificans progressiva.

Fibrodysplasia ossificans progressiva is a rare heritable disorder of connective tissue characterized by skeletal malformations and by progressive heterotopic ossification. It has been suggested that the genetic marker human leukocyte antigen B27 may be associated with fibrodysplasia ossificans progressiva, as it is with ankylosing spondylitis, another disorder with less severe hyperostosis. Genomic deoxyribonucleic acid from 23 classically affected patients with fibrodysplasia ossificans progressiva was screened for the human leukocyte antigen B27 allele by polymerase chain reaction. Only two of the 23 patients (9%) with fibrodysplasia ossificans progressiva who were examined showed the presence of the human leukocyte antigen B27 allele, an incidence that corresponds to the 8% frequency of individuals within the general population not affected with ankylosing spondylitis. These data suggest that the human leukocyte antigen B27 allele does not occur more commonly in the genotype of patients with fibrodysplasia ossificans progressiva than in the general population, and that the pathogenesis of heterotopic bone in fibrodysplasia ossificans progressiva differs from that of ankylosing spondylitis and other human leukocyte antigen B27 positive disorders.

Female↗
PubMed1986

Fibrodysplasia ossificans progressiva: CT appearance.

Twelve patients with fibrodysplasia ossificans progressiva were studied with computed tomography (CT). Characteristic swelling of the muscular fascial planes could be identified on CT scans prior to the development of ectopic ossification. Ossification could be seen on CT scans before it was apparent on plain radiographs. The pattern of ossification was similar to that seen at pathologic study, with multifocal sites developing adjacent to and extending around muscles. The appearance on CT scans confirms the hypothesis that the initial focus in fibrodysplasia ossificans progressiva is in the connective tissue.

Adolescent↗
PubMed1998

Initiation of fracture repair by bone morphogenetic proteins.

The potential for regeneration and repair of bone is well known. This article conveys the current progress in the realm of bone morphogenetic proteins and their potential for initiating fracture repair cascade. Demineralized bone matrix induces bone formation and has served as a model for the bone repair cascade. A family of bone morphogenetic proteins has been identified, isolated, and cloned from the demineralized bone matrix. Bone morphogenetic proteins are pleiotropic regulators of chemotaxis, mitosis, and differentiation. The bone morphogenetic protein receptors, Types I and II, bind bone morphogenetic proteins and act in collaboration to transduce the phosphorylation of Smad 1 and Smad 5, which enter the nucleus in partnership with Smad 4 to initiate bone morphogenetic protein responses including fracture healing. The accumulated information on bone morphogenetic proteins may aid in accelerating fracture repair and the potential use of bone morphogenetic protein antibodies to inhibit heterotopic bone formation and fibrodysplasia ossificans progressiva.

Antibodies↗
PubMed1998

Adelaide's blackbirding pathologist.

Archibald Watson (1849-1940) was the inaugural Elder Professor of Anatomy at the University of Adelaide. He held the position from 1885 to 1919, having previously trained in Gottingen, Paris, and London under some of the most eminent physicians of those times. Before he started his medical studies, he, Dr James Patrick Murray, and several others were involved in the kidnapping ("blackbirding") of Pacific Islanders (Kanakas) to work on South Sea cotton plantations. A number of the victims were killed. Watson and two others were charged with murder and remanded in Levuka to appear in Sydney. Watson absconded, traveled to Europe, and never stood trial; the other two were convicted and sentenced to death. Watson's life as an anatomist, surgeon, pathologist, member of the Adelaide Club, world traveler, Australian Army doctor during the Boer War and World War I, fisherman, and motorcyclist was full of interest and incidents. The events surrounding the autopsy he performed on a case of fibrodysplasia ossificans progressiva and the subsequent preservation of the skeleton in the Anatomy Museum of the University of Adelaide are described.

Anatomy↗
PubMed1996

Overexpression of an osteogenic morphogen in fibrodysplasia ossificans progressiva.

BACKGROUND: Fibrodysplasia ossificans progressiva is a heritable disorder of connective tissue characterized by congenital malformation of the great toes and postnatal formation of ectopic bone. Although the disorder was first described more than 300 years ago, the genetic defect and pathophysiology remain unknown. Bone morphogenetic proteins are potent bone-inducing morphogens that participate in the developmental organization of the skeleton, and increased production of one or more of these proteins has been proposed as the cause of fibrodysplasia ossificans progressiva. METHODS: We studied lymphoblastoid cell lines established from peripheral-blood mononuclear cells of patients with fibrodysplasia ossificans progressiva and fibroblast-like cell lines derived from lesional and nonlesional tissue. We used Northern blot analysis and ribonuclease protection assays to measure the expression of messenger RNA (mRNA) of bone morphogenetic proteins 1 to 7 and immunohistochemical analysis to examine protein expression. RESULTS: Among the bone morphogenetic proteins and mRNAs examined, only bone morphogenetic protein 4 and its mRNA were present in increased levels in cells derived from an early fibroproliferative lesion in a patient with fibrodysplasia ossificans progressiva. Bone morphogenetic protein 4 mRNA was expressed in lymphoblastoid cell lines from 26 of 32 patients with fibrodysplasia ossificans progressiva but from only 1 of 12 normal subjects (P<0.001). Bone morphogenetic protein 4 and its mRNA were detected in the lymphoblastoid cell lines from a man with fibrodysplasia ossificans progressiva and his three affected children (two girls and a boy), but not from the children's unaffected mother. No other bone morphogenetic proteins were detected. CONCLUSIONS: Overexpression of a potent bone-inducing morphogen (bone morphogenetic protein 4) in lymphocytes is associated with the disabling ectopic osteogenesis of fibrodysplasia ossificans progressiva.

Bone Morphogenetic Proteins↗