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Results for “MYOSITIS OSSIFICANS”

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PubMed1986

Technetium-99m MDP demonstration of heterotopic ossification in fibrodysplasia ossificans progressiva.

The extensive heterotopic bone formation in patients with fibrodysplasia ossificans progressiva (FOP) has been documented previously with radiographs. A case in which a Tc-99m MDP bone scan showed increased uptake at sites well before ossification could be documented radiographically is described. This finding suggests that bone scans would likely be useful to monitor the extent of involvement with FOP and to detect areas of new activity.

Child↗

PubMed1983

Mitral and aortic valve abnormalities in a patient with fibrodysplasia ossificans progressiva.

A 36-yr-old female with a 20-yr history of fibrodysplasia ossificans progressiva (FOP) died as the result of ruptured gastric ulcers. The patient had been treated with disodium ethane-l-hydroxy-1, 1-diphosphonate (EHDP). Post mortem there was thickening of the aortic valve cusps and mitral valve leaflets as well as shortening and thickening of the chordae tendineae of the mitral valve. This is a previously unreported association.

Adult↗

PubMed1990

Failure of surgery and isotretinoin to relieve jaw immobilization in fibrodysplasia ossificans progressiva: report of two cases.

Two patients with fibrodysplasia ossificans progressiva who presented with jaw immobilization due to formation of bone between the maxilla and mandible were treated with surgical resection of their ectopic bone in conjunction with experimental, adjunctive medical therapy using isotretinoin. Both patients had recurrence of their ectopic ossification within 2 months of surgery. Surgery to remove joint-bridging ossifications in FOP is not recommended.

Adolescent↗

PubMed1977

Fibrodysplasia ossificants progressiva.

Two cases of fibrodysplasia ossificans progressiva are reported. Both patients were females and had suffered from the disease since birth. The characteristic anomalies of great toes and thumbs associated with multiple ectopic ossifications of the connective tissue are described. Fasciae, tendons, ligaments, and joint capsules of the proximal parts of the extremities and the dorsal aspect of the trunk were involved. The muscle atrophy was probably a secondary phenomenon.

Adult↗

PubMed2003

Ankylosis of the jaw in a patient with fibrodysplasia ossificans progressiva.

A case of fibrodysplasia ossificans progressiva (FOP) is presented. This uncommon connective tissue disease tends to produce progressing ectopic osteogenesis. Because there are no reported curative procedures for TMJ ankylosis occurring in this condition, a palliative surgical approach is described. Etiology, diagnosis, and prognosis of the disease is reviewed. Recent research in BMP cytokine-induced bone repair may allow new approaches to treating this debilitating disease in the future.

Adult↗

PubMed2005

[Fibrodysplasia ossificans progressiva in children. The interest of early diagnosis and treatment].

INTRODUCTION: A rare genetic disease (with around a hundred cases in France), fibrodysplasia ossificans progressiva is characterized by heterotopic ossification and congenital malformation of the bones. It is worsened by physical trauma, progresses in successive flares and slowly results in total confinement of the children because of the calcification of the muscles and ankylosis of all the joints. OBSERVATIONS: We report the case of two children exhibiting fibrodysplasia ossificans progressiva in whom diagnosis was delayed at the age of 4 and 18 months respectively. DISCUSSION: Fibrodysplasia ossificans progressiva must be diagnosed during the neonate period. Early treatment will help to avoid the factors of aggravation, slow the progression of the disease and provide the children with improved quality of life. Unfortunately, there is no efficient treatment, bisphosphonates and corticosteroids are only beneficial during the flares. Hope for the future relies on gene therapy.

Accidental Falls↗

PubMed1998

Encrypted morphogens of skeletogenesis: biological errors and pharmacologic potentials.

Bone morphogenetic proteins (BMPs) are members of a class of ancient, highly conserved signalling molecules that play major roles in embryonic axis determination, organ development, tissue repair, and regeneration throughout the animal kingdom. The bone morphogenetic proteins are potent developmental morphogens that act in a concentration-dependent manner to specify cell fates in developing and regenerating systems. Complementary DNAs have been cloned for approximately twenty BMPs, and recombinant proteins have been produced for many of these genes. Transgenic and naturally occurring animal models demonstrate a wide variety of potential functions for BMP genes during development and tissue regeneration, and a wide range of pharmacologic effects are predicted from knock-out or over-expression of the BMP genes. Fibrodysplasia ossificans progressiva (FOP), a rare and devastating genetic disease of ectopic osteogenesis in humans, is associated with over-expression of at least one of the BMPs. The BMPs, their transmembrane receptors, their intracellular signal transducers, and their secreted antagonists hold great promise as pharmacologic agents in modulating a vast array of developmental and regenerative pathways in human diseases.

Adult↗

PubMed1990

Fibrodysplasia ossificans progressiva.

A 14-yr-old boy with fibrodysplasia ossificans progressiva (FOP) presented for surgery for bilateral division of his ossified masseter muscles. Patients with FOP may present problems to the anaesthetist, including difficulties with tracheal intubation, restrictive pulmonary disease and abnormalities of cardiac conduction. With our patient sedated the trachea was intubated using a fibrescope and anaesthesia was induced and maintained with nitrous oxide and enflurane in oxygen. Ventilation was controlled throughout surgery and recovery was uneventful.

Adolescent↗

PubMed1977

Use and complications of high-dose disodium etidronate therapy in fibrodysplasia ossificans progressiva.

A boy with fibrodysplasia ossificans progressiva received nearly twice the usual therapeutic dose of disodium etidronate for 13 months in an attempt to prevent reossification following a second operation to correct severe torticollis. The operation was successful, but during therapy he developed weakness and distinctive bone lesions characterized by general osteopenia, widened physes, and unique radiolucent bands in the metaphyses. The osseous abnormalities were distinct from those of rickets and healed after withdrawal of the drug.

Child, Preschool↗

PubMed2004

Influenza-like viral illnesses and flare-ups of fibrodysplasia ossificans progressiva.

Flare-ups of fibrodysplasia ossificans progressiva are most commonly triggered by soft tissue trauma. After observing severe flare-ups of fibrodysplasia ossificans progressiva in two half-sisters with culture-confirmed influenza B infections, we hypothesized that influenza-like viral illnesses also can trigger fibrodysplasia ossificans progressiva flare-ups. To address this hypothesis, we designed a questionnaire to assess whether patients with fibrodysplasia ossificans progressiva experienced influenza symptoms during the 2000 to 2001 influenza season, and whether these symptoms were correlated with flare-ups of the condition. The questionnaire was sent to patients with fibrodysplasia ossificans progressiva worldwide. Of the 264 patients surveyed, 123 (47%) responded. The survey revealed that the risk of a disease flare-up of fibrodysplasia ossificans progressiva during an influenza-like viral illness was increased at least threefold and possibly much more. The survey data strongly supported the hypothesis that influenza-like viral illnesses are associated with disease flare-ups in patients who have fibrodysplasia ossificans progressiva. Influenza-like viral illnesses may be a source of previously unrecognized muscle injury leading to heterotopic ossification and permanent loss of mobility in these patients. These findings have important implications for understanding and preventing environmental triggers of disease activity in this population of patients genetically susceptible to progressive heterotopic ossification.

Adolescent↗

PubMed2005

The radiographic morphology of foot deformities in patients with fibrodysplasia ossificans progressiva.

BACKGROUND: The most consistent deformities that allow early diagnosis of fibrodysplasia ossificans progressiva are the presence of bilateral short first rays and hallux valgus. The purpose of this study was to describe the radiographic features observed in the feet of patients with fibrodysplasia ossificans progressiva. METHODS: The radiographs of 26 feet (15 patients with fibrodysplasia ossificans progressiva) were reviewed to evaluate the radiographic changes that occur in the first ray. Variables analyzed were the hallux valgus (HV) angle, the distal metatarsal articular (DMA) angle, the proximal phalangeal articular (PPA) angle, the intermetatarsal (IM) angle, ratio of the lengths of the first and second metatarsal lengths (MT1:MT2), and the first and second ray length ratio. The length ratios were then subtracted from similar ratios in radiographs of age- and gender-matched normal patients previously reported. RESULTS: The proximal phalanx was consistently shortened but morphologically dissimilar from subject to subject. Asymmetry was noted in some patients with bilateral radiographs. The mean HV angle was 28 degrees, and the mean IM angle was 10 degrees. The mean DMA angle was 33 degrees, and the mean PPA angle was 14 degrees. The MT1:MT2 ratio was 0.89, and the mean first ray to second ray length ratio was 0.87. The mean of the differences in the MT1:MT2 and first and second ray length ratios in patients with fibrodysplasia ossificans progressiva compared to the normal controls were 0.05 and 0.01, respectively. Fusion occurred between the abnormal tibial epiphysis of the proximal phalanx and metatarsal head with advancing age, and 68% of the metatarsal heads were fused with the abnormal proximal phalangeal epiphysis. CONCLUSIONS: Foot pathology in patients with fibrodysplasia ossificans progressiva is variable but consistently involves an abnormality of the tibial aspect of the proximal phalangeal epiphysis of the hallux. This results in the clinical observation of hallux valgus in these patients. The first metatarsal is consistently shortened, and fusion between the epiphysis of the abnormal proximal phalanx and the shortened first metatarsal head occurs with advancing age.

Child↗

PubMed1985

Fibrodysplasia ossificans progressiva: current concepts and the role of CT in acute changes.

Fibrodysplasia ossificans progressiva (FOP) may be treated with diphosphonate in an attempt to control the deposition of calcium phosphate. Amounts of calcium deposition may be assayed by plain radiographs and CT. In a sporadic case of FOP treated for 6 years with a diphosphonate (EHDP) we have analyzed the findings from CT. This modality presents a suitable means of monitoring response to new therapeutic agents, but CT also gives insights into the pathogenesis of the disorder.

Child↗

PubMed2000

Osteogenic induction in hereditary disorders of heterotopic ossification.

The formation of heterotopic bone within soft connective tissue is a common feature of at least three distinct genetic disorders of osteogenesis in humans: fibrodysplasia ossificans progressiva; progressive osseous heteroplasia; and Albright hereditary osteodystrophy. The pathobiologic characteristics of osteogenic induction, the histopathologic features of osteogenesis, the anatomic distribution of heterotopic lesions, and the developmental patterns of disease progression differ among all three conditions. The molecular and cellular basis of redirecting a mature connective tissue phenotype to form bone is a remarkable biological phenomenon with enormous implications for the control of bone regeneration, fracture healing, and disorders of osteogenesis.

Diagnosis, Differential↗

PubMedDate not supplied

Fibrodysplasia ossificans progressiva: report of two cases.

Fibrodysplasia Ossificans Progressiva (FOP) is a rare hereditary connective tissue disease, genetically inherited as an autosomal dominant trait with complete penetrance but variable expressivity. Onset is typically in childhood and progressive involvement of the spine and proximal extremities leads to immobility and articular dysfunction. We present two cases with the typical features of FOP and a review of the pathogenesis, clinical manifestations and treatment options of this rare disease.

Adolescent↗

PubMed2001

A severely disabling disorder: fibrodysplasia ossificans progressiva.

Fibrodysplasia ossificans progressiva (FOP) is an extremely rare hereditary disorder characterised by progressive heterotopic ossification of the soft tissues. The resulting progressive immobilisation of the limbs, jaw and chest wall generally leads to severe disability. We present an 18-year-old girl with advanced FOP. She had three operative interventions to excise her ectopic bones but all resulted in failure. Treatment strategies for this disorder should include the avoidance of exacerbating factors.

Adolescent↗

PubMed1978

Elevated prostaglandin production in cultured cells from a patient with fibrodysplasia ossificans progressiva.

Prostaglandin E production was measured in cells cultured from both a fibromatoid lesion and a normal area of skin in a patient with fibrodysplasia ossificans progressiva. The synthesis of prostaglandin E-like material (iPGE) was ten- to twenty-fold greater in cells cultured from the patient's fibromatoid lesion than in fibroblasts obtained from her normal skin. Addition of indomethacin in vitro resulted in a greater than 95% reduction of iPGE production in both cell cultures. These observations appear to warrant further investigation in additional patients, with this disease.

Cells, Cultured↗

PubMed2003

In vivo somatic cell gene transfer of an engineered Noggin mutein prevents BMP4-induced heterotopic ossification.

BACKGROUND: The formation of the skeleton requires inductive signals that are balanced with their antagonists in a highly regulated negative feedback system. Inappropriate or excessive expression of BMPs (bone morphogenetic proteins) or their antagonists results in genetic disorders affecting the skeleton, such as fibrodysplasia ossificans progressiva. BMP signaling mediated through binding to its receptors is a critical step in the induction of abnormal ossification. Therefore, we hypothesized that engineering more effective inhibitors of this BMP-signaling process may lead to the development of therapies for such conditions. METHODS: BMP4-induced heterotopic ossification was used as a model for testing the ability of the BMP antagonist Noggin to block de novo bone formation, either by local or systemic delivery. Since Noggin naturally acts locally, a Noggin mutein, hNOGDeltaB2, was engineered and was shown to circulate systemically, and its ability to block heterotopic ossification was tested in a mouse model with use of adenovirus-mediated somatic cell gene transfer. RESULTS: A mouse model of BMP4-induced heterotopic ossification was developed. Local delivery of wild-type NOG inhibited heterotopic ossification, but systemic administration was ineffective. In contrast, systemic delivery of the adenovirus encoding hNOGDeltaB2 resulted in systemic levels that persisted for more than two weeks and were sufficient to block BMP4-induced heterotopic ossification. CONCLUSIONS: BMP4-induced heterotopic ossification can be prevented in vivo either by local delivery of wild-type Noggin or after somatic cell gene transfer of a Noggin mutein, hNOGDeltaB2. Furthermore, the data in the present study provide proof of concept that a naturally occurring factor can be engineered for systemic delivery toward a desirable pharmacological outcome. CLINICAL RELEVANCE: Blocking bone formation is clinically relevant to disorders of heterotopic ossification in humans, such as fibrodysplasia ossificans progressiva. Furthermore, development of BMP antagonists as therapeutic agents may provide modalities for the treatment of other pathologic conditions that arise from aberrant expression of BMPs and/or from a lack of their antagonists.

Adenoviridae↗

PubMed2003

Anesthetic management in fibrodysplasia ossificans progressiva (FOP): a case report.

Fibrodysplasia ossificans progressiva (FOP) is a rare congenital disorder of connective tissue, which has significant implications on anesthetic management of affected patients, e.g., application of difficult airway algorithm, avoiding any trauma during venous cannulation. FOP has received very limited reviews in the published anesthesia literature. We describe perioperative care of a 21-year-old female with established diagnosis of FOP presenting for hysteroscopy followed by dilatation and curettage. Multiorgan abnormalities of FOP are reviewed and available anesthetic options are analyzed and compared.

Adult↗