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Results for “MYOSITIS OSSIFICANS”

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At least 1,117 records · Page 62Linked to original sources

PubMed1995

Permanent heterotopic ossification at the injection site after diphtheria-tetanus-pertussis immunizations in children who have fibrodysplasia ossificans progressiva.

In patients who have fibrodysplasia ossificans progressiva, routine childhood diphtheria-tetanus-pertussis immunizations administered by intramuscular injection caused a significant risk of permanent heterotopic ossification at the site of injection (p < 10(-8)), whereas measles-mumps-rubella immunizations administered by subcutaneous injection posed no significant risk. Intramuscular injections should be avoided, if possible, once a diagnosis of fibrodysplasia ossificans progressiva has been established.

Adolescent↗

PubMed1995

Advanced fibrodysplasia ossificans progressiva.

Fibrodysplasia ossificans progressiva is a rare hereditary variant of heterotropic bone formation that is often difficult to diagnose. A bone scan of an advanced case of this condition is presented. Nuclear medicine physicians should be familiar with various aspects of this disease.

Adult↗

PubMed2006

Fibrodysplasia ossificans progressiva. An unusual cause of restricted mandibular movement.

A 9-year-old girl is presented who was initially misdiagnosed and finally diagnosed with fibrodysplasia ossificans progressiva only after presentation with progressive limitation of her mouth opening. The clinical, histopathological, and molecular biological aspects of this uncommon disorder will be discussed. Furthermore, dental and surgical guidelines will be described.

Child↗

PubMed1982

Fibrodysplasia ossificans progressiva.

Three hundred fifty-three previously reported cases of fibrodysplasia ossificans progressiva were reviewed and six new cases were analyzed radiologically. Characteristic findings of fibrodysplasia ossificans progressiva were confirmed and radiographic signs of the disease were demonstrated, including medial cortical thickening of the proximal tibia, narrow lumbar spinal canal, accessory epiphysis of the second phalanx of the fifth finger, and decreased humeral/epicondylar angle.

Bone and Bones↗

PubMedDate not supplied

Clinical presentation of fibrodysplasia ossificans progressiva: pitfalls in diagnosis.

A patient presented with a rapidly growing mass in the cervicothoracic paraspinous region. Associated clinical and radiographic skeletal abnormalities and histopathologic findings showing mild to moderate proliferation of spindle cells in a myxoid stroma led to the diagnosis of fibrodysplasia ossificans progressiva. Recognizing the clinical features of this disease is important and should avoid the need for obtaining tissue to make the diagnosis, because performing a biopsy may be particularly morbid in these patients.

Biopsy↗

PubMed1989

[Fibrodysplasia ossificans progressiva or Münchmeyer's disease. Personal observation].

We report here a case of fibrodysplasia ossificans progressiva in a 14-year-old boy, affected from birth by microdactyly of the big toes. This skeletal abnormality also existed in his paternal great-grandfather. When he was 7, some ectopic ossifications occurred and inexorably progressed despite all therapies. Fibrodysplasia ossificans progressiva is a serious and rare disease with a terrible development, leading to a sort of petrifaction of the patient. The lack of knowledge on this ectopic ossification process explains the want of suitable treatments to stop the course of this disease.

Adolescent↗

PubMed1998

Urinary basic fibroblast growth factor. A biochemical marker for preosseous fibroproliferative lesions in patients with fibrodysplasia ossificans progressiva.

Angiogenesis is a prominent histopathologic feature of preosseous fibroproliferative lesions in patients who have fibrodysplasia ossificans progressiva. Basic fibroblast growth factor is an extremely potent in vivo stimulator of angiogenesis, and has been implicated in the growth of solid tumors. An enzyme linked immunosorbent assay for basic fibroblast growth factor was performed on urine samples from patients who had active (n = 28) and inactive (n = 39) fibrodysplasia ossificans progressiva, and compared with urine samples from normal age and gender matched control subjects (n = 54). Median basic fibroblast growth factor levels were 2705 pg/g of creatinine in the normal control group, 5058 pg/g of creatinine in patients with inactive fibrodysplasia ossificans progressiva (no significant difference), and 8793 pg/g of creatinine in patients with active fibrodysplasia ossificans progressiva. Female subjects, both normal and with fibrodysplasia ossificans progressiva, had higher levels of urinary basic fibroblast growth factor than did male subjects. There was no correlation of urinary basic fibroblast growth factor levels with age or severity of preexisting disability. These data document an elevation of urinary basic fibroblast growth factor during acute flareups of fibrodysplasia ossificans progressiva and provide a biochemical basis for considering antiangiogenic therapy for inhibiting endochondral osteogenesis in this disorder.

Adolescent↗

PubMed2003

Fibrodysplasia ossificans progressiva (FOP).

Fibrodysplasia ossificans progressiva (FOP) is a rare deforming disease, affecting the skeleton and is associated with progressive endochondral ossification of the striated muscles. Swelling of the soft parts is rare and can be the initial manifestation. We report three patients where initial swelling preceded the ossification.

Child↗

PubMed1976

Alkaline phosphatase activity in cultured skin fibroblasts from fibrodysplasia ossificans progressiva.

Alkaline phosphatase activity in four strains of cultured skin fibroblasts obtained from a patient with fibrodysplasia ossificans progressiva was at the low normal range. The enzyme activity in normal fibroblasts significantly increased at late confluency. It appears that the high levels of alkaline phosphatase activity reported in biopsies of lesions are not genetically determined but are secondary events of local tissue reaction.

Adolescent↗

PubMed1986

Fibrodysplasia ossificans progressiva presenting as osteomyelitis-like syndrome.

An 8-year-old boy presenting with fever, neck pain, and torticollis was eventually diagnosed as having fibrodysplasia ossificans progressiva (FOP). The initial bone scan was interpreted as being suggestive of vertebral osteomyelitis. Subsequent computerized tomographic studies (CT scan), however, demonstrated paravertebral soft-tissue calcification adjacent to, but not involving, intact cervical vertebrae. The acute process of FOP was thereby distinguished from that of osteomyelitis by use of CT scan.

Cervical Vertebrae↗

PubMed1998

Embryonic overexpression of the c-Fos protooncogene. A murine stem cell chimera applicable to the study of fibrodysplasia ossificans progressiva in humans.

Murine embryonic overexpression of the c-fos protooncogene leads to early postnatal heterotopic chondrogenesis and osteogenesis with phenotypic features similar to those seen in children who have the disabling heritable disease fibrodysplasia ossificans progressiva. The overexpression of Fos in embryonic stem cell chimeras leads to heterotopic endochondral osteogenesis at least in part through a bone morphogenetic protein 4 mediated signal transduction pathway. In contrast, early fibrodysplasia ossificans progressiva lesions express abundant bone morphogenetic protein 4, without abundant expression of c-Fos, suggesting that the primary molecular defect in fibrodysplasia ossificans progressiva may be independent of the sustained Fos effects on chondrogenesis and osteogenesis. Comparisons of the clinical, molecular, and pathogenetic features of the c-Fos embryonic stem cell chimeras with those of fibrodysplasia ossificans progressiva provide insight into the earliest events in the molecular pathogenesis of genetically induced heterotopic chondrogenesis and osteogenesis. The relevance of the c-Fos embryonic stem cell chimera to the study of the currently untreatable human disease fibrodysplasia ossificans progressiva demonstrates the power of using embryonic stem cell technology for generating gain of function mutations in the study of human bone disease.

Animals↗

PubMed1988

Dense metaphyseal bands and growth arrest associated with isotretinoin therapy.

A 9-year-old boy, treated with high-dose isotretinoin therapy for fibrodysplasia ossificans progressiva, developed dense metaphyseal bands and growth arrest. Discontinuance of isotretinoin therapy was followed by gradual decrease of metaphyseal bands and resumption of clinical growth. The dense metaphyseal bands may be related to the known action of retinoids as modulators of chondrocyte phenotype and gene expression.

Adolescent↗

PubMedDate not supplied

Using the Internet to enable access to medical conferences.

OBJECTIVE: The goal of expanding access to individuals with disabilities to scientific and medical conferences is supported by both the Americans with Disabilities Act and the National Institutes of Health. DESIGN: Live-streaming video broadcast over the internet is widely available, although it has been used only in a limited fashion by the medical community. A consumer-oriented medical and rehabilitation conference concerning the rare disabling disease, fibrodysplasia ossificans progressiva, was broadcast via the world wide web. The address of a web page was announced before the conference to three computer users' groups that were considered likely to have an interest in the conference. The web page presented a live-streaming video broadcast of the conference. A phone line was installed, thereby allowing viewers to ask questions of the presenters during the question and answer periods. RESULTS: Sixteen users logged in 83 times to view the conference over a 2-day period. Five (23%) of 22 members of a fibrodysplasia ossificans progressiva internet users' group tuned in from distant places as The Netherlands and Israel. CONCLUSIONS: The internet is a viable tool to expand access to and increase the participation of individuals with disabilities in scientific and medical conferences. This technology should be used routinely in conferences of interest.

Congresses as Topic↗

PubMed1979

Urinary proline to hydroxyproline ratio varies with age.

Urinary excretion of proline and hydroxyproline was studied in five groups of subjects, viz. controls, pituitary dwarfs, familial dwarfs, patients with fibrodysplasia ossificans progressive and patients with generalized scleroderma. The ratio Pro/Hyp in the urinary fraction precipitated with 5 parts of acetone (1 + 5 fraction) was close to one in children and youngsters in all the groups, while in adults it was higher than two. Total Pro/Hyp does not give an accurate index of age.

Adolescent↗

PubMed1998

Catastrophic falls in patients who have fibrodysplasia ossificans progressiva.

There have been numerous anecdotal reports of catastrophic falls in patients with fibrodysplasia ossificans progressiva. To determine the incidence of serious morbidity and mortality associated with falls in this patient population, the authors surveyed the 135 patient members of the International Fibrodysplasia Ossificans Progressiva Association and an age and gender matched control group. Eighty-one percent of the fibrodysplasia ossificans progressiva population suffered a fall resulting in injury compared with 44% of the controls. Sixty-seven percent of the falls initiated a painful flareup of fibrodysplasia ossificans progressiva leading to permanent loss of movement in almost all patients. Fifty-four percent of all falls suffered by the fibrodysplasia ossificans progressiva group led to permanent disability compared with 4% of all falls in the control group. Although trauma to the head was a common site of injury in both groups, the injury profile in the fibrodysplasia ossificans progressiva group included traumatic brain injuries, intracranial hemorrhage and death whereas the control group suffered mostly minor soft tissue lacerations. Deficiencies in coordinate gait and protective function likely accounted for the severity of injuries especially to the head in the fibrodysplasia ossificans progressiva population. Precautions are recommended that are intended to minimize the risk of injury without compromising a patient's functional level and independence. These recommendations include limitation of high risk activities, protective head gear, safety improvements in living environments, and augmentation of stabilizing and protective functions.

Accidental Falls↗

PubMed1982

Fibrodysplasia ossificans progressiva in South Africa. Case reports.

Six patients with fibrodysplasia ossificans progressiva, a condition not previously reported in Blacks in this country, have been reviewed. All had serious physical disability and medical or surgical therapy did not influence the course of the disease. By extrapolation from prevalence data established in Britain, it can be estimated that there are at least 10 additional affected individuals in southern Africa.

Adolescent↗

PubMed1993

The histopathology of fibrodysplasia ossificans progressiva. An endochondral process.

In order to better characterize the biological features of fibrodysplasia ossificans progressiva, we reviewed the histopathological specimens from eleven patients (twelve biopsies) who had a confirmed diagnosis of the disease. All of the biopsies had been performed in children, to exclude the diagnosis of a malignant lesion. In no instance was the diagnosis of fibrodysplasia ossificans progressiva considered before the biopsy. The results of a lesional biopsy in all eleven patients revealed normal endochondral osteogenesis at heterotopic sites. The results of biopsy of an early lesion in six children were misinterpreted as revealing a diagnosis of fibromatosis or sarcoma before the roentgenographic appearance of ossification. Immunohistochemical studies of sections of the earliest lesion demonstrated S-100 antigen positivity before the histological appearance of differentiated osteochondral tissue. The presence of congenital malformation of the great toes and of postnatal heterotopic endochondral osteogenesis strongly suggests that fibrodysplasia ossificans progressiva is a disorder of defective induction of endochondral osteogenesis. This study established the predominant histopathological findings associated with fibrodysplasia ossificans progressiva and can serve as a basis for postulation of a candidate gene in the pathogenesis of the disorder. A lesional biopsy is not needed to make the diagnosis; biopsy uniformly exacerbates the condition and should be avoided.

Antigens, CD↗

PubMedDate not supplied

MR imaging of early fibrodysplasia ossificans progressiva.

Fibrodysplasia ossificans progressiva is a rare disorder characterized by progressive heterotopic bone formation in connective tissue and muscle in association with congenital skeletal anomalies. The disease usually leads to extensive immobility and eventual disability. We report the magnetic resonance findings in a boy prior to ossification of his soft tissue masses.

Child, Preschool↗