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A sciatic nerve lesion secondary to compression by a heterotopic ossification in the hip and thigh region--an electrodiagnostic approach.

A sciatic nerve lesion secondary to compression by a heterotopic ossification is rare. Operative release of the encased sciatic nerve in some cases may restore the function of the nerve partially or completely. However, in some cases the injury may be permanent. An electrophysiologic study is very useful to determine the location and severity of nerve damage, including axonal loss, demyelination, or both. An electrophysiologic study can emphasize the portion of the sciatic nerve that has been involved the most (lateral versus medial or peroneal versus tibial). In some cases an electrophysiologic study can suggest whether surgery should be postponed if a recovery pattern from the nerve injury is obvious. The prognostic value of follow-up studies is considerable. The authors reviewed literature available to them since 1971 and found 6 cases, including their own. This is the first attempt to put together all the information available in the literature about this condition.

Accidents, Traffic↗

Heterotopic ossification after rhizotomy and femoral osteotomy.

In 15 patients with cerebral palsy who underwent selective posterior rhizotomy (SPR) and subsequent proximal femoral varus derotation osteotomy (VDO), heterotopic ossification (HO) around the hip after VDO was noted in four of eight patients with spastic quadriplegia (seven of 26 hips, 27%). HO was not noted in the seven patients with spastic diplegia. A radiographic review of 118 hips with a femoral VDO in the 69 patients with cerebral palsy who did not undergo SPR during the same period showed no HO. Frequent evaluation of postrhizotomy patients with cerebral palsy quadriplegia after a femoral VDO is necessary to recognize HO as a possible etiology for late-onset pain and limited hip range of motion (ROM).

Adolescent↗

Heterotopic ossification related to toxic epidermal necrolysis in a patient with Behçet's disease.

We describe a 44-year-old woman with an 18-year-history of Behçet's disease (BD) in whom heterotopic ossification (HO) developed as a complication of toxic epidermal necrolysis (TEN). The patient presented with high fever, a progressive erythematous rash including target-like lesions, flaccid blister formations, and severe detachment. The patient was diagnosed with TEN, and methylprednisolone therapy was started. In the 2nd month of her hospital course, painful limitation developed in both her elbow and shoulder joints. Laboratory and radiographic findings confirmed HO of these areas. BD associated with TEN and HO is a previously unreported entity. In such a condition, determination of underlying genetic abnormality is important. Additionally, HO should be considered as a potential cause of the symptoms related to the joints.

Adult↗

A slowly progressive degenerative condition characterized by mental deficiency, wasting of limb musculature and bone abnormalities, including ossification of the pinnae.

A condition is described in a 33-year-old Caucasian male in which there is severe mental deficiency present from an early age. There is non-progressive hydrocephaly and the eye findings include posterior polar cataracts. There is slowly progressive muscle wasting of the limbs along with degenerative bone changes. Ossification has occurred in the pinnae. Secondary sexual characteristics are underdeveloped and there is a low normal serum testosterone. The parents are not related and there is no similar occurrence in the family. Biochemical investigations do not show any abnormality, and the chromosome picture is that of a normal male karyotype. The case appears to be unique.

Adult↗

Ameloblastoma with prominent stromal ossification.

A large ameloblastoma with prominent stromal ossification is reported. The patient was a 39-year-old Japanese male with diffuse swelling of the mandible from the left molar to the right premolar region. Radiographic examination showed a radiopaque lesion suggestive of a fibro-osseous lesion. However, histologic examination revealed the lesion to be a variant of follicular ameloblastoma. Most of the tumor cells appeared to be squamous metaplasia. The stroma was abundant, with prominent formation of bone trabeculae rimmed by osteoblasts. Differentiation of mesenchymal cells into active osteoblasts with formation of new bone trabeculae was frequently found in the stroma. The prominent stromal bone formation appeared to be reactive in nature, although it remains uncertain whether this type of ameloblastoma can be regarded as a discrete clinicopathologic entity.

Adult↗

Neuropathic bladder dysfunction in patients with ossification of the posterior longitudinal ligament.

BACKGROUND: Ossification of the posterior longitudinal ligament (OPLL) may cause neuropathic bladder dysfunction due to spinal cord involvement. OPLL, unlike a traumatic spinal cord lesion, progresses insidiously and sometimes affects longer cord segments. As the manifestation of bladder dysfunction may depend on the development of OPLL, we studied the relationship between bladder function and roentgenographic changes in the spinal canals of OPLL patients. PATIENTS AND METHODS: Eighteen surgical candidates (11 males and 7 females, 34 to 85 years old) were studied urodynamically. Sixteen underwent CO2-filling cystometry, uroflowmetry and measurement of their residual urine volume. Cystometry was omitted in the remaining 2 patients. The vertical extent of OPLL and the degree of stenosis in the spinal canal was estimated by x-ray films and CT. RESULTS: The cystometric study revealed detrusor hyperreflexia in 2 patients and areflexic or underactive detrusors in 5 patients. Intermittent flows or considerable amounts of residual urine were also observed in the arefilexia/underactive group. Uroflowmetry showed a normal flow with little residual urine in both patients in whom cystometry was omitted. Bladder sensation was maintained in all patients. The occurrence of abnormal detrusor activity had no relationship to the degree of canal stenosis, while the occurrence of an areflexic or underactive detrusor correlated with the vertical extent of OPLL. CONCLUSION: Although detrusor hyperreflexia is common in an upper spinal cord lesion, attention should also be paid to the development of detrusor underactivity in patients with a wide vertical extent of OPLL.

Adult↗

Patellar tendinitis--a case report of elongation and ossification of the inferior pole of the patella.

Patellar tendinitis is a condition that occurs in patients who are involved in repetitive use of the extension mechanism. The most common symptoms are pain at the inferior pole of the patella, and radiographic changes may also occur. This case report demonstrates an extreme example of elongation of the inferior pole of the patellar tendon. The ossification caused the patient pain, which was resolved with surgical removal of the bone.

Adult↗

Retardation of endochondral ossification at the distal ulnar growth plate in dogs.

Bilateral forelimb deformities associated with retained cartilage cores in the distal ulna were diagnosed in 8 immature giant breed dogs. Five of the dogs were autopsied. Histopathological examination of the distal ulnar growth plate revealed a localised central thickening due to the accumulation of hypertrophic chondrocytes which had failed to undergo the usual development to degeneration. Also the intercellular matrix septa of this cartilage were not calcified. Projections of the abnormal cartilage even persisted into the primary spongiosa. These abnormalities in the process of endochondral ossification of the growth plate produced the lesion of retained cartilage core which was responsible for the development of forelimb deformity.

Animals↗

Role of prostaglandin I2 in the gene expression induced by mechanical stress in spinal ligament cells derived from patients with ossification of the posterior longitudinal ligament.

Ossification of the posterior longitudinal ligament of the spine (OPLL) is characterized by ectopic bone formation in the spinal ligaments, and mechanical stress has been suggested to play an important role in the progression of OPLL. To identify the genes that participate in OPLL, the differential display reverse transcription-polymerase chain reaction (RT-PCR) method was used. A 283-base pair cDNA fragment corresponding to prostaglandin I2 (PGI2) synthase was highly expressed in OPLL cells compared with non-OPLL cells. To examine the effect of mechanical stress on the expression of PGI2 synthase, cells were subjected to uniaxial cyclic stretch (0.5 Hz, 20% stretch), and PGI2 synthase mRNA expression was assessed by quantitative RT-PCR. Cyclic stretch induced an increase in PGI2 synthase in OPLL cells in a time-dependent manner, whereas no change was observed in non-OPLL cells. Cyclic stretch for 9 h also induced a 2.86x increase in PGI2 production. Beraprost (a stable PGI2 analog) and dibutyryl cAMP (a membrane-permeable cAMP analog) increased the mRNA expression of alkaline phosphatase (ALP) as a marker for osteogenic differentiation up to 240 and 200%, respectively, in OPLL cells, whereas no change was observed in non-OPLL cells. The increases in ALP mRNA induced by beraprost and cyclic stretch were both inhibited by SQ22536, a potent adenylate cyclase inhibitor. These data suggest that the increase in PGI2 synthase induced by mechanical stress plays a key role in the progression of OPLL, at least in part through the induction of osteogenic differentiation in spinal ligament cells via the PGI2/cAMP system.

Adenylyl Cyclase Inhibitors↗

Homeobox protein MSX2 acts as a molecular defense mechanism for preventing ossification in ligament fibroblasts.

Ligaments and tendons are comprised of tough yet flexible connective tissue. Little is known, however, about the precise characteristics of the cells in ligaments and tendons due to the absence of specific markers and cell lines. We recently reported a periodontal ligament cell line, PDL-L2, with suppressed Runx2/Osf2 transcriptional activity and an inability to form mineralized nodules. The present study demonstrates that the homeobox protein Msx2 is a key factor in suppressing those two functions. Msx2 colocalizes with Runx2/Osf2 and suppresses its activity cooperatively, acting with another corepressor, TLE1, as a complex to recruit histone deacetylase 1 activity. Reverse transcription-PCR and in situ hybridization demonstrated that Msx2 expression is higher in periodontal ligament and tendon cells than in osteoblasts. Stable reduction of Msx2 expression in PDL-L2 cells induces osteoblastic differentiation, thereby causing matrix mineralization. Conversely, stable, forced Msx2 expression in MC3T3-E1 cells prevented osteoblast differentiation and matrix mineralization. Msx2-induced suppression of osteoblast differentiation was repressed by bone morphogenetic protein 2. In addition, Msx2 was downregulated in a symptom- and calcification-dependent manner at the affected region in patients with ossification of the posterior longitudinal ligament. Our findings indicate that Msx2 plays a central role in preventing ligaments and tendons from mineralizing.

Aged↗

Cervical myelopathy, ossification of the posterior longitudinal ligament, and diffuse idiopathic skeletal hyperostosis: problems in investigation.

This report describes a patient presenting with a spastic quadriplegia who was found to have both diffuse idiopathic skeletal hyperostosis (DISH) and ossification of the posterior longitudinal ligament (OPLL) in the cervical spine. There was a dramatic worsening of his symptoms during a myelogram examination of the neck. It is suggested that computed tomographic imaging of the neck is the preferred investigative procedure if OPLL is suspected as a cause of cervical myelopathy.

Cervical Vertebrae↗

Ossification of the posterior longitudinal ligament in three geographically and genetically different populations of ankylosing spondylitis and other spondyloarthropathies.

STUDY DESIGN: Cross sectional. RESEARCH QUESTIONS: (a) Is any clinical variable of ankylosing spondylitis (AS) associated with the presence of ossification of the posterior longitudinal ligament (OPLL)? and (b) Is OPLL present in patients with AS from different geographical or genetic backgrounds? METHODS: Three groups were assembled: (1) a prospective group of 103 consecutive AS patients from two community based rheumatology clinics from Guadalajara, who were evaluated using: a questionnaire with disease characteristics variables; clinical assessment by a neurologist; lateral radiographic views of the cervical spine and somatosensory evoked potentials (SSEP). (2) Fifty one spondyloarthropathies (SpA) patients from Mexico city whose cervical spine films were retrospectively reviewed. (3) Thirty nine AS patients from Edmonton, Canada whose cervical spine films were retrospectively reviewed and compared with 72 controls. RESULTS: Group 1: 74% of the 103 patients were men and 86% were HLA-B27 positive. The mean age was 35 years, and mean (SD) disease duration 10 (8) years. OPLL was reported in 16 patients (15.5%; 95% CI 9, 22). OPLL was statistically associated with older age (p = 0.001), longer disease duration (p = 0.001), clinical myelopathy (p = 0.03), worst functional index (p = 0.042), restricted axial movement measurements (all p < 0.001), radiological sacroilitis (p < 0.001 for linear association), osteitis pubis (p = 0.009), hip involvement (p = 0.006 for linear association), and abnormal SSEP (p = 0.008). Group 2: 92% of 51 patients were men; the mean age was 30 years and the mean (SD) disease duration 11 (7) years. OPLL was reported in 15 (29%, 95% CI 17, 41) patients (nine AS, two psoriatic arthritis, three juvenile AS, and one Reiter's syndrome). Group 3: 95% of the 39 patients were men; the mean of age was 46 years and disease duration of 18 (10) years. OPLL was reported in nine (23%; 95% CI 10, 36) patients, including one with psoriatic arthritis, and two with Crohn's disease. OPLL was observed in two of the control group. CONCLUSIONS: The prevalence of OPLL in AS and SpA is higher than previously recognised and seems to be associated with variables identifying more severe axial disease.

Adult↗

Calcification and ossification within the orbit.

The radiological diagnosis of orbital lesions can be aided by an understanding of the significance of the various kinds of calcium deposits. Orbital calcification may be metastatic or dystrophic, with ossification occurring in degenerated eye tissue in the presence of an abundant blood supply. It is the dystrophic type which is usually visualized radiographically. Intraorbital calcification had been categorized according to its configuration and location (a) ocular; (b) extraocular; and (c) extraorbital extending into the orbit on frontal radiographs. Lesions discussed include cataract, phthisis bulbi, vascular abnormalities, infection, and tumor.

Bone Diseases↗

Heterotopic ossification in paraplegia: a possible disturbance of the paravertebral venous plexus.

The distribution of the heterotopic ossifications that complicate paraplegia and other neurological disorders is remarkably similar to that of the paravertebral venous plexus of Batson. Stasis within this system consequent to immobilization and spinal trauma, combined with demineralization of the skeleton, are postulated to be the main factors promoting precipitation of calcium salts in the soft tissues in these conditions.

Humans↗

Idiopathic pulmonary ossification.

Idiopathic pulmonary ossification is an uncommon and asymptomatic disorder of unknown etiology in which trabeculated bone is found in the lung. It is usually mistaken for more serious entities radiographically, most commonly appearing as branching linear shadows of calcific density involving a limited area of the lung and exhibiting very slow progression; however, the shadows may be round or irregular and bulky. Sometimes the trabeculae are recognizable, and occasionally the lungs demonstrate widespread involvement. The authors describe 8 proven cases, including one in which a bone scan revealed uptake by heterotopic bone in the lung.

Diagnosis, Differential↗

Prophylactic radiation therapy for prevention of heterotopic ossification after hip arthroplasty: results in 141 high-risk hips.

In a 4 1/2-year, prospectively randomized study, 137 patients with 141 hips at high risk for heterotopic ossification (HO) received prophylactic radiation therapy (RT). Patients were randomly assigned to a low-dose regimen of five fractions of 2 Gy each (n = 73) or a high-dose regimen of either 10 fractions of 2 Gy each (n = 7) or five fractions of 3.5 Gy each (n = 61). Treatment failure was assessed by comparing immediately postoperative radiographs with radiographs obtained at least 6 months after hip surgery (Brooker grading score). Positive responses (ie, effective prophylaxis of HO) were seen in 129 (91.5%) hips. Treatment failures were observed in 12 (8.5%). Use of a nonsteroidal antiinflammatory drug (NSAID) lowered the failure rate in both RT groups. High RT dose with a short duration (< or = 9 days) and use of an NSAID was significantly (P = .009) correlated with treatment success. RT delivered within a few days after hip surgery is effective in preventing HO, even in high-risk patients, and provides an excellent alternative for patients with contraindications to long-term medication with either NSAIDs or corticosteroids.

Adult↗

Postoperative hip irradiation in prevention of heterotopic ossification: causes of treatment failure.

Over 4 years, 33 patients (mean age, 52 years) underwent hip surgery followed by single-fraction 700-cGy radiation therapy (RT). Records, port films, and plain radiographs were reviewed to assign Brooker classification levels for severity of heterotopic ossification (HO) and assess the amount of new HO developing after surgery. Nineteen patients (58%) developed radiographic evidence of new HO after surgery. All five patients with three or more risk factors and none of 12 with postoperative Brooker level 0 (no radiographic HO) developed new HO. Ten of 16 treated on postoperative day 1, six of 10 on postoperative day 2, none of three on day 3, two of three on day 4, and one of one on day 5 developed new HO. New HO developed outside the irradiated volume in 11 patients. Surgeons should remove all heterotopic bone whenever possible; RT should be administered within 3 days after surgery; portals should cover all potentially involved soft tissue; and future studies should evaluate larger single fractions (900-1,000 cGy) for prevention of HO in patients with three or more risk factors and/or radiographic evidence of residual HO.

Adult↗