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SOST/sclerostin, an osteocyte-derived negative regulator of bone formation.

Sclerosteosis and Van Buchem disease are two closely related bone disorders characterized by progressive bone thickening due to increased bone formation. Sclerosteosis is associated with mutations in the SOST gene and Van Buchem disease with a 52 kb deletion downstream of the SOST gene that probably affects transcription of the gene. Expression of the gene product sclerostin in bone is restricted to osteocytes and it is a negative regulator of bone formation. It inhibits BMP-stimulated bone formation, but cannot antagonize all BMP responses. The exclusive bone phenotype of good quality of patients with sclerosteosis and Van Buchem disease and the specific localization of sclerostin make it an attractive target for the development of bone forming therapeutics.

Adaptor Proteins, Signal Transducing↗

Complications associated with segmentation of the maxilla: a retrospective radiographic follow up of 82 patients.

The aim of the present study was to retrospectively examine the clinical and radiographic incidence and frequency of injuries to teeth and their surrounding tissues adjacent to interdental osteotomies in conjunction with segmentation of the maxilla. Vertical interdental osteotomies have been performed in combination with Le Fort I osteotomy for correction of various dento-facial deformities. All our orthognathic patients are followed prospectively in a standardized manner. Eighty-two consecutive patients who underwent dento-facial correction by segmented maxillary osteotomy alone or in combination with simultaneous mandibular surgery between 1992 and 1998 were included in the study. They were followed for up to 30 months postoperatively. A total of 158 interdental osteotomies were performed, involving a total of 316 teeth. Only a small number of complications such as osteolytic processes, marginal bone destruction, root resorption or mechanical injuries to the teeth were seen.

Adult↗

Chronic sclerosing osteomyelitis of a metacarpal.

Chronic sclerosing osteomyelitis in a metacarpal is presented. Bacterial cultures were negative, and radiographs and an isotope scan were non-specific. Histopathological assessment confirmed the diagnosis. Iliac bone grafting resulted in symptomatic relief to the patient.

Adult↗

Subchondral bone changes in hand and knee osteoarthritis detected by radiography.

OBJECTIVE: To describe the changes in subchondral bone that occur with the onset and progression of osteoarthritis (OA) from macroradiographic assessment of patient's hand and knee joints. DESIGN: The high magnification and spatial resolution of macroradiography permits detailed anatomical changes to be detected in OA joints. Data on the subchondral cortical and cancellous bone, recorded from both cross-sectional and longitudinal studies of hand and knee OA, are described and discussed with reference to recent findings on the altered biomechanical properties of OA bone. RESULTS: In OA joints, both subchondral cortical plate and subjacent horizontal trabeculae increase in thickness early, prior to joint space narrowing (JSN). With progression, cortical plate sclerosis increased in 60% of OA hands and did not change in knee OA until JSN <1.5mm in the medial diseased compartment. In knee OA, trabeculae, at sites of tibial subchondral sclerosis, increased in number and extent, changes that overlay a subarticular region that was osteoporotic. With cartilage loss, the articular surfaces in some knees appeared corrugated, and later, with bone-on-bone, the surfaces became flattened and deformed. CONCLUSIONS: The weaker than normal bone within thickened subchondral cortical plate and trabeculae of OA joints leads, in advanced OA, to deformation of the articular surfaces and absorption of local stresses producing an effect similar to stress-shielding. This effect, it is suggested, results in the subarticular osteoporosis.

Bone and Bones↗

The ratio of medial to lateral tibial plateau bone mineral density and compartment-specific tibiofemoral osteoarthritis.

INTRODUCTION: Bone is thought to play an important role in osteoarthritis (OA) pathophysiology. Our aim was to look at specific features of OA and their relation to the ratio of medial:lateral tibial plateau bone mineral density (M:L BMD Ratio). METHODS: We examined our research question in the Framingham OA Study Cohort. All participants had BMDs and weight-bearing plain radiographs of the knees (2002-2005). M:L BMD Ratios were calculated using BMD from medial and lateral regions in the tibial plateau. Knee x-rays were read for osteophytes (OSTs), joint space narrowing (JSN), and sclerosis (Osteoarthritis Research Society International (OARSI) scoring system). Knees were classified as having medial and/or lateral JSN if they had JSN >or=1 in the medial and/or lateral tibiofemoral compartments, respectively. Medial and/or lateral OSTs were defined as medial and/or lateral tibial and/or femoral OSTs >or=2, respectively. Medial sclerosis and lateral sclerosis were defined as medial and lateral tibial sclerosis >or=1, respectively. We performed a logistic regression with medial JSN as the outcome and with M:L BMD Ratio groups as predictor variables, using the median group as the referent. Analyses were adjusted for age, sex, and body mass index (BMI). Generalized estimating equations were used to adjust for correlation between knees. Identical analyses were performed with medial OSTs, medial sclerosis, lateral JSN, lateral OSTs, and lateral sclerosis as the outcomes. RESULTS: Mean age of 1612 subjects (3048 knees) was 63.9 (Standard Deviation (SD)+/-8.9), 56% were women, and mean BMI was 28.5 (SD+/-5.5). M:L BMD Ratio was positively associated with medial JSN (P for linear trend <0.0001) and negatively associated with lateral JSN (P for linear trend <0.0001). The relations of the ratio with medial and lateral OSTs were j-shaped with P for quadratic trends <0.0001. There were also strong associations between M:L BMD Ratio and compartment-specific sclerosis (P for linear trends <0.0001) with most knees with medial and lateral sclerosis being in the highest and lowest M:L BMD Ratio groups, respectively. CONCLUSION: In summary, the extremes of the M:L BMD Ratio are strongly associated with individual radiographic features of OA. These findings add to existing evidence supporting the importance of understanding bone in OA pathophysiology.

Absorptiometry, Photon↗

Benign fibrous histiocytoma in the mandible.

PURPOSE: Benign fibrous histiocytoma (BFH) is a very rare benign tumor occurring in the jaw. The purpose of this article is to describe the clinical, radiographic, and microscopic appearance of BFH occurring in the mandible. STUDY DESIGN: A 42-year-old man had an asymptomatic swelling of the left mandibular posterior area. RESULTS: Conventional radiographs revealed a well-defined multilocular radiolucency involving the left mandibular body, ramus, coronoid process, and condylar head. The lesion showed the thinning and expansion of the cortex. There were many thin, indistinct septa in the lesion. The computed tomography (CT) scans showed bone destruction with marginal sclerosis and a heterogeneous soft-tissue mass expanding the mandible. Microscopic examination revealed a cellular tumor composed of uniform spindle-shaped cells arranged in a storiform pattern. CONCLUSIONS: BFH is a very rare benign tumor occurring in the jaw, so more cases should be reported to understand the characteristics of the lesion.

Adult↗

Evaluation of the relationship between periapical lesions/sclerotic bone and general bone density as a possible gauge of general health among 80-year-olds.

OBJECTIVES: To evaluate the incidence among 80-year-olds of periapical lesions as detected on panoramic radiographs and to determine the relationship between sclerotic bone around the periapical lesions to heel bone density, body height, and hand-grip strength. STUDY DESIGN: Six hundred fifty-nine panoramic radiographs (262 males, 397 females), obtained from 80-year-old residents of Fukuoka Prefecture, Japan, were used for evaluation of periapical lesions. These findings were correlated with physical examination results to determine the relationship to general health. RESULTS: Of 659 panoramic radiographs, 31 (5%) were noted to have periapical lesions. Average size of the 31 periapical lesions was 6.1 +/- 2.2 mm. Of the 31 periapical lesions, 21 (68%) were accompanied by linear or diffuse types of sclerotic bone. Of the 21 sclerotic bones, 10 (48%) were of a linear type of sclerotic bone and 11 (52%) of a diffuse type of sclerotic bone. Of the 11 diffuse types of sclerotic bone, 10 (91%) were in the mandible and 1 (9%) in the maxilla. Periapical lesions in the mandible were more frequently accompanied by a diffuse type of sclerotic bone than those in the maxilla (P < .01). The hand-grip strength of those having periapical lesions, accompanied by a diffuse type of sclerotic bone, was stronger than those having no periapical lesions (P < .01) and those accompanied by a linear type of sclerotic bone (P < .03). However, there was no relationship between presence of sclerotic bone and heel bone density or body height. CONCLUSIONS: Periapical lesions accompanied by a diffuse type of sclerotic bone were more frequently seen in the mandible of 80-year-olds. To evaluate the clinical significance of sclerotic bone around periapical lesions in 80-year-olds, further study to evaluate the significance of endodontic treatment needs to be done.

Aged↗

The bone scan in inflammatory osseous disease.

The 99mTc-phosphate bone scan has become a sensitive, reliable, and safe method for evaluating the patient with suspected inflammatory disease of bone. The scan may become positive as early as the first 24 hr after the symptoms and 10-14 days before roentgenographic changes occur. It can be used to differentiate successfully a variety of diseases from osteomyelitis, and in conjunction with 67Ga-citrate scan has become a mainstay in the work-up of the patient with infectious disease. Applications of the bone scan to infectious diseases in pediatric practice are especially helpful, since these diseases are common problems in this age group. Increased experience with the 99mTc-phosphate bone scan has already defined several areas of "limitations" in evaluating inflammatory disease. "Cold" defects, negative scans in early stages of osteomyelitis, and "extended uptake" may all pose problems in interpretation, but careful correlation of the bone scan results with clinical history and physical findings, blood cultures, and roentgenography will significantly reduce these problems.

Acute Disease↗

Interferon therapy for orbital infiltration secondary to Erdheim-Chester disease.

PURPOSE: To describe a 55-year-old male with Erdheim-Chester disease with bilateral orbital infiltration and visual loss who was successfully treated with interferon-alpha. METHODS: Interventional case report. RESULTS: The patient was treated with interferon-alpha and had an improvement in his clinical signs, including his visual acuity, after 4 weeks of interferon therapy. CONCLUSION: Interferon-alpha can be effective in the treatment of orbital infiltration secondary to Erdheim-Chester disease.

Exophthalmos↗

Osteo-articular tuberculosis: its presentation in coloured races.

Four hundred and ninety-nine cases of osteo-articular tuberculosis have been analysed with special emphasis on the "unusual" presentation. Sclerotic bone reaction in association with active tuberculous osteitis is not at all uncommon and may occur in any affected bone. Maintenance of disc spaces with tuberculous spondylitis is not uncommon. Periosteal reactions, often very gross, occur commonly both in multicystic tuberculosis as well as in isolated lesions. Multiple bone involvement occurred in 8.2% of this series. Tomography delineates the total extent of involvement especially in central involvement of vertebral bodies. Healed tuberculosis may give rise to block vertebrae indistinguishable from the congenital type except for the absence of waisting at the level of fusion.

Adolescent↗

Bone formation associated with osseous metastases from bladder carcinoma.

Twenty-four cases of carcinoma of the urinary bladder, involving bone, have been reviewed. Eighteen of these were distant metastases beyond the pelvis. The lesions were predominantly osteolytic. Ossification in the soft tissues adjacent to the bone metastasis was observed in three cases, all involving the left tibia. The reason for this site of predilection remains obscure.

Adult↗

Case report: Erdheim-Chester disease: polyostotic sclerosing histiocytosis.

Erdheim-Chester disease is characterised by diffuse metaphyseal and diaphyseal sclerosis of long tubular bones of the appendicular skeleton. In addition, there is an inconstant tendency to visceral involvement by lipo-granulomatous infiltration. Histologically, this condition has marked similarities to Hand-Schuller-Christian disease. A further case is presented which supports the view that Erdheim-Chester disease is part of the spectrum of histiocytoses and not a distinct pathological entity. Polyostotic sclerosing histiocytosis would be an appropriate alternative label for this condition.

Adult↗

Unusual imaging findings in association with spinal osteoblastoma.

This paper describes four patients with spinal osteoblastoma that had unusual imaging features. Two cases arose in the cervical spine and two in the thoracic spine. Three tumours were associated with diffuse sclerosis of the vertebral body (an 'ivory vertebra'). All of these lesions either arose in the vertebral body or extended into the body from the adjacent pedicle. Reactive sclerosis at multiple levels was seen in three cases (one involving adjacent vertebral bodies, two involving adjacent pedicles or laminae and one involving multiple ribs). Osteophyte formation was identified bridging the disc space on the concave side of a scoliosis in two cases. Another case had an unusual scintigraphic appearance with an area of linear activity adjacent to the tumour. The finding of an 'ivory vertebra' in a child or young adult should raise the possibility of osteoblastoma involving the vertebral body, especially if there is an associated scoliosis.

Adult↗

The incidence and relevance of bone sclerosis in orbital pseudotumour.

We reviewed the CT studies of 176 patients with biopsy-proven inflammatory pseudotumour of the orbit, in an attempt to establish the incidence of bone sclerosis in this condition. We found 20 patients (11.5%) to show definite bone sclerosis and/or hyperostosis, and in a further 10 (6%) there was probable sclerosis, giving a total of 30 patients (17%) with some evidence of bony change. Bone-window films were available for a minority of patients, and the frequency of sclerosis was therefore probably underestimated. This feature, which presumably represents a low-grade, chronic sterile osteitis, has not previously been emphasised.

Humans↗