Cases reported "Sclerosis"

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1/18. Primary chronic osteomyelitis associated with synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO syndrome).

    Diffuse sclerosing osteomyelitis may indicate the mandibular localisation of the SAPHO syndrome. Twelve patients with diffuse sclerosis of the mandible were examined for symptoms of the SAPHO syndrome. Nine patients were found to have primary chronic osteomyelitis and eight of these represented a SAPHO syndrome. Results in this series support the hypothesis of an association between primary chronic osteomyelitis and the SAPHO syndrome.
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ranking = 1
keywords = mandible
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2/18. Sclerosing, pseudovascular rhabdomyosarcoma in adults. Clinicopathological and immunohistochemical analysis of three cases.

    rhabdomyosarcoma in adults represents a rare soft tissue neoplasm which is seen most frequently in its pleomorphic subtype in this age group. Very rarely, clear cell and spindle-cell variants have been reported. In this study we describe three cases of rhabdomyosarcoma in adult patients, characterised by prominent hyaline sclerosis and a pseudovascular growth pattern. All cases were identified in the consultation files of one of the authors and routinely processed. Immunohistochemical studies were performed on paraffin sections with the alkaline phosphatase-antialkaline phosphatase method. The patients, two women and one man, were 40, 41, and 56 years old. One developed a deep-seated soft tissue mass in the left lower leg, and one, a tumour of the left upper jaw. In one patient a bone tumour in the proximal body of the sacrum without extension into soft tissues was seen. The patients were treated by wide excision, piecemeal excision and incomplete excision in one case each; additional radiotherapy was performed in all three cases, and chemotherapy in two patients. In one patient multiple pulmonary metastases were noted, which showed progression despite systemic chemotherapy. Histologically, the neoplasms were composed of round/polygonal and spindle-shaped tumour cells including typical rhabdomyoblasts. In all cases a pseudovascular pattern and prominent hyaline sclerosis of the intercellular matrix was seen. Immunohistochemically, tumour cells stained positively for desmin and muscle actin (HHF35) and also for markers of striated muscle differentiation (myogenin, MyoD1, fast myosin). In this paper an unusual morphological variant of rhabdomyosarcoma arising in adult patients is described, which should be added to the morphological spectrum of these neoplasms.
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ranking = 0.05764914490548
keywords = jaw
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3/18. tooth eruption in a patient with craniometaphyseal dysplasia: case report.

    Craniometaphyseal dysplasia (CMD) is a very rare genetic disorder of bone remodeling caused by osteoclast dysfunction. The clinical and radiographical features of oral findings are presented in a sporadic case of CMD in a child (age 10 years, 7 months). An intraoral examination showed severe malocclusions, including anterior crossbite and deep bite. Furthermore, a radiographic examination showed increased radiopacity of the maxilla and mandibular bones due to hyperostosis and sclerosis of the jaw. There was no root resorption of the canines or molars in the primary dentition, although root formation of the permanent teeth was proceeding. Dental age was calculated to be approximately 1 year, 4 months younger than his chronological age. The eruption speed of the permanent lateral incisors after the gingival emergence was shown to be within normal values, and we discuss whether the canines and premolars in the permanent dentition could erupt or not.
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ranking = 0.05764914490548
keywords = jaw
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4/18. Therapeutic possibilities of long-term roxithromycin treatment for chronic diffuse sclerosing osteomyelitis of the mandible.

    The clinical efficacy of long-term roxithromycin treatment was examined objectively in nine patients with chronic diffuse sclerosing osteomyelitis of the mandible. roxithromycin was administered orally at a dose of 300 mg/day for between 68 days and 66 months. In seven of the nine cases (77.8%), the symptoms disappeared 1-12 months after the start of therapy. radiography showed that osteolytic changes (evident from 'moth-eaten' appearance of bone) had improved but that osteosclerosis had persisted or become more predominant by the end of therapy. Therefore, the optimum duration of treatment should be decided according to the amelioration of symptoms along with the disappearance of osteolytic findings in radiographs. Diarrhoea and stomach discomfort occurred in one case, and liver dysfunction in another, but these adverse reactions were relatively mild. The mechanism of action of roxithromycin in this study is not yet fully understood, but our results indicate that long-term roxithromycin treatment may be useful for diffuse sclerosing osteomyelitis of the mandible and should be attempted before surgical treatment is considered.
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ranking = 6
keywords = mandible
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5/18. Juvenile mandibular chronic osteomyelitis: a distinct clinical entity.

    Sclerosing osteomyelitis of the mandible is an uncommon disease of unknown aetiology. A series of eight female children (6 to 12 years old) with a distinct mandibular inflammatory disease were studied. Each presented with pain and a recurrent soft tissue swelling overlying a predominantly unilateral mandibular enlargement. On imaging, this deformity demonstrated a mixture of patchy sclerosis and radiolucency. A raised erythrocyte sedimentation rate was the only consistent serological finding. Treatment varied from symptomatic control with non-steroidal anti-inflammatory medication, to surgical management that included decortication and contouring and, in one case, resection with reconstruction. A potential protocol for treatment of this disease is given. The early age of onset of the disease process and the uniformity of the features distinguish this condition from other groups of disorders that, previously, have been collectively designated as chronic diffuse sclerosing osteomyelitis. It is proposed that this inflammatory disease of mandibular bone, in the paediatric patient, should be regarded as a separate clinical entity: 'juvenile mandibular chronic osteomyelitis'.
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ranking = 1
keywords = mandible
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6/18. Differences between florid osseous dysplasia and chronic diffuse sclerosing osteomyelitis.

    Florid osseous dysplasia (FOD) is confused in the literature with chronic diffuse sclerosing osteomyelitis. Two cases of each condition are presented to demonstrate the differences between them. In FOD, there are multiple lobulated sclerotic masses in several quadrants of the jaws, usually in black females. In some cases, the sclerotic masses are exposed to the oral cavity, resulting in a secondary osteomyelitis. Periapical cemental dysplasia is often found in association with FOD. Chronic diffuse sclerosing osteomyelitis is a primary inflammatory condition of the mandible. patients have cyclic episodes of unilateral pain and swelling. The affected region of the mandible exhibits a diffuse opacity with poorly defined borders. Although women are affected more often than men, black persons are not particularly susceptible.
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ranking = 2.0576491449055
keywords = mandible, jaw
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7/18. Sclerosing osteomyelitis of Garre (periostitis ossificans).

    The diagnosis of sclerosing osteomyelitis of Garre, or periostitis ossificans, is suggested by a history of hard swelling of the mandible accompanied by a carious dentition. Computed tomography, nuclear medicine scanning, and pantomography are used to illustrate the appearance of this condition arising in a 25-year-old woman. The differential diagnosis of mandibular thickening and associated bony sclerosis is discussed.
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ranking = 1
keywords = mandible
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8/18. Ultrastructure of sclerotic cemental masses.

    Electron microscopic findings in a focus of early calcification in a case of sclerotic cemental masses of the jaws are described. The lesional cells generally resemble typical collagen-producing cells, except that many contain unusually large numbers of intracytoplasmic actinlike filaments. This and other fine structural features are reminiscent of fibroblasts involved in active tissue repair. These observations suggest that the lesion is basically reactive in its biologic nature. The most characteristic portion of the mineralized component, the rounded calcified globules, ultrastructurally resembles primary cementum.
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ranking = 0.05764914490548
keywords = jaw
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9/18. Differential diagnosis of some simple osseous lesions associated with sickle-cell anemia.

    Sickle-cell disease is characterized by chronic hemolytic anemia, rapid destruction of defective erythrocytes, with consequent circulatory disturbances. Thromboses and infarctions with resultant osseous manifestations tend to be localized in the ends of the long bones or in the medullary area, the latter often becoming apparent in the jaws. Three case reports are presented to illustrate osseous changes observed in the jaws of these sickle-cell patients. The radiographic findings demonstrate the adaptive mechanisms of bone metabolism in response to vascular infarction, infection, or both in some sickle-cell anemia patients. A differential diagnosis is suggested for osseous lesions observed in the jaws of these patients.
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ranking = 0.17294743471644
keywords = jaw
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10/18. Collapse of the condylar head of the mandible in children and subsequent ankylosis.

    Five cases are reported of children who presented with limitation of jaw movement and had some degree of deformity with shortening of the mandible on the affected side. X-ray examination demonstrated changes in the head and neck of the mandibular condyle. There were varying degress of sclerosis and new bone formation in the area extending from the neck of the condyle into the sigmoid notch. The left side was affected in each case. Parallels are drawn with Perthes' disease, and although the temporo-mandibular joint is not weight-bearing, in both conditions avascular necrosis is likely to be involved. Both have similar X-ray appearances. The fragmentation of the epiphysis in the later stages causes widening and collapse of the head of the femur and the mandibular condyle.
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ranking = 5.0576491449055
keywords = mandible, jaw
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