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[Osteitis deformans].

Osteitis deformans is a bone disease of still unknown aetiology which shows characteristic symptoms in the maxillofacial region. An increase in size of the entire calvarium and also of single bones of the facial skeleton belongs to these symptoms. A triad may be seen in the jawbone: osteoporosis, foci of densification and cementum hyperplasia. No possibility of treatment was known in the past. Nowadays, the use of cytostatics, calcitonin and diphosphonates seems to be promising.

Alkaline Phosphatase

[Treatment of Paget's osteitis deformans].

Specific treatment of osteitis deformans (OD, Paget's disease of bone) is indicated when symptoms are associated with signs (biochemical or by bone scintigraphy) of active disease. OD in the base of the skull and osteolytic lesions in weight-bearing bones should be treated irrespective of symptoms. The effect of calcitonin (CT) on symptoms and skeletal blood-flow is achieved rapidly and healing of osteolytic lesions may occur. However, CT is only effective in approximately 60%, is discontinued due to side effect in 20% of patients, and must be administered parenterally. EHDP (Etidronate) is administered orally but the use of even small doses carries a risk of mineralisation defect and possibly fracture. EHDP should, therefore, be given intermittently and should not be used in osteolytic lesions of weight-bearing bones. Second-generation bisfofonates are devoid of this side effect and are very effective in OD. The effect of mithramycin is only transient and because of the risk of serious side effects the use should be restricted to severe cases of OD resistant to the other lines of treatment.

Adenosine Diphosphate

Hemifacial spasm and osteitis deformans.

PURPOSE/METHODS: A patient with osteitis deformans (Paget's disease) and hemifacial spasm underwent magnetic resonance tomographic angiography. Bone-modulating bisphosphonates and botulinum injection were administered to treat the hemifacial spasm. RESULTS/CONCLUSIONS: Computed tomography showed marked temporal bone overgrowth. Magnetic resonance tomographic angiography showed no vascular compression of the facial nerve root. The hemifacial spasm failed to resolve with intravenous pamidronate. Subsequent botulinum injection rendered the patient spasm free for 22 weeks. Further research on the use of bisphosphonates in the treatment of pagetoid hemifacial spasm is required.

Aged

[Paget's osteitis deformans. Epidemiology and clinical picture].

Osteitis deformans is a focal disease of the osteoclasts characterised by increased bone resorption subsequently followed by increased bone formation leading to abnormal bone. A viral etiology seems increasingly probable, but remains unproven. 5-30% of the patients present with symptoms such as pain, deformity and fracture. Hearing loss, nerve- or root-compression, arthrosis and hyperuricaemia may complicate the disease while malignant degeneration, hypercalcemia and high output cardiac failure are rare. The diagnosis is based on X-ray findings but biopsy may be necessary in selected cases. The extent of the disease is revealed by bone scintigraphy and the activity of the disease reflected by urine hydroxyproline excretion and serum alkaline phosphatase.

Adult

Sarcomas of bone complicating osteitis deformans (Paget's disease): fifty years' experience.

Osteitis deformans (Paget's disease of bone) may be complicated by osseous cancer. Of 3964 patients between 1927 and 1977, 38 had primary malignant bone tumors. This represents an incidence of sarcomatous change of 0.95%. The bones of the pelvis were most often involved; the humerus and the femur were next in frequency. No region of the skeleton was spared, with the exception of the forearms and hands. Of the 38 tumors, six were fibrosarcomas and 32 were osteosarcomas. Irrespective of the site of involvement, the histopathologic extremely poor. The 5-year survival rate was 7.9%, with a mortality rate of 86.8% 36 months after diagnosis. The main cause of death was pulmonary metastasis or local extension of tumor growth. Three patients were long-term survivors, but no clinical or pathologic elements could be identified which differentiated these survivors from the other patients in the study.

Adult

Osteitis deformans in the South African negro. A report of 3 cases.

Osteitis deformans is a relatively common disease in Caucasians, but rare in the indigenous people in Africa. Three cases of Paget's disease occuring in South African Negroes are reported here. Gene marker studies confirmed the absence of Caucasoid genes in these patients.

Black People

Osteitis deformans. Report of a long-standing case with extensive oral involvement.

Presented is a case of osteitis deformans (Paget's disease of bone) with a 38-year history after diagnosis. The patient had nearly the entire spectrum of symptoms that can result from widespread involvement of the skeletal system with this disease. Diffuse involvement of the maxilla and mandible resulted in postextraction complications which are common in Paget's disease of the bone.

Aged

[An analysis of the radiological appearance of osteoplastic metastases and osteitis deformans (Paget's disease) (author's transl)].

The validity of individual radiological signs for the differentiation of osteoplastic bone metastases from osteitis deformans has been investigated quantitatively (103 skeletal metastases from carcinoma of the prostate, 45 cases of Paget's disease, two osteoplastic bone metastases from a carcinoma of rectum and bronchus). The similarities were demonstrated by three cases observed by us. Problems in the quantitative evaluation of the radiographs are discussed.

Aged

Radionuclide imaging in osteitis deformans.

New knowledge of osteoclastic function and new treatments to reduce osteolytic activity have combined to yield new degrees of biochemical control over the increased rate of bone resorption and formation that characterized osteitis deformans (OD; Paget's disease). We have briefly outlined current concepts, and provided an example of the role radionuclide imaging can play in delineating the skeletal areas involved in this disease.

Bone and Bones

Paget's osteitis deformans: a carbonate-rich calcium alkali phosphate in the pre-malignant bone mineral and implications for lymphoma production by CO2.

Comparison of powder diffraction patterns of unheated normal bone mineral with the damaged by Paget's Osteitis Deformans showed that due to a difference in the spacing of one weak inner reflection the possibility exists that carbonate-containing alpha-calcium sodium orthophosphate may be a minor constituent of the osteitic bone mineral. The chemical and physical properties of this substance make it a likely minor constituent of bone salt. The presence of this calcium alkali phosphate in the osteitic mineral was tentatively proposed after demonstrating the similarity of the diffraction patterns of synthetic mixtures of this substance with tricalcium phosphate hydrate to those of the osteitic bone salt.

Aged

The clinical relevance of radiologically revealed Paget's disease of bone (osteitis deformans).

Radiological and hospital records of 400 patients with Paget's disease that was shown radiologically have been examined in an effort to establish the clinical significance of the disease. Twenty-eight per cent of the patients were considered to have symptoms related to their disease, the most frequent symptom being bone pain, deformity and fracture. It appears that Paget's disease may predispose to the formation of urinary calculi. Malignant change and neurological and cardiovascular complications are rare, and osteitis deformans does not appear to predispose to osteoarthrosis.

Adult

Osteitis deformans of the hip joint.

It is estimated that 66% of patients with Paget's disease have involvement of the pelvis and 46% of the proximal femur. Therefore, it is not unexpected that hip pain is one of the major presenting complaints. Analysis of the radiographs of 25 hips with one or more articular sufaces involved by Paget's disease demonstrated narrowing in 24. Unlike the findings in primary degenerative joint disease, the majority of cases had a radiographic pattern characterized by uniform narrowing of the articular cartilage and minimal hypertrophic changes. Pathologic correlation was obtained from specimens of four patients who underwent total hip replacements. The pathogenesis of arthritic changes associated with osteitis deformans is not established. The evidence presented suggest that cartilagenous narrowing results from a disturbance in endochondral bone formation related to the hyperemia of Paget's disease. Secondary deformities of bone produce further derangement of joint mechanics. The secondary degenerative changes which ensue differ mechanically, and therefore radiographically, from primary degenerative joint disease.

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