Cases reported "Osteitis Deformans"

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1/34. Acute ischemia of the lower limb. An unusual complication of Paget's disease of bone.

    Paget's disease of bone (osteitis deformans) is one of the most common skeletal diseases, characterised by bone distortion and the loss of interior structure. Asymptomatic evolution is usual, so diagnosis is likely to be made only when complications of the disease appear. This paper describes such a diagnosis made in a patient with acute lower limb ischemia caused by the compression of the superficial femoral artery between the adductor muscles and an exostosis of the femur.
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2/34. Florid osseous dysplasia of the mandible: report of a case.

    In 1986, a 60-year-old African American woman visited the Marquette University School of dentistry with a complaint of a vague, dull pain in her lower left quadrant. tooth No. 19 was extracted 10 years earlier because of extensive decay, and tooth No. 18 had received root canal therapy. A panoramic radiograph revealed the presence of ill-defined, multilocular, mixed (radiopaque-radiolucent) lesions present throughout the lower jaw. She was treated with antibiotics and scheduled for follow-up visits. When the symptoms persisted, tooth No. 18 was re-treated with root canal therapy and a representative biopsy was taken from the left mandibular area. The biopsy showed the presence of chronic osteomyelitis. The patient was treated with antibiotics and was scheduled for periodic check-up visits. In February 1995, she returned with the same symptoms in the left mandible. A panoramic radiograph showed persistence of the mixed radiopaque-radiolucent lesions throughout her mandible; however, the mass on the left side was more radiopaque and had assumed a "cotton wool" appearance. tooth No. 18 was extracted and a biopsy was taken from the area. After correlating the clinical behavior, radiographic appearance, and histopathologic features, a diagnosis of florid osseous dysplasia with osteitis was made. This case represents a classic example of the difficulty in diagnosing fibro-osseous lesions using radiographic interpretation alone and the need to correlate the clinical, radiographic, and histopathologic features to reach a diagnosis. Additionally, the present case clearly shows treatment problems of an otherwise self-limiting condition when secondary involvement with osteomyelitis is also present. A brief description of the conditions that were included in the differential diagnosis and their management is presented.
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keywords = mandible, jaw, lower
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3/34. Gross vertebral collapse associated with long-term disodium etidronate treatment for pelvic Paget's disease.

    Inhibition of skeletal mineralisation is a well-recognized complication of disodium etidronate therapy that was identified in the earliest studies of its use in osteoporosis and Paget's disease. The effect is seen at lower doses in Paget's disease than in osteoporosis. Several cases of spontaneous fractures occurring in unaffected bones of Paget's patients have been reported. However, we believe the case described here is the most severe example of etidronate-induced osteomalacia published in the literature, featuring widespread vertebral collapse occurring as a consequence of nearly 10 years of uninterrupted etidronate treatment for isolated hemipelvic Paget's disease.
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4/34. MR imaging of pseudosarcoma in Paget's disease of bone: a report of two cases.

    Pseudosarcoma is a rare manifestation of Paget's disease of bone. We report the MR imaging of two cases highlighting the difficulties in diagnosis. One of the cases is the first time this condition has been described outside the long bones of the lower limb.
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5/34. osteosarcoma arising in Paget's disease of the mandible.

    Although osteosarcoma is a well-known complication of Paget's disease of bone, it uncommonly develops in the jaw bones. We present an osteosarcoma arising in Paget's disease of the mandible with unique features of a normal serum alkaline phosphatase level, and histologic features of telangiectatic change in the osteosarcoma and association with cemento-osseous dysplasia. Sixteen reported cases of osteosarcoma arising in Paget's disease of the jaw bones (OPJ) are also reviewed and compared to osteosarcoma arising in Paget's disease occurring in the entire skeleton (OPS) and osteosarcoma arising de novo in the jaw bones (OJ). Females are more commonly involved in OPJ in contrast to a male predominance in OPS and OJ. OPJ also has a distinctively higher percentage involving blacks compared to OPS. The prognosis of OPJ is poor, with 69% of patients dying within two years after diagnosis. Early recognition, early and aggressive treatment are important to improve the prognosis and are hence emphasized.
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ranking = 254.57238578512
keywords = mandible, jaw
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6/34. Familial idiopathic hyperphosphatasia (FIH): response to long-term treatment with pamidronate (APD).

    A 5-year-old child suffering from familial idiopathic hyperphosphatasia (FIH) was treated by: (1) intravenous infusion of pamidronate (APD) (3 h) (0.75 mg/kg/day) for 5 days; and (2) oral administration of APD (8 mg/kg/day) for 1 year, in association with calcium (1 g/day) as calcium gluconate. A decrease of both serum calcium and phosphate, and a slight PTH increase were observed immediately after the IV treatment; serum alkaline phosphatase did not change, but a marked and rapid decline in the hydroxyprolinuria was observed: basal 659 /- 207 during IV treatment 169 /- 59 (mean /- SD mg/24 h, P < 0.005). At the end of one year of oral APD treatment clinical and radiological findings showed a remarkable improvement. serum calcium, phosphate and PTH returned to the initial values. plasma alkaline phosphatase levels showed a 70% decrease: basal 1370 IU/l, 1 year 410 IU/l whereas the hydroxyprolinuria values were similar to those determined at the end of the intravenous treatment (212 /- 13 mg/24 h), but still significantly lower than the basal levels (P < 0.01). No side-effects were observed. APD appears to be a promising treatment for patients with FIH.
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7/34. osteomyelitis mimicking Paget's disease or a pagetoid phenomenon: a case report.

    osteomyelitis is an inflammatory infection of the bone marrow. When the maxillofacial region is involved the mandible is the preferred site in adults and the elderly. Maxillary involvement is usually seen in children with only few cases reported in adults. The disease occurs in stages, with some stages highly mimicking other diseases. Clinicians ought to be aware of the full biological course of osteomyelitis of the jaws to avoid mis-diagnosis or underdiagnosis.
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ranking = 53.251511930171
keywords = mandible, jaw
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8/34. Giant cell tumours in mandible and spine: a rare complication of Paget's disease of bone.

    The case of a man who was diagnosed as having polyostotic Paget's disease at the age of 52 years is described. He developed a rare neoplastic complication of Paget's disease--a giant cell tumour in his mandible, which was excised. Nine years after the diagnosis of this tumour he developed a new giant cell tumour arising from the L3 vertebral body. He was born in Avellino in italy, from where five other cases of giant cell tumours arising from Pagetic bone disease have been reported. No family relationship between our patient and the other cases was established. His Paget's disease was particularly aggressive and resistant to treatment with two single high dose infusions of pamidronate almost two years apart.
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ranking = 237.0446249865
keywords = mandible
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9/34. Paget's disease of bone affecting a single vertebra: clinical, radiologic, and histopathologic correlations.

    We report a 67-year-old man who presented with a 3-month history of progressively increasing pain in the lumbar spine. His past medical history was unremarkable, and physical examination disclosed local tenderness over the lower spine. No neurologic dysfunction was identified. Routine laboratory evaluation including alkaline phosphatase activity was normal. An x-ray film of the lumbar spine showed enlargement and increased density of L-5 vertebra. A whole-body bone scan revealed markedly increased uptake at the L-5 level. To further evaluate the nature of the disorder and the cause of his pain, a computed tomography (CT) scan was obtained. It disclosed multiple lucent areas with some sclerotic changes mainly affecting the vertebral body of L-5. No spinal stenosis was found. Subsequently, a bone biopsy of L-5 was performed that showed typical findings consistent with Paget's disease. The patient was treated with etidronate (200 mg b.i.d. for 6 months) followed by salmon calcitonin (50 IU 3 times/week s.c. for 6 months). The pain declined gradually in severity and the patient became symptom free after 12 months of treatment. A repeat x-ray film, obtained at that time, showed no significant change. However, a bone scan showed almost complete normalization. The present case illustrates that a high index of suspicion is required when only a single vertebra is affected by Paget's disease, especially, when alkaline phosphatase activity is normal. It may present with severe pain without evidence of neurologic dysfunction. CT scan may be a useful adjunct in establishing the diagnosis and elucidating the cause of pain.
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10/34. A case of Paget's disease treated by distraction osteogenesis.

    Paget's disease is a localized bone disorder marked by increased turnover usually associated with a deformity of the affected bone. Distraction osteogenesis may be a useful method for correcting the deformity. A 57-year-old woman with Paget's disease had a 3 cm limb-length discrepancy with a 47 degrees procurvatum deformity of the ipsilateral femur. Distraction osteogenesis using a Taylor Spatial Frame was performed, leading to complete correction of the procurvatum deformity and limb-length discrepancy. After improvement of the limb-length discrepancy, the patient felt more comfortable than she had before surgery with less low back pain. This result suggests distraction osteogenesis may be appropriate for the treatment of some severe, complex deformities of the lower limbs in patients with Paget's disease.
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