Cases reported "Osteitis Deformans"

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1/68. Periprosthetic fracture of the acetabulum during total hip arthroplasty in a patient with Paget's disease.

    The case of a patient with Paget's disease of the pelvis (acetabulum) who had an intraoperative posterior wall fracture during the insertion of a noncemented acetabular component into an under-reamed acetabular bed of sclerotic Pagetoid bone is reported. This unusual complication has not, to my knowledge, been previously reported. patients with sclerotic bone, like those with osteoporotic bone, may also be at risk for periprosthetic acetabular fractures when an under-reaming technique is used.
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2/68. 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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3/68. A case of a dog with thickened calvaria with neurologic symptoms: magnetic resonance imaging (MRI) findings.

    A 6-year-old female mongrel dog weighing 9.0 kg was presented ananastatic, with clouding of consciousness, bilateral loss of hearing and depressed reactivity of the eyes to light. magnetic resonance imaging (MRI) examination showed that the calvaria was markedly thickened with compression to the cerebrum and cerebellum. The case of a dog with thickened calvaria with compression of the cerebrum and cerebellum which could not be diagnosed by conventional measures was amenable to diagnosis by MRI. With increased application of MRI examination, such canine cases might increase in number.
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4/68. Early experience with total knee replacement.

    This paper presents a review of two years' experience with the geometric total knee replacement. The results of 23 arthroplasties in 22 patients are discussed. Relief of pain was consistent and dramatic, movement was increased postoperatively in only three patients, but imporvement in overall function occurred in 20 of the 22 patients. There was one failure requiring arthrodesis. In this patient, six months after surgery the medial tibial condyle collapsed and the polyethylene tibial component fractured. This complication has not been reported before. Early results are encouraging. Total knee joint replacement is a useful procedure in advanced arthritis when arthrodesis is the only alternative.
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5/68. The treatment with alendronate in hemifacial spasm associated with Paget's disease of bone.

    The association of Paget's disease of bone and hemifacial spasm has rarely been reported. hemifacial spasm is often associated with compression of the facial nerve by a vascular loop at the point where the nerve leaves the brainstem before traversing the cerebellopontine angle. It is believed that narrowing of the cerebellopontine angle cistern caused by Paget's disease increases the chance of vascular compression of the facial nerve. Whilst specific antipagetic therapy such as calcitonin has been used with good response in hemifacial spasm associated with Paget's disease, the usefulness of the newer bisphosphonates is not clear. A 65-year-old woman with hemifacial spasm associated with Paget's disease was treated with alendronate, and the hemifacial spasm became very infrequent 4 months after commencement of the therapy.
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keywords = compression
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6/68. Failure after customized curved femoral stems in total hip arthroplasty for Paget's disease.

    We describe 2 patients with Paget's disease who underwent total hip arthroplasty for osteoarthrosis. In view of the femoral deformity in each case, a custom-made, long, curved femoral stem was used. Both patients have subsequently sustained periprosthetic fractures at the level of the tip of the prosthesis.
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7/68. 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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8/68. osteoprotegerin deficiency and juvenile Paget's disease.

    BACKGROUND: Juvenile Paget's disease, an autosomal recessive osteopathy, is characterized by rapidly remodeling woven bone, osteopenia, fractures, and progressive skeletal deformity. The molecular basis is not known. osteoprotegerin deficiency could explain juvenile Paget's disease because osteoprotegerin suppresses bone turnover by functioning as a decoy receptor for osteoclast differentiation factor (also called rank ligand). methods: We evaluated two apparently unrelated Navajo patients with juvenile Paget's disease for defects in the gene encoding osteoprotegerin (TNFRSF11B) using polymerase-chain-reaction (PCR) amplification followed by direct sequencing and Southern blotting of genomic dna. genetic markers near TNFRSF11B were evaluated by both a PCR method that involved sequence-tagged site-content mapping of a deletion of TNFRSF11B and PCR spanning the dna break points. RESULTS: Both patients had a homozygous deletion of TNFRSF11B, with identical break points, on chromosome 8q24.2. The defect spans approximately 100 kb, but neighboring genes are intact. We found that serum levels of osteoprotegerin and soluble osteoclast differentiation factor were undetectable and markedly increased, respectively. CONCLUSIONS: Juvenile Paget's disease can result from osteoprotegerin deficiency caused by homozygous deletion of TNFRSF11B.
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9/68. Rapid osteolysis after revision hip arthroplasty in Paget's disease.

    Rapid bone turnover in Paget's disease has been of concern to many surgeons performing hip arthroplasties. We present the case of a 71-year-old man with Paget's disease affecting the proximal femur. He had undergone total hip arthroplasty 14 years before. He sustained a fracture at the tip of the femoral component, which was managed with revision total hip arthroplasty. His postoperative course was complicated by rapid and profound osteolysis of the femur distal to the fracture site, secondary to disease activity. This case highlights the need for awareness of Paget's disease activity and this potential complication.
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10/68. Malignant transformation in Paget disease of bone.

    BACKGROUND. The previously reported incidence of malignant transformation of Paget disease up to 5.5% and its dismal prognosis have prompted the clinical investigation of a large population with Paget disease. methods. A chart review of symptomatic and asymptomatic patients with a diagnosis of Paget disease between 1970 and 1985 at four large Montreal hospitals revealed 1078 patients. Eight patients with malignant transformation were studied in detail. RESULTS. The incidence of malignant transformation was 0.7%, and the most frequent histologic type was osteogenic sarcoma. The most prevalent site was the femur, and pathologic fracture with focal osteolysis was present in 50% of patients at the initial appearance of the tumor. Healing at the fracture site was demonstrated in one patient. Another patient died of uncontrollable hemorrhage. A case of malignant lymphoma in Paget disease is recorded. CONCLUSIONS. Surgery or biopsy should be preceded by preoperative scintigraphic bone blood flow evaluation and, if necessary, administration of a preoperative course of mithramycin and selective embolization to minimize bleeding. The prognosis of malignant transformation in Paget disease is poor, although one patient with malignant lymphoma survived after aggressive treatment. Early biopsy and aggressive treatment should not be delayed.
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