Cases reported "Osteolysis, Essential"

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11/30. Gorham's disease: an autopsy report.

    We present the case report of a 35-year-old man with Gorham's disease (disappearing bone disease, massive osteolysis) with initial clinical findings of small bowel lymphangiomatosis and multicentric osteolysis. The patient, who otherwise was healthy, had a chylothorax develop and he died 9 months later of thoracic and pulmonary complications. An autopsy revealed absence of the proximal thoracic duct and significant lymphangiectatic abnormalities of the pleural, peritoneal, diaphragmatic, splenic, and small bowel tissue with lymphangiomatous masses in the thoracic and mediastinal regions. The findings suggest a subtype of Gorham's disease characterized by a dysplastic lymphatic system. osteolysis was correlated anatomically with lymphangiectatic tissue, suggesting mediation of osteoclastic resorption via local lymphatic tissue factors.
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keywords = bone disease
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12/30. An investigation of vanishing bone disease.

    Vanishing bone disease is a rare condition producing local deformity and instability. Fibrovascular tissue replaces bone completely but the mechanism of bone destruction and resorption is unknown and there is controversy regarding the presence or absence of osteoclasts in the disease. radiography, clinical chemistry, light microscopy, transmission electron microscopy (TEM) and cytochemistry were used to investigate the condition of a young woman presenting early in the disease process. We detected atypical ultrastructure in osteoblasts and endothelial cells. The rare osteoclasts, numerous mononuclear phagocytes and vascular endothelium found in the condition reacted positively for the enzyme acid phosphatase. Aggressive local excision of diseased tissue and insertion of a free vascularized bone graft at an advanced stage of the disease, accompanied by subsequent radiotherapy for residual disease only were successful in rehabilitating the affected forearm and hand.
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keywords = bone disease
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13/30. "Disappearing bone disease" in the hand.

    "Disappearing bone disease" is a rare entity that is characterized by extensive osteolysis associated with hemangiomatosis. A case affecting a 13-year-old girl, with wide-spread hand involvement is reported. She did not respond to bone grafting but the condition was arrested after a course of radiation therapy.
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ranking = 5
keywords = bone disease
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14/30. Disappearing bone disease: a case report and review of the literature.

    Disappearing bone disease is a rare condition manifested by massive osteolysis. Its etiology is unknown. Histologically, involved bones show a nonmalignant proliferation of thin-walled vessels. The mechanism of bone absorption remains unclear. No treatment has proven effective in arresting the disease. Cases in which there is extraosseous involvement have a much poorer prognosis.
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keywords = bone disease
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15/30. Disappearing bone disease: massive osteolysis of the ribs treated with radiotherapy. Report of a case.

    The case of a boy with disappearing bone disease concerning the ribs is reported. The clinical, roentgenographic, immunological and histological studies are described. The patient was treated by radiotherapy. The process of disease stopped in irradiated area of the chest, but five months later appeared beyond that area. The patient was irradiated additionally for the next area and disease was arrested successfully.
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ranking = 5
keywords = bone disease
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16/30. Progressive vertebral collapse in diffuse angiomatosis.

    A case of vanishing bone disease is presented, with radiologic, endoscopic, and peritoneoscopic evidence of angiomatous involvement of the bones, surrounding soft tissues, and gastrointestinal tract. The diagnosis was made by a combination of radiologic techniques and endoscopy. Areas of absent bone were shown to opacify after intralymphatic injections of contrast material, providing in vivo demonstrations that these areas of vanishing bone are occupied by abnormal lymphatics. The etiology remains obscure, but our review of the literature suggests that this condition may be one of a large spectrum of disorders due to a basic underlying endothelial dysplasia. The lymphatic system, vascular system, or both may be involved, and the condition may manifest itself as an abnormality of the bones, soft tissues, viscera, or a combination of these.
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keywords = bone disease
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17/30. Idiopathic carpotarsal osteolysis with Bartter's syndrome. A case report and review of the literature.

    Idiopathic carpotarsal osteolysis is 1 of the rare types of disappearing bone diseases characterized by painful limitation of movement and deformity of the wrist and tarsal joints, and associated with frequent episodes of joint inflammation and a radiographic appearance of osteolysis. Reported here is an 8-year-old girl with idiopathic carpotarsal osteolysis and Bartter's syndrome with significantly decreased re-absorption of chloride at distal renal tubules, low level of serum potassium, a metabolic alkalosis, and high levels of aldosterone and renin. Bartter's syndrome associated with idiopathic carpotarsal osteolysis has not been published previously. The typical plantar nodule in this patient confirmed the presence of fibromatosis with aggressive histologic signs in light and electron microscopic examination.
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keywords = bone disease
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18/30. Disappearing bone disease: a case report.

    Disappearing bone disease is a rare condition and usually affects young adults. Its aetiology is not known. A case of a 12-year-old female child, who had a fall and sustained a fracture mid shaft of femur and supracondylar region, is reported. On follow-up subsequent X-ray showed extensive osteolysis which was progressively affecting the other side of the pelvic girdle and femur. She was put on trial of calcitonin 50 IU by nasal spray for six months but there was no improvement. However, during the past three and half years the disease process had progressively extended to the other side of pelvic girdle and femur with fatal outcome. To the knowledge of the authors such progression in a short time has not been reported in any case so far.
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ranking = 5
keywords = bone disease
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19/30. Gorham's disease or vanishing bone disease: plain film, CT, and MRI findings of two cases.

    Gorham's disease may develop in any region of the skeleton, but shows a predilection for the bones of the shoulders and pelvic girdles. Less frequently, the disease may involve the spine, and it is the proximity to the spinal cord which may worsen the patient's outcome. We report the clinical outcomes of two children with typical, yet differently localized involvement, and also review the plain film, CT and MRI findings.
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ranking = 4
keywords = bone disease
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20/30. Gorham's disease involving the thoracic skeleton. Plain films and CT in two cases.

    Two cases of Gorham's disease (massive osteolysis, idiopathic osteolysis, vanishing bone disease) involving the thoracic skeleton are reported. Both patients were adolescent females with involvement of multiple ribs. Involvement of the thoracic spine and pleural effusions--unusual manifestations carrying a poor prognosis--were also present. The radiographic and computed tomographic appearances are included with discussion and review of this disease entity.
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ranking = 1
keywords = bone disease
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