Cases reported "Osteosclerosis"

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1/48. erdheim-chester disease: a case report.

    A 42-year-old man with erdheim-chester disease (EC) is presented. This is the first case of this disease reported in korea. The patient complained of knee pain and plain roentgenogram of the bilateral legs revealed diffusely increased density, coarsened trabecular pattern, and cortical thickening in the diaphysis, and metaphysis as well as epiphysis. magnetic resonance imaging revealed that the lesions showed low signal intensity on T1-weighted images and heterogeneously low and high signal intensity on T2-weighted images. Histological examination of the biopsy specimen showed a xanthogranulomatous lesion consisting aggregations of foamy histiocytes and Touton-type giant cells. Immunohistochemical staining showed positive reaction to anti-S-100 and lysozyme in the cytoplasm of the giant cells.
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2/48. Erdheim Chester disease: a rare cause of knee and leg pain.

    A case of Erdheim Chester disease in a 51-year-old Turkish patient is described. Erdheim Chester disease is a rare form of lipoid granulomatosis. knee and leg pain are the most common symptoms, and physicians working in orthopaedics and traumatology are the first to be consulted. Our patient demonstrated a typical bilateral, symmetric sclerosis of the metaphyseal region of long bones of the lower extremity, histologic examination revealed foamy, lipid-loaded histiocytes. The patient also suffered from arterial hypertension, diabetes insipidus and exophthalmos of the left eye. The diagnosis was confirmed by a bone biopsy, and the patient was treated with non-steroidal anti-inflammatory drugs, corticosteroids and vincristine.
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3/48. Monostotic fibrous dysplasia of the sphenoid sinus: a serendipitous finding on a bone scan.

    A 22-year-old woman had a Tc-99m MDP whole-body scan for low back pain. A focal area of increased activity was seen in the skull base in the region of the sella turcica. A computed tomographic examination showed ground-glass opacification of the sphenoid sinus and bony sclerosis along its walls, characteristic of fibrous dysplasia. Monostotic fibrous dysplasia, the more common form compared with the polyostotic variety, occurs in 70% to 80% of all patients with fibrous dysplasia. Monostotic lesions usually involve the ribs, femur, tibia, cranium, maxilla, and mandible. The frontal and sphenoid bones are the cranial bones most commonly involved.
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keywords = back pain, pain, back
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4/48. sarcoidosis presenting with large vessel vasculitis and osteosclerosis-related bone and joint pain.

    A 34-year-old African-American female diagnosed earlier with idiopathic thrombocytopenic purpura (ITP), lymphadenopathy, splenomegaly, uveitis, and pulmonary nodules, developed a subclavian artery aneurysm, and generalized annular osteosclerotic lesions with disabling arthralgias. Biopsies from bone and lymph node revealed non-caseating granulomas and no evidence of malignancy or infection, confirming the clinical impression of sarcoidosis.
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5/48. Rapidly destructive hip disease: a case report and review of the literature.

    Rapidly destructive hip disease (RDHD) is an arthropathy of unknown cause that involves rapid and total deterioration of both the acetabular and femoral aspects of the hip joint. We report a case in which radiographs taken 6 weeks apart vividly and poignantly demonstrate the rapidity of the disease. Because there is little in the orthopedic literature regarding RDHD, we would like orthopedic surgeons to be aware of the condition and the importance of repeat radiographs for patients with continued severe hip pain without an apparent cause.
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6/48. Expansile skeletal hyperphosphatasia: a new familial metabolic bone disease.

    We describe a new familial metabolic bone disease characterized by expanding hyperostotic long bones, early onset deafness, premature tooth loss, and episodic hypercalcemia. The condition affects a mother and daughter studied at the age of 36 years and 11 years, respectively. Both individuals lost all hearing in early childhood and suffered premature shedding of teeth. Skeletal pains began just before puberty. Swelling and aching of most middle phalanges in the hands is an especially troublesome manifestation. The mother also had episodes of symptomatic hypercalcemia first documented in late childhood and subsequently during intercurrent illness and postpartum lactation. Radiographs show hyperostosis and/or osteosclerosis predominantly in the skull and appendicular skeleton. Long bones also are expanded considerably, especially the middle phalanges in the fingers. The mother's skeletal abnormalities are more severe. Biochemical parameters of bone turnover, including serum alkaline phosphatase (ALP) activity, are elevated substantially. In the proposita, dynamic histomorphometry of nondecalcified sections of iliac crest revealed rapid skeletal remodeling. In the mother, who had been treated with bisphosphonates, electron microscopy (EM) showed disorganized collagen bundles as well as necrotic and apoptotic bone cells but no osteocytic osteolysis. measles virus gene transcripts were not detected in peripheral blood monocytes. karyotyping was normal, 46,XX. Hyperphosphatasia with bone disease previously has been reported as either a sporadic or autosomal recessive condition. Expansile skeletal hyperphosphatasia (ESH) is probably inherited as an autosomal dominant trait with a high degree of penetrance.
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7/48. Gelatinous bone marrow transformation in a case of idiopathic myelofibrosis: a morphological paradox.

    A 74-year-old woman with severe, but asymptomatic weight loss was found to have splenomegaly, thrombocytosis, and neutrocytosis. A trephine biopsy showed a hypercellular bone marrow with a proliferation of abnormal megakaryocytes, mild reticulin fibrosis, and osteosclerosis, i.e., features of an early stage of idiopathic myelofibrosis. In contrast to this predominant pattern, the marrow also contained a distinct hypocellular focus of gelatinous transformation (GMT) in which hematopoietic and fat cells were replaced by gelatinous substances that were characterized as hyaluronic acid mucopolysaccharides histochemically. GMT is a rare disorder of unknown pathogenesis, and is an unspecific indicator of severe illness which most often occurs in patients with a background of weight loss. This is the first reported case of gelatinous changes in an otherwise fibrotic bone marrow, a constellation which a priori seemed to be incompatible.
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keywords = back
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8/48. Calvarial sclerosing osteomyelitis.

    We report a 15-year-old boy who suffered from calvarial sclerosing osteomyelitis and presented with painful head swelling. x-rays of the skull revealed areas of irregular radiolucency. MR imaging and CT showed a well-demarcated intradiploic lesion with thickening of the skull extending from the frontal to the parietal calvarium with a low signal on T1-weighted images, strong but heterogeneous enhancement after gadolinium application and a mixed signal on T2-weighted images. Computer-navigated neurosurgery was planned, and the craniotomy defect was reconstructed by a preformed titanium implant. Sclerosing osteomyelitis of the calvarium has to be included in the differential diagnosis of osteolytic and sclerosing lesions of the skull coinciding with persistent swelling of the head.
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9/48. An unusual form of actinomycosis of the mandible with a resultant gross sequester in a 4-year-old child: a case report.

    Mandibular osteomyelitis due to actinomyces group is considered rare in the pediatric population. The initial complaint of the 4-year-old child described here was increased swelling of his cheek and pain. The patient was managed successfully by surgical treatment with antibiotic therapy.
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10/48. osteosarcoma arising in a femur with melorheostosis and osteopathia striata.

    Osteopathia striata is an asymptomatic autosomal dominant or sporadically inherited disorder that causes dense striations at sites of endochondral bone formation, with a predilection for the metaphyses of long bones. melorheostosis is a mixed sclerosing dysplasia with disturbance of both endochondral and intramembranous ossification, in which disordered intramembranous ossification dominates. It presents typical radiological changes of cortical hyperostosis distributed along a sclerotome with variable associated cutaneous and clinical features. Overlap syndromes including one or more of these diseases are described. We report a 44-year-old man with both melorheostosis and osteopathia striata who presented with pain secondary to superimposed osteosarcoma. In reporting this case we discuss the relationship between sclerosing dysplasia and either coexisting or complicating sarcoma.
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