Cases reported "Osteosclerosis"

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11/99. 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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ranking = 1
keywords = osteosclerosis
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12/99. osteosclerosis in primary hyperparathyroidism.

    osteosclerosis in adults with primary hyperparathyoidism is rare; the usual skeletal manifestation, when presented, is diffuse osteropenia. We describe a patient with generalized osteosclerosis in association with primary hyperparathyroidism. The findings are documented by conventional and fine-detail radiography, absorptiometric bone mineral analysis, quantitative microradiography and histologic examination of bone. The unique features are contrasted with the manifestations recorded in a recently studied group of 87 hyperparathyroid patients. The data presented here support a causal relationship in this patient between parathyroid hormone excess and the development of densely sclerotic bones.
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ranking = 1
keywords = osteosclerosis
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13/99. MR imaging of medullary streaks in osteosclerosis: a case report.

    We present a case of medullary sclerosis of the appendicular skeleton in a patient with chronic renal insufficiency for whom MR imaging findings were characteristic. T1- and T2-weighted MR images showed multiple vertical lines (medullary streaks) of low signal intensity in the metaphyses and diaphyses of the distal femur and proximal tibia.
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ranking = 4
keywords = osteosclerosis
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14/99. syndrome of myelofibrosis and osteosclerosis: a series of case reports and review of the literature.

    Myelofibrosis (MF) is a chronic disorder characterized by bone marrow evidence of myeloid metaplasia associated with reactive fibrosis, angiogenesis, and osteosclerosis. We report serum biochemistry, noninvasive markers of bone turnover, tetracycline-labeled bone histomorphometry, and bone densitometry (DXA) data of four men presenting with newly diagnosed biopsy-proven MF and osteosclerosis. The mechanisms and putative growth factors responsible for this syndrome are examined.
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ranking = 6
keywords = osteosclerosis
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15/99. Differential diagnosis and treatment of autosomal dominant osteosclerosis of the mandible.

    A 20-year-old patient who presented with concerns about her large mandible was found to have a generalized mild cortical sclerosis. She was treated successfully with staged orthognathic surgery, despite the dense sclerosis of the jaws.
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ranking = 4
keywords = osteosclerosis
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16/99. Neuropathology of Raine syndrome.

    We present three cases of Raine syndrome occurring in siblings of consanguineous parents. Raine syndrome is characterised by generalised osteosclerosis with craniofacial anomalies and intracranial calcifications. So far, only nine cases have been reported, and no evaluation of the distribution and extent of the cerebral mineralisations, as well as their impact on the surrounding tissue, has been undertaken yet. In our cases, calcifications were unevenly distributed throughout the central nervous system, not associated with neuronal loss or dystrophic events and appeared mostly as single calcospherites within the neuropil with occasional confluent deposits at advanced gestational age. There was intense perifocal microgliosis around single immature calcospherites, as well as mild astrogliosis around and within the confluent lesions, in which occasional macrophages could be found. Rarely, mineralisations occurred in blood-vessel walls, mainly affecting basal ganglia. Preferential sites of calcification were parietal and occipital periventricular white matter and corpus callosum, while frontal lobes were mildly affected. The cortex, temporal lobes as well as internal capsule, brain stem, cerebellum, leptomeninges, pituitary gland and choroid plexus were devoid of mineralisations. The subcortical grey matter was moderately involved in the putamen and pallidum, mildly in the caudate nucleus and subependymal germ cell matrix and not at all in the thalamus, Ammon's horn, amygdala and substantia nigra. The distribution of mineral deposits was thus inversely correlated to regional blood circulation and capillary density, with calcifications being concentrated in more sparsely perfused areas but lacking in highly vascularised tissue. This inverse relationship between mineralisation and regional blood flow was reflected in the varying distribution of calcospherites in grey and white matter as well as in the white matter of different lobes.
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ranking = 1
keywords = osteosclerosis
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17/99. strontium-89: a novel treatment for a case of osteosclerotic myeloma associated with life-threatening neuropathy.

    Osteosclerotic myeloma is a rare disorder characterized by paraproteinaemia and osteosclerosis, and may be associated with a progressive peripheral neuropathy. patients with widespread osteosclerotic lesions can succumb from neurological complications despite systemic chemotherapy. We present a case of disseminated osteosclerotic myeloma associated with POEMS (peripheral neuropathy, organomegaly, endocrinopathy, M band, skin changes) syndrome, which was complicated by a rapidly progressive, life-threatening neuropathy. The patient's symptoms remained unchanged in the face of combination chemotherapy. However, a substantial improvement was seen following outpatient treatment with the commonly available radioisotope strontium 89 in combination with steroids.
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ranking = 1
keywords = osteosclerosis
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18/99. Diffuse osteosclerosis with lymphocytic lymphoma. A case report.

    A unique case of diffuse osteosclerosis of the hemopoietic skeleton in a patient with lymphocytic lymphoma is described. biopsy findings excluded a concurrent myeloproliferative disorder.
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ranking = 5
keywords = osteosclerosis
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19/99. Multiple periarticular osteosclerosis accompanied by joint destruction treated with bilateral total knee arthroplasty.

    This report documents the case of a 53-year-old man with multiple periarticular osteosclerosis in the bilateral wrists, elbows, knees, and ankles. He underwent bilateral total knee arthroplasty to relieve difficult walking as a result of osteosclerosis accompanied by joint destruction in both knees. Because the bones were abnormally hard and brittle, the start of the surgery caused fracture, so that considerable caution was necessary during the subsequent procedures. The patient's symptoms were thought to be associated with hemodialysis because he had been suffering from renal failure since 1989. Involvement of other associated diseases was also suspected because of the unusually formation, brittleness and crystallinity of the patient's bones. However, no definite diagnosis could be made for this patient.
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ranking = 6
keywords = osteosclerosis
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20/99. Bone densitometry of a patient with osteosclerosis.

    A 30-yr-old Caucasian man with a history of dorsal and lumbar back pain, which responded partially to antiinflammatory agents, was seen at our Unit. The biochemical bone markers showed an increment in bone alkaline phosphatase and urinary CTX. serum phosphate tended to be low. Radiographic abnormalities were marked osteosclerosis in the pelvis and vertebral bodies without changes in size. Bone scintigraphy results were normal. The increase in bone mineral density (BMD) was greater in L2-L4 ( 3.9 SDs) than in total skeleton ( 1.4 SDs). Analysis of skeletal subareas showed a marked increase in axial skeleton BMD: trunk, 4.0 SDs; spine, 2.5 SDs and pelvis, 4.5 SDs. BMD of the remaining subareas was found to be normal: skull, 0.04 SDs; arms, -0.3 SDs and legs, -0.05 SDs. The patient refused to have a bone biopsy. The radiologic, densitometric, and biochemical findings in the patient presented herein are compatible with axial osteomalacia. Evaluation of total skeleton BMD, and especially skeletal subareas, clearly indicated that the abnormal BMD was restricted to the spine and pelvis whereas the rest of the skeleton was not affected.
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ranking = 5
keywords = osteosclerosis
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