Cases reported "Calcinosis"

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1/7. Diffuse intervertebral disk calcification in a patient with rheumatoid arthritis.

    A patient with seronegative rheumatoid arthritis (RA) who presented with intervertebral disk calcification (IDC) of several thoracic and lumbar intervertebral disks in herein described. There was no evidence of any other coexisting diseases such as ochronosis and hemochromatosis, but a remarkable degree of polyclonal hypergammaglobulinemia was observed as a notable finding. Although the appearance of IDC on T1-weighted images on magnetic resonance is controversial, no increased signal intensity was observed in our patient. To the best of our knowledge, this is the first report of IDC in RA.
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ranking = 1
keywords = ochronosis
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2/7. Multiple vacuum discs, and early sign of ochronosis. Radiologic findings in two brothers.

    Two brothers with ochronosis are reported and the radiological features of ochronotic vertebral disease are reviewed. A striking feature was the presence of multiple vacuum discs. This "vacuum phenomenon" constitutes one of the earliest radiologic changes in the spine and presumably represents areas of severe degeneration within the intervertebral discs. Other features included a dark pigmentation of the urine after standing or following the addition of sodium hydroxide, and calcification within the cartilages of the ears and intervertebral discs. There was no abnormality of the peripheral joints and pigmentary deposits in the ears, sclerae, or skin were not observed.
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ranking = 5
keywords = ochronosis
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3/7. ochronosis and alkaptonuria: report of a new case with calcified aortic valve stenosis.

    A patient with calcified aortic valve disease secondary to ochronosis and alkaptonuria is presented. Urgent aortic valve replacement was necessary. The cardiovascular complications of this disease are reviewed.
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ranking = 1
keywords = ochronosis
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4/7. alkaptonuria.

    A case of alkaptonuria with its various sequelae in the form of ochronosis, ochronotic arthropathy, spondylitis and prostatic calculi is reported. The case is of interest as it presented with hepatocellular failure and hepatitis b surface antigenaemia.
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ranking = 1
keywords = ochronosis
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5/7. low back pain and ochronosis.

    We have examined the presentation of low back pain in three patients with ochronosis, and have reviewed the literature. low back pain and/or stiffness is the first presenting feature and usually appears after the age of 30. Discolouration of the ear is almost always present. Characteristic radiographic changes in the lumbar spine include calcification of the intervertebral discs and lucencies in the discs. The process gradually extends to the entire spine.
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ranking = 5
keywords = ochronosis
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6/7. Cardiovascular ochronosis.

    A 64-year-old man with alkaptonuric ochronosis required aortic valve replacement for severe aortic stenosis and single-vessel aortocoronary artery bypass grafting for a subtotally occluded obtuse marginal branch of the circumflex coronary artery. Operative findings included ochronosis of a partly calcified aortic valve and the aortic intima. The aortic valve and a punch biopsy specimen of the ascending aorta were removed at surgery and were studied with transmission electron microscopy and light microscopy. The ultrastructural studies of the aortic valve revealed intracellular and extracellular deposits of ochronotic pigment. A portion of the extracellular ochronotic pigment represented degenerated cells. Large deposits of extracellular ochronotic pigment were associated with areas of valvular calcification. Electron microscopic study of the aorta disclosed ochronotic pigment in macrophages and smooth-muscle cells. Aggregates of extracellular ochronotic pigment in the intima and media appeared to be in locations of necrotic cells. light microscopy also showed intracellular and extracellular deposits of ochronotic pigment. Our study suggests that extensive extracellular deposits of ochronotic pigment in the aortic valve may serve as a stimulus for dystrophic calcification. This may play a role in the development of aortic valve calcification and aortic stenosis associated with alkaptonuric ochronosis. To our knowledge, this is the first ultrastructural study of the aortic valve and aorta in alkaptonuric ochronosis.
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ranking = 8
keywords = ochronosis
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7/7. Tendon involvement in a case of ochronosis.

    A patient with severe skeletal and systemic involvement due to ochronosis is reported. On radiographic examination there was a linear density attached to the left lesser trochanter. The possible role of ochronotic pigment deposition in tendon, leading to metaplastic bone formation, is discussed.
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ranking = 5
keywords = ochronosis
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