Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:4.1.2.13 (aldolase)
3,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 60-year-old woman was admitted to our hospital with a two month history of dry cough and dyspnea on exertion. A chest roentgenogram revealed diffuse interstitial shadows with a reduction of lower lung volume. Laboratory examinations revealed an increase in CPK and aldolase. There was decreased proximal muscle power, and the findings of a biopsy of the right deltoid were compatible with polymyositis. Myositic symptoms were stable, but the respiratory symptoms worsened, and an open lung biopsy was performed for diagnosis and to determine the best treatment. The histological findings of biopsy materials demonstrated active interstitial pneumonia complicated by cellular interstitial pneumonia, bronchiolitis obliterans organizing pneumonia, usual interstitial pneumonia and lymphoid hyperplasia. The patient responded well to adrenocorticosteroid and immunosuppressive therapy, and is now attending as an out patient. It is well known that PM-DM can be associated with interstitial pneumonia, and this complication is an important prognostic factor clinically. The pathological patterns of interstitial pneumonia in PM-DM may be divided into usual interstitial pneumonia and bronchiolitis obliterans organizing pneumonia. Furthermore, it is well documented that these patterns are concurrent with the response to adrenocorticosteroid and prognostic factors. However, our case of PM-DM, in which various patterns such as rheumatoid arthritis (RA) were pathologically revealed, cannot be considered as having uniform pathological pattern. We consider that pulmonary pathological patterns of PM-DM are very varied, as with RA. It is a very important to evaluate the nature of these patterns and the subsequent clinical course in PM-DM with interstitial pneumonia.
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PMID:[A case of interstitial pneumonia of polymyositis-dermatomyositis with various pathological findings in open lung biopsy]. 833 51

A 61-year-old woman was admitted to our hospital with a two week history of knee-joint pain, dry coughing and dyspnea on exertion. A chest roentgenogram revealed bilateral patchy and infiltrative shadows. Laboratory examination revealed high CK and aldolase levels. Although myositic symptoms were absent, the respiratory symptoms rapidly worsened and respiratory failure developed. An open-lung biopsy and a muscle biopsy were done. The open-lung biopsy specimen showed bronchiolitis obliterans organizing pneumonia (BOOP), but the muscle biopsy specimen revealed non-specific findings. After 4 weeks of intravenous intermittent high-dose corticosteroid therapy, 50mg of cyclophosphamide was given daily, along with 20mg of prednisolone on alternate days. There were marked clinical, physiological and roentgenographic improvements. A test for anti-Jo-1 antibody was positive, which suggested that this patients had pulmonary manifestations of polymyositis/dermatomyositis. Although BOOP can have an indolent course and a good prognosis, it may rapidly worsen and respiratory failure may develop, in which case it should be treated aggressively with a combination of intravenous high-dose corticosteroids and immunosuppressants.
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PMID:[Rapidly progressing BOOP in a patient with positive anti-Jo-1 antibody: response to corticosteroid pulse and immunosuppressant therapy]. 895 14

A 36-year-old man was admitted to our hospital complaining of cough, dyspnea on exertion, skin eruptions, and joint pain. Characteristic skin lesions such as erythema around the nails, telangiectasis, and edema of the eyelids were observed in this patient. He had never complained of muscle symptoms, and his laboratory examinations showed no elevation of either CPK or aldolase. From several lines of evidence including the skin biopsy findings, amyopathic dermatomyositis was diagnosed. Chest X-ray films showed subpleural funicular opacities and consolidation in both lower lung fields. Lung biopsy specimens taken under video-assisted thoracoscopic surgery revealed nonspecific interstitial pneumonia, group II. Oral prednisolone treatment was initiated at 60 mg daily together with oral cyclosporin A (100-150 mg daily). The minimum serum concentration of cyclosporin A was maintained between 100 and 200 ng/ml. Respiratory symptoms gradually improved, and the oral prednisolone dose was tapered off. Pulmonary function and chest CT findings showed marked improvement.
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PMID:[A case of nonspecific interstitial pneumonia associated with amyopathic dermatomyositis efficiently treated with a combination of cyclosporin A and prednisolone]. 1232 32