Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0011633 (dermatomyositis)
 4,181 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A Caucasian male developed florid dermatomyositis documented by serum enzyme elevation, electromyography, and histology of skin and muscle. Serum enzymes, including creatine phosphokinase (CPK), aldolase, glutamic oxaloacetic transaminase (SGOT), and lactic dehydrogenase (LDH), decreased initially during high dose systemic corticosteroid therapy, although profound muscle weakness persisted. Subsequent elevation of serum LDH and SGOT levels during treatment provided a clue to underlying neoplasia. Primary hepatoma with widespread metastases was found at necropsy.
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PMID:Aberrant serum enzyme patterns in dermatomyositis associated with hepatoma. 18 84

We conducted a statistical review of 114 cases of dermatomyositis (DMS) treated primarily at the Department of Dermatology at Nagoya University Hospital over 27 years from 1965 to 1991 in order to determine the primary characteristics of juvenile DMS with the following results. 1) Juvenile DMS was found slightly more often in males than females; the male-to-female ratio was 1.4:1. Therefore, unlike adult DMS with its preponderance of females, there was no clear gender predominance. 2) Muscular manifestations tended to follow the appearance of cutaneous manifestations, but the frequency of minor muscular manifestations was high over the entire course of the disease. 3) Laboratory findings showed increases in serum aldolase and serum creatinine kinase with significant frequency when compared with adult patients (p < 0.01 and p < 0.05, respectively). Elevated serum aldolase most often occurred prior to or at the time of the appearance of muscular manifestations, suggesting its usefulness in early diagnosis. The positive rates for the antinuclear antibody on HEp-2 cells and anti-DNA antibody were significantly lower in children than in adults (p < 0.001 and p < 0.05, respectively). 4) There were no cases of juvenile DMS complicated by malignant tumors, interstitial pneumonia, or pulmonary fibrosis. There were also no deaths, and the rate of "remission or improvement" was significantly higher than in adult DMS cases (p < 0.05). Adult cases which remained the same or worsened usually presented with intractable muscular manifestations. In children, however, the cutaneous manifestations were more difficult to treat.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Juvenile dermatomyositis: a statistical study of 114 patients with dermatomyositis. 140 7

A case of dermatomyositis (DM) associated with thymic cyst with lymphoid follicle formations has been reported. A 57-year-old man developed polyarthralgia, muscle weakness, heliotrope rash and Gottron's sign. Laboratory findings showed elevated values of creatine-phosphokinase, aldolase and transaminase. He was diagnosed as DM and was treated with prednisolone 50 mg/day. Muscle weakness was improved immediately, although rash persisted unchangeably. He was examined for coexistence of malignant tumor which had been reported frequently as associated with DM. Enlargement of thymus was found by computer tomography and then thymectomy was performed. In his thymus, lymphoid follicle formations, which are as often encountered in other autoimmune diseases, were found. So far, the beneficial effect of thymectomy on the improvement of immunological abnormalities not only in patients with myasthenia gravis but also in patients with other autoimmune diseases has been recognized. In this case, intractable rash in DM improved after thymectomy. Further study needs to clarify the relationships between DM and thymic abnormalities.
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PMID:[A case of dermatomyositis associated with thymic abnormalities]. 159 6

Statistical observation about the clinical items of 105 patients with dermatomyositis (DMS) attending Department of Dermatology, Nagoya University Hospital and some hospitals in Aichi Prefecture, during the year of 1965 to 1989, was carried out. Clinical features of the patients with juvenile DMS obtained from the observation were as follows. 1) The male:female ratio was 1.3:1 in juvenile DMS. The evident difference such as the predominance of females in adult DMS was not found. 2) The tendency that cutaneous manifestations usually preceded muscular manifestations was observed, and the muscular manifestations except severe symptoms were seen with a high incidence throughout the entire clinical course. 3) In the laboratory examinations, the incidence of elevation of serum aldolase concentrations in children was significantly higher than that in adults (p less than 0.05). Serum aldolase concentrations were usually elevated at onset or prior to the onset of muscular manifestations. Therefore the measurement of serum aldolase levels was considered to be useful for early diagnosis of juvenile DMS. The positive rate of antinuclear antibody in children was significantly lower than that in adults (p less than 0.001). 4) None of the children had any complications such as malignant tumors, interstitial pneumonia and pulmonary fibrosis, and none of them died. With regard to the outcome, the incidence of "remission or improvement" in children was significantly higher than that in adults (p less than 0.05). In the group of "same or worse", the children mainly had the cutaneous manifestations which were difficult to treat, compared with the adults. These results suggest that juvenile DMS may be a different disorder, probably a syndrome, from adult DMS.
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PMID:[Juvenile dermatomyositis--statistical observation of 105 patients with dermatomyositis]. 194 86

An effort was made to identify all patients with polymyositis/dermatomyositis (PM/DM) admitted to hospitals in Israel from 1956-1976. The diagnosis of PM/DM was retrospectively reviewed in 92 (46 definite, 26 probable, and 20 possible) cases. The most common complaints and physical findings in the course of the disease were muscle weakness (86 patients), rash (53 patients), arthritis or arthralgia (39 patients), and dysphagia (35 patients). Elevated serum aldolase levels were found in 64% of the patients for whom data were available; 92% had abnormal electromyogram results, and 60.9% had muscle histopathology consistent with PM/DM. Malignancy was diagnosed in 13 patients. Malignancy, ischemic heart disease, and pulmonary complications were the most common causes of death. The actuarial survival curve was heterogeneous, with an accelerated mortality during the first year after diagnosis and a slower mortality during the following 7 years. Independent unfavorable prognostic signs were: failure to induce remission, leukocytosis, fever, older age, a shorter disease history, and dysphagia.
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PMID:Prognostic factors in polymyositis/dermatomyositis. A computer-assisted analysis of ninety-two cases. 397 73

An increased frequency of diffuse interstitial lung disease (DILD) was found in 7 of 15 cases of polymyositis and dermatomyositis seen in a 7-yr period. Affected patients had higher values of creatine phosphokinase and aldolase and more severe muscle necrosis than 8 patients with DILD. Early corticosteroid therapy sufficient to normalize enzymes apparently led to better resolutions of DILD. Active muscle necrosis seemed to be related to the development of DILD.
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PMID:Interstitial lung disease in polymyositis and dermatomyositis. 696 22

A 54-yr-old man was admitted to Hokkaido University Hospital, complaining of fever, multiple arthralgia, edematous erythema and face and muscular weakness of extremities during the last 2 months. He was diagnosed as dermatomyositis by acceleration of ESR, elevation of GOT, GPT, CPK, aldolase, moderate increases of collagen fibers in biopsy specimen of skin and his clinical signs. Although stools were positive for occult blood, the routine radiographic examination failed to detect the bleeding site in the upper GI. tract. However, in the double contrast picture of the stomach, a very fine abnormal linear shadow was observed at the upper corpus of the lesser curvature. This linear shadow was a margin of the tumor, retrospectively. About 4 months later, abnormal pain occurred and a mass was palpable in the left lumbar region, suggesting a pancreatic tumor. He was operated on excising the tumor, but was performed only exploratory laparotomy because of the presence of intra-abdominal metastases. Death occurred 40 days after the operation and necropsy was done. The gross anatomical findings of the abdomen showed a stomach tumor as large as an infant's head and its metastases to pancreas, lymph nodes, and greater and lesser omentum. Esophageal mucosa including esophagocardiac junction was intact. Histological examination of the intragastric tumor revealed a typical squamous cell carcinoma with keratinization. According to the absence of the components of adenocarcinoma and squamous metaplastic gastric mucosa of non-cancerous areas in the stomach, it seemed likely to be a heterotopic squamous cell carcinoma. It was unknown about the precedence between the stomach cancer and dermatomyositis. There have been 11 cases of primary pure squamous cell carcinoma in the world literature since 1968, but this is the first case report of coexistence of these two diseases.
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PMID:[A case report of a primary pure squamous cell carcinoma of the stomach associated with dermatomyositis (author's transl)]. 726 22

We attempt to correlate the patient's disability and serum enzymes (creatinekinase, lactic dehydrogenase, aldolase, glutamic oxalacetic and glutamic piruvic transaminase) in several neuromuscular disorders using the Vignos and Archibald scale (V&A). In 806 cases we studied, serum enzyme levels and the V&A disability using a computer for Pearson's correlation and regressive analysis. A good correlation of the V&A with age suggested a progressive evolution (increased disability) in Duchenne muscular dystrophy, fascioscapulohumeral dystrophy, myotonic dystrophy, myopathies due to respiratory chain enzyme deficiency and amyotrophic lateral sclerosis. A negative correlation (decrease disability with age) was found for multicore myopathy, benign myopathy of childhood with type 1 predominance, carnitine myopathy deficiency and dermatomyositis. It was found a correlation (p < 0.05) of the V&A and the level of specific serum enzymes with Duchenne muscular dystrophy, oculocraniosomatic dystrophies, polymyositis and polyarteritis nodosa. Using regression analysis, we found a weak interrelation between serum enzymes studied simultaneously and the V&A. These weak relations suggest some limitation in the long term use of the five serum enzymes in the evaluation of neuromuscular disorders when compared with V&A; although they are very important in the diagnosis.
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PMID:[Correlation between functional disability, age, and serum enzymes in neuromuscular diseases]. 757 10

Dermatomyositis (DM) developed in a 35-year-old woman after 2 months of psoralen ultraviolet-A (PUVA) therapy for presumed psoriasis. Her disease was characterized by symmetrical proximal muscle weakness and cutaneous lesions compatible with DM. In addition, laboratory abnormalities included a positive antinuclear antibody, and elevated levels of creatine kinase and aldolase. This is the first report of DM developing during PUVA therapy.
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PMID:Dermatomyositis occurring during psoralen A (PUVA) therapy. 818 50

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


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