Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0011168 (dysphagia)
 15,644 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Leiomyosarcoma of the esophagus is a rare neoplasm. We reported a very rare case of esophageal leiomyosarcoma associated with pulmonary edema by the compression of the left atrium. A 67-year-old man was admitted with a 3-month history of chest pain, exertional dyspnea and dysphagia. Chest X-ray computed tomography showed posterior mediastinal tumor. Esophageogastroscopy and fiberoptic bronchofiberscopy showed no direct invasion of the tumor. By echocardiography, the left atrium was found to be compressed by the tumor and pulmonary hypertension (58/25 mmHg) and increased pulmonary wedge pressure (25 mmHg) was present. Open biopsy specimen demonstrated elongated cells suggestive of sarcoma. At autopsy, the tumor was confirmed to be leiomyosarcoma and to be originated from the lower esophagus.
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PMID:[Leiomyosarcoma of the esophagus associated with pulmonary edema by the compression of the left atrium]. 175 20

It is well known that patients with progressive systemic sclerosis (PSS), as well as other autoimmune disease, have various kinds of autoantibodies. We presented two PSS patients with anti Wa antibody, which had recently been reported as a new anti cytoplasmic antibody in only one case with PSS. Our first case, 49 years old female had clinical features of arthralgia, Raynaud's phenomenon, sclerodactyly, transient myalgia and sicca complex. Her laboratory findings showed hypergammaglobulinemia (1.9 g/dl), positive RAHA (1: 640) anti Wa antibody (1: 256), and anti SS-A antibody (1: 256). Second case, 64 years old female had also clinical features of Raynaud's phenomenon, sclerodactyly, dysphagia, dyspnea on exertion and dry mouth. Furthermore lung fibrosis and dysmobility of upper GI tract were observed. FANA (1: 20) was detected as nucleolar pattern, and the titer of anti Wa antibody was 1: 64. Clinical findings of myositis were not seen in both cases. This study suggested that anti Wa antibody might be one of the diagnostic marker on PSS.
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PMID:[Clinical study on two cases of progressive systemic sclerosis with anti Wa antibody]. 263 85

A patient with a DeBakey IIIb type dissecting aneurysm of the aorta was treated with Carpentier's method. But after surgery the thromboexclusion procedure at the pseudo-lumen was insufficient. Fourteen months after surgery the left lung area suddenly became opaque, suggesting the possibility of impending rupture of the aneurysm. Therefore the patient underwent emergency surgery in which another permanent clamp was attached to the peripheral end of the aneurysm. After this operation, the aneurysm became reduced in size and the left lung inflated again, but an aorto-pulmonary fistula was formed 24 days later. After 6 months after second surgery, the patient began to experience dysphagia and dyspnea on exertion. Detailed examinations showed that these symptoms were caused by compression of the esophagus, bronchus and pulmonary artery by the permanent clamp. That is, this clamp, which possibly moved during organic change and size reduction of the aneurysm, seems to have compressed the adjoining organs enough to cause dysfunction. Such a compression of the neighboring organs can be regarded as one of problems originating from Carpentier's method, and shows that the procedure is not problem free yet.
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PMID:[A clinical experience of signs of compression in the neighboring organs after operation with Carpentier's method in dissecting aneurysm of the aorta]. 277 39

Scleromyxedema is a rare fibromucinous connective tissue disorder characterized by papular skin lesions associated with sclerosis and a serum monoclonal gammopathy. Little is known about either the natural history or the systemic manifestations of this disease. We reviewed the medical records of 19 patients with biopsy-proven scleromyxedema seen from 1950 to 1985 for evidence of systemic disease. There were 10 males and 9 females with a median age at diagnosis of 53 years. Monoclonal gammopathy was present in 13 patients. Eight patients complained of dysphagia; 3 had proximal esophageal dysfunction and 1 had total esophageal aperistalsis on barium swallow. Proximal muscle weakness was noted in 5, with an inflammatory myopathy in 3. Six patients complained of dyspnea on exertion. Of these, 5 had reduced diffusing capacity, 3 had reduced volumes, and 2 developed cor pulmonale. Pathologic changes characteristic of "scleroderma kidney" were demonstrated in 1 patient at postmortem. One patient had Raynaud's phenomenon and 2 had arthralgias/arthritis with noninflammatory synovial fluids. Although 8 of 12 patients treated with melphalan noted regression of their skin changes, no consistent improvement in the extracutaneous manifestations was demonstrated. Furthermore, 2 patients died of sepsis related to melphalan-induced myelosuppression, and 4 developed hematological malignancies following melphalan therapy. In conclusion, systemic manifestations in scleromyxedema are more prevalent than previously recognized, and can resemble those of scleroderma. Significant toxicity occurred with the use of alkylating agents in these patients, with treatment-related complications developing in 45% of patients treated with melphalan. The lack of definitive data regarding the natural history of this disease complicates the question of optimal therapy, but the use of alkylating agents should be reserved for those patients with severe debilitating skin disease.
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PMID:Scleromyxedema: a scleroderma-like disorder with systemic manifestations. 333 81

Solitary extramedullary plasmacytomas are uncommon neoplasms. They occur most frequently in the upper aerodigestive tract and account for 4% of the nonepithelial tumors in this site. The evolution of a plasmacytoma is unsteady and symptoms at presentation have included dystonia, dysphagia, oral pain, cough, and dyspnea on exertion. Plasmacytoma of the upper aerodigestive tract has not been previously reported as a cause of obstructive sleep apnea.
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PMID:Plasmacytoma as a cause of obstructive sleep apnea. 876 30

A 68-year-old white woman was referred to us by her rheumatologist for possible participation in a clinical study of photopheresis for scleroderma. In February 1993, she noticed edema of her distal phalanges, Raynaud's phenomenon in both hands, flu-like symptoms, fatigue, intermittent diarrhea, abdominal pain, tearing in both eyes, dyspnea on exertion, dysphagia, and odynophagia. Bilateral silicone-gel breast implants had been placed 12 years before; 2 months before her present evaluation, they were removed and found to be ruptured. Physical examination revealed edema, limited to the fingers and hands bilaterally, and slight induration of the skin on the dorsum of both hands and distal forearms. The remainder of the physical examination was normal. According to our study protocol, a skin biopsy specimen from the dorsum of the right hand was taken, but all other laboratory investigations were refused. Histopathologic examination revealed multiple clear spaces of varying sizes in the dermis and multinucleated macrophages containing small refractile particles, characteristic of silicone granuloma (Figs. 1 and 2); however, the specimen showed no evidence of scleroderma. X-ray energy dispersive analysis by scanning electron-microscopy confirmed the presence of elemental silicon in the small refractile particles. The patient did not receive any treatment after her diagnosis and shortly thereafter, she was lost to follow-up.
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PMID:Silicone granuloma in acral skin in a patient with silicone-gel breast implants and systemic sclerosis. 883 27

This is the first report on idiopathic inflammatory myopathies (IIM) in French Canadians. We reviewed retrospectively 30 French Canadian adults (20 women and 10 men) with IIM seen consecutively over 12 years. The median age at diagnosis was 45 years. The IIM were 8 (27%) primary polymyositis (PM), 9 (30%) primary dermatomyositis (DM), 5 (17%) IIM with neoplasia (lymphoma, breast, esophageal, colonic, and skin cancer) and 8 (27%) IIM with a connective tissue disease (4 with systemic sclerosis, 2 with mixed connective tissue disease, and 2 with rheumatoid arthritis). The most common presenting symptom was proximal muscle weakness (n = 10,33%). Of the remaining 20 patients, 6 (20%) had the onset of their weakness within 1 month of the presenting symptom. Only 3 (10%) patients did not have proximal muscle weakness. Twenty-six (87%) patients had weakness in the pelvic girdle, 25 (83%) in the shoulder girdle, and 7 (23%) in the neck muscles. Other common symptoms included dyspnea on exertion and dysphagia, each present in 13 (43%) patients. Gottron's papules and the heliotrope rash were the most common skin lesions documented in 11 (37%) and 10 (33%) patients, respectively. The serum creatine kinase (CK) level was between 171 and 1,000 U/L in 13 (43%) patients and between 1,001 and 6,000 U/L in 13 (43%) patients. Antinuclear antibodies (ANA) on HEp-2 cells were positive in 16 (53%) patients, of which 2 (13%) expressed autoantibodies to nuclear pore complexes. Autoantibody specificities were anti-La (n = 4, 13%), anti-U1RNP (n = 3, 10%), and anti-Ro (n = 2, 7%). None of the patients expressed anti-Jo-1, anti-topoisomerase I, or anticentromere antibodies. Twenty-eight (93%) patients received corticosteroid therapy, and 8 (27%) patients responded to prednisone alone. Thirteen (43%) patients were treated with methotrexate, and 9 (69%) responded. The mean follow-up was 62 months: 23 (77%) had their disease controlled, 3 (10%) patients were lost to follow-up, and 4 (13%) died (no death occurred because of IIM or its treatment). Therapy was discontinued because of remission in 5 (17%) patients. Cumulative survival rates at 2, 5, and 10 years were 89%, 89%, and 85%, respectively. The presence of autoantibodies to nuclear pore complexes and anti-La autoantibodies, the rare occurrence of anti-Jo-1 autoantibodies, the response to conventional therapies, and a high survival rate may distinguish IIM in French Canadians from that of other reported series.
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PMID:Distinctive features of idiopathic inflammatory myopathies in French Canadians. 887 Jan 12

A 52-year-old man presented with a four-month history of malaise, low-grade fever, decreased appetite, and a 20-pound weight loss. He complained of joint pain and swelling, proximal muscle weakness, exertional dyspnea, and a dry cough. He also noted that his fingers had turned white and then blue when chilled and red when rewarmed. He had not had pleuritic chest pain, dysphagia, dry eyes or mouth, rash, or skin photosensitivity.
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PMID:A man with vague rheumatic complaints. 975 May 51

A 61-year-old woman in excellent health and taking no medications presented with progressive dyspnea on exertion. She did not exhibit diplopia, dysphagia, dysarthria or muscle weakness. Her condition deteriorated, and respiratory distress developed. The patient was intubated and placed on mechanical ventilation. A computed tomogram of her chest revealed a moderately enlarged thymus gland unsuspected on a plain x-ray film. Myasthenia gravis was diagnosed on the basis of a high acetylcholine receptor antibody titer. Treatment started with prednisolone, anticholinesterase agent, and plasma exchange. The patient underwent a thymectomy 3 weeks after her diagnosis. Her symptoms were brought under control by anticholinesterase agent and prednisolone. This case illustrates the need to consider myasthenia gravis as well as other motor-neuron disorders when evaluating individuals presenting acute respiratory failure of unknown origin.
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PMID:[Myasthenia gravis presenting as isolated respiratory failure]. 989 33

Vascular rings have been well documented to cause respiratory and gastrointestinal symptoms in infants and children. Few reports document symptomatic vascular rings in adults, and most have emphasized dysphagia as the predominant symptom. We present the case of a 36-year-old white male with a double aortic arch and progressive dyspnea on exertion. This led us to review previous reports of vascular rings in adults. Criteria for review consisted of anatomically complete vascular rings of the aortic arch in adults age 18 years or older. We identified 25 prior cases for review and included our recent patient. The most common vascular ring anomalies in our review of adults is double aortic arch (n = 12; 46%) followed by right aortic arch with aberrant left subclavian artery and ligamentum arteriosum (n = 8; 30%). Of 24 patients (66%), 16 were symptomatic. Reported symptoms involving the respiratory tract (n = 10 of 24; 42%) included dyspnea on exertion (n = 5), bronchitis (n = 2), recurrent pneumonia, stridor, and unspecified respiratory ailment (n = 1 each). Dysphagia was less common, occurring in eight patients (33%). Previously proposed mechanisms for respiratory tract symptoms include tracheomalacia, static or dynamic compression of the airways, intravascular volume infusion, and aspiration. We also propose exercise-induced dilatation of the aortic arch and age-dependent changes in thoracic compliance as potential mechanisms of dyspnea.
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PMID:Vascular rings of the thoracic aorta in adults. 1055 60


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