Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0243026 (sepsis)
 52,417 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

We reported a 74-year-old woman with plasmacytoid lymphocytic lymphoma who initially presented with exertional dyspnea, conscious disturbance, ascites, and lytic skull lesions. Protein electrophoresis and immunoelectrophoresis showed monoclonal IgG-lambda gammopathy with IgG level of 13300 mg/dl and marked suppression of the residual normal immunoglobulins. Abdominal computed tomography (CT) revealed a pattern mimicked cancerous peritonitis. She responded to plasmapheresis with much clinical improvement of the hyperviscosity syndrome but died of H. influenza sepsis 3 weeks after diagnosis. The unusual and interesting features of this case included: (1) IgG hyperviscosity syndrome and diffuse peritoneal involvement as the initial manifestations in plasmacytoid lymphocytic lymphoma, and (2) presence of lytic bone lesions in conjunction with high level of M--component and marked suppression of normal residual immunoglobulins in a patient with lymphoma rather than multiple myeloma.
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PMID:Plasmacytoid lymphocytic lymphoma presenting with IgG hyperviscosity syndrome and peritoneal lymphomatosis. 764 Nov 9

Non-resolved chronic pulmonary thromboembolism is a frequent cause of pulmonary hypertension. In long-standing disease hypertension is progressive due to intimal and medial changes in the perfused vessels. Non-resolution of thromboemboli is often associated with underlying coagulopathies; the presence of a lupus anticoagulant may pose a significant problem in the peri-operative management of these patients. Pulmonary thrombendarterectomy presents an efficient option of treatment which is feasible in the majority of patients. By means of pulmonary angiography and computed tomography operability is verified by the often difficult recognition of thromboembolic changes in the central pulmonary arteries. Patients with solely peripheral thromboembolic changes or primary pulmonary hypertension must be excluded. In presence of significant exertional dyspnea and/or pulmonary pressure elevation surgery is indicated. Mortality is high and mainly related to unrelieved pulmonary hypertension or pulmonary complications; pulmonary reperfusion edema, respiratory failure or pneumonia and sepsis. In all survivors the reduction of pulmonary hypertension is highly significant and persistent. Thromboembolic pulmonary hypertension may be treated curatively in most patients by thrombendarterectomy. Correct selection of surgical candidates is mandatory, and the patients should preferably be diagnosed and undergo surgery in an early stage of their disease.
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PMID:[Surgical treatment of thromboembolism-induced pulmonary hypertension]. 786 94

A 26-year-old male who had been diagnosed as pulmonary tuberculosis three years ago with an antituberculous chemotherapy of only two months, complained of tiredness, exertional dyspnea and fever since a month ago. Bloody sputum, bloody stool and hematuria have developed three days before admission. Petechiae over the body trunk and lower extremities were observed on admission. Peripheral blood examination revealed lymphocytopenia (672/microliters), low hemoglobin content (6.2 g/dl), thrombocytopenia (3,000/microliters), elevated FDP (36.2 micrograms/ml) and D-dimer (25.0 micrograms/ml) values. Chest radiograph showed a massive pleural effusion in the right hemithorax, bilateral pulmonary infiltrates and a cavity on CT scan. Together with positive acid-fast bacilli in sputum, diagnoses of relapsed pulmonary tuberculosis, tuberculous pleurisy associated with DIC (disseminated intravascular coagulation) were made. Left hydronephrosis which was presumed to be a consequence of infundibulum stenosis due to renal tuberculosis, was detected by abdominal ultrasonography. Treatment with antituberculous drugs and protease inhibitors were started with thoracic tube drainage. DIC condition was improved by the 20th hospital day and sputum culture turned to be negative after the 4th week, however, fever up to 38 degrees C continued until the end of the 7th week and a D-dimer which is a representative marker for secondary fibrinolysis, continuously showed a high level up to the 10th week of hospitalization. The patient was uneventful during the three months follow up period after discharge. DIC is a well known complication of sepsis including miliary tuberculosis, whereas it is rarely associated with cavitary tuberculosis and no case of prolonged elevation of D-dimer have been reported.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of pulmonary, pleural, and renal tuberculosis associated with DIC and a prolonged increase in D-dimer]. 804 Oct 60

A 57-year-old male presented with palpitations and dyspnea on exertion. Examination of the peripheral blood and bone marrow showed pancytopenia with marked red cell aplasia. Hypogammaglobulinemia was also recognized. Chest X-ray and CT showed a mass in the anterior mediastinum. A biopsy showed thymoma. Two months after admission, the patient died of sepsis secondary to worsening pancytopenia and hypogammaglobulinemia. Autopsy showed non-invasive spindle cell type thymoma and a marked decrease of hematogenous cells. Review of the literature indicates that pancytopenia associated with thymoma is resistant to all forms of treatment and its prognosis is poor.
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PMID:[Autopsy case of non-invasive thymoma associated with pancytopenia and hypogammaglobulinemia]. 851 9

A retrospective analysis of patients with hypertrophic obstructive cardiomyopathy treated by left ventricular myotomy and myectomy from 1972 to 1994 is reported. There were 158 patients (81 male and 77 female) with a mean age of 50.2(+/-17.2) years (range 12 to 80 years). One hundred nine patients (69%) were 60 years of age or younger, and 49 patients (31%) were older than 60 years. The overall mean follow-up period was 6.1(+/-4.8) years (range 0.1 to 19.3 years) and was 94% complete with a cumulative total of 956 patient-years. Preoperative exertional dyspnea was present in 84%, chest pain in 70%, presyncope in 54%, syncope in 31%, and cardiac arrest in 5% of patients. Preoperative cardiac catheterization was done in 150 patients, with mitral regurgitation detected in 104 patients (67%). The average maximal provocable left ventricular outflow tract gradient was 118 (+/-46) mm Hg (range 25 to 250 mm Hg). The average preoperative echocardiographic gradient at rest was 64 mm Hg, 20 mm Hg in the early postoperative period and 10 mm Hg in the late postoperative period. The mean septal thickness was 2.2 (+/-0.6) cm, 1.9 (+/-0.7) cm in the early postoperative period (p < 0.05 vs preoperative) and 1.7 (+/- 0.5) cm in the late postoperative period (p < 0.05 vs preoperative). The overall 30-day operative mortality rate was 3.2% (5/158), and 0% for 109 patients 60 years of age or younger. Causes of death included myocardial infarction and left ventricular free wall rupture, myocardial failure from septal perforation, sepsis, cerebrovascular accident caused by thromboembolism, and delayed cardiac tamponade in one patient each. Concomitant coronary artery bypass grafting was performed in 22 patients (19.3% of patients > or = to 40 years of age) and mitral valve replacement in 5 patients (3.2%). One hundred nine patients (69%) are alive, 10 patients (6.3%) were lost to follow-up, and 39 patients died (24.7%), including operative deaths). Actuarial survivals at 1, 5, 10, and 15 years were 92.4% +/- 2.2%, 85.4% +/- 3.1%, 71.5 +/- 4.6%, and 46% +/- 9%, respectively. The overall linearized death rate for discharged patients was 1.9%/pt-yr, and for cardiac related deaths it was 1.7%/pt-yr. Thirty-nine (36%) of the 109 survivors received beta-adrenergic blockers, and 30 (28%) received calcium channel blockers. Ninety-four patients had improvement in New York Heart Association functional class, 10 had improvement in symptoms but not in functional class, and 5 had no improvement in functional class or symptoms. Neither preoperative hemodynamic values nor routine echocardiographic measurements significantly correlated with quality of postoperative results. Left ventricular myotomy and myectomy is a safe and reproducibly effective operative treatment for medically refractory hypertrophic obstructive cardiomyopathy, especially for patients 60 years of age or younger. Improvement in functional class and symptoms can be expected in nearly all patients 60 years of age or younger. Improvement in functional class and symptoms can be expected in nearly all patients. The results of myotomy and myectomy serve as a standard for comparison with other interventions for medically refractory cardiomyopathy.
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PMID:Long-term results of left ventricular myotomy and myectomy for obstructive hypertrophic cardiomyopathy. 860 73

Allopurinol is a xanthine oxidase inhibitor used in management of chronic gout. It acts by reducing the amount of uric acid by inhibiting purine metabolism. A middle-aged hypertensive female who was on allopurinol for 7 months presented with generalized weakness and exertional dyspnea. Investigations revealed pancytopenia: normocytic normochromic anemia (Hb-3.2g/dL, TLC-3400/mm3) and severe thrombocytopenia (Platelets-1000/mm3) with mild hepatosplenomegaly and grade 2 medico renal disease with normal cardiac status. Nutritional, hemolytic and infective causes were ruled out. She was transfused with fresh whole blood, platelets, administered empirical antibiotics and started on steroids. Initially, she responded to treatment but later developed an episode of convulsions with anuria and succumbed to leukopenic sepsis secondary to hypo/aplastic anemia probably due to allopurinol. Allopurinol is used extensively in the management of chronic gout and is well tolerated due to its safety profile. But we here report a case of allopurinol induced aplastic anemia leading to the demise of a patient. Allopurinol though safe needs careful monitoring.
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PMID:Allopurinol: Sorrow to the marrow. 3275 32