Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 51-year-old woman, originating from Algeria, developed Q fever endocarditis on porcine bioprosthetic mitral valve. She had chronic course with nonspecific symptoms, such as dyspnea and fever, hepatosplenomegaly, and developed progressive cardiac failure. Worsening of hemodynamic state led to prosthetic valve replacement. Hemocultures were all negative, and the diagnosis of Q fever was unexpectedly performed by systematic screening for specific serum antibody to Coxiella burnetti. High phase I and II specific IgG and IgA antibody titers against C burnetii were found. The patient was treated by doxycycline and ofloxacin, and improved rapidly. Prosthetic valve constitutes likely predisposing factor for the development of chronic Q fever endocarditis. This observation emphasizes the need to search for Q fever in prosthetic valve dysfunction, in particular when hemocultures are negative.
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PMID:[Coxiella burnetii endocarditis on a bioprosthetic valve: review of the literature apropos of a case]. 819 Nov 3

A 76-year-old man was admitted to our hospital because of productive cough and dyspnea. Chest X-ray revealed cardiomegaly, bilateral pleural effusion, mediastinal lymphadenopathy, and marked bilateral hilar lymphadenopathy (BHL). His symptoms improved after therapy including diuretic for heart failure, but BHL was unchanged. M-protein in both serum and pleural effusion, and B-J protein in urine were detected. Protein immunoelectrophoresis demonstrated a heterogeneous increase in IgA with lambda light chain predominance. Bronchoscopy disclosed some prominent mucosal lesions of the bilateral bronchi, of which biopsies demonstrated amyloid deposition. Gastric mucosal biopsy also demonstrated amyloid deposition. Therefore this case was diagnosed as systemic primary amyloidosis due to AL-protein. In systemic amyloidosis, there are only a few reports of lymph node enlargement due to amyloid deposition, and it is particularly rare with BHL. From the presence of M-protein in pleural effusion, it is suggested that not only congestive heart failure due to cardiac amyloidosis but also amyloid deposition in the pleura would be related to the fluid production.
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PMID:[A case of systemic primary amyloidosis with BHL and M-protein in pleural effusion]. 833 50

A 77-year-old woman with hypertension was admitted to our hospital because of exertional dyspnea end peripheral edema. Chest X-ray showed cardiomegaly, pulmonary congestion and right pleural effusion. Hypertensive heart failure was diagnosed and treated, and right pleural effusion disappeared in 2 weeks. Abnormalities on laboratory data, i.e. anemia and increased ESR et al. continued after the improvement of heart failure. Serum IgG was elevated (2570 mg/dl), while IgA and IgM were decreased. Immunoelectrophoresis indicated the presence of monoclonal IgG-lambda in the serum. Bone marrow puncture revealed an increase in atypical plasma cells (38.4%). Multiple myeloma was diagnosed from these findings and treated with melphalan and prednisolone. But increases in atypical plasma cells (43.2%) and serum IgG (2573 mg/dl) continued. During treatment, right pleural effusion increased again. Thoracocentesis showed bloody effusion with numerous atypical plasma cells, and the presence of monoclonal IgG-lambda was indicated by immunoelectrophoresis. The patient died of renal and heart failure 2 months after the onset of malignant pleural effusion. Cytological examination and immunoelectrophoresis are necessary for pleural effusion in multiple myeloma.
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PMID:[A case of multiple myeloma associated with abnormal plasma cells and M-protein in pleural effusion]. 864 97

In order to examine the clinical characteristics and genetic background of secondary amyloidosis associated with rheumatoid arthritis, we analyzed clinical features and HLA typing of 85 patients in a multicenter study. Eighty-five patients with secondary amyloidosis associated RA were studied. The diagnosis of secondary amyloidosis were made on histological findings by biopsy or autopsy. The most common biopsy site was gastrointestinal tract (79.5%). Clinical symptom and the frequency at the time of diagnosis were; diarrhea (35 cases), abdominal pain (22 cases) and vomiting and nausea (16 cases). Abnormalities and the frequency in a laboratory test included proteinuria (49 cases), increased serum creatinine (32 cases), anemia (30 cases) and hematuria (15 cases). Twenty-eight patients were dead and 57 patients were alive at the time of the study. The average duration between diagnosis of amyloidosis and death was 19.4 +/- 18.5 (SD) months among the dead patients. The average duration after diagnosis of amyloidosis was 24.2 +/- 19.5 (SD) months in surviving patients. The causes of death were renal failure complicated with heart failure (6 patients), heart failure alone (3 patients) and renal failure alone (2 patients). Fifty-nine patients in the control group who were negative to amyloid deposition on biopsies at more than one site in the gastrointestinal tract, were clinically compared with patients in the amyloidosis group. No difference were noted in the age of RA occurrence and the stage between the two groups. As to the class, however, the number of patients with severe functional disorder (class 3 or severe) was larger in the amyloidosis group. There were no significant difference between the two groups in Lansbury's activity index. On hematology, biochemistry and urinalysis, the incidences of increased white blood cell count, anemia, increased platelet count, increased serum creatinine, hypoproteinemia, hypoalbuminemia, increased IgA, and increased urine and blood BMG were statistically significantly higher in the amyloidosis group than in the control group. HLA-A, -B, -C, and DR-locus antigens were compared in the 53 patients in the amyloidosis group and in the 59 subjects in the control group. There were no significant differences in frequency of HLA-A, and -B antigens between two groups. Frequency of CW7 antigen was significantly decreased in the amyloidosis group (13.2%) than in the control group (39.0%). Frequency of DR1 antigen was decreased in the amyloidosis group (3.8%) than in the control group (22.0%), although the difference was not significant. These findings suggest the possible involvement of genetic factors in the occurrence of amyloidosis. It is suggested that the occurrence of amyloidosis is suppressed by some genes which are linked with CW7 antigen.
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PMID:[Clinical characteristics and genetic background of secondary amyloidosis associated with rheumatoid arthritis in Japanese]. 871 35

Valve detachment or pseudoaneurysm is a well known complication of surgical treatment for cardiovascular disease due to aortitis syndrome. Although the preoperative management of inflammation by steroid therapy is very important, occasionally, the operation in active phase might be recommended because of progressive heart failure or impending rupture of aortic aneurysm. The pathology of aortitis syndrome is related to immunological abnormality, but there has been little information concerning the immunological factors. In order to evaluate the relationship between immunological factors and surgical results in patients with aortitis syndrome, immunological examination was obtained before surgery in 12 patients. Cardiovascular lesions due to aortitis syndrome were aortic regurgitation in 6 patients, annuloaortic ectasia in 1 patient, aortic arch aneurysm in 1 patient, mitral regurgitation in 2 patients, and coronary artery disease in 2 patients. Hospital mortality including initial operative death was not found. However, 6 late death (50%) occurred at late time with the mean of 23.8 months because of severe complications such as valve detachement or pseudoaneurysm after operation. In a comparison of the preoperative immunological values between survival group and late death group, immunoglobulins such as IgG, IgA, C3 and C4 were significantly higher in late death group. According to the recurrence of inflammation, the patients were divided into three groups. Group A included 3 patients who remains in active phase after initial operation in active phase. Group B included 3 patients who changed to active phase after initial operation in inactive phase. Group C included 6 patients who remains in inactive phase after initial operation. In a comparison among 3 groups, IgG and C4 showed no significant difference. However, IgA and C3 of group C were significantly lowest among three groups. This outcome is considered to be related to existence of latent inflammation. At operation, insertion of rigid prosthesis to fragile position due to latent inflammation may stimulate immunological reactions. We conclude that the preoperative values of the immunological factors, especially IgA and C3, are reliable predictors in postoperative prognosis.
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PMID:[Relationship between postoperative prognosis and preoperative immunological factors in aortitis syndrome]. 871 62

Peripartum cardiac failure due to cardiomyopathy is common in sub-saharan Africa. The etiology is unknown. This study was performed in Niger to assess a possible relationship between peripartum cardiomyopathy and Chlamydia. A total of 50 African women presenting peripartum cardiomyopathy underwent testing for infection by Chlamydia pneumoniae, Chlamydia trachomatis, and Chlamydia psittaci. The inclusion criteria were cardiac failure during the last three months of pregnancy or first 6 months postpartum with echocardiographic evidence of dilated cardiomyopathy. Similar testing was carried out in a control group of 25 African women from the same geographical location without cardiac disease. Detection of specific IgG, IgA and IgM antibodies was performed using the microimmunofluorescence technique. The cut-off values were > or = 1/32 for specific IgG antibody and > or = 1/16 for specific IgA and IgM antibody. Statistical comparison of the patient and control groups was achieved using the chi 2 test. For Chlamydia pneumoniae, 48 patients (96 p. 100) versus 20 controls (80 p. 100) controls were positive for IgG antibodies (p < 0.025) and 39 patients (80 p. 100) versus 14 controls (56 p. 100) were positive for IgA antibodies (p < 0.05). No patient or control demonstrated IgM antibodies for Chlamydia pneumoniae. For Chlamydia trachomatis and Chlamydia psittaci, differences in positive rates were not statistically significant. This is the first study demonstrating infection in patients with peripartum cardiomyopathy. The possible role of Chlamydia pneumoniae is discussed.
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PMID:[Chlamydia infection and peripartum dilated cardiomyopathy in Niger]. 1110 Apr 38

Osteolytic bone destruction, caused by the aberrant production and activation of osteoclasts, results in significant morbidity for patients with multiple myeloma (MM). Pamidronate [(3-amino-1-hydroxypropylidene)-1,1-bis-phosphonate] inhibits osteoclastic activity and reduces bone resorption. A potency of zoledronic acid (2-[imidazol-1-yl]-1-hydroxyethylidene-1,1-bisphosphonic acid, a new third generation bisphosphonate, as inhibitor of resorption was 850-fold greater than pamidronate, as was shown in preclinical models of bone resorption. Randomized, double-blind study was conducted to compare the efficacy and safety of zoledronic acid and pamidronate for treating myeloma bone disease. Since March 1999 the efficacy and safety of pamidronate and zoledronic acid is evaluated in MM patients all receiving anti-myeloma chemotherapy acc. to VMCP/VBAP alternating regimen. Nine patients with stage III myeloma and osteolytic lesions (3 female, 6 male, median age 57 years, range 52-67, with monoclonal protein: IgG-7, IgA-2) were randomly assigned (1:1:1 ratio) to treatment with either 4 or 8 mg of zoledronic acid via 15-minute intravenous infusion or 90 mg of pamidronate via 2-hour intravenous infusion every 3 to 4 weeks for 12 months. All patients have received 500 mg of calcium supplements and 500 IU of vit.D, orally, once daily, for the duration of administration of study medication. In extension phase of the study (June 2000-April 2002) patients did not received bisphosphonates. In 7 patients 18 cycles of assessed treatment was administered to each of them and one patient received 16 cycles. One patient died after receiving of 12 pamidronate therapy cycles at 11 month of the trial duration (and at 49 month since MM diagnosis and anti-tumour treatment). The patient's death occurred during the progression of plasma cell proliferation due to acute left ventricle cardiac failure. During the 12-month-period of bisphosphonate treatment skeletal related events (SRE) and progression of osteolysis occurred with the same frequency in 3 treatment groups. One patient experienced spinal cord compression and received radiation to bone and 2 patients experienced vertebral fracture. Time from study entry to the first SRE was 304 days in pamidronate and 366 and 392 days in 4 and 8 mg zoledronic acid group, respectively. The skeletal morbidity rate was identical in all treatment groups. Single hypocalcemic events occurred in 2 patients, mild hypertransaminasemia was observed in 3, worsening of renal function parameters in 2 patients (transient in one of them). Muscular pain and fever up to 39 degrees C (transient and self-limiting "flu-like" symptoms) occurred in 6 patients after several or some dozens of hours from study drug administration. Adverse events were similar in nature and frequency with zoledronic acid and pamidronate and were experienced by a similar proportion of patients in each treatment group. Median time of patient's observation duration after completing of administered treatment with zoledronic acid and pamidronate amounts to 20 months. At present actual median survival time of analysed patients since MM diagnosis is 42 months, since the beginning of treatment with pamidronate and zoledronic acid--33 months, and since completing treatment--20 months and is similar in 3 treatment groups. As was shown in our single center study in MM patients the safety and efficacy of pamidronate 90 mg and zoledronic acid 4 mg and 8 mg in monthly i.v. infusion are comparable. Thus the recommended dosage of zoledronic acid is 4 mg administered as a 15 minute i.v. infusion at intervals of 3 to 4 weeks.
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PMID:Comparative evaluation of safety and efficacy of pamidronate and zoledronic acid in multiple myeloma patients (single center experience). 1266 77

Antibody-mediated rejection (AMR) in human heart transplantation is an immunopathologic process in which injury to the graft is in part the result of activation of complement and it is poorly responsive to conventional therapy. We evaluated by immunofluorescence (IF), 665 consecutive endomyocardial biopsies from 165 patients for deposits of immunoglobulins and complement. Diffuse IF deposits in a linear capillary pattern greater than 2+ were considered significant. Clinical evidence of graft dysfunction was correlated with complement deposits. IF 2+ or higher was positive for IgG, 66%; IgM, 12%; IgA, 0.6%; C1q, 1.8%; C4d, 9% and C3d, 10%. In 3% of patients, concomitant C4d and C3d correlated with graft dysfunction or heart failure. In these 5 patients AMR occurred 56-163 months after transplantation, and they responded well to therapy for AMR but not to treatment with steroids. Systematic evaluation of endomyocardial biopsies is not improved by the use of antibodies for immunoglobulins or C1q. Concomitant use of C4d and C3d is very useful to diagnose AMR, when correlated with clinical parameters of graft function. AMR in heart transplant patients can occur many months or years after transplant.
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PMID:Antibody-mediated rejection in human cardiac allografts: evaluation of immunoglobulins and complement activation products C4d and C3d as markers. 1621 40

Ascites is rare in patients with multiple myeloma (MM). It may be due to diverse mechanisms, most frequently because of an increased permeability of the peritoneum or because of portal hypertension due to liver infiltration. Myelomatous ascites occurs more frequently in patients having Ig-G or Ig-A paraprotein and their prognosis is poor. It is submitted the case of a female patient aged 50 years with IgA-kappa MM, who evolved with cardiac failure (CF), plasma cells leukemia and ascites of mixed cause, because of peritoneal infiltrate of myelomatous cells, hepatic compromise and CF. A review of the different causes of ascites in patients with MM is performed. There are also summarized all myelomatous ascites cases published in the literature. Our report presents the first case of myelomatous ascites in a patient with plasma cells leukemia.
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PMID:[Myelomatous ascites]. 1843 66

Hyperammonemic encephalopathy is a rarely reported complication of multiple myeloma (MM). We describe an illustrative case of hyperammonemia in the setting of an immunoglobulin (Ig) D-lambda MM, and perform a systematic review of the English-written literature. Our search yielded 26 more cases. Median age was 64 years, and 54% of patients were male. All presented with progressive impairment of their level of consciousness. Median ammonium concentration was 109 micromol/L (interquartile range, 73-149 micromol/L). Most were IgA type (10 cases), and there were 2 cases of IgD type. Most cases were aggressive or chemotherapy-resistant forms of MM. Eight patients were diagnosed with MM at the same time as the episode of hyperammonemia. Only 1 patient had signs of portal hypertension as a result of concomitant hyperdynamic heart failure. Determination of amino acid in 10 patients showed high levels of glycine, low levels of tyrosine, and a low Fischer ratio. Two patients did not receive chemotherapy and died. Twenty-two out of 25 patients who received chemotherapy against MM showed a decrease in ammonium blood concentration, and of those, 15 survived the episode (68%). Overall mortality was 44%. In conclusion, hyperammonemia is a severe complication of MM, associated with a high mortality. It should be considered in any patient with MM and a low level of consciousness. Chemotherapy directed against MM seems to be the most effective measure in order to achieve normal ammonium levels and clinical improvement.
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PMID:Multiple myeloma and hyperammonemic encephalopathy: review of 27 cases. 1906 3


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