Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

To assess the efficacy, plasma drug concentrations and adverse effects of a new sustained release preparation of procainamide, 33 patients with heart disease were studied in an acute dose-ranging protocol and a chronic treatment protocol. Patients initially received a daily dose of 3 g of sustained release procainamide; this dose was increased by 1.5 g daily until ventricular premature depolarizations were suppressed by 75 percent or more, adverse drug effects occurred or a total daily dose of 7.5 g of sustained-release procainamide was reached. Twenty-five patients (76 percent) had at least a 75 percent reduction (range 75 to 100percent [mean +/- standard deviation 91 +/- 8.2]) in ventricular permature depolarization frequency at a dosage of 4.8 +/- 1.46 g/day (range 3.0 to 7.5). Despite the 8 hour dosing interval, the variation between maximal and minimal plasma procainamide and N-acetylprocainamide concentrations under steady state conditions was very small. Mean maximal procainamide and N-acetylprocainamide plasma concentrations were 10.4 +/- 6.02 and 12.0 +/- 7.40 micrograms/ml, respectively. The respective mean minimal concentrations were 6.8 +/- 4.50 and 8.7 +/- 5.99 micrograms/ml. In nine patients (27 percent) treatment with sustained release procainamide resulted in conversion of the antinuclear antibody test from negative to positive. Adverse drug effects occurred in 17 (52 percent) of the subjects. In general, adverse effects were minor and abated within 24 hours after administration of the drug was stopped. One patient had the procainamide-induced systemic lupus erythematosus-like syndrome.
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PMID:Efficacy, plasma concentrations and adverse effects of a new sustained release procainamide preparation. 615 83

Four cases of endocarditis due to Kingella kingae are described in compromised patients. All had primary heart disease, and two had systemic lupus erythematosis and congenital heart defect respectively, in addition. Confirmation of Kingella kingae was made in one case at autopsy. The literature on 11 cases of endocarditis, 2 bacteremia, 4 osteomyelitis, 5 septic arthritis and 1 intervertebral disc infection, all caused by Kingella kingae, is reviewed. Our findings confirm that the organism is of low pathogenicity. Children may be predisposed to infection with Kingella kingae.
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PMID:Endocarditis due to Kingella kingae. 646 70

This multicenter regional study analyzes survival of a large group of patients who began chronic dialysis or received their first renal transplant during the 5 1/2-year period that ended in June 1983. Survival was determined from the onset of renal replacement therapy, irrespective of changes in treatment modality. Univariate life-table analysis was used to examine more than 35 risk factors. Age of patients at entry into the therapeutic program significantly affected survival. General survival rates were lower for patients with diabetes than for nondiabetics, irrespective of treatment modality, with the exception of those older than 60 years of age. Five-year graft survival was lower for diabetics than for nondiabetics in all age groups, irrespective of source. Patients with systemic lupus erythematosus, focal glomerulosclerosis, or glomerulonephritis had the highest survival rates, whereas those with primary renal malignant lesions, primary hypertensive disease, or diabetes mellitus as the cause of renal failure had the lowest. Concurrent morbid conditions that adversely affected survival included arteriosclerotic heart disease, peripheral vascular disease, noncutaneous malignant lesions, chronic pulmonary disease, and multiple coexisting morbid conditions. Although the objective of this study was to analyze survival for single patient characteristics, irrespective of treatment modality, analyses of survival rates by treatment modality for a control group indicated that minimal differences were evident by the third year among the four treatment groups: in-center hemodialysis, home hemodialysis, living related donor transplantation, and cadaver transplantation; however, recipients of cadaver grafts had lower survival rates than all other groups, even those maintained by in-center hemodialysis (P = 0.025).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Survival rates of 2,728 patients with end-stage renal disease. 649 73

One of the rare examples of the transfer of autoimmune disease from mother to (unborn) child is the neonatal lupus syndrome. This syndrome comprises the development of fetal heart disease (congenital heart block) or neonatal skin rash and is specifically associated with maternal anti-Ro/SS-A autoantibodies. Previous studies have suggested that especially maternal autoantibody reactivity against the 52 kDa protein of the Ro/SS-A antigen and/or against the La/SS-B antigen is responsible for the development of congenital heart block (CHB). To determine the CHB-associated antibody response in more detail, we analysed the presence of autoantibodies in sera from mothers of children with isolated heart block. All 14 mothers of children with congenital heart block were positive for anti-Ro/SS-A antibodies. Remarkably, their antibody profile, including recognition of different Ro/SS-A proteins and autoantibody levels against these proteins, did not differ from anti-Ro/SS-A positive mothers of healthy children. In contrast, all 8 anti-Ro/SS-A negative mothers had children with acquired heart block. We conclude from our data that maternal anti-Ro/SS-A antibodies are essential for CHB but that fine analysis of this autoantibody response does not predict the occurrence of CHB.
Lupus 1993 Aug
PMID:Maternal autoantibodies and congenital heart block: no evidence for the existence of a unique heart block-associated anti-Ro/SS-A autoantibody profile. 750 95

This study was undertaken to determine the role of antibodies against both recombinant Ro (r-Ro) and La (r-La) proteins and polypeptides derived from the recombinant La protein in predicting fetal and neonatal outcome in children at risk to develop neonatal lupus erythematosus (NLE). All sera were obtained in the perinatal period and quantitative ELISA assays were used. We collected 41 maternal sera within 2 months of delivery of a child with NLE (21 with congenital heart disease block (CHB) and 20 with dermatologic NLE) and 19 sera from anti-Ro and/or anti-La antibody-positive mothers with systemic lupus erythematosus (SLE) who delivered a child without NLE. All sera were tested for anti-r-La and anti-r-Ro antibodies by ELISA, and most sera were tested for antibodies directed against La polypeptides by immunoblot. We found significantly higher anti-r-La antibody levels in the sera from mothers of children with NLE compared with sera from mothers of unaffected children (0.67 +/- 0.43 versus 0.14 +/- 0.30; P < 0.0001). There was a statistically significant difference in the mean anti-r-La levels between the sera of mothers of children with CHB compared with dermatologic NLE (0.51 +/- 0.45 versus 0.83 +/- 0.37 respectively; P = 0.0091). When we examined antibodies directed against the recombinant 52-kD Ro protein, there was a statistically significant elevation of titres in the sera of mothers of NLE children (0.77 +/- 0.35) compared with non-NLE mothers (0.29 +/- 0.39; P < 0.0001). There was no difference in the r-Ro levels between mothers of children with dermatologic NLE compared with CHB (0.82 +/- 0.37 versus 0.71 +/- 0.74; P = 0.32). When we examined polypeptides derived from the recombinant La protein, the mean number of polypeptides recognized by sera from mothers of children with NLE was significantly higher than the mean number of polypeptides recognized by sera from mothers of unaffected children (5.1 +/- 0.54 versus 2.3 +/- 0.54 respectively; P < 0.001). More importantly, when we examined the individual polypeptides, we found that only sera from mothers of children with NLE and not from mothers of unaffected children recognized a polypeptide designated DD (30% versus 0%, respectively). These studies indicate that the autoantibody response to the Ro/La particle can differentiate sera from mothers of children with NLE and sera from mothers of unaffected children. Furthermore, there was a difference in the anti-La autoantibody response between mothers of children with CHB and dermatologic NLE.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Autoantibody response to the Ro/La particle may predict outcome in neonatal lupus erythematosus. 777 62

Two cases of primary antiphospholipid antibody syndrome are reported. One patient presented multiple abortions and epilepsy. The second patient was affected by a brain vascular accident, with a residual hemiparesis. Both cases showed livedo reticularis in arms, NMR evidence of diffuse lesions of the white matter, high serum levels of anticardiolipin antibodies and cardiopathy. Lupus anticoagulant was also found in the serum of the first patient, and cortisone and antiaggregants enabled her to reach term in a fifth pregnancy after four miscarriages. In the other case histological examination of specimens of skin, peripheral nerve and skeletal muscle revealed occlusive, non arteriosclerotic vasculopathy and an absence of inflammatory lesions. Histological study has rarely been performed in primary antiphospholipid syndrome but suggests that the mechanism of thrombosis is not vascular; in our subjects it revealed findings similar to those in Sneddon syndrome.
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PMID:Primary antiphospholipid syndrome: two case reports, one with histological examination of skin, peripheral nerve and muscle. 799 67

To clarify the histopathological characteristics of pulmonary hypertension (PH) in Japan, and to clarify the role of serotonin and endothelin in monocrotaline induced PH, human histopathological studies and experimental studies were carried out. An epidemiological study based on the Annual of the Pathological Autopsy Cases in Japan, and a morphological study on autopsy cases of congenital heart disease and idiopathic PH were performed. Plasma levels of serotonin and endothelin, vascular responsiveness to serotonin, and the effects of a selective serotonin antagonist, DV-7028, were investigated after monocrotaline injection. Plexogenic pulmonary arteriopathy was prevalent, and recurrent pulmonary thromboembolism and pulmonary veno-occlusive disease extremely rare among primary pulmonary hypertension in Japan. In secondary PH, systemic lupus erythematosus and mixed connective tissue disease were frequent and showed particularly severe intimal and medial thickening. After an injection of monocrotaline, plasma serotonin and endothelin levels were raised, and pulmonary arteries showed hyperreactivity to serotonin. DV-7028 (5-HT2 receptor antagonist) attenuated the rise in pulmonary artery pressure and the various effects of monocrotaline. There may be some genetic difference between PH in Japan and other countries. Roles for serotonin and endothelin in the initiation and progression of monocrotaline induced PH are suggested.
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PMID:Pathology of pulmonary hypertension: a human and experimental study. 804 94

To study the cardiac involvement in systemic lupus erythematosus, the clinical and necroscopy records of 29 patients were analyzed. Of these, 28 were female and 1 was male with a mean age of 28.6 +/- 10.4 years. The mean duration of the disease was 23.8 +/- 20.1 months. Twenty patients had the diagnosis of renal failure, 11 of arterial hypertension and 8 of congestive heart failure. No valvular or coronary arterial disease was diagnosed in any patient. The most frequent cause of death was sepsis, followed by renal failure. Only one patient died of causes directly related to heart disease. At necroscopy, 12 patients had pericarditis and 10 had cardiomegaly. Nonspecific valvular abnormalities were observed in 2 patients, Libman-Sacks' endocarditis in 1 and bacterial endocarditis in 2. Cardiovascular abnormalities were absent in only 8 (7.5%) patients. It was concluded that although frequent and contributing to mortality in some patients, cardiovascular involvement in systemic lupus erythematosus is multifactorial and, usually, nonspecific.
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PMID:[Cardiac involvement in systemic lupus erythematosus: anatomo-pathological study]. 828 Dec

Cerebral infarction before the age of 45 years accounts for 4-6% of all strokes. The etiology remains unexplained in a significant proportion of patients even after extensive investigations. The reported risk factors of this age group are cardiopathies, hypertension, smoking, hypercholesterolemia, reduction of anticoagulant proteins, hypercoagulable states, antiphospholipid antibodies primary syndrome, antiphospholipid antibodies secondary syndrome, some hemoglobinopathies, hyperviscosity syndromes, vasculitis, collagen vascular diseases, fibromuscular dysplasia, arterial dissections, migraine, myopathy encephalopathy lactic acidosis stroke like episodes, homocystinuria, familial amyloid angiopathy, microangiopathy with retinopathy encephalopathy and deafness, systemic lupus erythematosus, use of cocaine, traumas or manipulations of neck, AIDS. From 1/1/94 to 04/30/95 we observed 19 patients with cerebral infarctions and 9 patients with transitory ischemic attacks in young people. The aim of our study was to apply a diagnostic protocol by sequential tests of first level and second level. According to this protocol we found that the more common risk factors were ischemic cardiopathy, hypertension, smoking and hypercholesterolemia. Moreover we observed other independent risk factors, although less frequent, like the antiphospholipid antibodies, neurolupus, AIDS, deficit of protein S.
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PMID:[The application of a new diagnostic protocol for stroke in the young]. 876 46

Natural antibodies that appear to have arisen in the absence of direct antigenic stimulation often are present in the circulation. In healthy individuals, they are of low prevalence and generally show low affinity to their respective antigens, whereas in autoimmune disease, their frequency and affinity toward specific antigens are in many cases increased. In some autoimmune disease, the spontaneous occurrence of autoantibodies to antigens that are apparently unrelated to symptoms or pathology has been observed. For example, antibodies to interferon-alpha (IFN-alpha) have been observed in patients with systemic lupus erythematosus but appear to be of no clinical significance. Natural autoantibodies to IFN-alpha have also been found in other patients, including those with insulin-dependent diabetes mellitus, heart disease, or cancer and patients who have just received allogeneic bone marrow transplantation. A major property of IFN-alpha autoantibodies is their capacity to both bind to and neutralize the biologic activity of a range of IFN-alpha subtypes and natural IFN-alpha preparations. Their origins and significance remain a matter for debate.
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PMID:Natural autoantibodies to interferons. 924 17


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