Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Kawasaki syndrome (KS), the major cause of acquired heart disease in children, is an acute multisystem vasculitis frequently associated with the development of myocarditis and coronary artery abnormalities. Despite the widely held belief that KS is an infectious disease, its etiology has remained elusive. Recently, we and others have reported the selective expansion of V beta 2+ T cells in the peripheral blood of most patients in the acute, but not in the convalescent, phase of KS. These data were consistent with the concept that this illness is triggered by a bacterial superantigen. We report here that a patient who died of acute KS had selective expansion of V beta 2+ T cells in her myocardium and coronary artery. Sequence analysis of TCR beta-chain genes of V beta 2+ T cells from the myocardium showed extensive junctional region diversity. These observations, along with the demonstration of V beta 2 expansion in both the CD4+ and CD8+ T cell subsets, support the concept that the activation of infiltrating V beta 2+ T cells are involved in the cardiovascular damage associated with KS.
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PMID:Evidence for superantigen involvement in cardiovascular injury due to Kawasaki syndrome. 759 9

In 1967, Tomisaku Kawasaki described a syndrome of mucocutaneous inflammation in 50 Japanese children. Seven years later, he reported his experience in English, emphasizing that 1% to 2% of affected children died of cardiac failure. Since then, Kawasaki disease has been described worldwide in children of all racial groups and has been recognized as a leading cause of acquired heart disease among children in the United States (Figure 1). The disease affects mostly toddlers; about 80% of patients are less than five years old, and only rare cases are seen in adolescents over age 15. Intravenous gamma globulin has recently been demonstrated to reduce systemic inflammation and the prevalence of coronary artery aneurysms in patients with Kawasaki disease. The treatment, however, is effective only if administered early in the illness. Hence, prompt and accurate diagnosis is essential. The following cases offer a context in which to discuss some of the clinical issues surrounding Kawasaki disease.
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PMID:Kawasaki disease and its cardiac sequelae. 769 40

Acquired heart disease in children may result in significant morbidity and mortality. Advances continue to be made in understanding Kawasaki disease, acute and chronic rheumatic heart disease, infective endocarditis, myocarditis, and dilated cardiomyopathy. The role of superantigens, particularly bacterial toxins, in the pathogenesis of Kawasaki disease continues to be defined. Intravascular ultrasound promises to improve the assessment of coronary arteries in Kawasaki disease. Current recommendations for the long-term management of Kawasaki disease are discussed. Significant changes in the epidemiology of acute rheumatic fever and endocarditis are noted. Updates on the role of echocardiography as well as current therapeutic issues in these diseases are addressed. The application of immunologic and molecular biologic techniques have implicated genetic and immune factors in the pathogenesis of myocarditis and cardiomyopathy. The relationship between viral infection and subsequent dilated cardiomyopathy, as well as the role of autoimmune mechanisms in the pathogenesis of these disorders, remains controversial.
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PMID:Advances in acquired pediatric heart disease. 778 68

Kawasaki syndrome (KS), the main cause of acquired heart disease in children, is associated with the selective expansion of V beta 2+ T cells in peripheral blood. Our study suggests that KS may be caused by a superantigen--a staphylococcal or streptococcal toxin. Bacteria were cultured without knowledge of their origin, from the throat, rectum, axilla, and groin of 16 patients with untreated acute KS and 15 controls. Bacteria producing toxins were isolated from 13 of 16 KS patients but from only 1 of 15 controls (p < 0.0001). Toxic shock syndrome toxin (TSST) secreting Staphylococcus aureus was isolated from 11 of the 13 toxin-positive cultures, and streptococcal pyogenic exotoxin (SPE) B and C were found in the other 2. These toxins are known to stimulate V beta 2+ T cells. All TSST-producing KS isolates were tryptophan auxotrophs indicating they were clonally related. S aureus isolates from acute KS patients were unusual because they produced less lipase, haemolysin, and protease compared to other isolates (p < 0.01). S aureus colonies from KS patients were white, and could be easily mistaken for coagulase-negative staphylococci, whereas colonies of non-KS isolates were gold. These observations suggest that the expansion of V beta 2+ T cells in most patients with KS may be caused by a new clone of TSST-producing S aureus, and, in a minority of patients, SPEB-producing or SPEC-producing streptococci.
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PMID:Toxic shock syndrome toxin-secreting Staphylococcus aureus in Kawasaki syndrome. 790 29

Kawasaki disease (KD) is the leading cause of acquired heart disease in children in the United States. The etiology is unknown. Data regarding the presence of T cell activation and its potential role in the pathogenesis of the disease have been conflicting. Expansion of T cells bearing V beta 2 and V beta 8 has recently been reported in the acute phase of KD, which suggests that a superantigen may mediate the disease process. To further assess the potential role of T cells in KD, T cell phenotypes were evaluated by using flow cytometry in a large series of patients, acutely and during convalescence. Included in this analysis were assessments of changes in the percentage of T cells bearing TCR V beta 2, V beta 5.1, V beta 6.7, V beta 8, V beta 12.1, and V beta 19 over time; the percentage of each V beta family bearing the activation markers HLA-DR and IL-2R; and the percentage of each V beta family bearing the memory marker, CD45RO. No expansion of any V beta family was present acutely, nor were increases in HLA-DR and IL-2R observed. However, a significant increase was observed during convalescence in the percentage of cells bearing CD45RO in the CD8+, but not the CD4+, population. CD45RO expression was also increased on V beta 2, V beta 8, and V beta 19 CD8+ T cells in a subset of patients. These data suggest that one or more conventional Ags drive the T cell immune response in KD, and argue against a role for superantigens in the disease process.
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PMID:TCR V beta family repertoire and T cell activation markers in Kawasaki disease. 791 45

To assess the frequency of hospital encoded diagnoses of acute rheumatic fever (ARF) and Kawasaki disease (KD), the two leading causes of acquired heart disease in children in the United States, we performed a survey of the medical record departments of United States children's hospitals and of general hospitals that have at least 400 beds and a pediatric ward. With a simple questionnaire, data were gathered for the years 1984 through 1990 by ICD.9CM codes, with a 58% response rate. About 8000 diagnoses of KD and 6000 diagnoses of ARF were encoded during the study period. Encoded diagnoses of both KD and ARF showed yearly fluctuations in the earlier years (1984 through 1987). For KD there was a general trend toward increasing numbers after 1986. These data are consistent with increased physician awareness and diagnosis of KD. For ARF a gradual decline was observed between 1986 and 1990. About 80% of ARF diagnoses were reported from general hospitals. The much smaller pool of encoded diagnoses of ARF at the children's hospitals showed a 56% increase from 1985 to 1986. These data suggest that the highly publicized increase in cases of acute rheumatic fever in the United States during the mid-1980s may reflect focal rather than nationwide increased activity and that nationally the number of diagnoses of ARF actually may have continued to decline gradually from 1984 through 1990.
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PMID:Seven-year national survey of Kawasaki disease and acute rheumatic fever. 855 45

The causes of death in 140 patients (30 male and 110 female) with rheumatoid arthritis (RA) at Kawasaki Municipal Hospital are analyzed. Autopsies were performed in 58%. The causes of death were infection (25.7%), heart disease (20.0%), renal failure (10.7%), cerebrovascular disease (10.0%), and respiratory failure (8.5%). The causes of death in RA patients were compared with those of 243 non-rheumatoid patients. Infection, congestive heart failure, renal failure and respiratory failure were more common causes of death in RA than in the control group. It should be noted that neoplasms are infrequent causes of death in patients with RA. The survival time (onset to death) of RA patients appears to be increasing year by year.
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PMID:[Causes of death in 140 patients with rheumatoid arthritis]. 805 26

To evaluate the prevalence of type I silent myocardial ischemia and silent myocardial infarction, 4,842 men aged 40 to 59 years, identified in occupational samples in Florence and Rome, and free from major heart disease, severe illnesses and chest pain, underwent a 3-stage diagnostic procedure. The first stage included resting electrocardiogram, hyperventilation test, exercise electrocardiogram and 24-hour Holter electrocardiogram. The subjects who were suspected of having type 1 silent myocardial ischemia or previous silent infarction at the first stage (n = 439; 9.1%) were entered into the second stage, which included echocardiogram, thallium 201 scintigraphy in conjunction with exercise testing or dipyridamole test, exercise radionuclide ventriculography and ergonovine test. Three hundred eighty-seven men participated in the second stage; after the diagnostic procedures were performed, 104 men (2.1%) were still suspected of having type 1 silent myocardial ischemia or infarction on the basis of predefined criteria. Sixty-two men continued on into the third diagnostic workup including coronary angiography. The final diagnosis of type 1 silent myocardial ischemia or infarction was reached in 25 patients (prevalence 0.52%; adjusted estimate 0.89%). Of these 25, 19 had coronary atherosclerotic disease, 1 had Kawasaki disease, 1 had coronary anomaly, 1 had induced focal coronary spasm, and 2 had normal coronary arteriograms despite the presence of unquestionable old myocardial infarction. Altogether, 6 patients with silent myocardial infarction were identified.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Epidemiology of silent myocardial ischemia in asymptomatic middle-aged men (the ECCIS Project). 825 31

Only limited information is available concerning left ventricular (LV) response to exercise after an external conduit operation for cyanotic congenital heart disease. Sixteen patients who had undergone external conduit repair (EC group) were studied with multi-gated cardiac pool imaging using a supine bicycle ergometer on 20 occasions. Six patients with a history of Kawasaki disease without coronary artery stenosis served as controls (control group). Myocardial imaging and cardiac catheterization were also performed in the EC group. There was no significant difference in left ventricular ejection fraction (LVEF) at rest between the groups. However, on exercise, LVEF of the EC group was significantly lower than that of the control group. Nine patients in the EC group showed a perfusion defect (PD) on 12 occasions. LVEF on exercise of the patients with PD was significantly lower than that of the patients without PD. Furthermore, only the patients with PD showed a LVEF decrease of 5% or more in response to exercise. In the EC group, a significant inverse relationship was demonstrated between right ventricular systolic pressure (RVP) and LVEF response to exercise. However, two out of four patients who underwent external conduit replacement improved their LVEF response to exercise with successful reduction of RVP. These findings indicate that an impaired left ventricular response to exercise was common in patients after external conduit operations. Myocardial damage and right ventricular outflow tract obstruction could be the causes of this left ventricular dysfunction.
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PMID:Radionuclide assessment of left ventricular performance on exercise after external conduit operation. 837 18

Kawasaki Syndrome (KS) is an acute multi-system vasculitis of infancy and early childhood associated with the development of coronary artery abnormalities. The prevalence of cardiovascular abnormalities can be significantly reduced by treating patients during the first 10 days of illness with high-dose intravenous immune globulin (IVIG). Despite the widely held belief that KS is caused by an infectious agent, the aetiology of this illness remains controversial. Recent immunological and microbiological studies suggest a potential role for staphylococcal and streptococcal toxins (superantigens) in the pathogenesis of KS. Confirmation of these findings could result in more effective diagnostic and therapeutic approaches for the treatment of this common cause of acquired heart disease in children.
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PMID:Kawasaki syndrome: immunomodulatory benefit and potential toxin neutralization by intravenous immune globulin. 862 44


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