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 disease (KD) is the most common pediatric vasculitis and the most frequent cause of acquired heart disease in children in the U.S. Its etiopathogenesis is unknown, although T cell, B cell and monocyte/macrophage populations have all been implicated in the disease. The precise role played by T cells is unclear. Analysis of T-cell activation markers in peripheral blood has demonstrated conflicting data. Study of tissue samples, which could clarify this issue, has been limited. Expansion of T cells bearing V beta 2 and V beta 8 has been reported during the acute phase of the disease, suggesting that exposure to a superantigen may represent one of the etiologies. Other studies, however, have not confirmed V beta expansions of T cells; in fact, indirect evidence that a conventional antigen may be involved has been reported in certain patients. Together, these various studies suggest that the clinical entity of KD may be induced by a variety of etiologic agents.
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PMID:The role of T cells in Kawasaki disease. 883 55

The vascular function of the coronary arteries in children after Kawasaki disease (KD) as yet remains uncertain. Here we report our findings of the vascular response of the coronary arteries after intracoronary injection of acetylcholine in patients after KD. A total of 29 patients, 19 after KD and 10 as control, were examined using coronary angiography (CAG). These were divided into 4 groups according to the type of the coronary artery lesions: Group 1 consisted of those with regressed aneurysms and involved 29 sites. These aneurysms had developed in the acute stage and had subsequently regressed and demonstrated normal findings in follow-up CAG. Group 2 consisted of those with persistent aneurysms involved 30 sites. Group 3 involved 52 angiographically normal sites in patients after KD. And Group 4 consisted of control patients and involved 70 sites. These patients had congenital heart disease with normal coronary arteries. During CAG we infused 15 micrograms acetylcholine chloride into the right or left coronary artery. The luminal diameters were measured using a cine-videodensitometric analyser to study the distensibility of the coronary artery wall. The change in the diameter was an increase of 13.71% +/- 15.09% (mean +/- SD) in the normal KD Group 3, and 12.21% +/- 13.83% in the control Group 4, demonstrating marked vasodilatation. In contrast, the change in the regressed aneurysms Group 1, and in the persistent aneurysms Group 2, was -2.65% +/- 16.65%, and -0.08% +/- 6.51%, respectively, demonstrating no change or mild vasoconstriction. The change in Group 1, and in Group 2, was less than in normal Group 3 or control Group 4. The normal Group 3 showed no significant difference from control Group 4. These findings suggested that the coronary artery with regressed aneurysms, or with persistent aneurysms after KD has impaired vascular function and is therefore at risk for developing to atherosclerosis in the long-term.
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PMID:The vascular function of the long-term coronary artery after Kawasaki disease--evaluation by intracoronary injection of acetylcholine. 915 57

Kawasaki disease (KD) is the major cause of acquired heart disease in children. KD is suspected of being an infectious disease, but the etiology has not yet been clarified. Immunologically, the disease is associated with the activation of T cells, monocytes, and macrophages resulting in highly elevated levels of several cytokines. Recently, expansions of T cells expressing TCRBV2 and TCRBV8 chains have been reported, and this suggests the involvement of a superantigen in the pathogenesis of KD. To address the role of a superantigen in KD, we investigated clonal expansion of T cells by estimating the complementarity-determining region 3 size profile among T cells expressing TCRBV1, TCRBV2, TCRBV4, TCRBV5, TCRBV8, TCRBV14, TCRBV16, TCRBV17, TCRBV18, and TCRBV20 chains during acute KD, during subacute KD, and during the long term follow-up period. During the acute phase of KD, several clonal expansions were found mainly in the CD8+ T cells that disappeared during the long term follow-up period. Our data suggest that the conventional Ags rather than a superantigen were involved in the pathogenesis of acute KD.
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PMID:Clonal expansion of CD8+ T cells in Kawasaki disease. 920 Apr 89

A retrospective study was made of 6 children, with nonsurgical-related acute myocardial infarction (AMI), between January 1987 and December 1994. The ratio for gender was 1 and mean age at AMI was 49 days, 4 cases being associated with congenital heart disease (Fallot's tetralogy, truncus arteriosus and DiGeorge syndrome, one case each, and anomalous origin of left coronary artery, 2 cases). Kawasaki disease and coronary embolisation from thrombosis of the renal vein occurred in the other 2 cases respectively. All developed congestive cardiac failure and cardiomegaly. In the ECG pathologic q waves with more than 35 msec occurred in all, and QT prolongation occurred in 3. Five children (83%) all with AMI in the anterior and lateral wall of the left ventricle died, death being related with cardiac mechanical failure and not with arrhythmias.
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PMID:[Acute myocardial infarct in infants]. 925 32

This year marks the 30th anniversary of the first description of Kawasaki disease. The disease has emerged as an important cause of acquired heart disease in children. The cause of Kawasaki disease remains unknown and this presents many problems in the diagnosis and management of the disease. This paper reviews recent publications on the pathogenesis, diagnosis, and the short- and long-term management of Kawasaki disease.
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PMID:Kawasaki disease thirty years on. 952 34

Kawasaki syndrome (KS) is an acute, sometimes fatal vasculitis of young children. KS has replaced acute rheumatic fever as the most common cause of acquired heart disease in children in the United States. The illness is manifested by prolonged fever, conjunctival injection, enanthem, exanthem, erythema and swelling of the hands and feet, and cervical adenopathy. These acute features of illness are self-limiting, but coronary artery abnormalities occur in 20% of untreated patients. The etiology of the illness is unknown, but its clinical and epidemiologic features are most consistent with an infectious cause. Common cardiovascular manifestations of the illness include myocarditis, pericardial effusion, and coronary artery aneurysm formation. Treatment with intravenous gamma globulin (IVGG) and aspirin within the first 10 days of illness reduces the prevalence of coronary artery abnormalities from 20% in those treated with aspirin alone to 4%. Patients who develop coronary artery aneurysms, particularly those who develop giant coronary artery aneurysms, may suffer myocardial infarction secondary to thrombosis or stenosis in the abnormal vessel. Additional research to determine the cause of KS is urgently needed to allow for improved diagnosis, more specific therapy, and prevention of the disorder.
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PMID:Kawasaki syndrome. 966 74

To determine the exercise responses of patients with congenital heart disease, 20 patients-5 who had undergone a right ventricular outflow tract reconstruction (group R; age, 15 +/- 2 years), eight who had undergone a Fontan operation (group F; age, 13 +/- 2 years), and seven who had a history of Kawasaki disease (group C; age, 15 +/- 1 years)-performed a treadmill exercise test. Patients of group R had a significant residual right ventricular outflow obstruction. Oxygen uptake (VO2), heart rate (HR), and plasma norepinephrine (NE) concentrations were measured at rest, during warm-up, at ventilatory threshold (VT), and at peak exercise. Exercise capacity was determined as a percentage of the predicted normal peak VO2 (%pVO2). The %pVO2 for groups R and F was 65 +/- 10 and 56 +/- 11, respectively. Peak HR for groups R and F was 171 +/- 4 and 155 +/- 5, which were lower than the HR for group C (p < 0.001). Although NE concentrations at rest, during warm-up, and at VT were significantly greater in groups R and F (p < 0.05), there were no significant differences in the NE concentrations at peak exercise. Peak HR correlated with %pVO2 (p < 0.001). The ratio of the increase in HR to NE from rest to VT was significantly lower in groups R and F than in group C (p < 0.001) and correlated with %pVO2 (r = 0.80; p < 0. 001). These data suggest that sympathetic nervous activity in groups R and F is increased at rest and during mild to moderate exercises, and reduced sinus node sensitivity to NE may be partly responsible for the abnormal HR response during exercise of patients with uncorrected congenital heart disease.
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PMID:Responses of plasma norepinephrine and heart rate during exercise in patients after Fontan operation and patients with residual right ventricular outflow tract obstruction after definitive reconstruction. 970 66

Kawasaki disease (KD) is a paediatric multisystem necrotizing vasculitis constituting the most frequent cause of acquired heart disease in childhood. Conflicting data have been reported regarding expanded T-cell populations using particular T-cell receptor (TCR) beta-chain variable (BV) gene segments, suggesting either a superantigen- or a conventional antigen-mediated immune response in this disease. In order to further investigate the role of T lymphocytes, cells were stained with an extensive panel of 21 different TCRBV specific monoclonal antibodies (MoAbs) covering almost 70% of all T-cells. Flow cytometry was employed to analyse the expression of the TCRBV repertoire in the CD4+ and CD8+ subsets separately, and of activation markers, in freshly isolated peripheral blood lymphocytes of 25 Kawasaki disease patients during the acute and convalescent phases of the disease. No abnormal usage of any TCRBV family was found, neither acutely nor during convalescence, compared with a control group of healthy children. However, a significant increase in interleukin-2 receptor (IL-2R)-expressing T lymphocytes restricted to the CD4+ subset was observed in KD patients. Our data confirm a strong immune activation in KD that might be of importance in the pathogenesis of the disease.
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PMID:Characterization of the T-cell receptor V-beta repertoire in Kawasaki disease. 979 Mar 17

Kawasaki syndrome is a fascinating worldwide illness of young children. This acute self-limited vasculitis has become the most common cause of acquired heart disease in children in the United States and Japan. KS causes significant coronary artery disease that may lead to myocardial infarction and sudden death. Clinical and epidemiologic features of KS support an infectious cause, but the etiology remains unknown. Clearly, additional research on the cause of KS and its pathogenesis is needed urgently to allow for improved diagnosis; more specific therapy; and, ultimately, prevention of the disorder.
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PMID:Kawasaki syndrome. 1021 77

Adrenomedullin is a novel hypotensive peptide originally isolated from human pheochromocytoma. Accumulating evidence suggests the possible involvement of adrenomedullin in the physiology of the pulmonary circulation and the pathophysiology of hypoxaemia. The aim of the present study was to investigate the pathophysiological significance of adrenomedullin in hypoxaemia caused by congenital cyanotic heart disease. Subjects were 16 patients with congenital cyanotic heart disease aged 0.8-10 years (Group C) and 12 age-matched control subjects (patients with coronary artery dilatation after Kawasaki disease; Group N). Plasma adrenomedullin concentrations were measured, using radioimmunoassay, in femoral venous, pulmonary arterial and pulmonary venous blood obtained during cardiac catheterization. Plasma adrenomedullin concentrations in Group C were significantly (3-fold) higher than those in Group N at all sampling sites. In Group C, plasma adrenomedullin concentrations in pulmonary venous blood were significantly lower than those in pulmonary arterial blood. Pulmonary uptake of adrenomedullin in Group C was significantly greater than that in Group N. Patients with congenital cyanotic heart disease showed elevated plasma adrenomedullin concentrations and an increased uptake of adrenomedullin in the pulmonary circulation, which may act to dilate pulmonary vessels and increase pulmonary blood flow to alleviate hypoxaemia. Intrinsically increased adrenomedullin levels may function as a compensatory mechanism for hypoxaemia in congenital cyanotic heart disease.
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PMID:Elevated plasma levels of adrenomedullin in congenital cyanotic heart disease. 1033 59


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