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 is an acute vasculitis of unknown cause that occurs predominantly in infants and young children and produces coronary artery aneurysms in approximately 15% to 25% of those affected. In the United States, Kawasaki disease is more commonly the cause of noncongenital heart disease in children than is acute rheumatic fever. Therapy for Kawasaki disease in the acute phase is aimed at reducing inflammation of the coronary artery wall and preventing coronary thrombosis; treatment with high-dose intravenous gamma globulin and aspirin has become the standard of care and reduces the risk of development of coronary artery aneurysms by three- to fivefold. Even when treated with high-dose intravenous immune globulin G (IVIG) regimens within the first 10 days of illness, however, approximately 5% of children with Kawasaki disease develop at least transient coronary artery dilation, and 1% develop giant aneurysms. For those with persistent or recrudescent fever despite initial IVIG infusion, multiple courses of gamma globulin and treatment with cortico- steroids may be indicated. Early experience suggests that therapies aimed at reducing the amount of tumor necrosis factor alpha have a role. For those who develop coronary artery aneurysms, chronic antithrombotic regimens are instituted. When aneurysms are small or moderate in size, aspirin alone may be sufficient, but for patients with giant aneurysms, most experts choose to treat Kawasaki disease with aspirin plus warfarin. Acute coronary thromboses are treated with platelet IIb/IIIa antagonists and thrombolytic therapy. For children with coronary artery stenoses and consequent ischemic heart disease, the therapeutic armamentarium is similar to that used in adults with atherosclerotic coronary artery disease and includes coronary artery bypass grafting and transcatheter interventions.
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PMID:Kawasaki Disease. 1109 28

Kawasaki disease (KD) is an acute, self-limited, febrile, multi-system vasculitis that predominantly affects the the pediatric population, and is the leading cause of acquired heart disease in children. No etiologic agent for the disease has been identified, there are no diagnostic tests available, and the diagnosis is established by fulfilling a defined set of clinical criteria. We report on a 9-year-old boy who presented initially with symptoms felt to represent a streptococcal infection. He was subsequently shown to meet the criteria for KD, developed cardiac complications of the disease and subsequently demonstrated recovery over a year's period of time. The diagnostic criteria for KD, differential diagnosis, pitfalls in diagnosis, therapeutic recommendations and outcomes are discussed with relevance to this case. Recent print and electronic information sources and references are provided.
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PMID:Kawasaki disease: a diagnostic challenge. 1113 17

Kawasaki Disease (KD) is a potentially fatal acute vasculitis of childhood. Although KD is the leading cause of acquired heart disease in children in developed nations, its pathogenesis remains unknown. We previously reported the novel observation that IgA plasma cells infiltrate the vascular wall in acute KD. We have now examined the clonality of this IgA response in vascular tissue from three fatal cases of KD to determine whether it is oligoclonal, suggesting an Ag-driven process, or polyclonal, suggesting nonspecific B cell activation or a response to a superantigen. We first sequenced VDJ junctions of 44 alpha genes isolated from a primary, unamplified KD vascular cDNA library. Five sets of clonally related alpha sequences were identified, comprising 34% (15 of 44) of the isolated alpha sequences. Furthermore, point mutations consistent with somatic mutation were detected in the related sequences. Next, using formalin-fixed coronary arteries from two additional fatal KD cases, we sequenced VDJ junctions of alpha genes isolated by RT-PCR, and a restricted pattern of CDR3 usage was observed in both. We conclude that the vascular IgA response in acute KD is oligoclonal. The identification of an oligoclonal IgA response in KD strongly suggests that the immune response to this important childhood illness is Ag-driven.
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PMID:Oligoclonal IgA response in the vascular wall in acute Kawasaki disease. 1114 18

Kawasaki syndrome was first described in 1967. The diagnosis is based on clinical criteria including fever for more than five days, conjunctivitis, lymph node enlargement and changes in the oral mucosa as well as in the palms and soles. Coronary complications including aneurysmal dilatation have been reported in 20-25% of untreated patients. Kawasaki syndrome is now the most common cause of acquired heart disease in children. Six patients were seen in our department during the period 1980-99. Five were treated with intravenous immunoglobulin. All of them showed immediate improvement of their general condition and were afebrile after 24-48 hours. One patient had a coronary aneurysm of moderate size, and four patients had signs of coronary vasculitis. All coronary changes were normalized after a maximum of 17 months. An update on Kawasaki disease is presented.
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PMID:[Kawasaki disease]. 1118 81

Childhood stoke is increasingly recognized, but studies remain largely descriptive. Important differences from adult stroke include the following: (1) frequently delayed or missed diagnosis, (2) heterogenous and overlapping risk factors, and (3) developmental differences in the cerebrovascular, neurologic, and coagulation systems. These aspects limit the extrapolation of the results of adult stroke research and present challenges in caring for children with stroke. The incidence of childhood ischemic stroke exceeds 3.3 in 100,000 children per year, more than double the estimates from past decades. The increased incidence reflects, in part, increased survival in previously fatal conditions predisposing to stroke, including congenital heart disease, sickle cell anemia, and leukemia. Risk factors for stroke are recognized in more than 75% of children. Common risk factors include congenital heart disease and sickle cell disease. Progressive arteriopathies, including vasculitis and moyamoya syndrome, are rare in children with stroke; however, transient arteriopathies including post-varicella angiopathy are increasingly recognized. Prothrombotic abnormalities are frequently present but of unclear significance. Adverse outcomes after childhood stroke, including death in 10%, recurrence in 20%, and neurologic deficits in two thirds of survivors could be reduced with available stroke treatments. Aggressive prehospital emergency care and transfer could improve access to hyperacute stroke therapies including tPA. Currently, the diagnosis is delayed by more than 24 hours from onset in most children. As in adults, tPA will likely produce unacceptable rates of intracerebral hemmorrhage unless given within 3 hours of stroke symptom onset. The appropriate choices for in hospital treatment and secondary preventative strategies, including aspirin and anticoagulants, are controversial. Empiric recommendations are published; however, age-appropriate clinical trials are urgently needed. The large multinational networks of investigators necessary for designing and conducting these future trials are now being formed.
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PMID:Stroke in children: recognition, treatment, and future directions. 1120 20

Inflammatory disorders which may affect the heart muscle, the endocardium, the pericardium and/or the coronary arteries are rare, but potentially devastating diseases. As the incidence of rheumatic heart disease has decreased, children with congenital heart disease now constitute the primary patient population at risk of infective endocarditis. Streptococcus viridans and Staphylococcus aureus are still the most frequently observed organisms. The majority of children with infective endocarditis can be cured today, but good results depend on early diagnosis and accurate treatment. Myocarditis occurs when the heart muscle is involved in an inflammatory process. Causes are numerous, but most common in children are infections with cocksackie viruses. Approximately two-thirds of children with symptomatic acute myocarditis show complete recovery of impaired ventricular function, 10-20% progress of dilatative cardiomyopathy and about 10% die or require heart transplantation. Kawasaki disease is the most prevalent inflammatory coronary artery disease and the leading cause of acquired heart disease in children. The origin of this acute systemic vasculitis remains unknown. Visible coronary arterial abnormalities develop in approximately 20% of children with untreated Kawasaki syndrome. A single dose of gamma-globulin (2 g/kg over 12 h) given within the first 10 days of onset of illness as early as possible, in addition to aspirin has been shown to reduce the duration of fever, which may reflect the severity of ongoing vasculitis, and to reduce the prevalence of coronary artery anomalies.
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PMID:[Clinical presentation, diagnosis and management of inflammatory heart diseases in childhood]. 1123 56

Infection with Trypanosoma cruzi causes a generalised vasculitis of several vascular beds. This vasculopathy is manifested by vasospasm, reduced blood flow, focal ischaemia, platelet thrombi, increased platelet aggregation and elevated plasma levels of thromboxane A(2) and endothelin-1. In the myocardium of infected mice, myonecrosis and a vasculitis of the aorta, coronary artery, smaller myocardial vessels and the endocardial endothelium are observed. Immunohistochemistry studies employing anti-endothelin-1 antibody revealed increased expression of endothelin-1, most intense in the endocardial and vascular endothelium. Elevated levels of mRNA for prepro endothelin-1, endothelin converting enzyme and endothelin-1 were observed in the infected myocardium. When T. cruzi-infected mice were treated with phosphoramidon, an inhibitor of endothelin converting enzyme, there was a decrease in heart size and severity of pathology. Mitogen-activated protein kinases and the transcription factor activator-protein-1 regulate the expression of endothelin-1. Therefore, we examined the activation of mitogen-activated protein kinases in the myocardium by T. cruzi. Western blot demonstrated an extracellular signal regulated kinase. In addition, the activator-protein-1 DNA binding activity, as determined by electrophoretic mobility shift assay, was increased. Increased expression of cyclins A and cyclin D1 was observed in the myocardium, and immunohistochemistry studies revealed that interstitial cells and vascular and endocardial endothelial cells stained intensely with antibodies to these cyclins. These data demonstrate that T. cruzi infection of the myocardium activates extracellular signal regulated kinase, activator-protein-1, endothelin-1, and cyclins. The activation of these pathways is likely to contribute to the pathogenesis of chagasic heart disease. These experimental observations suggest that the vasculature plays a role in the pathogenesis of chagasic cardiomyopathy. Additionally, the identification of these pathways provides possible targets for therapeutic interventions to ameliorate or prevent the development of cardiomyopathy during T. cruzi infection.
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PMID:The role of endothelin in the pathogenesis of Chagas' disease. 1133 35

Kawasaki disease, an acute systemic vasculitis of unknown etiology, is the leading cause of acquired heart disease in children in many parts of the world. It predominantly affects children under 5 years of age and is diagnosed clinically by the presence of characteristic clinical signs. Atypical patients, often younger than 1 year of age, manifest few early signs, usually have an incorrect admitting diagnosis and suffer from high morbidity and mortality. We report the presentation, clinical course and follow-up of a 3-month-old infant with atypical Kawasaki disease.
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PMID:Kawasaki disease--atypical presentation. 1133 31

Kawasaki disease is an acute self-limited vasculitis of infancy and early hildhood that is now the leading cause of acquired heart disease in children in the United States and Japan. An infectious cause is suspected, although the etiology remains unknown. Children typically are seen with the acute onset of fever followed by signs of mucosal inflammation and vasodilatation that evolve over the first week of the illness. Laboratory tests reveal a marked systemic inflammatory response. If untreated, 1 in 4 children will develop permanent damage to the coronary arteries. Intravenous gamma globulin in conjunction with aspirin is effective in reducing the inflammation and preventing coronary artery abnormalities if administered within the first 10 days of illness. This poses a difficult situation for the pediatric care provider who must distinguish Kawasaki disease from other rash/fever syndromes in a timely manner so that appropriate therapy can be initiated.
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PMID:Kawasaki disease. 1148 Jul 56

We report an unusual case in which infectious endocarditis presented systemic vasculitis and glomerulonephritis as the initial manifestation of the disease. The patient was a 16-year-old girl with congenital cyanotic heart disease who presented with skin purpura, proteinuria, and hematuria. She had hypergammaglobulinemia, cryoglobulinemia, and positive circulating immune complexes. Renal biopsy revealed crescentic glomerulonephritis. Her serum C3 level, which was initially normal, became decreased, and prednisolone and azathioprine were administered with a tentative diagnosis of systemic lupus erythematosus (SLE). Soon after, she developed fever and renal failure. Blood culture grew Streptococcus pyogenes, and the diagnosis of infectious endocarditis was made. Eight cases of systemic vasculitis and glomerulonephritis associated with infectious endocarditis have been described in the literature. Infectious endocarditis should be included in the differential diagnosis of systemic vasculitis and glomerulonephritis.
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PMID:Vasculitis associated with septicemia: case report and review of the literature. 1179 7


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