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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 the most common cause of multisystem vasculitis in childhood. The resultant coronary artery lesions make Kawasaki disease the leading cause of acquired heart disease in children in the developed world. TNF-alpha is a pleiotropic inflammatory cytokine elevated during the acute phase of Kawasaki disease. In this study, we report rapid production of TNF-alpha in the peripheral immune system after disease induction in a murine model of Kawasaki disease. This immune response becomes site directed, with migration to the coronary arteries dependent on TNF-alpha-mediated events. Production of TNF-alpha in the heart is coincident with the presence of inflammatory infiltrate at the coronary arteries, which persists during development of aneurysms. More importantly, inflammation and elastin breakdown in the coronary vessels are completely eliminated in the absence of TNF-alpha effector functions. Mice treated with the TNF-alpha-blocking agent etanercept, as well as TNFRI knockout mice, are resistant to development of both coronary arteritis and coronary aneurysm formation. Taken together, TNF-alpha is necessary for the development of coronary artery lesions in an animal model of Kawasaki disease. These findings have important implications for potential new therapeutic interventions in children with Kawasaki disease.
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PMID:TNF-alpha is necessary for induction of coronary artery inflammation and aneurysm formation in an animal model of Kawasaki disease. 1667 Mar 41

The aim of this study was to determine risk factors for coronary lesions, the type of heart lesion present, and long-term outcome in Kawasaki disease. We studied 150 children, aged 3 months to 9.5 years, who met the criteria for a diagnosis of Kawasaki disease. Of the 18% who were diagnosed with heart disease, 100% had coronary artery abnormalities, 11.1% had pancarditis, and 3.7%, mitral insufficiency. The coronary abnormalities were classified according severity as follows: diffuse ectasia in 40.7%; a small-to-medium-sized solitary coronary artery aneurysm in 33.3%; numerous small-to-medium-sized aneurysms in 11.1%; giant aneurysms in 11.1%; and coronary artery stenosis in 3.7%. The presence of pancarditis was a predictor of a giant coronary artery aneurysm. Mortality was 3.7%. Coronary aneurysm was the predominant heart lesion. The risk factors for coronary aneurysm in Kawasaki disease included age less than 27 months, fever lasting more than 8 days, erythrocyte sedimentation greater than 70 mm, and pancarditis.
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PMID:[Kawasaki disease: heart disease during childhood]. 1670 93

The increase in B-natiuretic peptide (BNP) is well correlated with cardiovascular symptoms in adults. Its use in children is recent and only partially evaluated. The authors undertook a prospective study of BNP concentrations and its kinetics in 54 children with an average age of 15 months (5 days to 11 years) admitted as paediatric emergencies. The symptoms were dyspnoea (60%), shock (15%), suspicion of Kawasaki disease (15%) and other (10%). Twenty children had BNP levels of more than 100 pg/ml related to decompensation of known congenital heart disease in 7 patients (average BNP 462 +/- 323 pg/ml), due to neonatal coarctation in 2 patients (BNP > 3000 pg/ml), due to cardiomyopathy in 6 patients (BNP= 2576 +/- 1215 pg/ml), due to an arrhythmia in 1 patient (BNP= 3754 pg/ml) and to Kawasaki disease in 4 patients (BNP= 521 +/- 448 pg/ml). Thirty-four children had BNP values of less than 100 pg/ml; 29 had no cardiac disease and 5 had known congenital heart disease with other symptoms. Measuring BNP is quick and economical and is a valuable aid in the diagnosis of cardiac dysfunction in symptomatic children in the emergency room. High BNP values seem to be correlated with the severity of the cardiac disease. Low BNP values seem to have a good negative predictive value in children without underlying cardiac disease. The interpretation of intermediary values, especially when there is previous cardiac disease, is more difficult in view of the absence of known threshold values for different haemodynamic situations. Further studies are required to determine the value of this test for the follow-up and setting up of prognostic values in children with congenital heart disease.
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PMID:[B-natriuretic peptide and cardiological emergencies in childhood]. 1680 38

Heart transplantation (HTx) is a treatment for end-stage heart failure or a complex or inoperable congenital defect. The long-term survival and the adequate donor to recipient body weight (D/R BW) ratio remain to be determined. From March 1995 to May 2004, 14 children (6 months-16 years of age) underwent HTx due to underlying diseases of idiopathic dilated cardiomyopathy (n = 10; 71.4%), congenital heart disease (n = 3; 21.4%), and Kawasaki disease (n = 1; 7.1%). Donor-recipient body weight ratio ranged from 0.89 to 3.9. Big heart syndrome was present in one patient when D/R BW ratio was more than 3. Actuarial survival was 92.9% at 5 years after transplantation. Only the one patient who had Kawasaki disease died due to early primary graft failure. HTx is a feasible method with good long-term survival rates for end-stage heart failure or for complex or inoperable congenital defects. After careful pretransplant evaluation, a high D/R BW ratio (more than 3) is acceptable.
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PMID:Single-center experience of pediatric heart transplantation in taiwan. 1698 21

Kawasaki disease is an acute vasculitis of childhood that predominantly affects the coronary arteries. The etiology of Kawasaki disease remains unknown, although an infectious agent is strongly suspected based on clinical and epidemiologic features. A genetic predisposition is also likely, based on varying incidences among ethnic groups, with higher rates in Asians. Symptoms include fever, conjunctival injection, erythema of the lips and oral mucosa, rash, and cervical lymphadenopathy. Some children with Kawasaki disease develop coronary artery aneurysms or ectasia, ischemic heart disease, and sudden death. Kawasaki disease is the leading cause of acquired heart disease among children in developed countries. This article provides a summary of the diagnostic and treatment guidelines published by the American Heart Association.
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PMID:Kawasaki disease: summary of the American Heart Association guidelines. 1703 51

Kawasaki disease is a multi system disorder with varying clinical expressions. This disease is an acute systemic vasculitis of unknown etiology that has recently recognized as a leading cause of acquired heart disease in children of many developed countries. We describe an unusual instance of hair loss in a patient with Kawasaki disease. A 26 months old boy developed prolonged high fever, bilateral conjunctival infection, arthralgia and erythromatosis skin rash. He was admitted to the hospital with the diagnosis of Kawasaki disease. Laboratory results included an erythrocyte sedimentation rate (ESR) above 100 and platelet count > 1000,000. The patient developed acute and unprovoked scalp hair loss. He was treated with intravenous immunoglobulin (IVIG) 2 g/kg and aspirin 100 mg/kg/day with complete improvement of signs and symptoms. This report documents hair loss as an uncommon presentation of Kawasaki disease.
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PMID:Hair loss as a sign of Kawasaki disease. 1723 74

Kawasaki disease (KD) is an acute febrile vasculitic syndrome of unknown etiology that preferentially affects the coronary artery. Interleukin-10 (IL-10) is a key proinflammatory cytokine, and a polymorphism near the major transcriptional start site of the IL-10 gene was shown to influence IL-10 production in vitro. This study investigated the association of the IL-10 promoter polymorphism with KD and its clinical parameters in Korean children. A total of 194 children with congenital heart disease (CHD) and 95 children with KD were included in this study. IL-10 (-627 A/C) polymorphism genotypes were determined using the single-base extension method. There was no difference in the allele frequencies of IL-10 (-627 A/C) polymorphism between CHD children and KD children. KD children with one or two copies of the IL-10 (-627C) allele showed significantly lower albumin levels (p = 0.020) and higher frequencies of early coronary artery aneurysm [62.22% versus 37.78%, adjusted odds ratio (aOR) = 3.50, 95% confidence interval (CI): 1.50-8.16] compared with KD children with the common IL-10 (-627A) allele. These findings suggest that the IL-10 (-627 A/C) promoter polymorphism might be a genetic marker for the risk of early coronary artery complication in KD.
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PMID:The IL-10 (-627 A/C) promoter polymorphism may be associated with coronary aneurysms and low serum albumin in Korean children with Kawasaki disease. 1741 67

Kawasaki disease is the most common cause of multisystem vasculitis in childhood. Kawasaki disease has been reported throughout the world and affects children of all ethnicity. Coronary artery damage from Kawasaki disease is the leading cause of acquired heart disease in children in the developed world. Diagnostic tests and prognostic markers are lacking, and questions remain unanswered in our understanding of the etiopathogenesis of the disease, thus limiting our ability to improve therapy and coronary outcome. In this article I will review advances made in an animal model of disease, which has helped advance our understanding of the etiology and pathogenesis of this fascinating clinical syndrome.
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PMID:Lessons learned from an animal model of Kawasaki disease. 1742 74

Kawasaki disease (KD) is primarily the systemic vasculitis of childhood that affects mainly the medium-sized arteries, such as the coronary arteries. KD is the leading cause of acquired heart disease, whereas the incidence of rheumatic fever has declined. The most serious complication is coronary artery involvement. Among the children with KD who developed cardiac complications, pericarditis is a rare complication, with an incidence of 0.07%. We report our experience in a 5.5-year-old child with KD complicated with aneurysm of the left anterior descendant coronary artery and septated pericardial effusion, which has not been reported in the literature. The pericardial effusion disappeared very dramatically with intravenous immunoglobulin (IVIG) therapy. We would like to point out that septated pericardial effusion in cases of KD do not need any further therapy other than IVIG and high-dose acetylsalicylic acid.
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PMID:Septated pericarditis associated with Kawasaki disease: a brief case report. 1799 May 88

Kawasaki disease (KD) is a systemic vasculitis condition with a relatively unknown etiology. First described in 1967 by Tomisaku Kawasaki in Japan, KD has come to be widely diagnosed in every region of the world. The disease has a high prevalence in children ages 6 months to 5 years, particularly in those of Japanese descent. Patients often present with a high fever, rash, lymphadenopathy, and conjunctival injections, but there is no diagnostic test for KD. This paper presents data from our Kawasaki registry including 99 patients with emphasis on Kawasaki cardiopathy. Three patients died from complications of KD, and 1 patient underwent heart transplant for massive aneurysmal dilatation. The 4 explanted hearts showed a spectrum of pathological findings (acute thrombosis, vasculitis, and myocarditis), and 1 patient showed the long-term sequelae of vasculitis in the form of massive aneurysmal dilatation. Among the survivors, 30% showed aneurysmal dilatation. This paper reviews the most recent information regarding Kawasaki cardiopathy and underlying molecular mechanisms.
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PMID:Kawasaki disease: four case reports of cardiopathy with an institutional and literature review. 1800 Nov 58


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