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

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.
 ...
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.
 ...
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.
 ...
PMID:[Kawasaki disease]. 1118 81

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.
 ...
PMID:[Clinical presentation, diagnosis and management of inflammatory heart diseases in childhood]. 1123 56

Kawasaki disease is one of the commonest vasculitides seen in children. It presents with prolonged fever and a polymorphic exanthem. It is a major cause of acquired heart disease in western society. Its exact cause is not known, but exposure to a superantigen has been suggested as a possible aetiological factor. Diagnosis of Kawasaki disease still relies on clinical criteria (Table 1) and investigations are done mainly to exclude other diseases and to detect early or established cardiac complications. Coronary complications can be reduced significantly by the use of intravenous immunoglobulin therapy combined with oral aspirin. The serious consequences of Kawasaki disease require a heightened awareness of this condition when dealing with childhood exanthems.
 ...
PMID:Kawasaki disease: an update. 1126 Jan 68

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.
 ...
PMID:Kawasaki disease--atypical presentation. 1133 31

Kawasaki disease (KD) is an acute systemic febrile illness of unknown aetiology, predominantly affecting children under five years of age. Initially described in 1967 by Tomisaku Kawasaki, it is now the most common cause of acquired heart disease in children in the developed world. Although normally self-limiting, KD is associated with a range of complications, the most important of which is the development of life-threatening coronary artery abnormalities. Here, we examine the evidence supporting the concept that KD is caused by an infectious agent. Various infectious agents--including bacterial, viral and Rickettsial organisms--have been implicated as potential causes, as have certain immunological agents such as bacterial toxin-mediated superantigens, allergens such as anionic detergents and house-dust mites, and some chemicals (including heavy metals). Following extensive research, however, no links between any of these individual agents and the disease have been established irrefutably. Despite this, most of the epidemiological and immunological evidence currently available indicates that the causative agent is most likely to be infectious in nature; and additional evidence highlights the likelihood that development of KD is multifactorial in nature, requiring certain genetic and immunological factors, and possibly a vector.
 ...
PMID:Kawasaki disease: is it caused by an infectious agent? 1144 Feb 4

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.
 ...
PMID:Kawasaki disease. 1148 Jul 56

Kawasaki disease is a syndrome of unknown etiology affecting children below 5 years of age and is a leading cause of acquired heart disease in many developed countries. Incidence of this disease in India is extremely low as evidenced by the meagre case reports. Complications due to this disease in Indian patients are still rarer. Here we report two cases of Kawasaki disease both of whom had a benign course. A comparison of this disease in Indian and Western literature shows that the incidence of cardiac complications in the Indian patients is about 10% while in the west it is reported at around 30%. This paucity of complications in the Indian patients may be the reason of poor reporting of this disease in our country.
 ...
PMID:Kawasaki disease--Indian perspective. 1156 55

Kawasaki disease is now being recognized as the leading cause of acquired heart disease in children in North America and Japan. This review discusses the recent developments and controversies in Kawasaki disease. Increasing evidence has supported an infectious etiology for Kawasaki disease; however, the debate continues as to whether the inflammatory response results from a conventional antigen or a superantigen. Recent immunohistochemistry findings suggest many vascular growth factors then play a role in the formation of the coronary artery lesions. Many studies have focused on the identification and therapy for patients resistant to conventional therapy, as well as the long-term prognosis of Kawasaki disease survivors. The recent advances in Kawasaki disease are helping to provide some clues in the etiology, pathogenesis and therapy for Kawasaki disease.
 ...
PMID:Recent developments in Kawasaki disease. 1196 55


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>