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KATHRYN A TAUBERT, PH D University of Texas Southwestern Medical School, Dallas, Texas.
STANFORD T SHULMAN, D M Northwestern University Medical School, Chicago, Illinois.
See document patient information related to Kawasaki disease written by a writer of the AAFP staff and based on information provided by the American Heart Association.
Kawasaki disease is a leading cause of acquired heart disease in children in the United States and other developed countries Most children who contract the disease are less than two years and 80 percent of affected children are under five generalized vasculitis of unknown etiology, Kawasaki disease can cause abnormalities of the coronary arteries, including coronary aneurysms from 20 to 25 percent of untreated children have abnormalities of the coronary arteries, which can resolve or persist These anomalies are of particular concern because they can lead to thrombosis, move towards a segmental stenosis or, rarely, breaking the main cause of death of Kawasaki disease is a heart attack the cause of the disease remains unknown, but epidemiological surveys and clinical presentation suggest a microbial the criteria diagn ostiques, including fever and other main characteristics have been established in the acute phase of the disease, treatment with acetyl alicyclic acid and immunoglobulin administered intravenously is directed to reducing the inflammation of the coronary arteries and early treatment and myocardial recognition of Kawasaki disease can reduce the development of potentially life-threatening abnormalities of the coronary arteries.
Kawasaki disease or syndrome is a generalized vasculitis of unknown etiology and a leading cause of acquired heart disease in children in developed countries described by Kawasaki in 1967, the disease was called mucocutaneous lymph node syndrome 1 OF the results in 50 Japanese children, Kawasaki describes a single disease that has been characterized by fever, rash, conjunctival injection, cervical lymphadenitis, inflammation of the lips and oral cavity, and redness and swelling of the hands and feet Although initially thought to be a benign childhood disease, the disease has been found responsible for the death of a number of Japanese children, mainly under two years, which seemed to improve or have recovered from the 2 disease autopsies demonstrated thrombosed aneurysms occlusion of the coronary artery and myocardial infarction resulting It is now known as coronary artery anomalies in about 20 to 25 percent of children with untreated Kawasaki 3 of disease. 5
Kawasaki disease was reported in the world 6 In the United States, the disease is increasingly recognized since the 1970s, and a number of regional outbreaks have been reported since 1976 July 10 Kawasaki disease is more common in boys than girls ratio of about 1 5 1 approximately 80 percent of affected children are less old, less than 2 percent of children age five recurrences 11.
The disease occurs year round, but more cases are reported in the winter and spring of December 14 annual incidence rate in the range US and Canada about six to 11 cases per 100,000 children under five 10 December 15 each year in this country, up to 3 500 children are hospitalized for Kawasaki disease 15 16 Although the absolute number of US cases is the largest among white children, incidence rates in North America are highest among children of Asian origin particularly those of Japanese or Korean bottom 6.
In Japan, 12 investigations of the epidemiological impact nationally on Kawasaki disease were conducted at two-year intervals starting in 1970. At the end of the 12th survey in 1992, 116.848 children were reported to have contracted Kawasaki disease 17 the annual incidence rate for 1991 1992 was 90 cases per 100,000 children under five years 18 percent of children affected about 1 were found to have a family history of Kawasaki disease 18 siblings more than half of the cases of siblings developed within 10 days after the first case.
epidemiology and clinical presentation suggest an infectious etiology of Kawasaki disease To date, however, a causative agent was not documented a variety of infectious agents have been proposed, including Rickettsia, viruses mainly virus Epstein-Barr virus and retroviruses, Streptococcus staphylococci, Propionibacterium species and parvovirus standard laboratory studies have failed to identify a specific agent 19.
A superantigen involved in the study to find out the toxins produced by certain staphylococci and streptococci and proposed a variant of toxic shock syndrome toxin 1 produced by Staphylococcus aureus associated as a possible cause of Kawasaki disease 20 To date, however, results of this study was not supported 21 22.
Although the initiating event has not been identified, the immune system is known to be involved in the acute phase of Kawasaki disease in response to an unknown trigger process, immunomodulatory marked abnormalities are observed is assumed the various secreted cytokines target vascular endothelial cells, producing cell surface neoantigens the antibodies produced against these antigens can then target the vascular endothelium, leading to a cascade of events leading to vascular damage.
In the absence of a specific diagnostic test, Kawasaki disease is a clinical diagnosis based on the characteristic history and physical signs were clinical criteria developed by research Kawasaki Japan Committee23 of the disease and by American Heart Association AHA 24 These criteria are classified as main clinical findings and other important clinical and laboratory findings.
The main clinical findings in Kawasaki disease are listed in Table January 25 fever and at least four of the five major clinical results should usually be present for the diagnosis, however, atypical or incomplete cases of Kawasaki disease, where patients have less than four five main characteristics were increasingly reported conventional diagnostic criteria fail more often in children, especially those under six months in patients with fever and three of the main clinical features can be diagnosed with Kawasaki disease where coronary heart disease is detected by two-dimensional echocardiography or coronary angiography.
Diagnostic criteria for Kawasaki disease Major clinical outcomes.
The fever lasts at least five days, fever is usually high and often spiking to 40 C 104 F or higher and persists in untreated patients for one to two weeks or more.
The presence of at least four of the top five following features.
Changes in the ends of these changes are distinctive and acute include redness, swelling and sometimes induration of the hands and feet one to three weeks after the onset of fever, desquamation of fingers and toes occurs about one to two months after the onset of fever, Beautiful lines of white lines across the nails may appear.
rash polymorphic eruption of the skin involves the trunk and extremities and may take several forms, including urticaria maculopapular rash a measles-like rash occasionally with target lesions or diffuse rash and vesicles bullae scarlatiniform unseen L rash usually appears within five days after the onset of fever.
bulbar conjunctiva bilateral conjunctival injection, rather than palpebral or tarsal are involved Generally, the limbic region is spared the conjunctival injection is not associated with an exudate and is usually painless.
The changes in the lips and oral cavity these changes include language strawberry, redness and cracking of the lips, and erythema of the oropharyngeal mucosa ulcerative lesions are not visible.
cervical lymphadenopathy at least one lymph node of a diameter of 1 5 cm or more lymphadenopathy is usually unilateral, with firm and slightly soft nodes.
Exclusion of other diseases with similar results.
Many experts believe that when the classic features are present, Kawasaki disease can be diagnosed by experienced observers before day 5 of the fever.
The first four of the five major clinical findings are present in about 90 percent of patients with cervical lymphadenopathy Kawasaki disease was observed in 50 to 75 percent of patients.
Adapted from Dajani AS, Taubert KA, Gerber MA Shulman ST P Ferrieri, M Freed et al Diagnosis and treatment of Kawasaki disease in children Circulation 1993; 87 80 1776.
Diagnostic criteria for Kawasaki disease Major clinical outcomes.
The fever lasts at least five days, fever is usually high and often spiking to 40 C 104 F or higher and persists in untreated patients for one to two weeks or more.
The presence of at least four of the top five following features.
Changes in the ends of these changes are distinctive and acute include redness, swelling and sometimes induration of the hands and feet one to three weeks after the onset of fever, desquamation of fingers and toes occurs about one to two months after the onset of fever, Beautiful lines of white lines across the nails may appear.
rash polymorphic eruption of the skin involves the trunk and extremities and may take several forms, including urticarial rash, maculopapular rash morbilliform sometimes with target lesions or diffuse bullae scarlatiniform rash and blisters are not seen The rash usually appears within five days after the onset of fever.
bulbar conjunctiva bilateral conjunctival injection, rather than palpebral or tarsal are involved Generally, the limbic region is spared the conjunctival injection is not associated with an exudate and is usually painless.
The changes in the lips and oral cavity these changes include language strawberry, redness and cracking of the lips, and erythema of the oropharyngeal mucosa ulcerative lesions are not visible.
cervical lymphadenopathy at least one lymph node of a diameter of 1 5 cm or more lymphadenopathy is usually unilateral, with firm and slightly soft nodes.
Exclusion of other diseases with similar results.
Many experts believe that when the classic features are present, Kawasaki disease can be diagnosed by experienced observers before day 5 of the fever.
The first four of the five major clinical findings are present in about 90 percent of patients with cervical lymphadenopathy Kawasaki disease was observed in 50 to 75 percent of patients.
Adapted from Dajani AS, Taubert KA, Gerber MA Shulman ST P Ferrieri, M Freed et al Diagnosis and treatment of Kawasaki disease in children Circulation 1993; 87 80 1776.
A high index of suspicion is necessary to diagnose Kawasaki disease in patients who have a number of standard features, but not enough to meet the diagnostic criteria Some of these patients have prolonged unexplained fever or cervical lymphadenopathy not responding antibiotic treatment, or they may have a fever with two to three classic criteria of Kawasaki disease, it has become clear that patients with Kawasaki disease atypical or incomplete are a significant risk for the development of abnormalities26 coronary and would therefore benefit from treatment if they could be identified.
Since the main clinical results that meet the diagnostic criteria for Kawasaki disease are not specific, other diseases and disorders with similar clinical results should be excluded Table February 25 A careful examination of measles is particularly important because appropriate control measures can be taken quickly if the disease is misdiagnosed as Kawasaki disease.
differential diagnosis of Kawasaki disease selected.
Paronychial day peeling 12 of the disease in a child of three years with Kawasaki disease.
Figures 1 to 4 used with the Committee's permission on Rheumatic Fever, Endocarditis, and Kawasaki Disease AHA Council on Cardiovascular Disease in the diagnostic guidelines for Disease Young Kawasaki Association Dallas American Heart 1993 Right to American author Heart Association.
Paronychial day peeling 12 of the disease in a child of three years with Kawasaki disease.
Figures 1 to 4 used with the Committee's permission on Rheumatic Fever, Endocarditis, and Kawasaki Disease AHA Council on Cardiovascular Disease in the diagnostic guidelines for Disease Young Kawasaki Association Dallas American Heart 1993 Right to American author Heart Association.
The results not included in the major diagnostic criteria are often present in patients with Kawasaki disease are divided into cardiac outcomes, not heart in the laboratory.
Cardiovascular manifestations may be prominent in the acute phase of the disease and is the leading cause of morbidity and mortality Table 28 March pancarditis can occur, and the coronary arteries can be affected 3 pericardial effusion, which usually resolves spontaneously without treatment specifically, is detected by echocardiography in approximately 30 percent of patients 3 clinically recognizable myocarditis, sometimes with congestive heart failure, is often manifested by tachycardia out of proportion to the degree of fever, gallop rhythm or arrhythmia changes electrocardiographic are nonspecific and are observed in one third of patients These changes include depression decrease wave voltage R, ST segment to the flattening of the T wave or an inversion and a PR or prolonged QT interval, or both 3.
TABLE 3 cardiac outcomes in acute Kawasaki disease.
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coronary artery anomalies develop in 20 to 25 percent of children with untreated disease Kawasaki 3 of 5 malformations may include ectasia diffuse coronary aneurysms or 5 and 6 of 27 patients who have giant aneurysms maximum internal diameter of at least 8mm have the worst prognosis and are at the greatest risk of developing coronary thrombosis, stenosis or myocardial infarction Unlike smaller aneurysms, these giant aneurysms are usually not solved children with abnormalities coronary arteries, especially the coronary giant aneurysms are more likely to have aneurysms other medium muscular arteries, such as kidney, brachial or femoral artery aneurysms usually become apparent to three weeks after the onset of fever; their appearance more than five weeks after the onset of the disease is rare risk factors for coronary aneurysms in patients with Kawasaki disease are listed in Table 4 25th.
Two-dimensional echocardiography Left Tiny normal vessels abnormally dilated vessels right arrows aorta AO arrows; PA pulmonary artery; LAD left anterior descending coronary artery; CX circumflex coronary artery; The main left main coronary artery.
Two-dimensional echocardiography Left Tiny normal vessels abnormally dilated vessels right arrows aorta AO arrows; PA pulmonary artery; LAD left anterior descending coronary artery; CX circumflex coronary artery; The main left main coronary artery.
Echocardiograms and angiograms obtained in long-term follow-up studies indicate that coronary aneurysms resolve within five to 18 months about 50 percent of patients 4 In these studies, half of the patients with persistent abnormalities showed decrease in size aneurysms, a third had resolution of aneurysms but developed coronary obstruction or stenosis, and the rest was fine irregularities of the walls without coronary stenosis myocardial ischemia can occur in the presence of a stenosis obstruction.
If untreated in the first 10 days of illness, patients with Kawasaki disease are under one year especially those younger than eight months have a significantly increased risk of developing aneurysms of the coronary artery 29 compared to older children, young children tend to have subtle manifestations of Kawasaki disease therefore, the presence of this disease is often more difficult to suspect and confirm in very young patients.
Several noncardiac clinical results can be observed in patients Table of Kawasaki disease in May 28 For example, infants and young children with this disease are often more irritable than children with other febrile diseases, arthritis and arthralgia more common among older girls may occur in the first two to three weeks of the disease and usually involve the knees, ankles or hips About a quarter of patients undergoing lumbar puncture in the acute phase of Kawasaki disease have results consistent with aseptic meningitis Other common features include diarrhea, vomiting, abdominal pain and pneumonia.
TABLE 5 noncardiac clinical results Kawasaki acute illness.
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Acute distention acalculous of hydrops of the gallbladder may occur during the first two weeks of illness recently, the rate of elevated serum bile acids have been reported in patients with acute Kawasaki disease 30 less frequent findings include erythema and induration recent Bacille Calmette Guerin vaccination site Auditory abnormalities, testicular swelling and peripheral gangrene have been reported in patients with the disease.
Laboratory results and their frequency in Kawasaki disease are listed in Table June 28 Although these findings are not specific for Kawasaki disease, they can help to establish the diagnosis in the first seven to 14 days of disease, patients often have increased acute phase reactants; these values then return to normal within les six to eight weeks neutrophilia, mild anemia, hypoalbuminemia and elevated levels of serum immunoglobulin E can also be seen thrombocytosis is often present after the first week of the disease and can be marked proteinuria and sterile pyuria urethral origin are also common.
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In the absence of a known etiologic agent, initial treatment of Kawasaki disease is to reduce fever and other inflammatory properties to help prevent the occurrence of abnormalities of the coronary arteries and following myocardial ischemic injury treatment long-term target is to prevent coronary thrombosis by inhibiting platelet aggregation.
Current recommendations for the initial processing preferably during the first 10 days of the onset of the disease include intravenous immunoglobulin to help prevent abnormalities of the coronary arteries and the oral administration of the acid aspirin acetylsalicylic at high doses accelerate the resolution of acute manifestations of Kawasaki disease, especially table 7 25 fever AHA recommends that aspirin given at a dose of 80 to 100 mg per kg per day during the acute phase this disease dosage should be continued until the patient has been afebrile for at least several days the dosage is reduced to 3 to 5 mg of aspirin per kg per day and continued therapy for its antithrombotic effect of aspirin can be discontinued after six to eight weeks echocardiography showed no signs of abnormalities of the coronary arteries in case Coronary artery anomalies are detected, the low-dose aspirin therapy should be continued indefinitely 31 To reduce the very small risk of Reye's syndrome, aspirin therapy should probably be out for a week or if the patient or flu varicella.
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KATHRYN A TAUBERT, PH D is a senior researcher in the Department of Science and Medicine at the National Center of the American Heart Association and associate professor of physiology at the University of Texas Southwestern Medical School, both in Dallas Dr. Taubert has a Ph.D. medical physiology at the University of Texas Southwestern medical School and completed a fellowship in cardiology at the Veterans Affairs medical Center in Dallas.
STANFORD T SHULMAN, MD is Professor of Pediatrics and Associate Dean for Academic Affairs at the Northwestern University Medical School and chief of the division of infectious diseases at Memorial Hospital for Children, both in Chicago Dr. Shulman received his medical degree from the University of Chicago medical School, where he was also a residence and was Chief resident Dr. Schulman completed a fellowship in pediatric infectious diseases and immunology at the University of Florida College of Medicine, Gainesville.
Correspondence address A to Kathryn Taubert, PhD Department of Science and Medicine, the American Heart Association, 7272 Greenville Ave Dallas, TX 75231-4596 Reprints are not available from the authors.
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This article is part of a series developed in collaboration with the American Heart Association Guest editor of the series is Rodman Starke D, M D Senior Vice President of Science and Medicine, the American Heart Association, Dallas.
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