Clinical aspects of 100 patients with Kawasaki disease

Article Abstract:

Kawasaki disease is a systemic vasculitis, or inflammation of the blood vessels, which chiefly affects children under the age of five. In up to 40 percent of cases the coronary arteries may be involved, and thrombosis (clot formation), heart attack, aneurysm (dilation of a vessel wall), or death may result. Diagnosis of the disease is made difficult by the lack of diagnostic laboratory tests; the cause of Kawasaki disease is unknown. The medical findings in 100 cases of Kawasaki disease are reviewed in this article. Diagnosis was based on five of six known medical criteria, or on four criteria if an aneurysm was also found. The criteria include: fever persisting for more than five days; inflammation of the mouth; swelling of the lymph nodes; conjunctivitis (an eye inflammation); redness, swelling, and peeling of the palms and soles; and a widespread skin rash. Boys were affected slightly more frequently than girls, and the majority of cases occurred before the age of three. More cases occurred in spring and early summer. Thirty-five cases had heart involvement, including 18 coronary artery aneurysms, and these 35 patients had significantly higher platelet and white blood cell counts. Blood tests were positive for nonspecific indications of systemic or inflammatory diseases in most patients, while 23 patients had measurable levels of autoantibodies, antibodies formed against body tissues. No single infective organism was found in patients. Treatment varied, and included aspirin (used in most cases), dipyridamole, immunoglobulin, prostacyclin, plasma exchange or exchange transfusions, and streptokinase. One patient died, one relapsed seven years later, many had recurrence of symptoms during other viral infections, three developed ataxia (a movement disorder) or chest pain, and 74 patients recovered completely. Heart complications occurred at one month or later in 27 cases, and 26 of these children gradually recovered (one with persistent aneurysms), while one died. (Consumer Summary produced by Reliance Medical Information, Inc.)

Pediatric diseases, Children, Demographic aspects, Diseases

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Antiendothelial cell antibodies detected by a cellular based ELISA in Kawasaki disease (enzyme-linked immunosorbent assay)

Article Abstract:

Kawasaki disease mainly affects children under the age of five; it is a systemic vasculitis, meaning the blood vessels become inflamed. In up to 40 percent of cases, the coronary arteries which bring blood to the heart itself are involved, and thrombosis (clot formation), heart attack, aneurysm (dilation of a vessel wall), or death may result. Diagnosis of the disease is difficult because of the lack of a diagnostic laboratory test; the cause of Kawasaki disease is unknown but it may be due to an infectious agent. Studies have suggested that immune system function is affected by Kawasaki disease, as there are increased levels of antibodies associated with altered levels of lymphocytes (infection-fighting white blood cells). A common finding in rheumatic disorders is the formation of autoantibodies, antibodies that recognize and attack the body's own tissues. Recent studies in patients with Kawasaki disease have shown the presence of antibodies against endothelial cells, which are the type of cells forming the lining of blood vessels. To further investigate this, an antibody-based laboratory test, the ELISA (enzyme-linked immunosorbent assay), was used to study the presence of antiendothelial cell antibodies in 32 patients who were diagnosed with Kawasaki disease and in 12 healthy children and 10 children with fevers (fever is one symptom of Kawasaki disease, and these children served as controls). Of the 32 Kawasaki patients, 21 had significantly elevated levels of antiendothelial cell antibodies, as compared with the healthy or febrile children. Studies in six patients showed that antibody levels rose about 10 days after the onset of the disease and declined with convalescence. The presence of antiendothelial cell antibodies was not significantly associated with cardiovascular complications. The association between autoantibodies and the cause and disease process in Kawasaki disease requires further study. (Consumer Summary produced by Reliance Medical Information, Inc.)

Analysis, Autoantibodies, Physiological aspects, Virus diseases

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Kawasaki disease: echocardiographic features in 91 cases presenting in the United Kingdom

Article Abstract:

Kawasaki disease (KD) is an acute childhood disease characterized by fever, which results in subsequent cardiac complications in a significant proportion of patients. The cause is unknown, but a viral infection or an immune disorder are suspected. Frequently, cardiac complications like aneurysms (ballooning of blood vessel walls) and coronary artery disease may develop some time after the fever episode is finished. The incidence and severity of abnormalities of coronary arteries were studied during an eight-year period in 91 children who had KD to determine an appropriate way to follow-up these patients. Echocardiography, ultrasound imaging of the heart, was used to detect aneurysms and other lesions. Echocardiography was performed in 75 children during the acute phase of KD, the first month, and lesions were found in 21 patients. Aneurysms were found more frequently toward the second half of the month, and tended to be more severe than those found earlier. Two children with moderately-sized lesions had heart attacks, and one died. No lesions were found when echocardiography was performed more than one month after development of KD. Of the 37 patients who had repeated echocardiograms up to seven years after acute KD, eight still had aneurysms, but were symptom-free. However, echocardiography is not extremely sensitive in detecting coronary artery disease. The study suggests that school-age children who have had KD should be evaluated for constricted coronary arteries before participating in physical education, especially those who had large aneurysms during the acute phase. Annual evaluation of children with a history of KD is recommended. (Consumer Summary produced by Reliance Medical Information, Inc.)

Usage, Prognosis, Echocardiography

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