Article

Kawasaki Syndrome: Still a Fascinating Enigma

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Abstract

In the two decades since this condition was first recognized among Japanese schoolchildren, preventive measures to lower the incidence of coronary artery aneurysms and various cardiac sequelae have significantly reduced mortality. Although the etiology of Kawasaki syndrome remains poorly understood, new evidence points to an inherited defect in T-lymphocyte immunoregulation.

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... Хотя этиология системного васкулита Кавасаки остается далекой до окончательного выяснения, Клиническая микробиология и антимикробная химиотерапия № 3, Том 4,2002 ряд исследований свидетельствует о наследственно обусловленном дефекте в иммунной регуляции дее ятельности Tлимфоцитов у таких пациентов [77]. Результаты молекулярноогенетического типии рования позволяют уточнить генотипические осоо бенности класса II главного комплекса антигенов тканевой совместимости [78]. ...
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Chapter
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Chapter
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Article
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Renal insufficiency is a rare manifestation of Kawasaki disease. We report a 2.5-year-old boy with Kawasaki disease who developed acute renal failure during the acute phase of his illness. A percutaneous renal biopsy revealed acute interstitial nephritis. No etiological agent could be identified and renal recovery occurred with supportive care alone.
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Deep neck abscesses are serious complications of common upper respiratory tract infections in children. Children are especially prone to airway obstruction complicating deep neck abscess. Pediatricians must understand the presentations and treatment of deep neck abscesses to avoid potentially life-threatening complications.
Article
We compared the efficacy of intravenous gamma globulin plus aspirin with that of aspirin alone in reducing the frequency of coronary-artery abnormalities in children with acute Kawasaki syndrome in a multicenter, randomized trial. Children randomly assigned to the gamma globulin group received intravenous gamma globulin, 400 mg per kilogram of body weight per day, for four consecutive days; both treatment groups received aspirin, 100 mg per kilogram per day, through the 14th day of illness, then 3 to 5 mg per kilogram per day. Two-dimensional echocardiograms were interpreted blindly and independently by two or more readers. Two weeks after enrollment, coronary-artery abnormalities were present in 18 of 78 children (23 percent) in the aspirin group, as compared with 6 of 75 (8 percent) in the gamma globulin group (P = 0.01). Seven weeks after enrollment, abnormalities were present in 14 of 79 children (18 percent) in the aspirin group and in 3 of 79 (4 percent) in the gamma globulin group (P = 0.005). No child had serious adverse effects from receiving gamma globulin. We conclude that high-dose intravenous gamma globulin is safe and effective in reducing the prevalence of coronary-artery abnormalities when administered early in the course of Kawasaki syndrome.
Article
The object of our investigation was to determine the immunoregulatory abnormalities in 48 children with Kawasaki syndrome. We demonstrated a global lymphocytosis with marked expansion of the B cell subset. Despite an increase in B cell numbers, there was a decrease in in vitro immunoglobulin production in response to pokeweed mitogen and hydrocortisone stimulation. These abnormalities correlated with a marked increase in the percentage of CD4+2H4+ (CD4+CD45R+) T cells, a T cell subset thought to be responsible for inducing suppression. Other abnormalities of T cells include cutaneous and in vitro anergy and evidence of T cell activation. Our results suggest that the B cell abnormalities seen in Kawasaki syndrome may be partially explained by defects in T cell immunoregulation.
Article
A total of 110 children aged 3 months to 16 years underwent two-dimensional echocardiography of the coronary arteries. Forty-two normal subjects and 68 patients with Kawasaki disease were evaluated. All 68 patients with Kawasaki disease underwent selective coronary arteriography. The objectives of this study were to 1) develop a normal profile of the proximal left and right coronary arteries as to caliber and shape in infants, toddlers and children using echocardiography; 2) compare the dimensions and shape of the coronary arteries of patients with Kawasaki disease but no obvious aneurysms with those of the coronary arteries of normal children; and 3) develop criteria that would permit distinguishing a large but normal coronary artery from a true aneurysm in patients with Kawasaki disease. In the normal subjects and patients with Kawasaki disease, the caliber of the coronary arteries showed little variability from the ostium to 10 mm distally, and ranged in size from 2 mm in infants to 5 mm in teenagers. There was no significant difference between male and female subjects. The feature that distinguished the large but normal coronary artery without aneurysm from that with an aneurysm was its uniformity of caliber. Also, the caliber of the opposite coronary artery was generally at the lower limits of normal. It appears that the proximal coronary arteries of infants and children can be accurately assessed using high resolution two-dimensional echocardiography, and that sequential evaluation of subtle changes over time may be performed.
Article
Coronary aneurysms were demonstrated echocardiographically in 34 of 186 patients who presented with Kawasaki syndrome between 1979 and 1983. The aneurysms were confirmed by selective coronary angiography in 27 patients and by postmortem examination in one. The 27 surviving patients with proven aneurysms were followed for 2 to 40 months (mean 15), during which they received low dose (5 to 10 mg/kg) aspirin daily. Progressive improvement and resolution of aneurysms were observed by serial echocardiography in 18 patients and confirmed by angiography in 14. Coronary aneurysms persisted, however, in nine other patients for 14 to 40 months (mean 25.7). The incidence of aneurysm resolution was higher in children less than 1 year of age at the onset of the illness than in patients older than 1 year (100% vs 50%; p less than .001). Aneurysms were more likely to resolve in girls than in boys (100% vs 42%; p less than .001). Fusiform aneurysms tended to resolve more frequently than saccular lesions (80% vs 18%; p less than .025). Aneurysms located distally in the coronary arteries appear to regress more rapidly than proximal ones. We conclude that an age of less than 1 year at the onset of Kawasaki syndrome, female sex, and fusiform aneurysm morphology are significant factors that favor resolution of coronary artery aneurysms. However, important questions remain with regard to the long-term fate and functional capabilities of these healed lesions.
Article
We report four patients with Kawasaki disease in whom characteristic coronary artery abnormalities developed after illnesses that did not meet diagnostic criteria. An additional patient lacked a history of acute manifestations of Kawasaki disease, but severe Kawasaki-like arterial changes were noted at autopsy. Fever was present in four of the five patients, in three lasting from 7 to 14 days. Despite manifestation of few classic acute clinical features of Kawasaki disease, three of four patients had desquamation of the fingers and toes 10 to 14 days after onset of illness, and the fifth had desquamation several months prior to death. These patients were seen over a 2-year period during which 22 other children were seen with Kawasaki disease with coronary artery abnormalities. Thus, strict adherence to currently accepted criteria for diagnosis of Kawasaki disease may lead to failure to recognize incomplete forms of this illness, with potential sequelae of myocardial infarction or sudden death. This finding suggests that children with prolonged unexplained febrile illnesses, especially those associated with subsequent peripheral desquamation, should undergo echocardiography 3 to 4 weeks after onset of the illness. This practice would help to identify those patients with illnesses characterized by incomplete diagnostic criteria but in whom significant coronary abnormalities develop.