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Background: Coronary artery aneurysms (CAAs) are a well-known complication of Kawasaki disease (KD), but there are no data on incidence or outcomes of systemic artery aneurysms (SAAs) in the current era., Methods: From April 1, 2016, to March 31, 2019, we screened for SAAs in 162 patients with KD at risk for SAAs with magnetic resonance angiography or peripheral angiography and analyzed incidence and early outcomes of SAAs., Results: Twenty-three patients had SAAs, demonstrating an incidence of 14.2% (23 of 162) in patients who were screened at 1 month after onset. The proportion of patients with SAAs was estimated to be 2% (23 of 1148) of all patients with KD. The median age at onset of KD with SAAs was 5 months. All patients with SAAs had CAAs, with z scores >8. Of patients with giant CAAs, 38.6% (17 of 44) had SAAs. A total of 129 SAAs occurred in 17 different named arteries. The most common sites for SAAs were the axillary (18.6%), common iliac (12.4%), and brachial (11.6%) arteries. During a median follow-up time of 6 months, 92.9% (79 of 85) of SAAs had some degree of regression, with 80% (68 of 85) of SAAs returning to normal. The overall regression rate was higher for medium to large SAAs than for medium to giant CAAs., Conclusions: Although the incidence of SAAs may not be as dramatically reduced as we expected compared with previous data, SAAs have a high regression rate during short-term follow-up., Competing Interests: POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose., (Copyright © 2019 by the American Academy of Pediatrics.)

Background: Coronary artery aneurysms (CAA) are a serious complication of Kawasaki disease. Treatment with intravenous immunoglobulin (IVIg) within 10 days of fever onset reduces the risk of CAA from 25% to <5%. Corticosteroids and infliximab are often used in high-risk patients or those with CAA at diagnosis, but there are no data on their longer-term impact on CAA., Methods: Retrospective multicenter study including children who had CAA with a z score ≥2.5 and <10 at time of diagnosis and who received primary therapy with IVIg alone or in combination with either corticosteroids or infliximab within 10 days of onset of fever., Results: Of 121 children, with a median age of 2.8 (range 0.1-15.5) years, 30 (25%) received primary therapy with corticosteroids and IVIg, 58 (48%) received primary therapy with infliximab and IVIg, and 33 (27%) received primary therapy with IVIg only. Median coronary z scores at the time of diagnosis did not differ among treatment groups ( P = .39). Primary treatment intensification with either corticosteroids or infliximab were independent protective factors against progression of coronary size on follow-up (coefficient: -1.31 [95% confidence interval: -2.33 to -0.29]; coefficient: -1.07 [95% confidence interval: -1.95 to -0.19], respectively)., Conclusions: Among a high-risk group of patients with Kawasaki disease with CAA on baseline echocardiography, those treated with corticosteroids or infliximab in addition to IVIg had less progression in CAA size compared with those treated with IVIg alone. Prospective randomized trials are needed to determine the best adjunctive treatment of patients who present with CAA., Competing Interests: POTENTIAL CONFLICT OF INTEREST: Dr Friedman has served as a paid expert witness in cases of missed diagnosis of Kawasaki disease (unrelated to the current study); the other authors have indicated they have no potential conflicts of interest to disclose., (Copyright © 2019 by the American Academy of Pediatrics.)

Objective: Kawasaki disease is the leading cause of acquired heart disease in children worldwide. This study characterizes the epidemiology of Kawasaki disease in Taiwan between 2003 and 2006., Methods: Using Taiwan's 2003-2006 national health insurance claims, we investigated the epidemiologic features of Kawasaki disease (ICD-9-CM code 446.1) and coronary artery aneurysm formation (International Classification of Diseases, Ninth Revision, Clinical Modification code 414.11) and compared the incidences of these diseases with those occurring between 1996 and 2002 in Taiwan and those reported by other countries., Results: During our 4-year study period, 3877 children and adolescents <20 years of age were hospitalized for Kawasaki disease. Ninety percent of these children were <5 years of age, and the male/female ratio was 1.62:1. The annual incidence of Kawasaki disease was 153 in 100000 children <1 year of age, 111 in children 1 year of age, 58 in children 2 years of age, 30 in children 3 years of age, 19 in children 4 years of age, and 5.2 in children 5 to 9 years of age. The overall incidence was 69 cases per 100000 for children <5 years of age. Kawasaki disease recurred in 1.5% of all cases. Kawasaki disease occurred most frequently in the summer and least frequently in the winter. Coronary artery aneurysm occurred in 7.2% (279 of 3877) of all Kawasaki disease cases., Conclusions: The overall incidence of Kawasaki disease was 69 in 100000 children <5 years of age between 2003 and 2006 in Taiwan, comparable with the incidence of 66 in 100000 children between 1996 and 2002. Taiwan has the third highest incidence of Kawasaki disease in the world, after Japan and Korea. In Taiwan, it occurs more frequently during the summer.

Objective: Kawasaki disease (KD) is the most common acquired heart disease in children worldwide. The incidence of KD varies among different countries, with Asian countries supposedly having higher incidences than Western countries. However, the incidence of KD in Taiwan has not been well investigated., Methods: Since the implementation of Taiwan's National Health Insurance (NHI) in 1995, NHI has covered health care for >96% its population. Using the NHI database from 1996 to 2002, we investigated epidemiologic features of KD (International Classification of Diseases, Ninth Revision, code 446.1), the rate of coronary artery aneurysm formation (International Classification of Diseases, Ninth Revision, code 414.11), and the change in incidence during the recent 7 years. We also compared the annual incidences in Taiwan with those reported by other countries., Results: During the 7-year study period, KD occurred most frequently in the summer and least frequently in winter. It is interesting that the highest peak occurred in the summer of 1998 at the same time that Taiwan's enterovirus 71 epidemic was occurring. Ninety-one percent of KD cases occurred in children who were <5 years old, and the male-to-female ratio was 1.70:1. Recurrence of KD was found in 1.3% (94 of 7305) of these children, and coronary artery aneurysm was found in 7.3% (536 of 7305). The annual incidence per 100,000 children was 146 in children <1 year old, 98 in 1-year-old children, 51 in 2-year-old children, 28 in 3-year-old children, 19 in 4-year-old children, and 5.3 in 5- to 9-year-old children; the incidence of KD decreased with increased age. The overall incidence was 66 cases per 100,000 children <5 years old from 1996 to 2002 with the annual incidence not differing significantly during the 7-year study period., Conclusions: KD in Taiwan occurs more frequently in boys and in the summer months. During the 7-year study period, the annual KD incidence in Taiwan of 66/100000 in children <5 years old was the second highest in the world after Japan.

Objective: The purpose of this study was to examine the physical and psychosocial well-being of children who have had Kawasaki disease (KD), including the influence of coronary artery status on health and health perceptions., Methods: The Child Health Questionnaire (CHQ) measures overall physical and psychosocial well-being in children 5 to 18 years. To study the long-term impact of KD on overall health status, we mailed the CHQ to patients without a history of coronary artery abnormalities (normal group), with regressed aneurysms (regressed group), with current coronary aneurysms <8 mm (mild-moderate aneurysm group), and with giant aneurysms >or=8 mm (giant group)., Results: Of 201 questionnaires mailed, 174 were delivered and 110 (63%) were completed. Median age (range) at completion was 10.5 years (5.1-17.9 years) and at illness onset was 3.1 years (0.2-12 years). There were no significant differences in psychosocial summary scores in any of the Kawasaki groups when compared with the US population sample. Physical summary scores were also similar to the US population sample in the normal coronary, mild-moderate aneurysm, and regressed aneurysm groups. However, the giant aneurysm group had significantly lower physical summary scores compared with the US population sample. Among subscales, general health perceptions in the KD groups were lower than in the US population sample, reaching statistical significance in all but the mild to moderate aneurysm group. In addition, parents whose children have had KD reported a higher proportion of anxiety issues, allergies, and orthopedic/bone/joint issues in their children than did the general US population sample. We did not find any difference in the incidence of attentional, behavioral, or learning issues when compared with the US population sample., Conclusions: KD patients without coronary artery aneurysms were similar to the general population in their general physical and psychosocial health. However, the parents of children in all KD groups reported lower general health perceptions than parents in the US population sample, suggesting that long-term concerns about their children's health exist regardless of overall health status. In addition, children with giant coronary artery aneurysms had lower overall physical summary scores.

Objective: Cardiac sequelae develop more frequently after recurrent Kawasaki disease than from the initial onset of the disease. The purpose of this study was to observe the existence of the sequelae at the initial and second onsets of the disease simultaneously with a large cohort., Materials and Methods: From the database of patients with Kawasaki disease prepared by the Japanese Kawasaki Disease Research Committee, 559 cases with recurrences recorded between 1989 through 1994 and their initial occurrence listed in the database were selected. Their proportions of cardiac sequelae after the initial and second onsets of Kawasaki disease were compared., Results: Of the 68 patients with cardiac sequelae after the initial onset, 32 (47%) suffered the sequelae after the second onset, whereas 78 (16%) of the 491 who were without cardiac sequelae after the initial onset developed the sequelae after the recurrence. Both proportions were higher than proportions in all patients with Kawasaki disease. In addition to the sex (male) and the existence of the sequelae after the initial onset, age at the second onset (older age) and the interval between the two episodes (longer period) were suspected to be risk factors for sequelae attributable to recurrent Kawasaki disease., Conclusion: Linked data of the initial and second episodes of Kawasaki disease showed that the risk of developing cardiac sequelae attributable to recurrent Kawasaki disease is high among both those with and without the sequelae at the initial episode. mucocutaneous lymph node syndrome, recurrence, cardiac sequelae, risk factors.