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Background Health care transition (HCT) is a period of high vulnerability for patients with chronic childhood diseases, particularly when patients shift from a pediatric to an adult care setting. An increasing number of patients with Kawasaki disease (KD) who develop medium and large coronary artery aneurysms (classified by the American Heart Association according to maximal internal coronary artery diameter Z-scores ≥5 and ≥10, respectively) are becoming adults and thus undergoing an HCT. However, a poor transition to an adult provider represents a risk of loss to follow-up, which can result in increasing morbidity and mortality. Methods and Results This scientific statement provides a summary of available literature and expert opinion pertaining to KD and HCT of children as they reach adulthood. The statement reviews the existing life-long risks for patients with KD, explains current guidelines for long-term care of patients with KD, and offers guidance on assessment and preparation of patients with KD for HCT. The key element to a successful HCT, enabling successful transition outcomes, is having a structured intervention that incorporates the components of planning, transfer, and integration into adult care. This structured intervention can be accomplished by using the Six Core Elements approach that is recommended by the American Academy of Pediatrics, the American Academy of Family Physicians, and the American College of Physicians. Conclusions Formal HCT programs for patients with KD who develop aneurysms should be established to ensure a smooth transition with uninterrupted medical care as these youths become adults.

Background Accurate prediction of coronary artery aneurysms ( CAAs ) in patients with Kawasaki disease remains challenging in North American cohorts. We sought to develop and validate a risk model for CAA prediction. Methods and Results A binary outcome of CAA was defined as left anterior descending or right coronary artery Z score ≥2.5 at 2 to 8 weeks after fever onset in a development cohort (n=903) and a validation cohort (n=185) of patients with Kawasaki disease. Associations of baseline clinical, laboratory, and echocardiographic variables with later CAA were assessed in the development cohort using logistic regression. Discrimination (c statistic) and calibration (Hosmer-Lemeshow) of the final model were evaluated. A practical risk score assigning points to each variable in the final model was created based on model coefficients from the development cohort. Predictors of CAAs at 2 to 8 weeks were baseline Z score of left anterior descending or right coronary artery ≥2.0, age 40-fold greater in the validation cohort (odds ratio, 44.0; 95% CI, 10.8-180 [ P

BackgroundCoronary 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

ImportanceAmerican Heart Association guidelines recommend echocardiography in Kawasaki disease at baseline, 1 to 2 weeks, and 4 to 6 weeks after treatment to detect coronary artery abnormalities. However, these examinations are expensive and may require sedation in young children, which is burdensome and carries some risk.ObjectiveTo assess the benefit of additional echocardiographic imaging at 6 weeks in patients with uncomplicated Kawasaki disease who had previously normal coronary arteries.Design, setting, and participantsThis is a retrospective review of patients with Kawasaki disease who were cared for between 1995 and 2014 in 2 academic pediatric referral practices Eligibility criteria included receiving intravenous immunoglobulin treatment for acute Kawasaki disease at a center; the absence of significant congenital heart disease; available echocardiographic measurements of both the right and left anterior descending coronary arteries at 10 days or less after diagnosis (baseline), 2 (±1) weeks, and 6 (±3) weeks of illness; and normal coronary arteries at baseline and 2 weeks, defined as maximum coronary artery z scores less than 2.0 and no distal aneurysms. Data analysis was completed from March 2015 to November 2015.Main outcomes and measuresThe number of patients with right coronary artery or left anterior descending coronary artery z scores of 2.0 or more at 6 weeks.ResultsThe median age of the 464 included patients was 3.3 years (interquartile range, 1.8-5.4 years); 264 (56.9%) were male, 351 of 414 for whom data were available (84.8%) had complete Kawasaki disease, and 66 (14.2%) received additional intravenous immunoglobulin treatment. At 6 weeks of illness, 456 patients (98.3%) who had had normal coronary artery z scores at baseline and 2 weeks continued to have normal z scores. Of the remaining 8 patients (1.7%), the maximum z score within 6 weeks was 2.0 to 2.4 in 5 patients (1.2%), 2.5 to 2.9 in 1 patient (0.2%), and 3.0 or more in 2 patients (0.4% [95% CI, 0.1%-1.5%]). Coronary artery dimensions ultimately normalized in all but 1 patient, who had minimal dilation at 6 weeks (right coronary artery z score, 2.1). Sensitivity analyses using less restrictive cut points (eg, a maximum z score

BACKGROUND. Pediatric SARS-CoV-2 infection can be complicated by a dangerous hyperinflammatory condition termed multisystem inflammatory syndrome in children (MIS-C). The clinical and immunologic spectrum of MIS-C and its relationship to other inflammatory conditions of childhood have not been studied in detail. METHODS. We retrospectively studied confirmed cases of MIS-C at our institution from March to June 2020. The clinical characteristics, laboratory studies, and treatment response were collected. Data were compared with historic cohorts of Kawasaki disease (KD) and macrophage activation syndrome (MAS). RESULTS. Twenty-eight patients fulfilled the case definition of MIS-C. Median age at presentation was 9 years (range: 1 month to 17 years); 50% of patients had preexisting conditions. All patients had laboratory confirmation of SARS- CoV-2 infection. Seventeen patients (61%) required intensive care, including 7 patients (25%) who required inotrope support. Seven patients (25%) met criteria for complete or incomplete KD, and coronary abnormalities were found in 6 cases. Lymphopenia, thrombocytopenia, and elevation in inflammatory markers, D-dimer, B-type natriuretic peptide, IL-6, and IL-10 levels were common but not ubiquitous. Cytopenias distinguished MIS-C from KD and the degree of hyperferritinemia and pattern of cytokine production differed between MIS-C and MAS. Immunomodulatory therapy given to patients with MIS-C included intravenous immune globulin (IVIG) (71%), corticosteroids (61%), and anakinra (18%). Clinical and laboratory improvement were observed in all cases, including 6 cases that did not require immunomodulatory therapy. No mortality was recorded in this cohort. CONCLUSION. MIS-C encompasses a broad phenotypic spectrum with clinical and laboratory features distinct from KD and MAS. FUNDING. This work was supported by the National Institutes of Health, National Institute of Arthritis and Musculoskeletal and Skin Diseases; the National Institute of Allergy and Infectious Diseases; Rheumatology Research Foundation Investigator Awards and Medical Education Award; Boston Children's Hospital Faculty Career Development Awards; the McCance Family Foundation; and the Samara Jan Turkel Center., Introduction Since the onset of the Coronavirus disease 2019 (COVID-19) pandemic, millions of individuals have been infected with severe acute respiratory syndrome Coronavirus 2 (SARS-CoV-2) and more than 500,000 deaths [...]

ObjectiveIt has been claimed that the aneurysm rate for Kawasaki disease (KD) patients in Japan is lower than in the U.S. However it has been difficult to compare coronary artery (CA) outcomes between the two countries because of different definitions for CA abnormalities. Therefore, we compared CA internal diameters between Japanese and U.S. KD patients using standard definitions and methods.Study designWe retrospectively reviewed CA outcomes in 1082 KD patients from 2 centers in the U.S. and 3 centers in Japan and compared Z-max scores (maximum internal diameter for the left anterior descending or right coronary artery expressed as standard deviation units from the mean (Z-score) normalized for body surface area) obtained within 12 weeks after onset and calculated using two different regression equations from Canada (Dallaire) and Japan (Fuse). We defined a Z-max of < 2.5 as normal and a Z-max of ≥ 10 as giant aneurysm.ResultThe median Z-max for the U.S. and Japanese subjects was 1.9 and 2.3 SD units, respectively (p < 0.001). There was no significant difference in rates of patients with Z-max ≥ 5.0 between the countries. In a multivariable model adjusting for age, sex, and treatment response, being Japanese was still associated with a higher Z-max score.ConclusionPreviously reported differences in aneurysm rates between Japan and the U.S. likely resulted from use of different definitions and nomenclature. Adoption of Z-scores as a standard for reporting CA internal diameters will allow meaningful comparisons among different countries and will facilitate international, collaborative clinical trials.

Aneurysms of the vascular wall represent a final common pathway for a number of inflammatory processes, including atherosclerosis and idiopathic vasculitis syndromes. Kawasaki disease (KD) is an acute, self-limited vasculitis in children and the leading cause of acquired coronary artery aneurysms. We sought to identify shared molecular mechanisms of aneurysm formation by genotyping eight polymorphisms in matrix metalloproteinase (MMP)-1, 3, 7, 12 and 13 in the gene cluster on Chr.11q22, whose gene products have been implicated in aneurysm formation or are known to have elastase activity. We genotyped 482 US-UK KD patients (aneurysm+: n=111, aneurysm-: n=371) and tested our findings in an independent cohort of 200 Japanese KD patients (aneurysm+: n=58, aneurysm-: n=142). Analysis of the five MMP genes identified modest trends in allele and genotype frequencies for MMP-3 rs3025058 (-/T) and haplotypes containing MMP-3 rs3025058 (-/T) and MMP-12 rs2276109 (A/G) (nominal P=2 to 4 × 10(-5)) that conferred increased risk of aneurysm formation in US-UK subjects. This finding was validated in Japanese subjects and suggests the importance of this locus in aneurysm formation in children with KD. The region encompassing these risk haplotypes is a prime candidate for resequencing to look for rare genetic variation that may influence aneurysm formation.

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 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&apos;s health exist regardless of overall health status. In addition, children with giant coronary artery aneurysms had lower overall physical summary scores. Pediatrics 2003;111:579-583; Kawasaki disease, Child Health Questionnaire, functional health status, quality of life., ABBREVIATIONS. KD, Kawasaki disease; CHQ, Child Health Questionnaire; JRA, juvenile rheumatoid arthritis; ADHD, attention-deficit/hyperactivity disorder. Kawasaki disease (KD) is an acute, systemic vasculitis of unknown cause. First described in Japan [...]

Controversy surrounds the treatment of hypercholesterolemia in children. The National Cholesterol Education Program has recommended the possibility of drug treatment only after diet treatment for high cholesterol levels has been tried and proven ineffective. The efficacy and adverse effects of niacin treatment of hypercholesterolemia were investigated in a retrospective review of 21 children, ages 4 to 14 years, who received 550 to 2250 mg/day of niacin for severe hypercholesterolemia. Daily doses of more than 1000 mg reduced total cholesterol by 23% and low-density lipoprotein cholesterol by 30%, but it produced no measurable reduction of high-density lipoprotein cholesterol or triglycerides. Although the treatment was helpful, adverse, reversible side effects occurred, prompting researchers to conclude that niacin should be reserved for use in carefully supervised treatment of severe hypercholesterolemia by a lipid specialist., Objective. To determine the efficacy and adverse effects of niacin treatment of hypercholesterolemia in children. Design. Retrospective review. Setting. Two university hospital referral clinics. Patients. All children who received single-drug niacin treatment for severe hypercholesterolemia between 1980 and 1991. Results. Twenty-one children, aged 4 to 14 years, were treated with niacin, 500 to 2250 mg daily. Pretreatment total serum cholesterol value (mean [+ or -] SD) was 7.84 [+ or -] 1.14 mmol/L (303 [+ or -] 44 mg/dL), and low-density lipoprotein cholesterol value was 6.28 [+ or -] 1.16 mmol/L (243 [+ or -] 45 mg/dL). Niacin treatment in daily doses >1000 mg reduced total cholesterol by 23% and low-density lipoprotein cholesterol by 30% (P < .001) but had no effect on high-density lipoprotein cholesterol and triglycerides. As in adults, reversible adverse effects were common, occurring in 16 (76%) of the 21 children. Six children (29%) had reversible dose-related elevations of serum aminotransferase levels. Niacin therapy was discontinued in 8 children (38%) because of flushing, abdominal pain, vomiting, headache, or elevated serum aminotransferase levels. Conclusions. This study suggests that although niacin treatment in children is efficacious, adverse effects are common. Until further study demonstrates long-term safety, niacin treatment should be reserved for the closely-supervised treatment of severe hypercholesterolemia by a lipid specialist. Pediatrics 1993;92:78-82; cholesterol, hypercholesterolemia, niacin.

* Objective.--To determine the impact of nutritional counseling on lipoprotein profiles in dyslipoproteinemic children. Design.--Retrospective case review. Setting.--An academic hospital-based pediatric lipid clinic in Boston, Mass. Participants.--One hundred four newly referred children with primary dyslipoproteinemia. Interventions.--Nutritional recommendations were adapted from the National Cholesterol Education Program's step 2 diet. Three-day diet records were used to assess baseline and follow-up diets. Results.--Two thirds of the children continued to have excellent diets or improved their diets after counseling, but low-density lipoprotein cholesterol (LDL-c) values decreased by 15% or more in only 19% of children. The observed change in LDL-c was not significantly associated with nutritional counseling. However, a strong correlation was evident between dietary interventions and concentration of high-density lipoprotein cholesterol (HDL-c) values in serum. Marked fat restriction lowered HDL-c levels, while liberalization of use of fat, with emphasis on monounsaturates, in a subset of children following an excessively fat-restricted diet on presentation, appeared to improve HDL-c levels. Conclusions.--After nutritional counseling, LDL-c levels decreased by 15% or more in only 19% of dyslipoproteinemic children referred for treatment. There were no clear predictors of LDL-c responsiveness, but changes in dietary fat intake appeared to significantly influence HDL-c levels. (AJDC. 1993;147:378-381), Changing dietary fat intake appears to have a significant effect on levels of high-density lipoprotein cholesterol (HDL-c) but little effect on levels of low-density lipoprotein cholesterol levels (LDL-c). Of 104 children with abnormally high levels of LDL-c and abnormally low levels of HDL-c, 96% had parents or close relatives with abnormal levels of LDL-c and HDL-c or premature heart disease. Children's diets were analyzed based on 3-day diet records, and then they and their families received nutritional counseling on the importance of lowering total fat, saturated fat and cholesterol intake. Following counseling, 66.7% of the children had maintained excellent diets or had improved their diets. However, LDL-c levels dropped by 15% or more in only 19% of the children. HDL-c levels increased by 15% or more in 28% of the children. HDL-c levels rose significantly in children who initially were on very low fat diets but were then counseled to increase their intake of unsaturated fats.