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Abstract: Objective: It 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 design: We 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 12weeks 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. Result: The 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. Conclusion: Previously 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. [Copyright &y& Elsevier]

OBJECTIVES: We sought to analyze trends in admissions and to describe therapies used for acute Kawasaki disease over a 6-year period. METHODS: The Pediatric Health Information System provides patient data including demographic variables, International Classification of Diseases, Ninth Revision codes, and services billed to patients. Patient identifiers enable tracking of medication use in and across multiple admissions within a center. We analyzed data for patients with (1) a diagnosis code for Kawasaki disease, (2) intravenously administered immunoglobulin treatment during hospitalization, and (3) discharge between January 1, 2001, and December 30, 2006, from 27 hospitals contributing complete data over the study period. RESULTS: During the study period, 5197 Kawasaki disease admissions were identified for 4811 patients; numbers increased 32.6% from 2001 (n = 678) to 2006 (n = 899). Retreatment with intravenous immunoglobulin was administered to 712 patients (14.8%) over the study period. Other antiinflammatory therapies included intravenously administered methylprednisolone (5.8%), orally administered prednisone (2.8%), and infliximab (1%). Use of infliximab steadily increased from 0.0% (0 of 678 patients) in 2001 to 2.3% (21 of 899 patients) in 2006. Coronary artery aneurysms were coded for 3.3% of patients. Male patients, patients <1 year of age, and Hispanic patients were significantly more likely to have coding for coronary artery aneurysms. CONCLUSIONS: Our report provides the first large multicenter description of agents used in the treatment of intravenously administered immunoglobulin-resistant Kawasaki disease in the United States. Trends include increased numbers of admissions attributable to Kawasaki disease and increased usage of infliximab. [ABSTRACT FROM AUTHOR]

There have been no published prospective randomized clinical trials that have: (1) established an association between invasive dental and nondental invasive procedures and risk of infective endocarditis; or (2) defined the efficacy and safety of antibiotic prophylaxis administered in the setting of invasive procedures in the prevention of infective endocarditis in high-risk patients. Moreover, previous observational studies that examined the association of nondental invasive procedures with the risk of infective endocarditis have been limited by inadequate sample size. They have typically focused on a few potential at-risk surgical and nonsurgical invasive procedures. However, recent investigations from Sweden and England that used nationwide databases and demonstrated an association between nondental invasive procedures, and the subsequent development of infective endocarditis (in particular, in high-risk patients with infective endocarditis) prompted the development of the current science advisory.

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: The American College of Cardiology Quality Network enables national benchmarking and collaborative quality improvement through vetted metrics. We describe here our initial experience with the Quality Network., Methods: Quarterly data for metrics pertaining to chest pain, Kawasaki disease, tetralogy of Fallot, elevated body mass index, and others were shared with the collaboratives for benchmarking. National improvement efforts focussed on counselling for elevated body mass index and 22q11.2 testing in tetralogy of Fallot. Improvement strategies included developing multi-disciplinary workgroups, educational materials, and electronic health record advances., Results: Chest pain metric performance was high compared with national means: obtaining family history (90-100% versus 51-77%), electrocardiogram (100% versus 89-99%), and echocardiogram for exertional complaints (95-100% versus 74-96%). Kawasaki metric performance was high, including obtaining coronary measurements (100% versus 85-97%), prescribing aspirin (100% versus 86-99%), follow-up with imaging (100% versus 85-98%), and documenting no activity restriction without coronary aneurysms (83-100% versus 64-93%). Counselling for elevated body mass index was variable (25-75% versus 31-50%) throughout quality improvement efforts. Testing for 22q11.2 deletion in tetralogy of Fallot patients was consistently above the national mean (60-85% versus 54-68%) with improved genetics data capture., Conclusion: The Quality Network promotes meaningful benchmarking and collaborative quality improvement. Our high performance for chest pain and Kawasaki metrics is likely related to previous improvement efforts in chest pain management and a dedicated Kawasaki team. Uptake of counselling for elevated body mass index is variable; stronger engagement among numerous providers is needed. Recommendations for 22q11.2 testing in tetralogy of Fallot were widely recognised and implemented.

Background: Transforming growth factor (TGF)-β is a multifunctional peptide that is important in T-cell activation and cardiovascular remodeling, both of which are important features of Kawasaki disease (KD). We postulated that variation in TGF-β signaling might be important in KD susceptibility and disease outcome., Methods and Results: We investigated genetic variation in 15 genes belonging to the TGF-β pathway in a total of 771 KD subjects of mainly European descent from the United States, the United Kingdom, Australia, and the Netherlands. We analyzed transcript abundance patterns using microarray and reverse transcriptase-polymerase chain reaction for these same genes, and measured TGF-β2 protein levels in plasma. Genetic variants in TGFB2, TGFBR2, and SMAD3 and their haplotypes were consistently and reproducibly associated with KD susceptibility, coronary artery aneurysm formation, aortic root dilatation, and intravenous immunoglobulin treatment response in different cohorts. A SMAD3 haplotype associated with KD susceptibility replicated in 2 independent cohorts and an intronic single nucleotide polymorphism in a separate haplotype block was also strongly associated (A/G, rs4776338) (P=0.000022; odds ratio, 1.50; 95% confidence interval, 1.25 to 1.81). Pathway analysis using all 15 genes further confirmed the importance of the TGF-β pathway in KD pathogenesis. Whole-blood transcript abundance for these genes and TGF-β2 plasma protein levels changed dynamically over the course of the illness., Conclusions: These studies suggest that genetic variation in the TGF-β pathway influences KD susceptibility, disease outcome, and response to therapy, and that aortic root and coronary artery Z scores can be used for phenotype/genotype analyses. Analysis of transcript abundance and protein levels further support the importance of this pathway in KD pathogenesis.

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.