Characterization of Post-Thrombotic Syndrome in Children with Cardiac Disease.

OBJECTIVE: To assess the validity of existing clinical scales assessing the presence of physical and functional abnormalities for diagnosing post-thrombotic syndrome (PTS) in children, including specific evaluation of use in children with congenital heart disease (CHD). STUDY DESIGN: One hundred children aged >2 years (average age, 6 years), including 33 with CHD and previously proven extremity deep vein thrombosis (DVT), 37 with CHD and no previous DVT, and 30 healthy siblings, were blindly assessed for PTS using the modified Villalta Scale (MVS). All patients aged <6 years underwent neurodevelopmental testing and an age-appropriate quality of life assessment. RESULTS: The MVS identified mild PTS in 20 children and moderate PTS in 1 child (including 14 of 33 [42%] in the CHD/DVT group, 5 of 37 [14%] in the CHD/no DVT group, and 2 of 30 controls [7%]). The diagnosis of PTS was confirmed clinically in 14 patients, all of whom had previous thrombosis and 1 of whom was MVS-negative. MVS had an accuracy of 91% and performed reasonably well as a screening tool but poorly as a diagnostic tool. MVS reliability was acceptable. Children with PTS had similar quality of life as those without PTS but had higher rates of neurodevelopmental delays in gross motor skills (70% vs 24%; P = .02) and problem-solving indicators (60% vs 15%; P = .008). CONCLUSIONS: Using the MVS scale for PTS screening in children with CHD is feasible and reliable, and the scale has good correlation with a clinical diagnosis of PTS despite a high prevalence of false-positive findings. Further research is needed to determine the clinical relevance of PTS in this population.

Socioeconomic Status and Kawasaki Disease Outcomes in a Single-Payer Health Care System.

Background: For patients with Kawasaki disease (KD), lower socioeconomic status (SES) may adversely affect the timeliness of presentation and initiation of intravenous immune globulin, and coronary artery outcomes. Multipayer systems have been shown to affect health care equity and access to health care negatively. We sought to determine the association of SES with KD outcomes in a single-payer health care system. Methods: Patients with KD presenting from 2007 to 2017 at a single institution were included. SES data were obtained by matching patient postal code district with data from the 2016 Census Canada. Results: SES data were linked for 1018 patients. The proportion of households living below the after-tax low-income cutoff in the patient's postal code district was 13% for not treated, 13% for delayed intravenous immune globulin treatment, and 12% for prompt treatment (P = 0.58). Likewise, the average median annual household income was unrelated to delayed or no treatment. The percentage >15 years of age with advanced education differed between groups at 33%, 29%, and 31% for delayed treatment, prompt treatment, and missed groups, respectively (P = 0.004). SES variables were not significantly different for those with vs without coronary artery aneurysms (max Z-score: >2.5), including the proportion of households living below low-income cutoff (12% vs 13%; P = 0.37), average median annual household income (CAD$81,220 vs $82,055; P = 0.78), and proportion with a university degree (33% vs 31%; P = 0.49), even after adjusting for sex, age, year, and KD type. Conclusions: Timeliness of treatment for KD and coronary artery outcomes were not associated with SES variables within a single-payer health care system.

Contexte: Chez les patients atteints de la maladie de Kawasaki (MK), un statut socioéconomique (SSE) plus difficile pourrait retarder le moment de la première consultation et le début du traitement par immunoglobuline intraveineuse (IgIV) ainsi que peser sur les résultats associés aux artères coronaires. Il a été démontré que les systèmes à payeurs multiples compromettent l'équité en matière de soins de santé et l'accès à ces derniers. Nous avons cherché à déterminer s'il existait un rapport entre le SSE et les résultats associés à la MK au sein d'un système de soins de santé à payeur unique. Méthodologie: L'étude comprenait des patients atteints de la MK qui se sont présentés à un même établissement entre 2007 et 2017. Les données sur le SSE ont été obtenues en associant le code postal des patients aux données du recensement canadien de 2016. Résultats: Les données sur le SSE de 1 018 patients ont été répertoriées. La proportion des foyers qui étaient sous le seuil de faible revenu (SFR) après impôt dans la circonscription correspondant à leur code postal était la suivante : 13 % pour les patients non traités, 13 % pour les patients chez qui le traitement par IgIV a été tardif et 12 % pour les patients qui ont rapidement reçu un traitement (p = 0,58). De même, aucune relation n'a été établie entre le revenu annuel médian des ménages et un traitement tardif ou une absence de traitement. Le pourcentage de personnes âgées de plus de 15 ans ayant un niveau de scolarité élevé différait d'un groupe à l'autre, soit respectivement 33 %, 29 % et 31 % pour les groupes à traitement tardif, à traitement rapide et sans traitement (p = 0,004). Les variables en matière de SSE n'étaient pas significativement différentes chez les patients présentant des anévrismes coronariens et chez ceux n'en présentant pas (score z maximal > 2,5), peu importe la proportion des foyers qui étaient sous le SFR après impôt (12 % contre 13 %; p = 0,37), le revenu annuel médian des ménages (81 220 $ CA contre 82 055 $; p = 0,78) ou le taux de diplomation universitaire (33 % contre 31 %; p = 0,49), et ce, même après ajustement en fonction du sexe, de l'âge et du type de MK. Conclusions: Aucune corrélation n'a été établie entre le SSE et le délai avant l'instauration d'un traitement contre la MK ou les résultats liés aux artères coronaires dans le contexte d'un système de soins de santé à payeur unique.

Understanding the Educational Support and Psychosocial Needs of Parents and Adolescents With Kawasaki's Disease and Coronary Artery Aneurysms.

INTRODUCTION: Kawasaki disease (KD) with coronary artery aneurysms (complex KD) presents relentless challenges for families. Psychosocial experiences and needs were explored. METHOD: A descriptive, exploratory study of adolescents and parents using a needs survey and psychosocial instruments (anxiety, depression, and functional impairment). RESULTS: Fifty-one parents and 38 adolescents participated. Predominant interests were for information sheets, newsletters, Web sites, and phone applications. Gaps in disease-specific knowledge and awareness of coronary artery aneurysms between parents and adolescents were identified. Psychosocial concerns for adolescents included symptoms of anxiety (22%), depression (13%), and functional impairment (22%). Multivariable analyses indicated higher depression scores associated with longer travel distance from specialists (p = .04). Parent-reported social concerns for their adolescent were associated with higher anxiety (p = .005) and functional impairment (p = .005). Written commentary complemented the findings. DISCUSSION: Care protocols require psychosocial assessment/referral and the use of virtual platforms. The groundwork was laid for developing patient and family-centered strategies.

Associations between the spatiotemporal distribution of Kawasaki disease and environmental factors: evidence supporting a multifactorial etiologic model.

The etiology of Kawasaki Disease (KD), the most common cause of acquired heart disease in children in developed countries, remains elusive, but could be multifactorial in nature as suggested by the numerous environmental and infectious exposures that have previously been linked to its epidemiology. There is still a lack of a comprehensive model describing these complex associations. We present a Bayesian disease model that provides insight in the spatiotemporal distribution of KD in Canada from 2004 to 2017. The disease model including environmental factors had improved Watanabe-Akaike information criterion (WAIC) compared to the base model which included only spatiotemporal and demographic effects and had excellent performance in recapitulating the spatiotemporal distribution of KD in Canada (98% and 86% spatial and temporal correlations, respectively). The model suggests an association between the distribution of KD and population composition, weather-related factors, aeroallergen exposure, pollution, atmospheric concentration of spores and algae, and the incidence of healthcare encounters for bacterial pneumonia or viral intestinal infections. This model could be the basis of a hypothetical data-driven framework for the spatiotemporal distribution of KD. It also generates novel hypotheses about the etiology of KD, and provides a basis for the future development of a predictive and surveillance model.

Spatiotemporal clustering of cases of Kawasaki disease and associated coronary artery aneurysms in Canada.

Detailed epidemiologic examination of the distribution of Kawasaki disease (KD) cases could help elucidate the etiology and pathogenesis of this puzzling condition. Location of residence at KD admission was obtained for patients diagnosed in Canada (excluding Quebec) between March 2004 and March 2015. We identified 4,839 patients, 164 of whom (3.4%) developed a coronary artery aneurysm (CAA). A spatiotemporal clustering analysis was performed to determine whether non-random clusters emerged in the distributions of KD and CAA cases. A high-incidence KD cluster occurred in Toronto, ON, between October 2004 and May 2005 (116 cases; relative risk (RR) = 3.43; p < 0.001). A cluster of increased CAA frequency emerged in Mississauga, ON, between April 2004 and September 2005 (17% of KD cases; RR = 4.86). High-incidence clusters also arose in British Columbia (November 2010 to March 2011) and Alberta (January 2010 to November 2012) for KD and CAA, respectively. In an exploratory comparison between the primary KD cluster and reference groups of varying spatial and temporal origin, the main cluster demonstrated higher frequencies of conjunctivitis, oral mucosa changes and treatment with antibiotics, suggesting a possible coincident infectious process. Further spatiotemporal evaluation of KD cases might help understand the probable multifactorial etiology.

Epidemiology of Kawasaki Disease in Canada 2004 to 2014: Comparison of Surveillance Using Administrative Data vs Periodic Medical Record Review.

BACKGROUND: We have previously documented an increase in the incidence of Kawasaki disease (KD) in Ontario followed by a stabilization from 1995 to 2006. We sought to validate the estimation of incidence of KD using administrative data and to describe the epidemiology of KD across Canada from 2004 to 2014. METHODS: We queried the Canadian Hospital Discharge Database for hospital admissions associated with a discharge diagnosis of KD. The data set was manually curated and estimates of incidence were compared with those obtained from the retrospective triennial surveillances of KD performed in 2007 and 2010. RESULTS: The average number of cases per year identified through administrative data was 245 ± 45 vs 229 ± 33 from retrospective surveillance. This overestimation, representing 7 ± 6%, is similar to the historical percentage of patients originally diagnosed with KD in whom the diagnosis is subsequently excluded. The annual incidence of KD in Canada was 19.6, 6.4, and 1.3 cases per 100,000 children younger than 5 years, 5-9 years, and 10-14 years old, respectively, with important regional and seasonal differences. The incidence remained stable over the study period in the youngest age group but increased in both older age categories. Coronary artery aneurysms affected 3.5% of all patients, and 0.8% experienced associated major cardiac complications. CONCLUSIONS: Reliance on administrative data to determine incidence of KD is feasible and accurate with manual curation of the data. The incidence of KD in Canada seems to have plateaued for younger children. Differences in annual incidence observed between provinces remain to be explained, and might reflect genetic or environmental differences.

Environmental epidemiology of Kawasaki disease: Linking disease etiology, pathogenesis and global distribution.

BACKGROUND: The pathogenesis of Kawasaki disease (KD) is commonly ascribed to an exaggerated immunologic response to an unidentified environmental or infectious trigger in susceptible children. A comprehensive framework linking epidemiological data and global distribution of KD has not yet been proposed. METHODS AND FINDINGS: Patients with KD (n = 81) were enrolled within 6 weeks of diagnosis along with control subjects (n = 87). All completed an extensive epidemiological questionnaire. Geographic localization software characterized the subjects' neighborhood. KD incidence was compared to atmospheric biological particles counts and winds patterns. These data were used to create a comprehensive risk framework for KD, which we tested against published data on the global distribution. Compared to controls, patients with KD were more likely to be of Asian ancestry and were more likely to live in an environment with low exposure to environmental allergens. Higher atmospheric counts of biological particles other than fungus/spores were associated with a temporal reduction in incidence of KD. Finally, westerly winds were associated with increased fungal particles in the atmosphere and increased incidence of KD over the Greater Toronto Area. Our proposed framework was able to explain approximately 80% of the variation in the global distribution of KD. The main limitations of the study are that the majority of data used in this study are limited to the Canadian context and our proposed disease framework is theoretical and circumstantial rather than the result of a single simulation. CONCLUSIONS: Our proposed etiologic framework incorporates the 1) proportion of population that are genetically susceptible; 2) modulation of risk, determined by habitual exposure to environmental allergens, seasonal variations of atmospheric biological particles and contact with infectious diseases; and 3) exposure to the putative trigger. Future modelling of individual risk and global distribution will be strengthened by taking into consideration all of these non-traditional elements.