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.

Recruitment feasibility to a cohort study of endocrine and metabolic health among survivors of childhood brain tumours: a report from the Canadian study of Determinants of Endometabolic Health in ChIlDrEn (CanDECIDE).

OBJECTIVES: The aim of this study was to test the feasibility of recruitment and performance of study procedures of the Canadian Study of Determinants of Endometabolic Health in ChIlDrEn (CanDECIDE) study, which was designed to assess the determinants of endocrine and metabolic health in survivors of childhood brain tumours. SETTING: A single paediatric tertiary care centre in Hamilton, Ontario, Canada. PARTICIPANTS: We included boys and girls, aged 5 years and older, who were lean (body mass index (BMI) below 85th centile for age and gender) or overweight/obese (BMI 85th centile or above for age and gender). We excluded children on steroids or immunosuppressant therapy, smokers and those who had an active infection for the 2 weeks prior to participation. OUTCOMES: Feasibility targets included recruitment rate of at least 50%, the consenting of 80% of participants to provide biological samples, 90% questionnaire completion rate and the ability to process biological samples from at least 80% of participants. RESULTS: We approached 210 potential participants, and of the 112 (53%) who agreed to participate, 30 (26.8%) completed the study visit over 7 months. All participants agreed to fast, provide biological samples and complete the questionnaires. Sample collection was successful in 97% (29/30) of participants and laboratory procedures were feasible in 100% of collected samples. We also tested resources required for the conduct of the full study including personnel, space, laboratory equipment and procedures and determined that they are all feasible. CONCLUSIONS: Recruitment and consenting of patients for the CanDECIDE study may be feasible. However, we are considering prolonging recruitment duration and collaboration with other centres to meet recruitment targets due to lower than expected recruitment rate. Completion of questionnaires and implementation of sample processing protocols are feasible.

Canadian Study of Determinants of Endometabolic Health in ChIlDrEn (CanDECIDE study): a cohort study protocol examining the mechanisms of obesity in survivors of childhood brain tumours.

BACKGROUND: Childhood obesity has reached epidemic proportions and is impacting children's health globally. In adults, obesity is associated with chronic low-grade inflammation that leads to insulin resistance, which is one of the important mechanisms through which dysregulation of metabolism occurs. There is limited information available about the contribution of inflammation to metabolic health in obese children, and how individual and lifestyle factors impact this risk. One of the paediatric groups at risk of higher rates of obesity includes the survivors of childhood brain tumours. The aim of this study was to evaluate the mechanisms that contribute to inflammation in obese survivors of childhood brain tumours. METHODS AND ANALYSIS: This is a prospective cohort study. We will recruit lean and obese survivors of childhood brain tumours, and a control group composed of lean and obese children with no history of tumours. We will measure circulating and urinary cytokine levels and cytokine gene expression in monocytes. In addition, the methylation patterns of cytokine genes and that of toll-like receptor genes will be evaluated. These will be correlated with individual and lifestyle factors including age, sex, ethnicity, puberty, body mass index, fasting lipid levels, insulin sensitivity, diet, exercise, sleep, stress and built environment. The sample size calculation showed that we need 25 participants per arm ETHICS AND DISSEMINATION: This study has received ethics approval from the institutional review board. Once completed, we will publish this work in peer-reviewed journals and share the findings in presentations and posters in meetings. DISCUSSION: This study will permit the interrogation of inflammation as a contributor to obesity and its complications in obese survivors of childhood brain tumours and compare them with lean survivors and lean and obese controls with no history of tumours, which may help identify therapeutic and preventative interventions to combat the rising tide of obesity.

Educational paper: decreasing the burden of cardiovascular disease in childhood cancer survivors: an update for the pediatrician.

The cardiovascular impact of cancer therapies on the heart is one of the major concerns in the long-term follow-up of childhood cancer survivors (CCSs). Long-term cardiovascular effects include the development of left ventricular dysfunction resulting in congestive heart failure and ischemic heart disease, as well as valvular and pericardial disease. This is mainly ascribed to the cardiotoxic side effects of chemotherapeutic agents (especially anthracyclines) and radiotherapy, but other factors such as radiation and inflammation play a role in the effect of childhood cancer on the cardiovascular health. The most concerning effect is the high incidence of symptomatic heart failure in CCS patients treated with anthracyclines. More than 50 % of CCSs treated with anthracyclines develop asymptomatic left ventricular dysfunction after cancer therapy, with approximately 5 % developing clinical signs of heart failure during long-term follow-up. Once CCS patients develop congestive heart failure, prognosis is poor and is not influenced by current medical treatment strategies. To reduce the long-term burden of cardiovascular disease in pediatric cancer patients, a diversified approach will be necessary. In the acute phase, prevention of cardiac damage through the use of cardioprotective agents (e.g., dexrazoxane) or by administering less cardiotoxic chemotherapeutic agents is to be considered. A recent randomized trial suggested that the use of dexrazoxane reduced cardiac toxicity without affecting cancer outcomes. Especially patients requiring high doses of chemotherapeutic agents could benefit from this approach. Recent data suggest that genetic testing might identify patients at higher risk for cardiotoxicity. This seems mainly related to genes involved in drug metabolism. This would allow personalized approach adjusting chemotherapy based on cardiovascular risk profiling. This could be combined with newer monitoring strategies in the acute phase using newer echocardiographic techniques and biomarker screening to identify patients with early damage to the myocardium. For the long-term CCS cohort, early detection and treatment of early dysfunction prior to the development of congestive heart failure could potentially improve long-term outcomes. Promoting healthy lifestyles and controlling additional cardiovascular risk factors (e.g., obesity, diabetes, arterial hypertension) is an important task for every physician involved in the care of this growing cohort.

Vascular and autonomic function in preschool-aged children with congenital heart disease.

OBJECTIVE: To compare indices of vascular health and heart rate variability in preschool-aged children with repaired congenital heart disease (CHD) including tetralogy of Fallot (n = 6) and coarctation of the aorta (n = 6). DESIGN: A cross-sectional study design was used. All measures were noninvasive and collected over a single testing session under the supervision of a parent/guardian. SETTING: Data collection took place in a quiet, temperature-controlled room (23°± 1°C) with the participant in a supine position. PATIENTS: Twelve (six females, six males) preschool-aged children with repaired CHD (CHD: 4 ± 1 years) and 12 age- and gender-matched healthy controls (CON: 5 ± 1 years) participated in the study. OUTCOME MEASURES: Supine, resting measures of heart rate variability (time, frequency, and nonlinear domains), whole-body pulse wave velocity (ventricular depolarization to dorsalis pedis artery), brachial blood pressures, and carotid artery distensibility, lumen diameter, intima-media thickness, and wall/lumen ratio were collected in both groups. RESULTS: The groups were similar in age, height, and weight; however, CON had significantly higher body mass index values (CON: 16.9 ± 2.2, CHD: 15.1 ± 1.0, P < .05) and body mass index percentiles (CON: 69 ± 27%tile, CHD: 36 ± 24%tile, P < .01) compared to CHD. No group differences were found for resting brachial blood pressures, whole-body pulse wave velocity, heart rate variability, and carotid artery distensibility, lumen diameter, and intima-media thickness (P > .05). Carotid artery pulse pressures (CHD: 38 ± 6 mm Hg, CON: 31 ± 6 mm Hg, P < .05) and wall/lumen ratios (CHD: 0.091 ± 0.007, CON: 0.085 ± 0.006, P < .01) were significantly higher in the CHD group. CONCLUSIONS: These results may indicate that preschool-aged children with repaired CHD display early signs of vascular remodeling, but not autonomic or vascular dysfunction. The effects of larger wall/lumen ratios on cardiovascular disease risk require further investigation.

Taquiarritmia fetal com conduçäo atrioventricular 1: 1. Infusäo em veia umbilical de adenosina como teste diagnóstico / Fetal tachyarrhythmia with 1: 1 atrioventricular conduction. Adenosine infusion in the umbilical vein as a diagnostic test

This is the report of a case of fetal tachyarrhythmia with 1:1 atrioventricular conduction detected by pre-natal echocardiography in a fetus at 25-weeks gestation. Adenosine infusion via cordocentesis was performed as a diagnostic test to differentiate between atrioventricular nodal reentrant supraventricular tachyarrhythmia and atrial flutter. After infusion, transient 2:1 atrioventricular dissociation was obtained and the diagnosis of atrial flutter was made. Transplacental therapy with digoxin and amiodarone was then successfully used.

Taquiarritmias supraventriculares no feto. Experiência de uma unidade de referência em cardiologia fetal / Fetal supraventricular Tachyarrhythmias. Experience of a fetal cardiology reference center

OBJETIVO: Avaliar a forma de apresentaçäo, diagnóstico e tratamento das taquiarritmias supraventriculares fetais, através do relato de uma série de casos acompanhados em um centro terciário de cardiologia fetal. MÉTODOS: Säo descritos 25 casos de taquiarritmia supraventricular diagnosticados intra-útero, no período de janeiro/89 a outubro/97, em uma populaçäo compreendendo 3117 gestantes. RESULTADOS: Foram diagnosticados 17 casos taquiarritmia supraventricular e 8 casos de flutter atrial fetal. As idades gestacionais variam de 26 a 40 semanas. Doze fetos apresentavam hidropisia no momento do diagnóstico (6 com taquicardia supraventricular (TSV) e 6 com flutter atrial). Quatro fetos com TSV apresentavam cardiopatias estruturais (dois casos de anomalia de Ebstein e dois com comunicaçäo interventricular). Todos os fetos foram internados na Unidade de Cardiologia Fetal para monitorizaçäo e tratamento. Entre os 17 fetos com TSV, 12 apresentaram reversäo da arritmia após administraçäo de digoxina, mas esta medida näo foi eficaz em nenhum paciente com flutter. Dois pacientes com TSV e seis com necessitaram interrupçäo da gestaçäo para cardioversäo elétrica pós-natal. A mortalidade foi de 3/17 no grupo da TSV (incluindo dois pacientes com anomalia de Ebstein) e de 0/8 no grupo com flutter. CONCLUSÄO: As taquiarritmias supraventriculares fetais säo enventos raros na populaçäo geral. Entretanto, podem provocar insuficiência cardíaca e óbito intrauterino. Como a resposta ao tratamento é satisfatória, tornam-se de extrema importância o diagnóstico precoce e o tratamento adequado.

Restrição pré-natal do forame oval / Prenatal Restriction of the Foramen Ovale

The parallel arrangement of the fetal intracardiac circulation requires unrestricted flow of blood through the foramen ovale in the atrial septum. Restriction to this flow is a significant cardiac abnormality, with potentially serious sequelae in post-natal life. A wide spectrum of abnormalities has been associated to the restriction of flow through the foramen ovale, including enlargement of right atrium, hypertrophy of right ventricle, enlargement of tricuspid valve annulus, hypoplastic syndrome of left heart, non-immune hydrops fetalis and supraventricular tachycardias. We report the diagnosis and follow-up of a fetus identified in the prenatal period, in whom the restriction of the foramen ovale originated severe tricuspid regurgitation and severe right ventricular hypocontractility, despite the morphologically normal tricuspid valve. After delivery, there was complete resolution of the clinical findings. The paramount importance of complete assessment of interatrial flow in high risk fetuses, allowing for the early detection and intervention, is emphasized.

Procedimentos diagnósticos e terapêuticos invasivos durante a vida fetal / Invasive diagnostic and therapeutic procedures to assess fetal diseases

A abordagem diagnóstica e terapêutica invasiva fetal tem apresentado avanços significativos nos últimos anos. Algumas malformaçöes, como obstruçöes urinárias, lesöes torácicas, hérnia diafragmática congênita, obstruçöes valvares cardíacas, constituem alguns exemplos de doenças que podem ter benefício com o manejo precoce. Os resultados da literatura säo promissores. Os procedimentos realizados incluem derivaçäo vésico-amniótica e vesicostomia (aberta ou por videofetoscopia) para o tratamento das obstruçöes urinárias; cirurgia de correçäo de hérnia diafragmática entre 22 e 28 semanas de gestaçäo; estudos ainda em fase experimental para colocaçäo de marcapasso fetal para o tratamento do bloqueio atrioventricular total. Existem protocolos internacionais para tratamento e acompanhamento de pacientes, sendo que as técnicas anestesicas e cirúrgicas têm tido avanços significativos, considerando as peculiaridades específicas do binômio mäe-feto