2016 European Society of Hypertension guidelines for the management of high blood pressure in children and adolescents

Increasing prevalence of hypertension (HTN) in children and adolescents has become a significant public health issue driving a considerable amount of research. Aspects discussed in this document include advances in the definition of HTN in 16 year or older, clinical significance of isolated systolic HTN in youth, the importance of out of office and central blood pressure measurement, new risk factors for HTN, methods to assess vascular phenotypes, clustering of cardiovascular risk factors and treatment strategies among others. The recommendations of the present document synthesize a considerable amount of scientific data and clinical experience and represent the best clinical wisdom upon which physicians, nurses and families should base their decisions. In addition, as they call attention to the burden of HTN in children and adolescents, and its contribution to the current epidemic of cardiovascular disease, these guidelines should encourage public policy makers to develop a global effort to improve identification and treatment of high blood pressure among children and adolescents

Treatment of Fabry Disease: Outcome of a Comparative Trial with Agalsidase Alfa or Beta at a Dose of 0.2 mg/kg

Two different enzyme preparations, agalsidase alfa (Replagal(TM), Shire) and beta (Fabrazyme(TM), Genzyme), are registered for treatment of Fabry disease. We compared the efficacy of and tolerability towards the two agalsidase preparations administered at identical protein dose in a randomized controlled open label trial.Thirty-four Fabry disease patients were treated with either agalsidase alfa or agalsidase beta at equal dose of 0.2 mg/kg biweekly. Primary endpoint was reduction in left ventricular mass after 12 and 24 months of treatment. Other endpoints included occurrence of treatment failure (defined as progression of cardiac, renal or cerebral disease), glomerular filtration rate, pain, anti-agalsidase antibodies, and globotriaosylceramide levels in plasma and urine. After 12 and 24 months of treatment no reduction in left ventricular mass was seen, which was not different between the two treatment groups. Also, no differences in glomerular filtration rate, pain and decline in globotriaosylceramide levels were found. Antibodies developed only in males (4/8 in the agalsidase alfa group and 6/8 in the agalsidase beta group). Treatment failure within 24 months of therapy was seen in 8/34 patients: 6 male patients (3 in each treatment group) and 2 female patients (both agalsidase alfa). The occurrence of treatment failures did not differ between the two treatment groups; chi(2) = 0.38 p = 0.54.Our study revealed no difference in reduction of left ventricular mass or other disease parameters after 12 and 24 months of treatment with either agalsidase alfa or beta at a dose of 0.2 mg/kg biweekly. Treatment failure occurred frequently in both groups and seems related to age and severe pre-treatment disease.International Standard Randomized Clinical Trial ISRCTN45178534 [http://www.controlled-trials.com/ISRCTN45178534]

Left ventricular systolic deformation in subclinical metabolic cardiomyopathies

Cardiovascular disease is a significant contributor to morbidity and mortality in patients with inherited metabolic disorders or chronic kidney disease. Conventional echocardiography typically identifies cardiac involvement at a more established stage of the disease. Strain echocardiography, which assesses the deformation of the myocardium, has the potential for early detection of subclinical myocardial dysfunction. This thesis consists of 3 studies of left ventricular myocardial deformation in patients with diseases causing metabolic myocardial alterations, associated with development of cardiomyopathy. In study 1, Doppler strain echocardiography was performed in 40 patients with Stage II and III chronic kidney disease. In study 2, speckle strain echocardiography was performed in 68 patients who underwent renal transplantation in childhood, and in study 3, speckle strain echocardiography was performed in 38 patients with Fabry disease. In all studies, the ability of strain echocardiography to detect subclinical cardiac dysfunction not detected by conventional echocardiography was studied. As demonstrated by the results of this thesis, echocardiography, using Doppler or speckle strain, detected impaired left ventricular long axis function in the studied patient groups. In particular, left ventricular longitudinal strain was reduced, while ejection fraction, measured by conventional echocardiography, was generally preserved in patients with early-stage chronic kidney disease and in patients with mild Fabry disease compared to healthy subjects. Furthermore, having metabolic disease was associated with lower left ventricular systolic strain independent of left ventricular mass. In patients who underwent childhood renal transplantation, hypertension was common and a main covariate of left ventricular diastolic dysfunction. In contrast, left ventricular systolic deformation was comparable between patients and healthy subjects. In conclusion, Doppler or speckle strain echocardiography, may detect impaired myocardial function in patients with diseases causing metabolic myocardial alterations, in spite of normal findings on conventional echocardiography and without clinical evidence of heart disease

Assessment of Physical Activity in Children and Adolescents with Congenital Heart Defects

Disorder of the cardiovascular system is the most common cause of death worldwide and sufficient physical activity plays an import role in preventing these deaths. Children and adolescents with congenital heart defect are at risk of living a sedentary life as a result of overprotection and uncertainty regarding physical activity recommendations. Assessment of physical activity level should therefore be part of regular follow-up in this population. A whole range of subjective and objective measure instruments of physical activity are available. However, not all are suitable in children and adolescents. Questionnaires have the advantage of being inexpensive and simple, but reduced recall capability of children and adolescents and low-to-moderate correlation with objective measures of physical activity are of concern. At present a single unobtrusive motion sensor allowing valid and long-term monitoring of physical activity may be the best choice

Validation of a Modified Triage Scale in a Norwegian Pediatric Emergency Department

Objective. Triage is a tool developed to identify patients who need immediate care and those who can safely wait. The aim of this study was to assess the validity and interrater reliability of a modified version of the pediatric South African triage scale (pSATS) in a single-center tertiary pediatric emergency department in Norway. Methods. This prospective, observational study included all patients with medical conditions, referred to the pediatric emergency department of a tertiary hospital in Norway from September 1, 2015, to November 17, 2015. Their assigned triage priority was compared with rate of hospitalization and resource utilization. Validity parameters were sensitivity, specificity, positive and negative predictive value, and percentage of over- and undertriage. Interrater agreement and accuracy of the triage ratings were calculated from triage performed by nurses on written case scenarios. Results. During the study period, 1171 patients arrived at the hospital for emergency assessment. A total of 790 patients (67 %) were triaged and included in the study. The percentage of hospital admission increased with increasing level of urgency, from 30 % of the patients triaged to priority green to 81 % of those triaged to priority red. The sensitivity was 74 %, the specificity was 48 %, the positive predictive value was 52 %, and the negative predictive value was 70 % for predicting hospitalization. The level of over- and undertriage was 52 % and 26 %, respectively. Resource utilization correlated with higher triage priority. The interrater agreement had an intraclass correlation coefficient of 0.99 by Cronbach’s alpha, and the accuracy was 92 %. Conclusions. The modified pSATS had a moderate sensitivity and specificity but showed good correlation with resource utilization. The nurses demonstrated excellent interrater agreement and accuracy when triaging written case scenarios

Interventions to increase physical activity for people with congenital heart disease (Protocol)

© 2018 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd. This is a protocol for a Cochrane Review (Intervention). The objectives are as follows: To assess the effects of interventions to increase exercise and all types of physical activity, versus no intervention (exercise and physical activity as usual) for people with congenital heart disease.status: publishe