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Despite the high prevalence of isolated systolic hypertension (ISH) among hypertensive adolescents, its clinical significance is not determined. In addition, it is hypothesized that ISH with normal central blood pressure (BP) in young patients is a benign phenomenon and was hence labeled spurious hypertension (sHTN).Using cardiac magnetic resonance we evaluated a group of 73 patients with suspected primary hypertension, aged 13-17 years (median: 16.9, interquartile range 15.8-17.4; 13 girls), in whom, based on 24-h ambulatory BP monitoring either ISH (n = 30) or white-coat hypertension (WCH) (n = 43) was diagnosed. Based on noninvasive central BP measurement 13 participants in the ISH group were classified as having sHTN and 17 were diagnosed with true hypertension.Compared with WCH adolescents, ISH patients presented with higher indexed left ventricular mass index (LVMI) (P 0.001), maximal left ventricular (LV) wall thickness (P 0.001), LV concentricity (P = 0.001) and more often had LV hypertrophy (47 vs. 14%, P = 0.002). They had higher average pulse wave velocity (PWV) in the proximal aorta (P = 0.016) and the whole thoracic aorta (P = 0.008). In addition, we observed higher indexed LV stroke volume (P = 0.025) in patients with ISH. The sHTN subgroup had significantly higher LVMI and aortic PWV, and more often had LV hypertrophy compared with the WCH group. The sHTN and true hypertension subgroups did not differ in terms of aortic PWV, LVMI or LV geometry.Compared with adolescents with WCH patients with ISH, including the sHTN subtype, have more pronounced markers of cardiac end-organ damage, higher aortic stiffness and stroke volume.

Obese youth with sleep-disordered breathing are treated with positive airway pressure to improve sleep and cardiovascular status. While improvements in sleep parameters have been confirmed, a study by Katz et al. showed no major improvement in ambulatory blood pressure. The aim of this ancillary study was to analyze short-term blood pressure variability, following positive airway pressure treatment, as a more sensitive marker of cardiovascular health. We analyzed 24-h blood pressure variability data in 17 children, taken at baseline and after 12 months of treatment. These data were derived from an already published prospective, multicenter cohort study conducted in 27 youth (8-16 years) with obesity who were prescribed 1-year of positive airway pressure for moderate-severe sleep-disordered breathing. Significant decreases were found in 24 h systolic blood pressure (p = 0.040) and nighttime diastolic blood pressure (p = 0.041) average real variability, and diastolic blood pressure (p = 0.035) weighted standard deviation. Significant decreases were noted in nighttime diastolic blood pressure time rate variability (p = 0.007). Positive airway pressure treatment resulted in a significant decrease in blood pressure variability, suggesting a clinically significant improvement of sympathetic nerve activity in youth with obesity and sleep-disordered breathing. IMPACT: Cardiovascular variability, as measured by blood pressure variability, is improved in children following positive airway pressure treatment. Our novel findings of improved blood pressure time rate variability are the first described in the pediatric literature. Future studies aimed at analyzing target organ damage in this patient population will allow for a better understanding as to whether alterations in blood pressure variability translate to decreasing target organ damage in children, as seen in adults.

BACKGROUND Although the clinical presentation of fibromuscular dysplasia (FMD) and its generalized character has been previously described in adults, data on FMD in children are limited. METHOD In this study, we aimed to assess visceral artery involvement in pediatric FMD patients with documented renal artery stenosis (RAS) and renovascular hypertension (RVH) in comparison with healthy individuals. We retrospectively analyzed the results of angiographic studies of 16 patients with a median age of 13.9 years (10 girls) in comparison with 16 age- and sex-matched healthy controls. RESULTS Out of the 16 FMD patients, 10 (63%) had stenotic lesions identifiable only in renal arteries, whereas six (37%) had additional stenoses in other vascular beds - in the celiac trunk (four patients), superior mesenteric artery (four patients), inferior mesenteric artery (one patient), splenic artery (one patient), common hepatic artery (three patients), and abdominal aorta (one patient). The comparison of ostial diameters of vessels, in which no periostial narrowing, stents, or occlusions were found, revealed that patients with FMD had a significantly smaller diameter of the celiac trunk (P = 0.017), splenic arteries (P = 0.007), and common hepatic artery (P = 0.026) than their age- and sex-matched healthy peers. CONCLUSION We found that 69% of children with RVH caused by FMD had clinically silent stenoses or tortuosity of visceral arteries. The results of our study suggest that pediatric FMD is a generalized arterial condition, and the patients may need screening for both renal as well as nonrenal manifestations of the disease.

Arterial hypertension in renal transplant recipients warrants antihypertensive treatment. The preferable choice of antihypertensives that should be used in patients after kidney transplantation remains a matter of debate; however, calcium channel blockers (CCB) and angiotensin-converting enzyme inhibitors (ACEI) are currently the most commonly used antihypertensives. This educational review summarizes the current evidence about the effects of these two classes of medications in transplant recipients. Several studies have demonstrated that both classes of drugs can reduce blood pressure (BP) to similar extents. Meta-analyses of adult randomized controlled trials have shown that graft survival is improved in patients treated with ACEIs and CCBs, and that CCBs increase, yet ACEIs decrease, graft function. Proteinuria is usually decreased by ACEIs but remains unchanged with CCBs. In children, no randomized controlled study has ever been performed to compare BP or graft survival between CCBs and ACEIs. Post-transplant proteinuria could be reduced in children along with BP by ACEIs. The results of the most current meta-analyses recommend that due to their positive effects on graft function and survival, along with their lack of negative effects on serum potassium, CCBs could be the preferred first-line antihypertensive agent in renal transplant recipients. However, antihypertensive therapy should be individually tailored based on other factors, such as time after transplantation, presence of proteinuria/albuminuria, or hyperkalemia. Furthermore, due to the difficulty in controlling hypertension, combination therapy containing both CCBs and ACEIs could be a reasonable first-step therapy in treating children with severe post-transplantation hypertension.

Background: Kidney size evaluation is an essential examination in pediatric nephrology. While body length/height is the best predictor of kidney length, age-based and body surface area (BSA)–based normative values may be useful in clinical practice or research. This study aimed to establish ultrasound-based kidney length lambda-mu-sigma (LMS) percentiles by age and BSA in healthy children. Methods: In 1758 Polish and Lithuanian children (868 boys, 49%) aged 0–19 years, kidney length was measured using ultrasonography. In all participants, anthropometric measurements were taken and kidney function was evaluated based on serum creatinine concentration. Participants with chronic or kidney diseases, abnormal kidney function, or pathologies in sonographic examination were excluded from the analysis. Results: Kidney length (median kidney length) increased progressively from infancy to the age of 18 years, from 60.1 to 114.2 mm in males, and from 57.3 to 105.2 mm in females. A gradual increase of kidney length (50th percentile) in relation to BSA (from 46.1 mm in infants with a BSA of 0–1.2 m2 to 118.3 mm in adolescents with a BSA of 2.6–2.8 m2) was also observed. LMS percentiles by age (stratified by sex) and BSA were determined and presented as graphs and tables of percentiles and LMS parameters by 1-year age intervals and 0.2 m2 of BSA, respectively. Conclusions: We present the first age- and BSA-based kidney length LMS normative values based on the largest pediatric cohort to date, which can be used in both clinical practice and research studies. Graphical abstract: A higher resolution version of the Graphical abstract is available as Supplementary information [Figure not available: see fulltext.]

Background: Left ventricular non-compaction (LVNC) is a rare cardiomyopathy typically involving the left ventricle (LV); however, the right ventricle (RV) can also be affected. This case-control study aimed to assess the morphology and function of LV and RV in children with LVNC. Methods: Sixteen children (13 ± 3 years, six girls) with LVNC were compared with 16 sex- and age-matched controls. LV and RV morphology and function were evaluated in cardiovascular magnetic resonance (CMR) studies. Additionally, LV and RV global radial (GRS), circumferential (GCS), and longitudinal strain (GLS) were assessed using tissue-tracking analysis. Results: Patients with LVNC did not differ from the healthy controls in terms of age, height, weight, and body surface area (BSA). In total, 4/16 subjects with LVNC had mid-wall late gadolinium enhancement (LGE). Compared to the control group, patients with LVNC had higher end-diastolic volume (EDV) indexed for body surface area (BSA), lower ejection fraction (EF), and lower LV strain parameters (all p < 0.05). Children with LVNC also presented with thicker RV apical trabeculation, whereas there were no differences in RV EF and EDV/BSA between the groups. Nevertheless, children with LVNC had impaired RV GRS and GCS (both p < 0.05). Conclusions: LVNC in pediatric patients is associated with LV enlargement and impaired LV systolic function. Additionally, children with LVNC have increased RV trabeculations and subclinical impairment of RV myocardial deformation.

Background and Aims Blood pressure lability has been observed in certain cohorts of pediatric patients with variable degrees of proteinuria; however, the impact of proteinuria on blood pressure is not fully elucidated. The objective of our study was to analyze blood pressure and heart rate in pediatric patients with proteinuria. Methods We performed a retrospective chart review of patients (age 1‐18) diagnosed with idiopathic nephrotic syndrome, with varying degrees of proteinuria. Blood pressure and heart rate data were analyzed in relation to anthropometric and biochemical parameters. A total of 72 urine sample analyses, along with associated blood pressure measurements, were obtained from the charts of 33 children (males = 25). Results Diastolic blood pressure Z‐scores were significantly higher in proteinuric patients (urine protein/creatinine >0.02 g/mmol) compared to non‐proteinuric patients (P = .006; Cohen‐d 0.97 [0.41; 1.53]). Systolic blood pressure was also significantly higher in proteinuric patients (P = .04), but with a less significant effect size (Cohen‐d 0.54 [−0.002; 1.08]). Proteinuria (>0.02 g/mmol) was the most significant predictor of diastolic (β = .79, P = .04), but not systolic blood pressure elevation on multivariate analysis. Conclusions We observed a disproportionate increase in diastolic blood pressure vs systolic blood pressure in patients with proteinuria.

Background Currently used pediatric kidney length normative values are based on small single-center studies, do not include kidney function assessment, and focus mostly on newborns and infants. We aimed to develop ultrasound-based kidney length normative values derived from a large group of European Caucasian children with normal kidney function. Methods Out of 1,782 children aged 0–19 years, 1,758 individuals with no present or past kidney disease and normal estimated glomerular filtration rate had sonographic assessment of kidney length. The results were correlated with anthropometric parameters and estimated glomerular filtration rate. Kidney length was correlated with age, height, body surface area, and body mass index. Height-related kidney length curves and table were generated using the LMS method. Multivariate regression analysis with collinearity checks was used to evaluate kidney length predictors. Results There was no significant difference in kidney size in relation to height between boys and girls. We found significant (p D effect size = 0.04 and 0.06) differences between prone vs. supine position (mean paired difference = 0.64 mm, 95% CI = 0.49–0.77) and left vs. right kidneys (mean paired difference = 1.03 mm, 95% CI = 0.83–1.21), respectively. For kidney length prediction, the highest coefficient correlation was observed with height (adjusted R2 = 0.87, p Conclusions We present height-related LMS-percentile curves and tables of kidney length which may serve as normative values for kidney length in children from birth to 19 years of age. The most significant predictor of kidney length was statural height. Graphic Abstract

Due to concerns about cumulative radiation exposure in the pediatric population, it is not standard practice to perform spine radiographs in most conditions that predispose to vertebral fracture (VF). In this study we examined the accuracy of two clinical predictors, back pain and lumbar spine bone mineral density (LS BMD), to derive four case-finding paradigms for detection of prevalent VF (PVF). Subjects were 400 children at risk for PVF (leukemia 186, rheumatic disorders 135, nephrotic syndrome 79). Back pain was assessed by patient report, LS BMD was measured by dual-energy X-ray absorptiometry, and PVF were quantified on spine radiographs using the modified Genant semiquantitative method. Forty-four patients (11.0%) had PVF. Logistic regression analysis between LS BMD and PVF produced an odds ratio (OR) of 1.9 (95% confidence interval [CI], 1.5 to 2.5) per reduction in Z-score unit, an area under the receiver operating characteristic curve of 0.70 (95% CI, 0.60 to 0.79), and an optimal BMD Z-score cutoff of -1.6. Case identification using either low BMD alone (Z-score < -1.6) or back pain alone gave similar results for sensitivity (55%, 52%, respectively), specificity (78%, 81%, respectively), positive predictive value (PPV; 24%, 25%, respectively), and negative predictive value (NPV; 93%, 93%, respectively). The paradigm using low BMD plus back pain produced lower sensitivity (32%), higher specificity (96%), higher PPV (47%), and similar NPV (92%). The approach using low BMD or back pain had the highest sensitivity (75%), lowest specificity (64%), lowest PPV (20%), and highest NPV (95%). All paradigms had increased sensitivities for higher fracture grades. Our results show that BMD and back pain history can be used to identify children with the highest risk of PVF so that radiography can be used judiciously. The specific paradigm to be applied will depend on the expected PVF rate and the clinical approach to the use of radiography. © 2019 American Society for Bone and Mineral Research.

Obese youth with sleep-disordered breathing are treated with positive airway pressure to improve sleep and cardiovascular status. While improvements in sleep parameters have been confirmed, a study by Katz et al. showed no major improvement in ambulatory blood pressure. The aim of this ancillary study was to analyze short-term blood pressure variability, following positive airway pressure treatment, as a more sensitive marker of cardiovascular health. We analyzed 24-h blood pressure variability data in 17 children, taken at baseline and after 12 months of treatment. These data were derived from an already published prospective, multicenter cohort study conducted in 27 youth (8-16 years) with obesity who were prescribed 1-year of positive airway pressure for moderate-severe sleep-disordered breathing. Significant decreases were found in 24 h systolic blood pressure (p = 0.040) and nighttime diastolic blood pressure (p = 0.041) average real variability, and diastolic blood pressure (p = 0.035) weighted standard deviation. Significant decreases were noted in nighttime diastolic blood pressure time rate variability (p = 0.007). Positive airway pressure treatment resulted in a significant decrease in blood pressure variability, suggesting a clinically significant improvement of sympathetic nerve activity in youth with obesity and sleep-disordered breathing. IMPACT: Cardiovascular variability, as measured by blood pressure variability, is improved in children following positive airway pressure treatment. Our novel findings of improved blood pressure time rate variability are the first described in the pediatric literature. Future studies aimed at analyzing target organ damage in this patient population will allow for a better understanding as to whether alterations in blood pressure variability translate to decreasing target organ damage in children, as seen in adults.

Although relatively rare in childhood, primary hypertension (PH) is thought to have originated in childhood and may be even determined perinatally. PH prevalence increases in school-age children and affects 11% of 18-year-old adolescents. Associated with metabolic risk factors, elevated blood pressure in childhood is carried into adulthood. Analysis of the phenotype of hypertensive children has revealed that PH is a complex of anthropometric and neuro-immuno-metabolic abnormalities, typically found in hypertensive adults. Children with elevated blood pressure have shown signs of accelerated biological development, which are closely associated with further development of PH, metabolic syndrome, and cardiovascular disease in adulthood. At the time of diagnosis, hypertensive children were reported to have significant arterial remodelling expressed as significantly increased carotid intima-media thickness, increased stiffness of large arteries, lower area of microcirculation, and decreased endothelial function. These changes indicate that their biological age is 4 to 5 years older than their normotensive peers. All these abnormalities are typical features of early vascular aging described in adults with PH. However, as these early vascular changes in hypertensive children are closely associated with features of accelerated biological development and neuro-immuno-metabolic abnormalities observed in older subjects, it seems that PH in childhood is not only an early vascular aging event, but also an early biological maturation phenomenon.

Background The clinical significance of isolated systolic hypertension with normal central blood pressure known as spurious hypertension (sHT) in adolescents and its evolution over time is not known. Methods The aim of this study was to analyze changes in office, ambulatory blood pressure (ABPM), central systolic blood pressure (cSBP), hemodynamic parameters, and target organ damage (TOD) over a 1-year follow-up in a group of non-obese children with sHT. Results Of 294 patients referred for primary hypertension, 138 patients (31 girls; 22%) had hypertension confirmed by ABPM. 48/138 (35%) patients (7 girls; 15%) were diagnosed with sHT (elevated office and ambulatory systolic BP, but normal cSBP); 43 of them (6 girls; 14%) were followed for 12 ± 3 months during non-pharmacological therapy. At baseline 7 (16%) patients had borderline values of cIMT or LVMi indicating mild TOD. After 12 months, 10/43 (3 girls; 23%) patients developed sustained HT (elevated office, ambulatory BP and cSBP), 11/43 (1 girl; 26%) maintained sHT, and 22/43 (2 girls; 51%) evolved to white coat hypertension or normotension. The cSBP values increased in 27 patients (4 girls; 63%), but the group average remained in the normal range. Prevalence of TOD did not change during observation. The multivariate regression analysis showed that the only predictor of cSBP change over time was a change in serum uric acid level. Conclusions In conclusion, after 1 year of non-pharmacological treatment, 23% of adolescents with sHT developed sustained hypertension, with the main predictor of cSBP change being the change in serum uric acid.

Hypertension Canada provides annually-updated, evidence-based guidelines for the diagnosis, assessment, prevention, and treatment of hypertension in adults and children. This year, the adult and pediatric guidelines are combined in one document. The new 2018 pregnancy-specific hypertension guidelines are published separately. For 2018, 5 new guidelines were introduced, and one existing guideline on the blood pressure thresholds and targets in the setting of thrombolysis for acute ischemic stroke was revised. The use of validated wrist devices for the estimation of blood pressure in individuals with large arm circumference is now included. Guidance is provided for the follow-up measurements of blood pressure, with the use of standardized methods and electronic (oscillometric) upper arm devices in individuals with hypertension, and either ambulatory blood pressure monitoring or home blood pressure monitoring in individuals with white coat effect. We specify that all individuals with hypertension should have an assessment of global cardiovascular risk to promote health behaviours that lower blood pressure. Finally, an angiotensin receptor-neprilysin inhibitor combination should be used in place of either an angiotensin-converting enzyme inhibitor or angiotensin receptor blocker in individuals with heart failure (with ejection fraction < 40%) who are symptomatic despite appropriate doses of guideline-directed heart failure therapies. The specific evidence and rationale underlying each of these guidelines are discussed.

Although the importance of office prehypertension/high normal blood pressure (BP) has been well documented, the significance of ambulatory prehypertension (AmbPreHT) has not been determined. We analyzed markers of target organ damage and hemodynamics in adolescents with AmbPreHT in comparison with hypertensive and normotensive subjects. Out of 304 white patients aged 15.0±2.5 years with office hypertension, 30 children had AmbPreHT and were compared with 66 normotensive healthy children and 92 children with true hypertension (elevated office, ambulatory, and central BP), 22 had ambulatory hypertension (AmbHT), and 70 had severe AmbHT (SevAmbHT). Stroke volume and cardiac output were greater in AmbPreHT compared with patients with normotension but did not differ between AmbPreHT, AmbHT, and SevAmbHT. Similarly, AmbPreHT, AmbHT, and SevAmbHT had similar total peripheral resistance, lower than patients with normotension ( P P Z scores were significantly higher than in normotensive ( P P Z scores were increased in patients with AmbPreHT, AmbHT, and SevAmbHT compared with patients with normotension ( P Z score, central systolic BP, and uric acid levels were significant independent predictors of left ventricular mass index. In conclusion, patients with AmbPreHT presented similar cardiovascular adaptations to those observed in patients with hypertensive and may be at risk of developing cardiovascular events.

Children with chronic kidney disease (CKD) are at risk for obstructive sleep apnea (OSA) and hypertension. The objectives of this study were to explore associations between OSA severity using the apnea-hypopnea-index(AHI) and obstructive apnea-hypopnea-index(OAHI) on polysomnography (PSG), OSA symptoms, and measures of hypertension in children with CKD. One-night in-laboratory PSGs and 24-h ambulatory blood pressure monitoring (ABPM) were performed on children with CKD stages 2–5 (non-dialysis dependent). Sleep questionnaires, including the modified Epworth Sleepiness Scale (ESS) and the Pediatric Sleep Questionnaire (PSQ), were administered during the sleep study. Nineteen children and adolescents completed a PSG and questionnaires and thirteen completed ABPMs. Mean (standard deviation) age at the time of the sleep study was 14.1 (3.2) years. Eleven (58%) participants had CKD stage two, and eight (42%) had stage 3–4. None of the participants were found to have OSA on PSG. One participant had a positive ESS score (≥ 11) and five participants had positive PSQ scores (≥ eight). Night systolic and diastolic pressures were strongly correlated with the OAHI (r = 0.67 and r = 0.69, respectively, p

Arterialni tuhost odraži vlastnosti cevni stěny. Studie u dospělých pacientů prokazaly, že arterialni tuhost je rizikovým faktorem morbidity a mortality z kardiovaskularnich přicin. V posledni době vzrůsta použivani neinvazivnich metod měřeni arterialni tuhosti, jako je rychlost pulsni vlny i v pediatricke populaci, a to jak ve výzkumu, tak v klinicke praxi. S rozsiřenim těchto neinvazivnich metod do dětskeho věku přibývaji rychle nase znalosti o faktorech a stavech, ktere jsou spojeny s vzestupem arterialni tuhosti. Mezi tyto faktory patři nejen tradicni kardiovaskularni rizikove faktory, ale take stavy, jakými jsou intrauterinni růstova retardace, metabolický syndrom, obezita, diabetes mellitus a chronicke onemocněni ledvin, vaskulitidy a vaskulopatie spojene s různými syndromy, kongenitalni srdecni vady a některe systemove choroby. Casne rozpoznani zvýsene tuhosti arterii v dětstvi by mohlo vest k casne lecebne intervenci. V tomto smyslu jsou ale nezbytne longitudinalni intervencni studie, ktere by potvrdily, že zlepseni tuhosti cev u normalni i rizikove pediatricke populace povede k sniženi kardiovaskularniho rizika v casne dospělosti.

Hypertension (HTN) in children is associated with early vascular aging (EVA) and underlying immunologic-metabolic abnormalities and accelerated biological maturation. Morphologic and functional vascular changes underlying EVA and HTN in children resemble those seen in the elderly including but not limited to an increase in intima-media thickness (IMT) and arterial stiffness and endothelial dysfunction. Although progeria syndrome leading to EVA and the development of clinically manifested cardiovascular (CV) disease in the second decade of life is a rare hereditary disorder, primary HTN, which is also associated with EVA, is much more common (reported in up to 10% in adolescents). EVA associated with HTN in children leads to the premature development of target organ injury in childhood and CV events in early adulthood. Limited evidence from prospective observational studies in children and adolescents indicates that early lifestyle measures (low salt/low sugar intake and exercise) or pharmacologic treatment of HTN, or both, partially reverses morphologic and functional changes underlying EVA such as an increase in carotid IMT and pulse wave velocity, a decrease in flow-mediated dilation of the brachial artery, and an increase in oxidative stress and visceral fat. Future mechanistic and therapeutic clinical trials are desirable to assess the mechanisms and treatment strategies of EVA in the context of HTN in children and their effect on CV events in early adulthood.

The aim of the article was to evaluate arterial stiffness, an early marker of increased cardiovascular risk, in relation to obesity. The continuous metabolic syndrome (cMetS) score was calculated as sum of Z score of mean arterial pressure, body mass index, serum glucose, triglyceride, and high-density lipoprotein cholesterol in 144 obese patients and 66 nonobese controls. Ambulatory arterial stiffness index (AASI) was calculated as 1 minus regression slope of diastolic on systolic blood pressure from ambulatory blood pressure measurements. The mean AASI increased progressively with severity of obesity. The receiver operator curve analysis of body mass index and AASI showed area under the curve of 0.64 ± 0.06; cMetS area under the curve was 0.72 ± 0.05 suggesting a better predictive power of the cMetS for an increased AASI (>0.3). Patients with obesity have significantly higher arterial stiffness. A composite score such as cMetS seems to be better predictor of an increased stiffness than individual risk factors.

Purpose of the program: To provide guidance on the management of pediatric kidney transplant patients during the COVID-19 pandemic. Sources of information: Program-specific documents, preexisting, and related to COVID-19; documents from provincial, national, and international kidney transplant societies/agencies and organ procurement agencies; national and international webinars, including webinars that we hosted for input and feedback; with additional information from formal and informal review of published academic literature. Methods: Challenges in the care of pediatric kidney transplant patients during the COVID-19 pandemic were highlighted within the Canadian Society of Transplantation (CST) Pediatric Group. It identified pediatric kidney transplant nephrologists (including a pediatric nephrologist ethicist) across the country and formed a workgroup. The initial guidance document was drafted and members of the workgroup reviewed and discussed all suggestions in detail via e-mail and virtual meetings. Disagreements were resolved by consensus. The document was reviewed by the CST Kidney Transplant Working Group, by the Canadian Society of Nephrology (CSN) COVID-19 Rapid Response Team (RRT), and an infectious disease expert. The suggestions were presented at an interactive webinar sponsored by CSN in collaboration with the CST and Canadian Association of Pediatric Nephrologists (CAPN), and attended by pediatric kidney health care professionals for further peer input. Final revisions were made based on feedback received. CJKHD editors reviewed the parallel process peer review and edited the manuscript for clarity. Key findings: We identified 8 key areas of pediatric kidney transplant care that may be affected by the COVID-19 pandemic: (1) transplant activity, (2) outpatient clinic activity, (3) monitoring, (4) multidisciplinary care, (5) medications (immunosuppression and others), (6) patient/family education/support, (7) school and employment, and (8) management of pediatric kidney transplant patients who are COVID-19 positive. We make specific suggestions for each of these areas. Limitations: A full systematic review of available literature was not undertaken for the sake of expediency in development of this guideline. There is a paucity of literature to support evidence-based recommendations at this time. Instead, these guidelines were formulated based on expert opinion derived from available knowledge/experience and are subject to the biases associated with this level of evidence. The parallel review process that was created to expedite the publication of this work may not be as robust as standard arms’ length peer review processes. Implications: These recommendations are meant to serve as a guide to pediatric kidney transplant directors, clinicians, and administrators for providing the best patient care in the context of limited resources while protecting patients and health care providers wherever possible by limiting exposure to COVID-19. We recognize that recommendations may not be applicable to all provincial/local health authority practices and that they may not be delivered to all patients given the time and resource constraints affecting the individual provincial/local health jurisdiction.

Children with untreated hypertension have a high risk of developing target organ damage and cardiovascular complications later on in life. The currently accepted threshold for the definition of hypertension in children is a blood pressure above the 95th percentile or >140/90. Recent reports suggest, however, that the risk of target organ damage and cardiovascular morbidity is already increased at lower than hypertensive blood pressure levels (90–95th percentile or 120–140/80–90) which are labelled as high-normal blood pressure or prehypertension. Similarly to established hypertension levels, high-normal blood pressure in children is likely to persist into adulthood (tracking) or convert into overt hypertension. On the other hand, high-normal blood pressures may return to normotensive values during follow-up. The intrinsic long-term blood pressure variability underlines the need for repeated BP measurements over time. Consistently high-normal blood pressure/prehypertension may be an indication for the need for early workup of target organ damage and at least non-pharmacological treatment. However, in case of subclinical target organ damage early pharmacological may be considered.

Objective: Spurious hypertension (sHTN) is characterized by elevated systolic blood pressure (BP) in peripheral arteries measured by office BP and ambulatory BP monitoring (ABPM), but normal central BP. The prevalence of sHTN and its clinical significance remains unknown. The aim of the study was to assess hemodynamics and target organ damage (TOD) in adolescents with sHTN in comparison with other forms of hypertension (HTN). Patients and Methods: We analyzed 265 children (54 girls; 15.1 ±2.5 years) referred with arterial HTN, in whom secondary HTN was excluded. We assessed BP levels - office BP, ABPM, central systolic BP (cSBP), hemodynamics - cardiac output (CO), stroke volume (SV) and target organ damage (TOD) - left ventricular mass index (LVMi), carotid intima-media thickness (cIMT), pulse wave velocity (PWV). Anova with TukeyHSD post hoc test was used for analysis. Results: 115 subjects had white coat hypertension (WCH; office BP>95 th perc+ABPMth perc), 25 had ambulatory prehypertension (preHTN; office BPth perc+ABPMth perc+ABPM load >25%), 43 had sHTN (office BP+ABPM >95th perc+cSBPth perc), 82 had true hypertension (tHTN; office BP+ABPM+cSBP >95th perc. There were no significant differences between groups in age, weight and BMI. Only pts with tHTN presented features typical of hyperkinetic circulation and pts with preHTN presented intermediate hemodynamic phenotype between WCH and sHTN and those with tHTN (p2.7 /95 th percentile by Khoury et. 2009) was similar in both WCH and sHTN (0.82 and 0.80, respectively), and significantly lower than in tHTN (0.89, p=0.04). The proportion of patients with LVH (LVMi>=95 th perc) was 12% in WCH, 4% in preHTN, 9% in sHTN, 22% in tHTN (p=0.05). cIMT Z-scores were also significantly lower in both WCH (0.92) and sHTN (0.90) groups compared to tHTN (1.22, p=0.01). Similar results were obtained for PWV Z-scores (WCH=1.8, sHTN=1.29, tHTN=2.28, p=0.01). Patients with preHT were not significantly different from tHTN patients in all parameters. Conclusions: Adolescents with sHTN have similar hemodynamic phenotype and TOD as patients with WCH. However, because of lack of data on the evolution of sHTN, close monitoring and follow-up is warranted.

Objective: Spurious hypertension (sHTN) is characterized by elevated systolic blood pressure (BP) in peripheral arteries but normal central BP. The BP variability and characteristics of BP rhythms in sHTN is unknown. We therefore analyzed BP variability and rhythmicity in adolescents with sHTN in comparison with other forms of hypertension (HTN). Patients and Methods: We studied 265 children (54 girls; 15.1 ±2.5 years) referred with arterial HTN, in whom secondary HTN was excluded. We assessed BP levels - office BP, ABPM, central systolic BP (cSBP), systolic BP average real variability (SBPARV) and mean MAP rhythmicity (amplitudes and acrophases). Anova with TukeyHSD post hoc test was used to compare groups. One tailed t-test was used to compare MAP amplitudes and acrophases with normative values for children with Bonferroni correction for multiple tests. Results: 115 subjects had white coat hypertension (WCH; office BP>95 th perc+ABPMth perc), 25 had ambulatory prehypertension (preHTN; office BPth perc+ABPMth perc+ABPM load >25%), 43 had sHTN (office BP+ABPM >95th perc+cSBPth perc), 82 had true hypertension (tHTN; office BP+ABPM+cSBP >95th perc. There were no significant differences between groups in age, weight and BMI. The median SBPARV was 8.87 in WCH, 8.63 in preHTN, 9.25 in sHTN and 9.28 in tHTN (not significantly different between groups). The prevalence of 24h rhythms ranged from 77 to 88%, not significantly from norms or among groups. The prevalence of 12h rhythms was not significantly different compared to norms and ranged from 50 to 60% (NS among groups). There were also no significant differences in the prevalence of 8h and 6h rhythms. All groups had significantly decreased 24h amplitudes compared to norms, but not significantly different among groups. There were no differences in 12h, 8h and 6 amplitudes between WCH, preHTN, sHTH, tHTN and norms. 24h acrophases were significantly prolonged in sHTN, 12h acrophases were significantly prolonged in preHTN, sHTN and tHTN. Conclusions: Adolescents with spurious hypertension have significantly lower prevalence of 24h rhythms, decreased 24h amplitudes and prolonged 24h and 12h acrophases suggesting increased sympathetic drive similarly to patients with other hypertensive phenotypes.

Recommended standards for assessing blood pressure in human research where blood pressure or hypertension is a major focus

After the 2016 guidelines for blood pressure measurement, diagnosis, and investigation of pediatric hypertension, we now present evidence-based guidelines for the prevention and treatment of hypertension in children. These guidelines were developed by Hypertension Canada's Guideline Committee pediatric subgroup after thorough evaluation of the available literature. Included are 10 guidelines specifically addressing health behaviour management, indications for drug therapy in children with hypertension, choice of therapy for children with primary hypertension, and goals of therapy for children with hypertension. Although the pediatric literature is inherently limited by small numbers of participants, fewer trials, and a prolonged latency to the development of vascular outcomes, this report reflects the current and highest level of evidence and provides guidance for primary care practitioners on the management of pediatric hypertension. Studies of therapeutic lifestyle modifications in children are available to guide current management and more antihypertensive drugs have been studied in children since the Food and Drug Administration Modernization Act. Consistent with Hypertension Canada's guideline policy, diagnostic and therapeutic algorithm tools will be developed and the guidelines will be reviewed annually and updated according to new evidence.

Hemolytic uremic syndrome (HUS) is characterized bymicroangiopathic anemia, thrombocytopenia, and acute kidney injury [1]. In addition, severe HUS can be associated with irreversible renal and CNS complications, leading to chronic kidney disease and permanent neurological deficits [2]. The most common form of HUS is induced by a verocytotoxin/ Shiga toxin (Vtx/Stx)-producing bacteria [3]. Less commonly, HUS can be induced by bacterial (e.g., S. pneumoniae) [3] or viral (e.g., influenza) infections [4]. We report a patient with a severe clinical presentation of HUS following pneumonia (presumed S. pneumoniae infection). Making the correct diagnosis proved to be difficult as the patient presented with unusual findings, including a negative Coombs test and low C3 complement level on two occasions. These findings did not meet the usual diagnostic criteria of S. pneumoniae-induced HUS, which include a positive Coombs test and a normal complement C3 level. While HUS caused by defective complement alternative pathway (CAP) regulation, presents with low serum C3 complement in approximately 75 % of patients [5], patients with the infection-induced types of HUS do not exhibit complement dysregulation, and their C3 levels are normal [3, 6]. In patients with S. pneumoniae-induced HUS, the C3 levels are usually normal, and the Coombs test is positive, owing to antibodies binding to the exposed T-antigen on the surface of red blood cells [7, 8]. Thus, the combination of normal C3 complement levels with a positive Coombs test would confirm the diagnosis of S. pneumoniae-induced HUS [7, 8]. However, complement activation leading to a lower C3 complement can occur even in non-genetic forms of HUS [9], which can be considered an aggravating factor for the disease progression. Furthermore, the Coombs test can be positive in 25 to 90 % of cases [10, 11], indicating that a relatively large proportion of patients with pneumococcal HUS can have a negative Coombs test. This refers to the article that can be found at http://dx.doi.org/10.1007/ s00467-015-3101-y

The diagnosis of hypertension is often unrecognized in the general paediatric and type 1 diabetes populations. Reference to a simple blood pressure table has been proposed as a screening tool for the identification of abnormal paediatric blood pressure. This simple table lists one cut-off blood pressure value for each sex and year of age, compared with the standard blood pressure tables, which list 476 abnormal and normal blood pressure values.To determine the sensitivity and specificity of the simple blood pressure table in identifying hypertensive and abnormal (hypertensive or prehypertensive) blood pressure values in children with type 1 diabetes.A retrospective cohort study was conducted. Data regarding baseline characteristics and blood pressure measurements were collected. Blood pressure values were categorized as nonhypertensive, prehypertensive or hypertensive using the standard and simple blood pressure tables. Sensitivity and specificity of the simple blood pressure table was determined for the identification of hypertensive and abnormal (hypertensive or prehypertensive) blood pressure values using a generalized estimating equation approach.The simple blood pressure table had sensitivities of 100% (95% CI 95.7% to 100%) and 100% (95% CI 97.6% to 100%), and specificities of 61.1% (95% CI 54.0% to 67.8%) and 81.3% (95% CI 74.4% to 86.6%), for the identification of hypertensive and abnormal blood pressure values, respectively.The simple blood pressure table is an effective screening tool that may enable earlier detection and timely treatment of abnormal blood pressure.Le diagnostic d’hypertension demeure souvent non diagnostiqué dans les populations générale et atteinte du diabète de type 1 d’âge pédiatrique. On a proposé d’utiliser un tableau simplifié de la tension artérielle comme outil de dépistage d’une tension artérielle anormale en pédiatrie. Ce tableau simplifié indique une valeur seuil de tension artérielle selon le sexe et l’âge, par rapport aux tableaux de tension artérielle standards, qui contiennent 476 valeurs de tension artérielle anormales et normales.Déterminer la sensibilité et la spécificité du tableau simplifié de la tension artérielle pour déterminer les valeurs hypertensives et anormales (hypertension et préhypertension) de la tension artérielle chez des enfants atteints du diabète de type 1.Les chercheurs ont mené une étude rétrospective de cohorte. Ils ont amassé des données relatives aux caractéristiques et aux mesures de tension artérielle de base. Ils ont classé les valeurs de tension artérielle entre non hypertensives, préhypertensives ou hypertensives au moyen des tableaux de la tension artérielle standard et simplifié. Ils ont déterminé la sensibilité et la spécificité du tableau simplifié de la tension artérielle pour déterminer les valeurs hypertensives et anormales (hypertension ou préhypertension) de la tension artérielle au moyen d’une approche d’équations d’estimation généralisées.Le tableau simplifié de la tension artérielle présente une sensibilité de 100 % (95 % IC 95,7 % à 100 %) et de 100 % (95 % IC 97,6 % à 100 %), et une spécificité de 61,1 % (95 % IC 54,0 % à 67,8 %) et de 81,3 % (95 % IC 74,4 % à 86,6 %) pour déterminer des valeurs de tension artérielle hypertensives et anormales, respectivement.Le tableau simplifié de la tension artérielle est un outil de dépistage efficace qui peut favoriser un dépistage plus rapide et un traitement opportun de la tension artérielle anormale.

Primary hypertension is associated with disturbed activity of the sympathetic nervous system and altered blood pressure rhythmicity. We analyzed changes in cardiovascular rhythmicity and its relation with target organ damage during 12 months of antihypertensive treatment in 50 boys with hypertension (median, 15.0 years). The following parameters were obtained before and after 12 months of antihypertensive treatment: 24-hour ambulatory blood pressure, left ventricular mass, carotid intima–media thickness, and MRI for visceral and subcutaneous adipose tissue. Amplitudes and acrophases of mean arterial pressure and heart rate rhythms were obtained for 24-, 12-, and 8-hour periods. After 1 year of treatment, 68% of patients were normotensive, and left ventricular mass and carotid intima–media thickness decreased in 60% and 62% of patients, respectively. Blood pressure and heart rate rhythmicity patterns did not change. Changes in blood pressure amplitude correlated with the decrease of waist circumference ( P =0.035). Moreover, the decrease of visceral fat correlated with the decrease of 24-hour mean arterial pressure and heart rate acrophases (both P

Background Practice variation is common for nephrotic syndrome (NS) treatment. Methods A cross-sectional, web-based survey on NS treatment was administered to 58 Canadian pediatric nephrologists with the aim to document existing practice variation and compare practice with the recommendations of the Kidney Disease Improving Global Outcomes Clinical Practice Guideline for NS. Results Of the 58 nephrologists asked to participate in the survey, 40 (69 %) responded. Among these, 62 % prescribed initial daily glucocorticoid (GC) therapy for 6 weeks, 26 % for 4w eeks by 26 %, and 10 %p rescribed‘other’ .A lternateday GC was continued for 6 weeks by 63 % of respondents and for >6 and

The aim of the study was to analyze whether auscultatory normative values (Fourth Task Force [4TF]) can be applied to blood pressure (BP) obtained by oscillometric devices. The authors performed a retrospective analysis of oscillometric office BP and ambulatory BP monitoring in 229 children (116 boys), median age 15.31 years. Office systolic BP (SBP) and diastolic BP (DBP) values were converted into Z scores using 4TF and oscillometric (German Health Interview and Examination Survey for Children and Adolescent [KiGGS]) reference values. There was good correlation between the two normative methods (r=0.9773 for SBP, r=0.9627 for DBP). Results from Bland-Altman test revealed only minimal differences in Z scores between 4TF and KiGGS for SBP, but a significant proportional error for DBP. 4TF and KiGGS Z scores were equally predictive of ambulatory hypertension. In conclusion, auscultatory and oscillometric normative data are interchangeable for SBP but not for DBP.

We present the inaugural evidence-based Canadian recommendations for the measurement of blood pressure in children and the diagnosis and evaluation of pediatric hypertension. Rates of pediatric hypertension are increasing concomitant with increased rates of childhood obesity. With this, there is growing awareness of the need to measure blood pressure in children. Consequently, the present recommendations have been developed to address an important gap and improve the clinical care of children. For 2016, a total of 15 recommendations are presented. These are categorized in a fashion similar to that of the existing adult recommendations. Specifically, we present recommendations on (1) accurate measurement of blood pressure in children, (2) criteria for diagnosis of hypertension in children, (3) assessment of overall cardiovascular risk in hypertensive children, (4) routine laboratory tests for the investigation of children with hypertension, (5) ambulatory blood pressure measurement in children, and (6) the role of echocardiography. We discuss the rationale for the recommendations and present additional supporting material for the clinician, including tables with standardized techniques for blood pressure measurement and determination of normative blood pressure values for children. Hypertension Canada's Canadian Hypertension Education Program Guidelines Task Force will update the recommendations annually and develop future evidence-based recommendations to guide prevention and treatment of pediatric hypertension.

Hypertension Canada's Canadian Hypertension Education Program Guidelines Task Force provides annually updated, evidence-based recommendations to guide the diagnosis, assessment, prevention, and treatment of hypertension. This year, we present 4 new recommendations, as well as revisions to 2 previous recommendations. In the diagnosis and assessment of hypertension, automated office blood pressure, taken without patient-health provider interaction, is now recommended as the preferred method of measuring in-office blood pressure. Also, although a serum lipid panel remains part of the routine laboratory testing for patients with hypertension, fasting and nonfasting collections are now considered acceptable. For individuals with secondary hypertension arising from primary hyperaldosteronism, adrenal vein sampling is recommended for those who are candidates for potential adrenalectomy. With respect to the treatment of hypertension, a new recommendation that has been added is for increasing dietary potassium to reduce blood pressure in those who are not at high risk for hyperkalemia. Furthermore, in selected high-risk patients, intensive blood pressure reduction to a target systolic blood pressure ≤ 120 mm Hg should be considered to decrease the risk of cardiovascular events. Finally, in hypertensive individuals with uncomplicated, stable angina pectoris, either a β-blocker or calcium channel blocker may be considered for initial therapy. The specific evidence and rationale underlying each of these recommendations are discussed. Hypertension Canada's Canadian Hypertension Education Program Guidelines Task Force will continue to provide annual updates.

In pediatric patients at risk of hyperkalemia there are limited treatment or preventive alternatives for this electrolyte imbalance. Oral or rectal sodium polystyrene sulfonate (SPS) has several potential adverse effects, and dietary potassium restriction may compromise nutrition. Pretreatment of infant formula with SPS has been previously studied with promising efficacy. The optimal dosing and contact time has not been fully elucidated for this practice, nor have brand and generic products been compared. The present study aimed to evaluate the effectiveness of varying amounts of brand and generic SPS for the removal of potassium from formula after 1 and 24 hours. SPS was added to infant formula in four different amounts measured in milliliters to reflect how a parent or caregiver would measure this product at home. After 1 and 24 hours samples were withdrawn and potassium and sodium levels were measured. Potassium decreased in all samples, with the greatest reduction after the addition of 10 mL of SPS. Sodium levels increased in all pretreated samples to a greater extent than the potassium reduction. Contact time of either 1 or 24 hours did not impact the amount of potassium removed or the increase in sodium concentration. There were also no differences found between generic and brand SPS products. The effectiveness of SPS for formula pretreatment appears to have a plateau effect beyond the addition of 20 mL (16.47 g of brand name product, 19.5 g of generic product). This study demonstrates an effective protocol for pretreatment of formula.

Tsampalieros A, Lepage N, Feber J. Intraindividual variability of the modified Schwartz and novel CKiD GFR equations in pediatric renal transplant patients. Pediatr Transplantation 2011: 15: 760–765. © 2011 John Wiley & Sons A/S. Abstract: GFR in children can be obtained from a formula using SCr and height or various formulas including serum CysC. Recently, two new GFR formulas have been developed: (i) height and SCr—mSchwartz GFR and (ii) height, SCr, CysC, and serum urea (CKiD GFR). While these formulas proved to be accurate when compared to the gold standard, their use in children post-kidney Tx is yet to be assessed. A total of 1174 blood samples (urea, SCr and CysC) were analyzed from the post-Tx period in 24 Tx children (12 boys, median age = 8.6 yr) currently followed at our institution. CKiD GFR and mSchwartz GFR were compared using Bland–Altman analysis and the CV. The mSchwartz GFR overestimated the CKiD GFR (mean bias = 1.09 ± 0.14; 95% limits of agreements from 0.82 to 1.36). Median CV of CKiD GFR (10.3%) was significantly lower than that of mSchwartz GFR (15.0%), p = 0.04, and negatively correlated with the slope of GFR (r2 = 0.34, p = 0.0026). In conclusion, CKiD GFR has a significantly lower intraindividual variation than mSchwartz GFR and may be better suited for longitudinal follow-up of patients post-Tx.