Posterior Reversible Encephalopathy Syndrome in Children and Adolescents.

PURPOSE OF REVIEW: Posterior reversible encephalopathy syndrome, or PRES, is a constellation of severe, acute hypertension and specific brain imaging findings. This may be caused by failure of the cerebral autoregulatory system to manage acute or severe changes in blood pressure. The incidence in children is unknown but estimated to be more common in children with predisposing factors including renal disease, autoimmune disease, malignancy, solid organ transplantation, stem cell transplantation, hypertension, sepsis, and exposure to certain medications. RECENT FINDINGS: Management of PRES includes addressing hypertension, removing offending agents when possible, and anti-epileptic medications. Most children with PRES recover completely, but recurrence is possible. Lack of resolution of imaging findings likely portends a worse prognosis.

Long term renal outcome and risk of elevated blood pressure in children undergoing complete primary repair of bladder exstrophy (CPRE).

INTRODUCTION: Since the initiation of staged reconstruction for bladder exstrophy (BE), hypertension has been a known complication of the procedure. Hypertension is a well-established risk factor for chronic kidney disease (CKD) progression and associated with cardiovascular/cerebrovascular morbidity and mortality. Few studies exist evaluating the risk of developing hypertension among patients with bladder exstrophy who underwent CPRE. We hypothesized that long-term blood pressure levels may be elevated in males vs females, and may be correlated with presence of hydronephrosis, bladder neck reconstruction, or continence status. OBJECTIVE: We sought to revisit our long-term experience with CPRE and determine factors associated with incidence of elevated blood pressures. METHODS: We reviewed all BE patients undergoing CPRE at our institution from 1999 to 2019. Patients were considered eligible for inclusion if last renal ultrasound was obtained at least 5 years after repair. Upper tract outcomes based on imaging, history of pyelonephritis and renal function tests measured by serum creatinine and estimated glomerular filtration rate (eGFR, Schwartz formula) were reviewed. Systolic/diastolic blood pressures (SBP/DBP) from all encounters were captured. All blood pressure values were age adjusted by percentile. RESULTS: A total of 36 patients were considered eligible for review. Median follow-up of this cohort was 10.01 (5.16-21.47) years. The mean creatinine for the patients available was 0.58 mg/dL (SD = 0.20), at mean age of 8.90 years Neither SBP or DBP were significantly elevated in males vs females, but had lower odds of elevation >90th percentile for those with higher eGFR, lower renal length, and reimplantation. Pyelonephritis incidence was 38% (n = 14) with first episode at mean age of 8.8 years, and mean of 3.7 episodes per patient. DISCUSSION: At long term follow up, blood pressures following CPRE were not significantly elevated, despite the relatively frequent occurrence of CKD, and hydronephrosis. Male gender does appear to suggest higher risk for long-term deterioration in this regard. Higher eGFR, higher renal length, and presence of ureteral reimplantation were associated with lower likelihood of systolic/diastolic blood pressure elevation. Continence status and bladder neck reconstruction were not associated with likelihood of blood pressure elevation. CONCLUSIONS: Blood pressure and upper-tract outcomes for patients undergoing CPRE at birth are positive for the majority of patients. To avoid complications from hypertension, patients should be closely evaluated as the risks associated with elevated blood pressure are significant. Ultimately, larger-scale prospective and multi-institutional studies are further needed to characterize risks of hypertension in this complex patient population.

Effect of Age on Hypertension Recognition in Children With Chronic Kidney Disease: A Report From the Chronic Kidney Disease in Children Study.

BACKGROUND: Young age has been associated with poorer control of hypertension in children with chronic kidney disease (CKD). Using data from the CKiD Study (Chronic Kidney Disease in Children), we examined the relationship between age, hypertensive blood pressure (BP) recognition, and pharmacologic BP control in children with nondialysis-dependent CKD. METHODS: Participants included 902 CKiD Study participants with CKD stages 2 to 4. A total of 3550 annual study visits met inclusion criteria and participants were stratified by age (0 to <7, ≥7 to <13, ≥13 to ≤18 years). Generalized estimating equations to account for repeated measures were applied to logistic regression analyses to evaluate the association of age with unrecognized hypertensive BP and medication use. RESULTS: Children <7 years of age had a higher prevalence of hypertensive BP and a lower prevalence of antihypertensive medication use compared with older children. At visits where participants <7 years of age had hypertensive BP readings, 46% had unrecognized, untreated hypertensive BP compared with 21% of visits for children ≥13 years of age. The youngest age group was associated with higher odds of unrecognized hypertensive BP (adjusted odds ratio, 2.11 [95% CI, 1.37-3.24]) and lower odds of antihypertensive medication use among those with unrecognized hypertensive BP (adjusted OR, 0.51 [95% CI, 0.27-0.996]). CONCLUSIONS: Children younger than 7 years of age with CKD are more likely to have both undiagnosed and undertreated hypertensive BP. Efforts to improve BP control in young children with CKD are needed to minimize development of cardiovascular disease and slow CKD progression.

Burnout in Pediatric Nephrology Fellows and Faculty: Lessons From the Sustainable Pediatric Nephrology Workforce Project (SUPERPOWER).

Physician well-being is an important contributor to both job satisfaction and patient outcomes. Rates of burnout among physicians vary by specialty, ranging from 35 to 70%. Among pediatric residents, longitudinal data demonstrates consistent rates of burnout around 50-60%, although little is known about burnout among pediatric subspecialty fellows. Specifically, the degree of burnout among pediatric nephrologists remains unknown, as does the impact faculty burnout may have on trainee burnout. We sought to evaluate prevalence and predictors of burnout among US pediatric nephrology fellows and faculty, and assess for interactions between groups. In this multi-center pilot survey of all United States pediatric nephrology training programs from February to April 2020, burnout was assessed through abbreviated Maslach Burnout Inventory and predictors were explored through survey items devoted to demographic, personal characteristics, and job and career satisfaction questions. A total of 30/34 available fellows and 86/102 faculty from 11 institutions completed the survey (overall response rate 85%). The prevalence of burnout was 13% among fellows and 16% among faculty. Demographic (age, gender, year of training, faculty rank, marital status) and program factors (fellowship size, faculty size, current block/rotation, vacation or weekend off timing) were not significantly associated with burnout. Faculty and fellows with burnout reported significantly lower quality of life (5.3 vs. 7.9, p < 0.05), higher perceived stress (2.4 vs. 1.4, p < 0.05) and lower satisfaction with career choice (66 vs. 22%) and work life balance (28 vs. 0%), compared to those without burnout (p < 0.05 for all). Other important factors positively associated with burnout included lower institutional support for wellness programs and lower satisfaction with both colleague and faculty support. Larger studies are needed to explore if burnout is truly less prevalent among pediatric nephrology fellows and faculty compared to pediatric residents and graduate physicians. A larger sample size is also necessary to determine whether any interactions exist between the faculty and trainee roles in the developments of burnout. Future studies should also explore how to promote well-being through addressing key factors such as overall learning/working environment, stress reduction, and building personal resilience.

Impact of the COVID-19 Pandemic on Nephrology Fellow Training and Well-Being in the United States: A National Survey.

BACKGROUND: The coronavirus disease 2019 (COVID-19) pandemic's effects on nephrology fellows' educational experiences, preparedness for practice, and emotional wellbeing are unknown. METHODS: We recruited current adult and pediatric fellows and 2020 graduates of nephrology training programs in the United States to participate in a survey measuring COVID-19's effects on their training experiences and wellbeing. RESULTS: Of 1005 nephrology fellows-in-training and recent graduates, 425 participated (response rate 42%). Telehealth was widely adopted (90% for some or all outpatient nephrology consults), as was remote learning (76% of conferences were exclusively online). Most respondents (64%) did not have in-person consults on COVID-19 inpatients; these patients were managed by telehealth visits (27%), by in-person visits with the attending faculty without fellows (29%), or by another approach (9%). A majority of fellows (84%) and graduates (82%) said their training programs successfully sustained their education during the pandemic, and most fellows (86%) and graduates (90%) perceived themselves as prepared for unsupervised practice. Although 42% indicated the pandemic had negatively affected their overall quality of life and 33% reported a poorer work-life balance, only 15% of 412 respondents who completed the Resident Well-Being Index met its distress threshold. Risk for distress was increased among respondents who perceived the pandemic had impaired their knowledge base (odds ratio [OR], 3.04; 95% confidence interval [CI], 2.00 to 4.77) or negatively affected their quality of life (OR, 3.47; 95% CI, 2.29 to 5.46) or work-life balance (OR, 3.16; 95% CI, 2.18 to 4.71). CONCLUSIONS: Despite major shifts in education modalities and patient care protocols precipitated by the COVID-19 pandemic, participants perceived their education and preparation for practice to be minimally affected.

Practical application of ABPM in the pediatric nephrology clinic.

The use of 24-h ABPM has become commonplace when diagnosing and managing hypertension in the pediatric population. Multiple clinical guidelines recommend ABPM as the preferred method for identifying white-coat hypertension, masked hypertension, and determining degree of blood pressure (BP) control. Accurate, timely diagnosis and optimal management are particularly important in certain populations, such as children with chronic kidney disease (CKD), diabetes, and other conditions with increased risk for cardiovascular disease. Understanding how best to utilize ABPM to achieve these goals is important for pediatric nephrologists and other hypertension specialists. This review will provide practical information on the equipment, application, interpretation, and documentation of ABPM in the specialty clinic.

Changing outpatient referral patterns in a small pediatric nephrology practice.

BACKGROUND: We have noted a large number of referrals for abnormal kidney imaging and laboratory tests and postulated that such referrals have increased significantly over time. Understanding changes in referral patterns is helpful in tailoring education and communication between specialists and primary providers. METHODS: We performed a retrospective chart review of new patient referrals to Mary Bridge Children's Nephrology clinic for early (2002 to 2004) and late (2011 to 2013) cohorts. The overall and individual frequencies of referrals for various indications were compared. RESULTS: The overall number of new visits was similar for early (511) and late (509) cohorts. The frequency of referrals for solitary kidneys and multi-cystic dysplastic kidneys, microalbuminuria and abnormal laboratory results increased significantly (Odds Ratio (OR) and 95% Confidence Interval of OR: 1.920 [1.079, 3.390], 2.862 [1.023, 8.006], 2.006 [1.083, 3.716], respectively) over the time interval while the proportion of referrals for urinary tract infections (UTIs) and vesicoureteral reflux (VUR) decreased by half (OR: 0.472, 95% CI: 0.288, 0.633). Similarly, referrals for urinary tract dilation and hydronephrosis occurred significantly less often (8% versus 6%, OR: 0.737, 95% CI: 0.452, 1.204) with similar changes in referrals for voiding issues (OR: 0.281, 95% CI: 0.137, 0.575). However, these changes were not statistically significant. Frequencies for other indications showed little variation. CONCLUSIONS: Changes in indications for referral likely reflect evolution of practice in management of UTIs and VUR and increased use of imaging and laboratory testing by pediatric providers. These findings have relevance for ongoing education of pediatricians and support the need for collaboration between primary providers and nephrologists to assure the judicious use of resources.

Chronic School Absenteeism of Children with Chronic Kidney Disease.

We evaluated the frequency of chronic school absenteeism (≥18 missed school days per year) among children with mild-to-moderate chronic kidney disease. Chronic absenteeism was present in 17.3% of children with chronic kidney disease, compared with 2.7% of children in the US National Health and Nutrition Examination Survey.

Utility of ambulatory blood pressure monitoring in the evaluation of elevated clinic blood pressures in children.

Although ambulatory blood pressure monitoring (ABPM) is recognized for its role in assessing white coat hypertension, other uses include evaluation of treatment adequacy, nocturnal hypertension, dipping status, and hypertension severity. We performed a retrospective study of ABPMs completed at a single center from November 2007 to October 2011 to determine the frequency of white coat hypertension, prehypertension, and hypertension in children and the correlation of these findings with office BPs. A total of 247 ABPMs were performed in 206 children, ages 4-20 years, including 48 recordings in 39 diabetic patients and 64 recordings in treated hypertensive patients. We found a poor correlation between hypertensive status based on clinic BP and diagnosis on ABPM, and evidence for a white coat effect. Among treated patients, ABPM results resulted in medication changes in 63%. We conclude that ABPM is a useful tool for characterizing hypertensive status and treatment adequacy in children.

The Association of Pediatric Obesity With Nocturnal Non-Dipping on 24-Hour Ambulatory Blood Pressure Monitoring.

BACKGROUND: Obesity has been linked with abnormal nocturnal dipping of blood pressure (BP) in adults, which in turn is associated with poor cardiovascular outcomes. There are few data regarding abnormal dipping status in the obese pediatric population. The goal of this study was to further describe the relationship between obesity and non-dipping status on ambulatory blood pressure monitor (ABPM) in children. METHODS: We conducted a cross-sectional study using a database of patients aged 5-21 years who had undergone 24-hour ABPM at Seattle Children's Hospital from January 2008 through May 2014. Subjects were grouped by body mass index (BMI) into lean (BMI 15th-85th percentile) and obese (BMI >95th percentile) groups. RESULTS: Compared to lean subjects (n = 161), obese subjects (n = 247) had a prevalence ratio (PR) for non-dipping of 2.15, adjusted for race (95% confidence interval (CI) = 1.25-3.42). Increasing severity of obesity was not further associated with nocturnal non-dipping. Nocturnal non-dipping was not associated with left ventricular hypertrophy (PR = 1.01, 95% CI = 0.71-1.44). CONCLUSIONS: These results suggest that in children, just as in adults, obesity is related to a relatively decreased dipping in nocturnal BP.

Treatment of hypertension in children with chronic kidney disease.

Hypertension (HTN) is increasingly recognized as a common feature of pediatric chronic kidney disease (CKD). A growing body of evidence demonstrates that HTN is both underdiagnosed and undertreated in this population. The consequences of untreated HTN include adverse effects on CKD progression, markers of cardiovascular morbidity, and neurocognitive functioning. Consensus guidelines issued over the past decade have incorporated recent research on the consequences of HTN in recommendations for the diagnosis and treatment of HTN in pediatric CKD and include lower BP targets. Agents which target the renin-angiotensin-aldosterone system (RAAS) should be considered first-line therapy in CKD-associated HTN in children, though multiple medications may be required to achieve sufficient BP control.

Treatment of obesity-related hypertension in children and adolescents.

The obesity epidemic has become a common concern among pediatricians, with an estimated 32 % of US children and adolescents classified as overweight and 18 % as obese. Along with the increase in obesity, a growing body of evidence demonstrates that chronic diseases, such as Type 2 diabetes, primary hypertension, and hyperlipidemia, once thought to be confined solely to adulthood, are commonly seen among the obese in childhood. Following a brief summary of the diagnosis and evaluation of hypertension in obese children and adolescents, this review will highlight recent research on the treatment of obesity-related hypertension. Pharmacologic and non-pharmacologic treatment will be discussed. Additionally, current and emerging therapies for the primary treatment of obesity in children and adolescents, which have been gaining in popularity, will be reviewed.

Predictors of blood pressure and its control in pediatric patients receiving dialysis.

OBJECTIVE: To evaluate and characterize the degree of blood pressure (BP) control in children on chronic dialysis and to identify significant predictors of hypertension and BP control in these patients. STUDY DESIGN: Linear and logistic regression models were used to examine trends in BP and BP control in a cross-sectional sample of patients on chronic dialysis aged 1-21 years enrolled in the North American Pediatric Renal Trials and Collaborative Studies registry from 1992-2008. RESULTS: At 6 months after dialysis initiation, 67.9% of patients had uncontrolled or untreated hypertension, and 57.8% were prescribed antihypertensive medications. More recent year of dialysis initiation was associated with a higher use of antihypertensive medication and lower systolic BP and diastolic BP z scores (P < .001) measured over time from 6 months to 3 years post dialysis initiation. Other factors associated with higher BP included black race, glomerular disease, younger age, hemodialysis (systolic BP only), and antihypertensive use. There were significant differences in BP control by dialysis modality and disease etiology, with patients on hemodialysis or those with glomerular diseases having the highest percentage of uncontrolled hypertension. CONCLUSIONS: Despite widespread antihypertensive use, many pediatric patients on dialysis are at risk for untreated or uncontrolled hypertension. Additional efforts are needed to improve management of hypertension in these children.