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Despite prevention strategies, cytomegalovirus (CMV) remains a common infection in pediatric solid organ transplant recipients (SOTR). We sought to determine the frequency, associations with, and long-term outcomes of CMV DNAemia in pediatric SOTR. We performed a single-center retrospective cohort study, including 687 first time SOTR ≤21 years receiving universal prophylaxis from 2011 to 2018. Overall, 159 (23%) developed CMV DNAemia, the majority occurring after completing primary prophylaxis. CMV disease occurred in 33 (5%) SOTR, 25 (4%) with CMV syndrome and 10 (1%) with proven/probable tissue-invasive disease. CMV contributed to the death of three (0.4%) patients (all lung). High-risk (OR 6.86 [95% CI, 3.6-12.9]) and intermediate-risk (4.36 [2.3-8.2]) CMV status and lung transplantation (4.63 [2.33-9.2]) were associated with DNAemia on multivariable analysis. DNAemia was associated with rejection in liver transplant recipients (p

We aimed to investigate intradialytic changes in ventricular and atrial function using speckle tracking echocardiography (STE) in pediatric hemodialysis (HD). Children with HD vintage > 3 months were enrolled, and echocardiography was performed prior to, during, and after HD. STE was analyzed using GE EchoPAC. Left ventricular (LV) global longitudinal strain (GLS), strain rate (Sr), and mechanical dispersion index (MDI) were calculated as the average from 3 apical views; diastolic strain (Ds) and Sr from 4-chamber tracing; left atrial strain (LAS) and Sr from the 4- and 2-chamber views. A total of 15 patients were enrolled at a median age of 12 years (IQR 8, 16) and median HD vintage of 13 months (IQR 9, 25). GLS worsened during HD (− 15.8 ± 2.2% vs − 19.9 ± 1.9%, p 0.1). In conclusions, intradialytic LV strain and LAS changes consistent with subclinical systolic and diastolic dysfunction were observed during HD in children. Changes in Ds, DSr, LAS, and LASr were not associated with BP change or BV removal and may be related to the disease progression. Longitudinal study using these novel indices may unfold the effect of these subclinical changes on long-term cardiovascular health in children requiring chronic HD.

Kidney transplantation in small children is technically challenging. Consideration of whether to use intraperitoneal versus extraperitoneal placement of the graft depends on patient size, clinical history, anatomy, and surgical preference. We report a large single-center experience of intraperitoneal kidney transplantation and their outcomes.We conducted a retrospective review of pediatric patients who underwent kidney transplantation from April 2011 to March 2018 at a single large volume center. We identified those with intraperitoneal placement and assessed their outcomes, including graft and patient survival, rejection episodes, and surgical or non-surgical complications.Forty-six of 168 pediatric kidney transplants (27%) were placed intraperitoneally in children mean age 5.5 ± 2.3 years (range 1.6-10 years) with median body weight 18.2 ± 5 kg (range 11.4-28.6 kg) during the study period. Two patients (4%) had vascular complications; 10 (22%) had urologic complications requiring intervention; all retained graft function. Thirteen patients (28%) had prolonged post-operative ileus. Eight (17%) patients had rejection episodes ≤6 months post-transplant. Only one case resulted in graft loss and was associated with recurrent focal segmental glomerular sclerosis (FSGS). Two patients (4%) had chronic rejection and subsequent graft loss by 5-year follow-up. At 7-year follow-up, graft survival was 93% and patient survival was 98%.The intraperitoneal approach offers access to the great vessels, which allows greater inflow and outflow and more abdominal capacity for an adult donor kidney, which is beneficial in very small patients. Risk of graft failure and surgical complications were not increased when compared to other published data on pediatric kidney transplants.

OBJECTIVES Paralleling improved outcomes in critically ill patients, survival for pediatric acute kidney injury has improved. Continuous renal replacement therapy is the preferred modality to optimize fluid and electrolyte management as well as nutritional support for children developing acute kidney injury in the PICU. However, some patients remain too fragile for transition to intermittent renal replacement therapies and require continuous renal replacement therapy for a prolonged period. Characteristics of this cohort and factors impacting outcomes are not well known. We aimed to describe the characteristics of pediatric patients requiring prolonged continuous renal replacement therapy and evaluate the factors impacting hospital survival. DESIGN Retrospective chart review. SETTING Tertiary PICU. PATIENTS Children requiring prolonged continuous renal replacement therapy. Prolonged continuous renal replacement therapy was defined as continuous renal replacement therapy dependence greater than or equal to 28 days. Primary outcome was hospital mortality. INTERVENTIONS None. MEASUREMENTS AND MAIN RESULTS From 2013 to 2016, 344 patients received continuous renal replacement therapy, 36 (10%) received continuous renal replacement therapy for greater than or equal to 28 days. Seventeen patients (47%) were female. Overall mortality was 44% (16/36); 69% (11/16) of nonsurvivors died of sepsis. Pediatric Logistic Organ Dysfunction score was significantly higher in nonsurvivors. Mortality rate was significantly higher in patients who were neutropenic at continuous renal replacement therapy start. Neutropenia (defined as absolute neutrophil count < 1,500/mm) at continuous renal replacement therapy start was the only independent predictor of mortality. One in four survivors did not recover renal function and remained dialysis dependent. CONCLUSIONS Prolonged continuous renal replacement therapy patients are at high risk of nonrecovery of renal function and require close monitoring. The majority of nonsurvivors in the study group died from sepsis. Neutropenia at continuous renal replacement therapy initiation was associated with increased risk of mortality. Progression of underlying disease process could explain the higher death rate in patients with neutropenia; however, inadequate treatment of infectious complications could be another explanation to explore further in future studies.

Introduction: Noninvasive hematocrit monitoring (NIVHM) during pediatric hemodialysis (pedHD) provides data in real time regarding changes in hematocrit and blood volume and also provides venous oxygen saturations. The latter has been proposed to indicate changes in tissue oxygen consumption. It is not known how well NIVHM oxygen saturations (O2sat) approximate blood gas measured oximetry saturation (mO2sat) in the course of pedHD. We aimed to assess the validity and reliability of NIVHM O2sat compared to mO2sat. Methods: This is a prospective study in 15 patients 90 days on hemodialysis (HD) without congenital heart disease. HD access was fistula (AVF) in 4 patients and tunneled catheters in the remainder. Pulse oximetry (spO2) was continuously monitored; mO2sat was measured via oximetry in a blood gas analyzer and NIVHM O2sat values collected at the start, middle, and end of HD treatment. Results: A total of 45 dyad measurements were obtained. NIVHM O2sat correlated well with mO2sat (R = 0.89, p < 0.0001); the same was seen at pre, mid, and post HD time points (R = 0.86–0.95, p < 0.001). NIVHM O2sat was lower than mO2sat; with catheter as access, the difference was 9.3 ± 8.6 (CI: 12.3–6.22, p < 0.0001) and with AVF was 2.1 ± 0.78 (CI: 2.6–1.7, p < 0.0001). Bland-Altman analysis demonstrated the difference but did not show any systematic bias. Continuous monitor of spO2 showed no hypoxia. Discussion/Conclusion: Intradialytic NIVHM O2sat correlates well with mO2sat but yield lower values. Future studies can include NIVHM O2sat changes as a surrogate for central venous O2 saturation changes and potentially yield useful information regarding tissue oxygen consumption in pedHD patients.

Background Cerebral and myocardial hypoperfusion occur during hemodialysis in adults. Pediatric patients receiving chronic hemodialysis have fewer cardiovascular risk factors, yet cardiovascular morbidity remains prominent. Methods We conducted a prospective observational study of pediatric patients receiving chronic hemodialysis to investigate whether intermittent hemodialysis is associated with adverse end organ effects in the heart or with cerebral oxygenation (regional O2 saturation [rSO2]). We assessed intradialytic cardiovascular function and rSO2, using noninvasive echocardiography to determine myocardial strain and continuous noninvasive nearinfrared spectroscopy for rSO2. We measured changes in blood volume and central venous oxygen saturation (mCVO2) were pre-, mid-, and post-hemodialysis. Results The study included 15 patients (median age, 12 years; median hemodialysis vintage 13.2 [9, 24] months were included. Patients were asymptomatic. The rSO2 did not change during hemodialysis, whereas mCVO2 decreased significantly, from 73% to 64.8 %. Global longitudinal strain of the myocardium worsened significantly by mid-hemodialysis and persisted post-hemodialysis. The ejection fraction remained normal. Lower systolic blood pressure and faster blood volume change were associated with worsening myocardial strain; only blood volume change was significant in multivariate analysis (β coefficient, -0.3; 95% confidence interval [95% CI], -0.38 to -0.21; P=0.0001). Blood volume change was also associated with a significant decrease in mCVO2 (β coefficient 0.42; 95% CI, 0.07 to 0.76; P=0.001). Access, age, hemodialysis vintage, and ultrafiltration volume were not associated with worsening strain. Conclusion s Unchanged rSO2 suggested that cerebral oxygenation was maintained during hemodialysis. However, despite maintained ejection fraction, intradialytic myocardial strain worsened in pediatric hemodialysis and was associated with blood volume change. The effect of hemodialysis on individual organ perfusion in pediatric versus adult patients receiving hemodialysis might differ.

Hydrothorax complicating continuous cycling peritoneal dialysis (CCPD) is an uncommon event. Its presentation may occur shortly after or years after initiation of dialysis. Surgical intervention offers the advantage of direct visualization and repair of the diaphragmatic defect. Video assisted thoracoscopy surgery (VATS) has been increasingly used in identifying these defects to facilitate this repair. We present 2 pediatric cases who underwent successful direct surgical repair of diaphragmatic defects using VATS with return to CCPD. Initial approach with VATS should be strongly considered in patients in whom a lifetime change in modality has significant repercussions.

BACKGROUND Management of chronic kidney disease mineral bone disorder (CKD-MBD) in pediatric patients with end-stage renal disease (ESRD) is challenging. While the use of calcimimetics is well-studied in adults on dialysis, few studies have been performed in pediatrics. Little is known about the use of cinacalcet in young children with ESRD. The aim of this study was to report the efficacy and safety of cinacalcet for treatment of secondary hyperparathyroidism in chronic dialysis patients younger than 5 years. MATERIALS AND METHODS We included children aged < 5 years on chronic dialysis, either hemodialysis (HD) or peritoneal dialysis (PD), who were prescribed cinacalcet for more than 1 month. Retrospective chart review was performed to gather demographics, dialysis prescription, relevant mineral imbalance laboratory parameters, and medications. Data was collected for 6 consecutive months. RESULT 18 patients (9 male), mean age at initiation of cinacalcet was 2.3 years; 13 PD and 5 HD. Average starting dose of cinacalcet: 6.2 mg daily, 0.55 mg/kg/day. Average time on dialysis was 14.4 months. Parathyroid hormone significantly decreased over the 1st month following initiation of cinacalcet from 929 (IQR 572 - 1,056) to 427 (IQR 256 - 778) pg/mL, p = 0.009. Three patients developed asymptomatic hypocalcemia (Ca < 9.4 mg/dL). Serum phosphorous decreased after initiation, and this was persistent at 6 months. Significant improvement in linear growth was observed while on cinacalcet and growth hormone (GH). CONCLUSION Cinacalcet can be effectively used in young children on dialysis with minimal side effects. Good linear growth was seen in patients on cinacalcet and GH therapy. Long-term large scale data is necessary to confirm. Institution-based management algorithm incorporating cinacalcet would be helpful to maintain uniformity in role of cinacalcet for management of CKD-MBD.

The autogenous arteriovenous fistula (AVF) is the preferred vascular access for pediatric and adult patients on chronic hemodialysis (HD). However, the prevalent rate of AVF use in children remains low in the United States. Duplex ultrasonography (DUS) of arm vessels is an essential step in the planning of AVF creation in adults, but its use in children is not described in much detail. In this retrospective study, we examined the role of preoperative DUS in children and adolescents who underwent AVF creation at a single large pediatric hospital, from 2008 to 2017. In addition, we sought to determine the potential risk factors for primary failure (PF), which was defined as the inability to use the AVF for at least a sustained 6-week period either due to thrombosis or any other reasons. We created 67 AVFs in 57 patients (34 male; 60%). The overall PF was 16% (11/67), and higher in cephalic AVF (10/40, 25%) compared to basilic (1/27, 3.7%), P = .04. The median maturation time was longer for basilic compared to cephalic AVF (105±47 vs. 88±38 days, respectively, P = .006). The size of the juxta-anastomotic vein and artery on preoperative DUS was not significantly different in cephalic versus basilic AVFs. However, the median diameter of the outflow vein was significantly smaller in the cephalic compared to basilic AVF (3.4 mm vs. 4.2 mm, P = .042). In addition, the outflow vein size was the only preoperative factor found to be significant as possible predictor for PF on univariable analysis. The results of this study show that the PF rate and maturation time for AVFs created in children and adolescents are comparable to adults, with the routine use of preoperative DUS. We postulate that vessel selection for AVF in the pediatric population should take into consideration the size of the vein at the outflow (and not just at the anastomosis).

Left ventricular hypertrophy (LVH) and arterial stiffness measured by pulse wave velocity (PWV) are independent predictors of cardiovascular (CV) mortality in adults receiving chronic dialysis. Hypertension strongly associates with elevated left ventricular mass index (LVMI) and PWV, with ambulatory blood pressure monitoring (ABPM), and central blood pressure (CBP) superior to office blood pressures (BP) in predicting CV morbidity. Few studies have described associations of office BP, ABPM, and CBP with LVMI and PWV in adolescent and young adult patients receiving hemodialysis (HD). Cross-sectional study of 22 adolescents and young adults receiving chronic HD. Pre- and post-dialysis office BP and CBP using applanation tonometry were obtained. Twenty-four-hour ABPM was obtained midweek post-dialysis. Pre- and post-dialysis carotid-brachial PWV were obtained same day as BP measurements. Annual echocardiograms for standard care were reviewed for LVH. Pre-dialysis CBP index correlated with LVMI (r = 0.3, p = 0.04) and PWV (r = 0.48, p = 0.02). Hypertensive patients identified by ABPM had worse LVMI; daytime ABPM systolic BP index correlated with LVMI (r = 0.5, p = 0.02). Office BP was not associated with LVMI; only office diastolic BP was associated with PWV (r = 0.46, p = 0.02). There was no correlation of LVMI or PWV with bone health parameters, anemia, interdialytic weight gain, or residual renal function. Ambulatory blood pressure monitoring is superior to casual office BP obtained at time of dialysis in delineating cardiovascular morbidity in adolescent and young adult HD patients. CBP is easily performed and correlates with LVMI and PWV in adolescent and young adult HD patients; however, large-scale normative data is needed.

In its first 3 years, the Standardizing Care to Improve Outcomes in Pediatric End Stage Renal Disease (SCOPE) Collaborative demonstrated a statistically significant increase in the likelihood of compliance with a standardized follow-up care bundle and a significant reduction in peritonitis. We sought to determine if compliance with care bundles and low peritonitis rates could be sustained in centers continuously participating for 84 months. Centers that participated from collaborative launch through the 84-month study period and provided pre-launch peritonitis rates were included. Children on maintenance peritoneal dialysis were eligible for enrollment. Changes in bundle compliance were assessed using a logistic regression model or a generalized linear mixed model (GLMM). Changes in average annualized peritonitis rates over time were modeled using GLMMs. Nineteen centers contributed 1055 patients with 1268 catheters and 17,247 follow-up encounters. The likelihood of follow-up compliance increased significantly over the study period (OR 1.05 95% confidence interval (CI) 1.03, 1.07; p < 0.001). Centers achieved ≥ 80% follow-up bundle compliance by 28 months and maintained a mean compliance of 84% between 28 and 84 months post-launch. Average monthly peritonitis rates decreased from 0.53 (95% CI 0.37, 0.70) infections per patient-year pre-launch to 0.30 (95% CI 0.23, 0.43) at 84 months post-launch, p < 0.001. Centers participating in the SCOPE Collaborative for 84 months achieved and maintained a high level of compliance with a standardized follow-up care bundle and demonstrated a significant and continued reduction in average monthly peritonitis rates.

Hemodialysis (HD) in infants poses unique challenges compared to extracorporeal dialysis in older children. Specific technical issues must be addressed including vascular access, HD equipment, and dialysis prescription. The main clinical problems of end-stage renal disease remain epidemic in this population, such as poor growth, anemia, hypertension, mineral bone disorder, and psychosocial burden; their management however is characterized by significant differences compared to older patients. Given their peculiarities, infants needing maintenance HD should always be treated by a skilled multidisciplinary team in very specialized pediatric dialysis units.

Studies regarding hemodialysis (HD) arteriovenous fistula (AVF) cannulation in adults indicate a higher risk of infection with the buttonhole (BH) technique compared to the rope-ladder (RL) technique. Pediatric data on this issue is sparse.We compared infection rates within the Standardizing Care to Improve Outcomes in Pediatric End stage kidney disease (SCOPE) centers performing BH cannulation versus RL cannulation of AVF. Generalized linear mixed modeling was used to assess differences in access-related blood stream infection (BSI) and access site infection (ASI) rates between the centers.Data was available from 211 AVF enrollments among 210 children. There were 61 AVF enrollments at 6 BH centers and 150 enrollments at 13 RL centers. Demographics were similar between the two groups. There were 12 total infections in 3383 patient months. BH centers had 3 infections (0 BSI, 3 ASI) and RL centers had 9 infections (5 BSI, 3 ASI). Mean [95% confidence interval] infection rates per 1000 patient months were not different between BH and RL centers (BH: 3.1 [0.6,15.6], RL: 3.2 [1.3,9.4], p = 0.947). A survey was also completed by the BH centers to describe their BH practices. The BH procedure at the majority of sites was characterized by a small patient/nurse ratio and strict antiseptic protocols.This data provides evidence of a low BSI rate associated with BH cannulation in pediatric HD patients. Further studies are needed to better delineate the differences in the pediatric and adult experience with the BH cannulation technique.

Serum creatinine is typically used to evaluate kidney function. Yet, it is a marker that can only provide estimations of kidney function because it can be influenced by other factors, such as dietary intake. The expanding field of infant formula selection in recent history has given many options for parents who are unable to provide breastmilk. Standard infant formulas and breastmilk generally fall within a select range of creatine content. With greater accessibility to internet-based medical advice (licensed or unlicensed), parents and families have more chances to be exposed to opportunistic websites and opinions that may provide harmful information. In this report, we describe the case of excessive dietary creatine intake in an infant who presented with elevated creatinine while otherwise appearing healthy and having normal cystatin C. After in-depth evaluation of nutritional intake, there was a suspicion for high creatine load of the infant’s homemade formula, which was composed of beef liver and various unregulated nutritional powders. Within 12 hours of stopping the infant’s homemade formula and providing intravenous fluids, the infant’s creatinine normalized. We highlight the importance of in-depth nutrition assessments and education on the health risks associated with improper formula selection.

Thrombocytopenia associated multi-organ failure (TAMOF) is a clinical syndrome with features of new onset thrombocytopenia, increased lactate dehydrogenase, and multi-organ failure in critically ill patients. TAMOF can be the initial presentation of an underlying disease process or can develop during the course of illness either during the hospital stay. TAMOF has a high mortality rate if not treated; therefore, early detection is critical. TAMOF has been rarely reported in diabetic ketoacidosis. We are describing the first case of a patient diagnosed with hyperglycemic, hyperosmolar non-ketotic syndrome who developed TAMOF on the third day of his hospital course. In addition to supportive care in the intensive care unit the patient received serial therapeutic plasma exchanges and improved quickly after treatment. Early diagnosis and treatment of TAMOF decreases morbidity and mortality.

Background Interventional catheterization procedures may be needed for patients with severe renal failure who are dependent on dialysis. To avoid the risk of fluid overload and electrolyte derangement during complex procedures in this oliguric/anuric patient population, we performed intra-procedural dialysis, either continuous renal replacement therapy (CRRT) or continous cycling peritoneal dialysis (CCPD). Methods We performed a retrospective review of a cohort of pediatric patients, ages 0–18 years, with dialysis-dependent renal failure who received CRRT or CCPD during catheterization procedures from January 2013 to March 2016. Results Eight patients underwent a total of nine interventional catheterization procedures while receiving intra-procedural dialysis. Median age was 4.5 years (range 8 months to 17 years) and weight, 11.6 kg (11.2–62.6 kg). Six patients had end-stage renal disease (ESRD) and two patients had acute kidney injury (AKI), one due to hepatorenal syndrome and one due to multifactorial causes associated with congenital heart disease. The most common reason for catheterization was occlusive venous thrombosis requiring recanalization. CRRT was used during five cases and CCPD during four cases. Median procedure time was 337 min (95–651 min) and median contrast dose 4.2 mL kg−1 (1.2–8.2 mL kg−1). Euvolemia was maintained based on pre- and post-catheterizations weights, and no significant electrolyte abnormalities occurred based on lab monitoring during and post-procedure. Conclusions Intra-procedural dialysis using CRRT or CCPD enables even small pediatric patients with severe renal failure to undergo long and complex interventional catheterizations by reducing the risk of fluid overload and electrolyte abnormalities. Collaboration between nephrology, cardiology, and dialysis teams is necessary for successful management of this challenging patient population.

Kidney transplantation is the treatment of choice in pediatric patients with end-stage renal disease. This population presents technical challenges particularly in those less than 20 kg due to anomalous anatomy, vascular access issues prior to transplantation, and a generally small size for age. Standard allograft outflow is usually achieved utilizing the iliac veins or IVC. When use of the iliocaval system is not feasible, alternative anastomosis must be considered. Herein, we report a case of a pediatric kidney transplantation where successful allograft outflow was achieved using the SMV when he was found to have an atretic IVC intraoperatively. In this setting, use of the portal system was required to achieve adequate allograft outflow. We created a donor iliac graft for added length to anastomose the renal vein with the SMV. In the setting of IVC occlusion with poor drainage, we utilized a patent vessel with larger caliber for outflow to reduce the risk of high venous pressures, allograft failure, venous rotation, and thrombosis. We conclude that the SMV may serve as an alternative outflow tract in the small pediatric patient and provides the vessel caliber needed to reduce the risks of complications.

The Standardizing Care to Improve Outcomes in Pediatric End Stage Renal Disease (SCOPE) collaborative seeks to reduce hemodialysis (HD) catheter-associated blood stream infections (CA-BSI) by increasing implementation of standardized HD catheter care bundles. We report HD catheter care practices and HD CA-BSI rates from SCOPE. Catheter care practices and infection events were collected prospectively during the study period, from collaborative implementation in June 2013 through May 2017. For comparative purposes, historical data, including patient demographics and HD CA-BSI events, were collected from the 12 months prior to implementation. Catheter care bundle compliance in 5 care bundle categories was monitored across the post-implementation reporting period at each center via monthly care observation forms. CA-BSI rates were calculated monthly, and reported as number of infections per 100 patient months. Changes in CA-BSI rates were assessed using generalized linear mixed model (GLMM) techniques. Three hundred twenty-five patients with tunneled HD catheters [median (IQR) age 12 years (6, 16), M 53%, F 47%] at 15 centers were included. A total of 3996 catheter care observations over 4170 patient months were submitted with a median (IQR) 5 (2, 14) observations per patient. Overall bundle compliance was high at 87.6%, with a significant and progressive increase (p

The purpose of this study was to review surgical outcomes after elective placement of peritoneal dialysis (PD) catheters in children with end-stage renal disease.Children with PD catheters placed between February 2002 and July 2014 were retrospectively reviewed. Outcomes were catheter life, late (30days post-op) complications (catheter malfunction, catheter malposition, infection), and re-operation rates. Comparison groups included laparoscopic versus open placement, age2, and weight10kg. Univariate and multivariate analysis were performed.One hundred sixteen patients had 173 catheters placed (122 open, 51 laparoscopic) with an average patient age of 9.7±6.3years. Mean catheter life was similar in the laparoscopic and open groups (581±539days versus 574±487days, p=0.938). The late complication rate was higher for open procedures (57% versus 37%, p=0.013). Children age2 or weight10kg had higher re-operation rates (64% versus 42%, p=0.014 and 73% versus 40%, p=0.001, respectively). Adjusted for age and weight, open technique remained a risk factor for late complications (OR 2.44, 95% CI 1.20-4.95) but not re-operation.Laparoscopic placement appears to reduce the rate of late complications in children who require PD dialysis catheters. Children2years age or10kg remain at risk for complications regardless of technique.

End-stage renal disease (ESRD) is a state of acquired immune deficiency. Children with ESRD have a shortened life expectancy without transplantation. Deaths due to infection are only second in frequency to cardiovascular causes in both children and adults with ESRD. Although loss of skin barrier due to the presence of indwelling catheters seems to be the most obvious explanation, other factors such as malnutrition, dysregulated inflammation, and acquired immune dysfunction due to uremia contribute to the immune deficiency of ESRD. Infectious complications also constitute a large morbidity, leading to frequent hospitalization and increased resource utilization in the ESRD population. This chapter reviews factors predisposing to infections in children with ESRD and examines the burden of infectious complications in two common dialysis modalities, hemodialysis and peritoneal dialysis. Definitions of dialysis-related infections are presented as well as their epidemiology, microbiology, and management including indications for catheter removal versus medical treatment with catheter in situ. Renal transplantation, conventionally considered hand in hand with ESRD, is outside the scope of this chapter and will not be discussed.

Background End-stage renal disease (ESRD) although rare among infants presents many management challenges. We sought to evaluate factors associated with PD catheter failure among infants initiated on chronic PD. Methods A retrospective chart review of all children under two years of age who had PD catheters placed for initiation of chronic PD from 2002 to 2015. Data was extracted for catheter related events occurring within 12 months of catheter placement. Cox and Poisson regression models were used to delineate factors associated catheter complications. Results Twenty-five infants with median age 18 days had PD catheters placed for chronic dialysis. Common complications included leakage around the exit site (31%), blockage (26%), migration or malposition (23%), catheter-related infections (18%), and other complications (2%). Predictors of initial PD catheter failure were age less than one month at catheter placement (hazard ratio (HR) 7.77, 95% CI, 1.70–35.39, p = 0.008), use of catheter within three days of placement (HR 5.67, 95% CI, 1.39–23.10, p = 0.015) and presence of a hernia (HR 8.64, 95% CI, 1.19–62.36, p = 0.033). In an adjusted Poisson regression model, PD catheter use within three days of placement was the only predictor of any catheter complication over the12 months of follow up. Conclusions Use of PD catheters within three days of placement was associated with catheter failure. We recommend that when possible, catheters should be allowed to heal for at least three days prior to use to reduce risk of complications and improve catheter survival.

Treatment for hyperphosphatemia in chronic kidney disease (CKD) involves dietary control of phosphorus intake, dialysis, and treatment with oral phosphate binders, none of which were approved by the Federal Food and Drug Administration in pediatric patients at the time of this study. This was a phase 2, multicenter study (NCT01574326) with a 2-week, randomized, placebo-controlled, fixed-dose period (FDP) followed by a 6-month, single-arm, open-label, dose-titration period (DTP), with the aim to evaluate the safety and efficacy of sevelamer carbonate (SC) in hyperphosphatemic pediatric patients with CKD. Following a 2–4 week screening phase, pediatric patients with a serum phosphorus level higher than age-appropriate levels were randomized to receive either SC or placebo as powder/tablets in 0.4–1.6 g doses, based on body surface area. The primary efficacy outcome was the change in serum phosphorus from baseline to end of the FDP in the SC versus placebo arms (analysis of covariance). The secondary outcome was mean change in serum phosphorus from baseline to end of DTP by treatment group and overall. Treatment-emergent/serious adverse events (AEs) were recorded. Of 101 enrolled patients (29 centers), 66 completed the study. The majority of patients were adolescents (74%; mean age 14.1 years) and on dialysis (77%). Renal transplant was the main reason for discontinuation. SC significantly reduced serum phosphorus from baseline levels (7.16 mg/dL) during the FDP compared to placebo (least square mean difference − 0.90 mg/dL, p = 0.001) and during the DTP (− 1.18 mg/dL, p

The Standardizing Care to Improve Outcomes in Pediatric End Stage Renal Disease (SCOPE) Collaborative is a quality improvement initiative to reduce dialysis-associated infections. The frequency of peritoneal dialysis (PD) catheter exit site infection (ESI) and variables influencing its development and end result are unclear. We sought to determine ESI rates, to elucidate the epidemiology, risk factors, and outcomes for ESI, and to assess for association between provider compliance with care bundles and ESI risk.We reviewed demographic, dialysis and ESI data, and care bundle adherence and outcomes for SCOPE enrollees from October 2011 to September 2014. ESI involved only the exit site, only the subcutaneous catheter tunnel, or both.A total of 857 catheter insertions occurred in 734 children over 10,110 cumulative months of PD provided to these children. During this period 207 ESIs arose in 124 children or 0.25 ESIs per dialysis year. Median time to ESI was 392 days, with 69% of ESIs involving exit site only, 23% involving the tunnel only, and 8% involving both sites. Peritonitis developed in 6%. ESI incidence was associated with age (p = 0.003), being the lowest in children aged2 years and highest in those aged 6-12 years, and with no documented review of site care or an exit site score of 0 at prior month's visit (p 0.001). Gender, race, end stage renal disease etiology, exit site orientation, catheter cuff number or mobilization, and presence of G-tube, stoma, or vesicostomy were unassociated with ESI incidence. Of the ESIs reported, 71% resolved with treatment, 24% required hospitalization, and 9% required catheter removal, generally secondary to tunnel infection.Exit site infections occur at an annualized rate of 0.25, typically well into the dialysis course. Younger patient age and documented review of site care are associated with lower ESI rates. Although most ESIs resolve, hospitalization is frequent, and tunnel involvement/catheter loss complicate outcomes.

The care of children with end-stage renal disease (ESRD) is highly specialized and often poorly understood by nonpediatric providers and facility/institution administrators. As such, this position paper has been created to offer provider, facility, and institutional guidance regarding the components of care necessary for children receiving dialysis. Key differences between adult and pediatric dialysis units are highlighted. Responsibilities and expectations of the members of the interdisciplinary dialysis team are outlined as they pertain specifically to the care of pediatric dialysis patients. Physical and staffing requirements of the dialysis facility are reviewed, again focusing on unique needs and challenges faced by the pediatric dialysis care team. Among these, vascular access options and proper planning of ESRD care are underscored. Pediatric quality-of-life metrics differ significantly from adult quality variables, and proper tools for assessment must be used. Endorsed by the Council of the American Society of Pediatric Nephrology (ASPN), this position paper serves as a reference tool for the provision of care to pediatric patients with ESRD.

Evaluation of hypertension in the pediatric age group should be guided by the age at presentation, the severity of hypertension at presentation, and whether the hypertension is sustained or transient. The common causes of hypertension differ by age group (Table 1). Hypertension is more likely to be secondary and sustained in the younger age groups, whereas primary (essential) hypertension and white coat hypertension are more likely to be the etiology in adolescents [1–5]. The global epidemic of childhood obesity has been associated with an increased incidence of primary hypertension and prehypertension in adolescents worldwide [3–7]. Obese children have as much as a threefold higher risk for developing hypertension compared to nonobese children [7]. The frequent occurrence of primary hypertension in obese adolescents dictates an entirely different evaluation than for younger or nonobese children. Renal disease must be considered in every child with hypertension, because of the prevalence of renovascular and renal parenchymal disorders as the etiology in any age group. Secondary hypertension from renal disorders is more likely to be severe at presentation than primary hypertension. Endocrine causes of hypertension are rare, so special diagnostic studies for these disorders should be reserved for those patients whose history, physical examination, and preliminary evaluation warrant further specific investigation. This chapter outlines the steps in evaluation of childhood hypertension, starting with the confirmation of the diagnosis and evaluation of its duration and severity.

Infants with hyperammonemia can present with nonspecific findings so ordering an ammonia level requires a high index of suspicion. Renal replacement therapy (RRT) should be considered for ammonia concentrations of >400 μmol/L since medical therapy will not rapidly clear ammonia. However, the optimal RRT prescription for neonatal hyperammonemia remains unknown. Hemodialysis and continuous renal replacement therapy (CRRT) are both effective, with differing risks and benefits. We present the cases of two neonates with hyperammonemia who were later diagnosed with ornithine transcarbamylase deficiency and received high-dose CRRT. Using dialysis/replacement flow rates of 8,000 mL/h/1.73 m2 (1,000 mL/h or fourfold higher than the typical rate used for acute kidney injury) the ammonia decreased to

Immunoglobulin (Ig) M nephropathy is defined by electron-dense mesangial deposits and mesangial IgM visible by immunofluorescence (IF) without other histopathologic and immunofluorescent microscopic abnormalities. Certain patients have only immuno-positive (IgM+) IF. Children presenting with steroid-dependent or steroid-resistant nephrotic syndrome have a high prevalence of IgM+ IF with or without electron-dense deposits. We reviewed the clinical course of children with steroid-dependent or steroid-resistant nephrotic syndrome who underwent renal biopsy at Texas Children‘s Hospital from 1989 to 2006 to further characterize IgM+ IF in children with nephrotic syndrome. Of the 55 children with steroid-resistant or -dependent minimal change disease (MCD), 23 had IgM+ IF. Of these 23 children, 61% had microscopic hematuria at presentation, 48% (11/23) were steroid-dependent, and 48% (11/23) steroid-resistant (one underwent biopsy prior to steroid therapy). We compared the efficacy of adjuvant treatment with cyclophosphamide and cyclosporine: 18% initially treated with cyclophosphamide obtained remission, while 55% had no response; 83% obtained subsequent remission with cyclosporine. Of those initially treated with cyclosporine, 88% obtained complete or partial remission. IgM+ IF may be surrogate marker for the severity of MCD. Based on our results, children with MCD and IgM+ IF have a better response to cyclosporine than cyclophosphamide.

Hypertension is an important manifestation of systemic lupus erythematosus (SLE) but reports of prevalence vary between 20-70% in published reports of adult and pediatric patients. For both children and adults with SLE, the clinical diagnosis and management of hypertension has traditionally been based on guidelines developed for the general population. In clinical trials, the criteria used for defining participants with hypertension are mostly undefined. As a first step towards formally assessing the blood pressure (BP) patterns of children diagnosed with SLE, 24-hr ambulatory BP monitoring data was analyzed on clinic patients who presented with prehypertension or stage I hypertension. In this pediatric SLE cohort (n=10), 20% met daytime criteria for a diagnosis of hypertension. Patterns of BP elevation varied widely with white coat, masked, isolated systolic, and diastolic nocturnal hypertension all identified. Nocturnal hypertension was detected in 60% and attenuated nocturnal BP dipping in 90% of both hypertensive and normotensive SLE patients. In SLE patients, the median nighttime systolic and diastolic loads were 25% and 15.5% compared with median daily loads of 12.5% and 11.5%. Daytime and nighttime systolic and diastolic BP load and nocturnal dipping was compared to a control population consisting of 85 non-SLE patients under 21 years old with prehypertension or stage 1 hypertension presenting to hypertension clinic. Median systolic BP dipped 5.3 mmHg in SLE patients compared to 11.9 mmHg in non-lupus (p-value = 0.001). Median diastolic BP dipped 12.9 mmHg versus 18.5 mmHg in non-lupus (p-value = 0.003). Patterns of BP dysregulation in pediatric SLE merit further exploration. Children with or without SLE displaying prehypertensive or stage 1 casual BP measurements had similar rates of hypertension by ambulatory BP monitoring. However, regardless of BP diagnosis, and independent of kidney involvement, there was an increased proportion with attenuated nocturnal dipping and nocturnal hypertension in SLE patients.