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Background: Residents are at the forefront of student education in the hospital, yet valid tools to assess their performance as teachers are lacking. Aims: To develop a valid evaluation tool for assessing resident performance as educators for clerkship students. Method: A mixed-methods design was used. Focus groups of residents and medical students explored desired behaviors in resident educators. Using grounded theory, a list of behaviors was generated inductively through iterative review and categorized into themes. After thematic saturation, behaviors were rated on a Likert scale by stakeholders based on 'importance' and 'accuracy of measurement.' Items which were both important and accurate were used in the final tool. Results: Eighty-five desirable behaviors for resident educators were identified and consolidated into a 14-item tool. Twenty met both 'importance' and 'accuracy' criteria and fell under themes of respect, safe environment, balancing supervision with autonomy, relevant teaching and feedback. Nineteen 'important' behaviors deemed not accurately measurable fell under themes of professionalism, communication, management skills and leadership. Conclusions: Evaluation of residents as teachers and development of resident-as-teacher curricula should emphasize aforementioned areas. Professionalism and organizational skills may not be measurable reliably by learners. Complementary tools to assess these aspects of resident performance are necessary. [ABSTRACT FROM AUTHOR]

Background: Non-calcium-containing phosphate binders, such as sevelamer preparations, are being increasingly used in patients on dialysis due to their lower association with hypercalcemia and cardiovascular morbidity and mortality. While minor gastrointestinal side effects are quite common with the use of sevelamer, more serious gastrointestinal toxicities have only rarely been reported. Case-diagnosis/treatment: We report a pediatric patient on maintenance dialysis receiving sevelamer hydrochloride who developed severe abdominal pain and a high-grade stricture of the sigmoid colon. The patient underwent exploratory laparotomy, resulting in a partial colectomy and colostomy. Histopathologic examination showed colonic mucosal injury and characteristic 'fish-scale'-like sevelamer hydrochloride crystals within the mucosa. Conclusions: Whether the sevelamer crystals were causal, contributory or purely incidental remains to be clearly elucidated. However, our case raises sufficient concern to warrant additional investigation into whether there is a causal relationship between sevelamer use and intestinal mucosal injury. [ABSTRACT FROM AUTHOR]

Children with steroid-resistant nephrotic syndrome (SRNS) are at risk of developing renal failure. We report here the results of a single-center retrospective observational study of the remission rate in pediatric patients with SNRS receiving tacrolimus. Serial renal biopsies from children on tacrolimus therapy were evaluated for tubulointerstitial fibrosis and transforming growth factor-β immunostaining. Of the 16 children with SRNS, 15 went into complete remission after a median of 120 days of therapy. Nine children were able to stop steroids, while the others were on tapering doses. Forty-seven percent had relapses, most of which were steroid-responsive. Serial renal biopsies were obtained from seven children after a median treatment duration of 24 months; two of these children had increased tubulointerstitial fibrosis and four showed increased transforming growth factor-β tissue staining. Children with worsening histological findings were younger. There was no significant association between tacrolimus exposure and biopsy changes, although the average trough level was higher in those children with worsening histological findings. In conclusion, tacrolimus may be a safe and effective alternative agent for inducing remission in children with SRNS. However, caution needs to be taken when prescribing this agent due to its narrow therapeutic index. Serial renal biopsies are necessary to check for subclinical nephrotoxicity, especially in younger children and those with higher trough levels. [ABSTRACT FROM AUTHOR]

Truong U, Moon-Grady AJ, Butani L. Cardiac tamponade in a pediatric renal transplant recipient on sirolimus therapy. Pediatr Transplantation 2005: 9: 541–544. © 2005 Blackwell Munksgaard Because of its lack of nephrotoxicity, the use of sirolimus, as an immunosuppressive agent, has increased considerably in solid-organ transplant (Tx) recipients. With its increased use, Tx professionals are encountering a variety of previously unreported side-effects such as angioedema and interstitial pneumonitis. We describe here the case of a pediatric renal Tx recipient who, while receiving sirolimus, developed a large pericardial effusion requiring pericardiocentesis. An extensive workup for an infectious etiology was performed; the only positive result was isolation of adenovirus type 2 from the patient's stool specimen. Following sirolimus dose reduction this child's effusion stabilized and has not recurred. The purpose of this report is to advise health-care professionals caring for Tx recipients about this potentially life-threatening complication associated with sirolimus. The role of adenovirus, if any, in contributing to the development of our patient's pericardial effusion is discussed herein. [ABSTRACT FROM AUTHOR]

There is a growing recognition that children with Lowe syndrome are at risk of nephrocalcinosis and nephrolithiasis from hypercalciuria. Increased fluid intake and correction of metabolic acidosis have remained the focus for intervention but are not always successful. Thiazide diuretics, which reduce urinary calcium excretion, have not been used in these children, due to concerns that (i) they may not work as a result of the underlying tubular abnormalities and (ii) their risk may outweigh the potential benefits they have to offer. Herein we report a child with Lowe syndrome whowas successfully treated with thiazides in managing his hypercalciuria. [ABSTRACT FROM AUTHOR]

The original version of this article unfortunately contained a mistake. The name of Vimal Chadha was presented incorrectly. The corrected author list is given above. [ABSTRACT FROM AUTHOR]

Our objective was to examine serum ferritin trends after conversion to permanent vascular access (PVA) among children who started hemodialysis (HD) using tunneled cuffed catheters (TCC). Retrospective chart reviews were completed on 98 subjects from 20 pediatric HD centers. Serum ferritin levels were collected at the creation of PVA and for two years thereafter. There were 11 (11%) arteriovenous grafts (AVG) and 87 (89%) arteriovenous fistulae (AVF). Their mean TCC use was 10.4 ± 17.3 months. Serum ferritin at PVA creation was elevated at 562.64 ± 492.34 ng/mL, increased to 753.84 ± 561.54 ng/mL (p = < 0.001) in the first year and remained at 759.60 ± 528.11 ng/mL in the second year (p = 0.004). The serum ferritin levels did not show a statistically significant linear association with respective serum hematocrit values. In a multiple linear regression model, there were three predictors of serum ferritin during the first year of follow-up: steroid-resistant nephrotic syndrome as primary etiology (p = 0.035), being from a center that enrolled >10 cases (p = 0.049) and baseline serum ferritin level (p = 0.017). Increasing serum ferritin after conversion to PVA is concerning. This increase is not associated with serum hematocrit trends. Future studies should investigate the correlation of serum transferrin saturation and ferritin levels in pediatric HD patients. [ABSTRACT FROM AUTHOR]

Hypersensitivity to para-phenylenediamine (PPD) and related compounds induced by temporary black henna tattoos has become a serious health problem worldwide. Different patterns of sensitization with various clinical aspects are described in literature due to PPD associated to henna tattoo and these manifestations are likely correlated with the immunological and dermatological pathomechanisms involved. Henna is the Persian name of the plant Lawsonia inermis, Fam. Lythraceae. It is a woody shrub that grow in regions of North Africa, South Asia, India and Sri Lanka. Nowadays it is rather frequent to see temporary "tattoos" performed with henna. To make tattoos darker and long-lasting PPD has been associated to henna in tattoo drawings mixtures, so obtaining "blackhenna" In these years there has been a rise of contact sensitization to PPD and in medical literature an increased number of cases have been reported on temporary henna tattoo application. Here we review the various clinical patterns related to PPD and henna tattoo, to investigate the possible link between clinic-morphological pictures and the immunological response to PPD and henna. The literature underlines that different clinical manifestations are related to black henna containing PPD, and its derivative products may cause delayed-type as well as immediate-type reactions. Further studies are needed to investigate the relationship between clinical and morphological aspects of PPD contact dermatitis and the T cell subsets predominance. [ABSTRACT FROM AUTHOR]

Presents a letter to the editor on life-threatening hyperphosphatemia and hypocalcemia from inappropriate use of fleet enemas.

Presents a letter to the editor on hemolytic uremic syndrome.

Diabetic kidney disease (DKD), previously encountered predominantly in adult patients, is rapidly gaining center stage as a childhood morbidity and one that pediatric nephrologists are likely to encounter with increasing frequency. This is in large part due to the obesity epidemic and the consequent rise in type 2 diabetes in children and adolescents, as well as the more aggressive diabetes phenotype in today's youth with more rapid β-cell decline and faster development and progression of diabetes-related complications along with lower responsiveness to the treatments used in adults. DKD, an end-organ complication of diabetes, is at the very least a marker of, and more likely a predisposing factor for, the development of adverse cardiovascular outcomes and premature mortality in children with diabetes. On an optimistic note, several new therapeutic approaches are now available for the management of diabetes in adults, such as GLP1 receptor agonists, SGLT2 inhibitors, and DPP4 inhibitors, that have also been shown to have a favorable impact on cardiorenal outcomes. Also promising is the success of very low-energy diets in inducing remission of diabetes in adults. However, the addition of these pharmacological and dietary approaches to the management toolbox of diabetes and DKD in children and adolescents awaits thorough assessment of their safety and efficacy in this population. This review outlines the scope of diabetes and DKD, and new developments that may favorably impact the management of children and young adults with diabetes and DKD. [ABSTRACT FROM AUTHOR]

Background: Hemodialysis (HD) guidelines recommend permanent vascular access (PVA) in children unlikely to receive kidney transplant within 1 year of starting HD. We aimed to determine predictors of primary and secondary patency of PVA in pediatric HD patients.Methods: Retrospective chart reviews were performed for first PVAs in 20 participating centers. Variables collected included patient demographics, complications, interventions, and final outcome.Results: There were 103 arterio-venous fistulae (AVF) and 14 AV grafts (AVG). AVF demonstrated superior primary (p = 0.0391) and secondary patency (p = 0.0227) compared to AVG. Primary failure occurred in 16 PVA (13.6%) and secondary failure in 14 PVA (12.2%). AVF were more likely to have primary failure (odds ratio (OR) = 2.10) and AVG had more secondary failure (OR = 3.33). No demographic, clinical, or laboratory variable predicted primary failure of PVA. Anatomical location of PVA was predictive of secondary failure, with radial having the lowest risk compared to brachial (OR = 12.425) or femoral PVA (OR = 118.618). Intervention-free survival was predictive of secondary patency for all PVA (p = 0.0252) and directly correlated with overall survival of AVF (p = 0.0197) but not AVG. Study center demonstrated statistically significant effect only on intervention-free AVF survival (p = 0.0082), but not number of complications or interventions, or outcomes.Conclusions: In this multi-center pediatric HD cohort, AVF demonstrated primary and secondary patency advantages over AVG. Radial PVA was least likely to develop secondary failure. Intervention-free survival was the only predictor of secondary patency for AVF and directly correlated with overall access survival. The study center effect on intervention-free survival of AVF deserves further investigation. [ABSTRACT FROM AUTHOR]

Objective: To determine whether pre-transplant body mass index (BMI) affects renal allograft function and survival in pediatric renal transplant recipients.Study design: This is a retrospective cohort study using the Organ Procurement and Transplantation Network data from 2000 to 2013 to compare time to total allograft loss (allograft failure or death), prevalence of delayed graft function, prevalence of acute rejection, and estimated glomerular filtration rate (eGFR) post-transplant in pediatric renal transplant recipients categorized by BMI z-score.Results: A total of 8804 kidney transplant recipients met our inclusion criteria, and of those, 6% were underweight, 14% were overweight, and 17% were obese pre-transplant. The adjusted hazard ratio (HR) for allograft failure was significantly higher for obese recipients compared to normal weight recipients (HR 1.25, 95% CI 1.1, 1.42); for every 1 point increase in BMI z-score, there was a 7% increased hazard of allograft failure (HR 1.07; 95% CI 1.03-1.1, p < 0.001). The prevalence of delayed graft function and acute rejection increased with higher BMI z-score category; however, this difference did not reach statistical significance. eGFR at 1 and 5 years post-transplant decreased with higher BMI z-score although it was only statistically significant at 1 year.Conclusions: Obesity is prevalent in pediatric renal transplant recipients, and obese, but not overweight or underweight, pediatric renal transplant recipients have an increased risk of allograft failure. Implementation of effective obesity interventions in pediatric renal transplant recipients is of critical importance to improve longevity of the renal allograft. [ABSTRACT FROM AUTHOR]

There is a need to reconsider the acceptance of organs from donors considered suboptimal, in the absence of data. Toxoplasma antibody-positive donors (TPD) constitute one such group. The objective of our study was to compare graft survival in deceased donor renal transplant (Tx) recipients, stratified by Toxoplasma IgG status, using the Organ Procurement and Transplantation Network (OPTN) database. A log-linear event history regression model for graft failure categorized by Toxoplasma IgG status, adjusting for confounders was applied to first kidney-only Tx recipients from 2018 to 2022. Of the 51,422 Tx, 4,317 (8.4%) were from TPD. Acute rejection and graft failure (5% each) were similar between groups. Crude graft failure was 7.3 failures per 100 person-years for TPD recipients compared to 6.5 failures per 100 person-years for the Toxoplasma-negative group ( p 0.008). The crude failure rate ratio was 1.14 with an adjusted hazard rate ratio of 1.04 (95% CI: 0.94, 1.15, p 0.39). In renal Tx recipients, TPD graft recipients have comparable survival to Tx from Toxoplasma-negative recipients. While caution and close monitoring of recipients post-Tx for surveillance of disseminated toxoplasmosis are still warranted, our study suggests that patients can be successfully managed using TPD organs., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Butani and Tancredi.)

Nephrocalcinosis occurs in as many as 40% of preterm neonates. Many causes and contributors predispose neonates to develop nephrocalcinosis, including metabolic, genetic, and iatrogenic factors. Because nephrocalcinosis can be a manifestation of an underlying genetic disorder, neonates with nephrocalcinosis must undergo an evaluation to identify and address contributors, to prevent further renal calcium deposition that can potentially lead to renal dysfunction. In this article, we review the epidemiology, pathogenesis, diagnosis, and evaluation of nephrocalcinosis in neonates. We also summarize the natural history of nephrocalcinosis of prematurity as well as the management of this condition., (Copyright © 2024 by the American Academy of Pediatrics.)

About 10% of all home peritoneal dialysis regimens in children with chronic kidney disease stage 5 are reported to involve some form of a tidal peritoneal dialysis (TPD) prescription. Despite this, there remain several gaps in how pediatric nephrologists approach the use of TPD. This stems from a combination of factors such as the confusing technical terminology pertaining to TPD, seemingly conflicting data on the risks, benefits, and indications for TPD, and lastly, limited published guidelines on the practical aspects of how to write a TPD prescription, based on the indication, in children. Our educational review, using evidence-based data, attempts to bridge this gap and provide an easy-to-use guide on the key practical aspects of TPD in children., (© 2023. The Author(s).)

Infections remain the most common cause of hospitalization after kidney transplantation, contributing to significant post-transplant morbidity and mortality. There is a growing body of literature that suggests that immunoglobulins may have a significant protective role against post-transplant infections, although the literature remains sparse, inconsistent, and not well publicized among pediatric nephrologists. Of great concern are data indicating a high prevalence of immunoglobulin abnormalities following transplantation and a possible link between these abnormalities and poorer outcomes. Our educational review focuses on the epidemiology and risk factors for the development of immunoglobulin abnormalities after kidney transplantation, the outcomes in patients with low immunoglobulin levels, and studies evaluating possible interventions to correct these immunoglobulin abnormalities., (© 2022. The Author(s).)

Introduction: Academic Health Centers (AHCs) have complex, often competing missions. Many have developed mission-based management (MBM) systems to support their clinical and non-clinical missions. There are limited data on MBM use for their educational missions. Our scoping review explored how AHCs employed such systems.  Materials and methods: Arksey and O'Malley's six-stage framework guided our review. Based on pre-defined criteria, English language articles from PubMed, EMBASE, SCOPUS, and the Healthcare Administration Database published between 2010 and 2020 were loaded into a reference manager. The search included all health professions education schools. Articles were excluded if they were review articles, commentaries, or clearly did not involve funding for education. From the final list of selected articles, data were extracted using a data extraction sheet we developed. Two researchers reviewed each article again to ensure extracted data were reported consistently and with sufficient detail.  Results: Of the 1729 manuscripts identified, 35 met inclusion criteria. Sixteen (46%) contained data in some form but did not have a formal methods section describing the specific approach to data collection and analysis. Moreover, there was marked variability in how educational effort was quantified, what counted as educational effort (educational scholarship versus teaching) and the impacts of such quantification (departmental funding versus individual faculty incentives). None of the studies reported on the impact on faculty promotion. Faculty satisfaction with the system was reported in seven studies (20%) and was generally positive., Conclusions: A systematic description of how systems were developed to support the educational mission was lacking. Clear goals, methods of development, uniform data on educational productivity and quality, and program evaluation were not defined by most articles. This lack of process clarity presents a challenge, but more importantly an opportunity for academic health centers to unify efforts and continue to further their educational mission., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Beck Dallaghan et al.)

Background: There are conflicting data on long-term outcomes of pediatric LURD renal Txs compared to Txs of kidneys from other donor sources., Methods: An analysis of the OPTN database was conducted in children (<18 years) who had received their 1st kidney-only Tx between January 1, 2000, and September 30, 2021. The primary outcome measure was time to graft failure or death. Cox event history regression model for time to primary outcome, categorized by donor source and adjusting for confounders was performed., Results: Of the 12 089 subjects, 327 (2.7%) received kidneys from LURDs, 4349 (36%) from LRDs and 7413 (61%) from DD. One year graft failure rate was 3.56%. On regression analyses, compared to LRD kidney recipients, LURD recipients had comparable graft survival (graft failure AHR 1.15, 95th percentile confidence interval 0.87-1.51; p .31) and DD recipients had lower graft survival (graft failure hazard ratio 1.26, 95th percentile confidence interval 1.10-1.43; p < .001). When using living unrelated kidney recipients as the reference group, DD kidney recipients had comparable graft survival, with a wide confidence interval (hazard ratio for graft failure 1.09; 0.83-1.43, p .53)., Conclusions: Pediatric LURD kidney recipients have comparable graft survival to LRD kidney recipients; DD kidney recipients had the poorest survival. Our study, the largest to date, should encourage centers to embrace non-commercial living-unrelated transplantation as a viable option for children, preferable to DD kidneys., (© 2022 Wiley Periodicals LLC.)

Background: Unlike in adults, there are limited pediatric data exploring the association between acute respiratory illnesses and blood pressure abnormalities. The aim of our study was to explore the association of bronchiolitis, a common respiratory illness, with elevated blood pressure in hospitalized children., Methods: In this single center retrospective case-control study, we evaluated the association between bronchiolitis and elevated blood pressure and hypertension in hospitalized children, compared to a control group admitted with nonrespiratory conditions, using multivariate regression analyses. Standard published normative data on pediatric blood pressure were used to classify children in various blood pressure categories., Results: A high prevalence of elevated blood pressure (16%) and hypertension (60%) was noted among children with bronchiolitis; this was not statistically different from the control group (18% for elevated blood pressure; 57% for hypertension; P-values, 0.71 and 0.53, respectively). On multivariate regression analyses, only length of stay was associated with hypertension. No patient with blood pressure abnormalities received antihypertensives nor were any nephrology consults documented., Conclusions: A high prevalence of blood pressure abnormalities, without documentation of their recognition, was noted in hospitalized children regardless of diagnosis, pointing to the need for more data on outcomes-driven significance of pediatric inpatient blood pressure measurements., (© 2022. The Author(s).)

Common causes of pediatric ESRD are distinct from those seen in the adult population. In the pediatric population, the most common are congenital anomalies of the kidney and urinary tract (CAKUT), affecting approximately 30% of children with CKD. These structural anomalies often require coordinated care with the pediatric urology team to address voiding issues, bladder involvement, and the potential need for surgical intervention. For pediatric nephrologists and urologists, common CAKUT that are encountered include antenatal hydronephrosis, obstructive uropathies (eg, posterior urethral valves), and vesicoureteral reflux. As more pediatric patients with CAKUT, CKD, and ESRD transition to adult care, it is important for receiving adult nephrologists to understand the clinical presentation, natural history, and prognosis for these diagnoses. This review outlines the diagnosis and potential interventions for these conditions, including strategies to address bladder dysfunction that is often seen in children with CAKUT. A discussion of these management decisions (including surgical intervention) for CAKUT, which are quite common to pediatric nephrology and urology practices, may provide unique learning opportunities for adult nephrologists who lack familiarity with these pediatric conditions., (Copyright © 2022 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Engagement of academic faculty in research remains low. While barriers to research have been explored, there are no data on how national organizations can help overcome these barriers. Our study explored faculty satisfaction and motivational drivers for engagement with research opportunities offered by the Council on Medical Student Education in Pediatrics (COMSEP), an organization of pediatric medical educators, and characterize strategies perceived by faculty to promote the use of these opportunities., Methods: In 2021, 5 survey questions were administered to faculty members of COMSEP to explore satisfaction with COMSEP's research offerings, the perceived value of educational research, and the facilitators, barriers and potential opportunities for COMSEP to promote research. Clark's Commitment and Necessary Effort model on motivation served as the theoretical framework for our study, which explores motivation, self-efficacy and contextual factors influencing an individual's pursuit of goals. Chi-square analysis and Wilcoxon Signed Ranks Test were used to compare categorical and scaled variables among groups who did and did not avail of COMSEP's research offerings., Results: 90 (25%) of 360 recipients responded. 61% expressed satisfaction with COMSEP's research offerings. 68% indicated research was an expectation of their academic appointment, that education was their primary research focus (74%) and that they did not have other research opportunities that met their needs (58%). Of respondents, 75.7% of females had submitted a proposal compared to 60% of non-responders who were females. The comparison by gender was not statistically significant. Exploration by academic rank revealed that 35% of instructor/assistant professors had submitted a proposal compared to 65% of associate professors/professors (p =.05). Barriers leading to non-submission to any of the offerings included having too much other work, lack of enjoyment in writing and inability to find mentors. Respondents endorsed the importance of several strategies to promote engagement in research-skill building opportunities, personalized consultations and increased funding., Conclusions: Faculty educators value the importance of educational research and recognize that research opportunities offered by COMSEP address an unmet need, but express ambivalence in the enjoyment of writing (reflecting their mood), and endorse structural barriers, that are amenable to change, affecting their personal agency., (© 2022. The Author(s).)

Background: Renal transplants (Tx) are performed infrequently in infants, due to concerns related to poor outcomes. The aim of our study was to compare graft failure rates in infant (<1 y) renal Tx recipients compared with older children., Methods: Retrospective cohort study of pediatric renal Tx recipients from 2000 to 2015, using the Organ Procurement and Transplant Network database. A log-linear event history regression model for time to graft failure, adjusting for age group and important confounders, was used to estimate post-Tx graft failure probabilities., Results: In 2696 Tx followed for a median of 6.1 y, 704 failures were observed. Significant predictors of graft failure were year of Tx (for each year after 2000, rates were 8.6% lower), Black race-ethnicity (63% higher compared with Whites), and number of HLA matches. For infants (n = 27), estimated graft failure percentage (95% confidence interval) within the first 1-, 2-, and 5-y post-Tx were 10.4 (0.1-21.1), 11.9 (1.2-22.6), and 16.4 (4.9-27.9). For the 1- to 11-y-olds (n = 1429), these were 3.8 (3.0-4.6), 6.3 (5.4-7.3), and 13.6 (12.2-15.0), respectively, and for the 12+ y olds (n = 1240), they were 3.8 (3.1-4.5), 8.1 (7.2-9.0), and 19.9 (18.1-21.7), respectively ( P  < 0.001 for 5-y graft failure rate across age groups)., Conclusions: Infant renal Tx recipients experience a higher graft failure rate in the first year, compared with older cohorts, but over longer intervals, cumulative failure rates are comparable or even lower. To minimize early graft losses such Tx should be performed in experienced centers., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2021 The Author(s). Transplantation Direct. Published by Wolters Kluwer Health, Inc.)

Background: Steroid resistant nephrotic syndrome (SRNS), while uncommon in children, is associated with significant morbidity. Calcineurin inhibitors (CNIs) remain the first line recommended therapy for children with non-genetic forms of SRNS, but some children fail to respond to them. Intravenous (IV) cyclophosphamide (CTX) has been shown to be effective in Asian-Indian children with difficult to treat SRNS (SRNS-DTT). Our study evaluated the outcome of IV CTX treatment in North American children with SRNS-DTT., Methods: Retrospective review of the medical records of children with SRNS-DTT treated with IV CTX from January 2000 to July 2019 at our center. Data abstracted included demographics, histopathology on renal biopsy, prior and concomitant use of other immunosuppressive agents and serial clinical/laboratory data. Primary outcome measure was attainment of complete remission (CR)., Results: Eight children with SRNS-DTT received monthly doses (median 6; range 4-6) of IV CTX. Four (50%) went into CR, 1 achieved partial remission and 3 did not respond. Three of the 4 responders had minimal change disease (MCD). Excluding the 1 child who responded after the 4th infusion, the median time to CR was 6.5 (range 0.5-8) months after completion of IV CTX infusions. Three remain in CR at a median of 8.5 years (range: 3.7-10.5 years) after completion of CTX; one child relapsed and became steroid-dependent. No infections or life-threatening complications related to IV CTX were observed., Conclusions: IV CXT can induce long term remission in North-American children with MCD who have SRNS-DTT., (© 2021. The Author(s).)

Background and Objectives: Arteriovenous fistulae (AVF) and grafts (AVG) are preferred permanent vascular access (PVA) for chronic hemodialysis (HD) patients. Our objective was to examine the change in markers of HD efficacy after successful establishment of a PVA among children who started HD with a tunneled cuffed catheter (TCC)., Materials and Methods: Retrospective chart reviews were completed on patients from 20 pediatric dialysis centers. All patients used TCC prior to AVF/AVG, and each patient acted as his/her own control. Data on markers of HD efficacy (single-pool Kt/V, urea reduction ratio (URR), serum albumin and hematocrit (Hct)) were collected at the creation of AVF/AVG and for 2 years thereafter. Statistical methods included hypothesis testing and statistical modeling after adjusting for relevant demographic variables., Results: First PVA was created in 98 individual children: 87 (89%) were AVF and 11 (11%) were AVG. The mean TCC vintage prior to AVF/AVG was 10.4 ± 17.3 months. At 1-year follow-up, Kt/V improved by 0.15 ± 0.06 (p = 0.02) and URR improved by 4.54 ± 1.17% (p < 0.0001). Furthermore, PVA was associated with improved serum albumin by 0.31 ± 0.07 g/dL (p < 0.0001) and Hct by 2.80 ± 0.65% (p < 0.0001) at 1 year. These HD efficacy markers remained statistically significant at 2 nd -year follow-up. These observations were further supported by the adjusted models. Conversion to AVF was associated with statistically significant improvement in all four markers of HD efficacy at 1-year follow-up. This trend was not demonstrated for subjects who were converted to AVG., Conclusion: Switching to PVA was associated with improved markers of HD efficacy, single-pool Kt/V, URR, serum albumin, and Hct. This improvement was mostly demonstrated at 1 year and maintained for the 2 nd year. The potential differential impact of the type of PVA on the trajectory of markers of HD efficacy should be further investigated.

Background: While there are several curricula using patients as educators, little has been published on how they affect student learning and professional development., Objective: To explore what 1 st year medical students learn about professional values from a patient-led educational experience and how it affects their professional development., Design: We piloted a pediatric patient and family-led educational session during the molecular medicine course, with the goal of sharing the experience of caring for a child with a chronic illness. Following the session, students were required to submit a written reflection on what they learned and the impact the session had on them. All reflections from one academic year were qualitatively analyzed by two investigators and organized using HyperRESEARCH software. A content analysis approach was used to generate codes and emergent themes. Two theoretical lenses guided the analyses: Arnold's framework on professional values and the lens of professional identity formation, described as a process by which health care professionals "think, act and feel like a physician., Results: Students gained an appreciation of professional values, especially humanism and excellence, and how clinician role models reinforce these values. Reflective writings demonstrated recognition among learners that their identity involved being active participants in health care delivery and not just as passive classroom learners. Students were motivated to study diligently and be patient advocates; some questioned their skills in dealing with ambiguity and with the health-care system, resulting in a sense of helplessness., Conclusion: Students learn the importance of professional attributes and of clinician role models through a pediatric family teaching experience. They are motivated, displaying glimpses of their future role as caregivers and patient advocates; however, some also express fear and doubt their own abilities. Based on this, a debriefing session has been introduced to prevent a negative effect on learner self-efficacy.

Purpose: To explore when and in what form pediatric faculty and residents practice reflection., Method: From February to June 2015, the authors conducted focus groups of pediatric faculty and residents at the University of California, Davis; Stanford University; and the University of California, San Francisco, until thematic saturation occurred. Transcripts were analyzed based on Mezirow's and Schon's models of reflection, using the constant comparative method associated with grounded theory. Two investigators independently coded transcripts and reconciled codes to develop themes. All investigators reviewed the codes and developed a final list of themes through consensus. Through iterative discussions, investigators developed a conceptual model of reflection in the clinical setting., Results: Seventeen faculty and 20 residents from three institutions participated in six focus groups. Five themes emerged: triggers of reflection, intrinsic factors, extrinsic factors, timing, and outcome of reflection. Various triggers led to reflection; whether a specific trigger led to reflection depended on intrinsic and extrinsic factors. When reflection occurred, it happened in action or on action. Under optimal conditions, this reflection was goal and action directed and became critical reflection. In other instances, this process resulted in unproductive rumination or acted as an emotional release or supportive therapy., Conclusions: Participants reflected in clinical settings, but did not always explicitly identify it as reflection or reflect in growth-promoting ways. Strategies to enhance critical reflection include developing knowledge and skills in reflection, providing performance data to inform reflection, creating time and space for safe reflection, and providing mentorship to guide the process.

Objective: To describe hypersensitivity reactions in patients receiving maintenance hemodialysis., Data Sources: PubMed search of articles published during the past 30 years with an emphasis on publications in the past decade., Study Selections: Case reports and review articles describing hypersensitivity reactions in the context of hemodialysis., Results: Pharmacologic agents are the most common identifiable cause of hypersensitivity reactions in patients receiving hemodialysis. These include iron, erythropoietin, and heparin, which can cause anaphylactic or pseudoallergic reactions, and topical antibiotics and anesthetics, which lead to delayed-type hypersensitivity reactions. Many hypersensitivity reactions are triggered by complement activation and increased bradykinin resulting from contact system activation, especially in the context of angiotensin-converting enzyme inhibitor use. Several alternative pharmacologic preparations and dialyzer membranes are available, such that once an etiology for the reaction is established, recurrences can be prevented without affecting the quality of care provided to patients., Conclusion: Although hypersensitivity reactions are uncommon in patients receiving hemodialysis, they can be life-threatening. Moreover, considering the large prevalence of the end-stage renal disease population, the implications of such reactions are enormous. Most reactions are pseudoallergic and not mediated by immunoglobulin E. The multiplicity of potential exposures and the complexity of the environment to which patients on dialysis are exposed make it challenging to identify the precise cause of these reactions. Great diligence is needed to investigate hypersensitivity reactions to avoid recurrence in this high-risk population., (Copyright © 2017 American College of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)

Objective: To determine whether renal transplantation survival is similar in children receiving pediatric en bloc kidneys compared with those receiving standard deceased donor kidneys., Study Design: We compared time to allograft failure and estimated glomerular filtration rate (eGFR) in pediatric recipients of en bloc and standard criteria deceased donor renal transplants using Organ Procurement and Transplantation Network data for 2000-2013. Cox regression analysis was used to compare time to allograft failure, and the Student t test was used to compare eGFR., Results: A total of 6882 recipients met the study inclusion criteria; 1.8% received an en bloc transplant. The adjusted hazard for allograft failure was similar for recipients of en bloc kidneys compared with standard criteria kidneys (hazard ratio, 1.15; 95% CI, 0.83-1.59; P = .41). The median wait time for transplantation was significantly shorter for recipients of en bloc kidneys (157 days vs 208 days; P = .03). Moreover, eGFR was superior for recipients of en bloc kidneys up to 5 years post-transplantation., Conclusion: Transplantation of en bloc pediatric kidneys should be considered a viable option for pediatric recipients and may afford unique benefits by reducing wait times and promoting preservation of graft function., (Copyright © 2016 Elsevier Inc. All rights reserved.)