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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.

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.

IntroductionAcademic 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.ConclusionsA 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.

BackgroundEngagement 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.MethodsIn 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.Results90 (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.ConclusionsFaculty 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.

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.

BackgroundUnlike 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.MethodsIn 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.ResultsA 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.ConclusionsA 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.

BackgroundRenal 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 (

BackgroundSteroid 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.MethodsRetrospective 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).ResultsEight 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.ConclusionsIV CXT can induce long term remission in North-American children with MCD who have SRNS-DTT.

BackgroundWhile there are several curricula using patients as educators, little has been published on how they affect student learning and professional development.ObjectiveTo explore what 1st year medical students learn about professional values from a patient-led educational experience and how it affects their professional development.DesignWe 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.ResultsStudents 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.ConclusionStudents 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.

Psychogenic polydipsia is a well-described phenomenon in those with a diagnosed psychiatric disorder such as schizophrenia and anxiety disorders. Primary polydipsia is differentiated from psychogenic polydipsia by the lack of a clear psychotic disturbance. We present a case of a 27-month-old boy who presented with polyuria and polydipsia. Laboratory studies, imaging, and an observed water deprivation test were consistent with primary polydipsia. Polydipsia resolved after family limited his fluid intake and began replacing water drinking with other transition objects and behaviors for self-soothing. This case highlights the importance of water deprivation testing to differentiate between causes of polyuria, thereby avoiding misdiagnosis and iatrogenic hyponatremia. Secondly, primary polydipsia can result during the normal stages of child development without overt psychiatric disturbances.

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 "black henna". 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.

While antineutrophil cytoplasmic antibody (ANCA) positivity has been documented in some patients with postinfectious glomerulonephritis (PIGN) and is associated with more severe disease, antiphospholipid antibodies (APA) are not known to be a common occurrence. We describe a child with severe acute kidney injury who was noted to have prolonged positivity of both ANCA and APA; a renal biopsy showed noncrescentic immune complex mediated glomerulonephritis with subepithelial deposits compatible with PIGN. He recovered without maintenance immunosuppressive therapy and at last follow-up had normal renal function. We discuss the cooccurrence and implications of ANCA and APA in children with PIGN.

Primary renal lymphoma is a rare entity, even more so in children. Children with primary renal lymphoma present with variable clinical features such as constitutional signs and symptoms, acute kidney injury, palpable abdominal masses, and gross hematuria. Herein we report a child who presented with seemingly advanced chronic kidney disease and was eventually diagnosed with primary lymphoma. He responded well to intensive chemotherapy and recovered renal function, although he was left with some functional limitations as a consequence of his treatment regimen. Our report highlights the importance of keeping neoplastic conditions under consideration when taking care of children with severe kidney disease of unclear etiology.

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 who was successfully treated with thiazides in managing his hypercalciuria.

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., Author(s): Erica Winnicki [sup.1] , Madan Dharmar [sup.2] , Daniel J. Tancredi [sup.2] , Stephanie Nguyen [sup.2] , Lavjay Butani [sup.2] Author Affiliations: (Aff1) 0000 0001 2297 6811, grid.266102.1, Department [...]

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.