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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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: In academic health centers, education remains an incompletely supported and funded mandate. In an attempt to promote education and better support educational endeavors of faculty, some academic health centers and departments have conceived of a metric, the educational value unit (eVU), to begin to "quantify" teaching. What goes into this metric, its intended goals and the logistics of its implementation vary considerably among centers. Lessons Learned: This practical advice paper highlights the various lessons learned from a review of the limited published literature on eVU systems supplemented with our personal experience in implementing a successful eVU system in the Department of Pediatrics at our institution, to help guide others who may be interested in doing that same. Even in limited-resource settings, our hope is that these lessons can serve as a guide on how to better quantify and reward teaching, whether through monetary or nonfiscal incentives and recognition. [ABSTRACT FROM AUTHOR]

While there are several curricula using patients as educators, little has been published on how they affect student learning and professional development. To explore what 1st year medical students learn about professional values from a patient-led educational experience and how it affects their professional development. 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. 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. 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. [ABSTRACT FROM AUTHOR]

Purpose: To explore the types of exemplary professional behaviors and the facilitators and barriers to professional behavior discussed by student–mentor dyads during appreciative inquiry (AI) dialogs. Materials and methods: We conducted a qualitative analysis of AI narratives discussing exemplary professional practice written by third-year medical students following a dialog with mentors. Narratives were thematically analyzed using directed content analysis to explore the types of exemplary professional behaviors discussed and the facilitators and barriers to professional practice. Narratives were coded independently by two investigators; codes were finalized, themes were derived, and a model on how exemplary professional behaviors are nurtured and reinforced was developed. Results: Themes addressed humanism toward others and excellence, with altruism being an underlying implicit guiding principle behind professional behavior. Humanism toward self was infrequently discussed as an aspect of professionalism, but when discussed, was perceived to foster resilience. Principle-based attitudes and emotional intelligence facilitated professional behaviors. Programmatic scaffolds facilitated professional behavior and included curricula on reflective practice, mentorship, promoting learner autonomy and connectedness, and a safe environment. Conclusions: AI is an effective strategy that can be used to stimulate learner reflection on professionalism, humanism, and wellness and promote learner acknowledgement of positive aspects of the learning environment. [ABSTRACT FROM AUTHOR]

We report a case of an adolescent boy with Down's syndrome and ESRD on hemodialysis who developed mild Graves' disease that was not amenable to radioablation, surgery, or ATDs. After 14 months of observation without resolution of Graves' disease, he successfully received a DDRT with a steroid minimization protocol. Thymoglobulin and a three-day course of steroids were used for induction and he was started on tacrolimus, MMF, and pravastatin for maintenance transplant immunosuppression. One month after transplantation, all biochemical markers and antibody profiling for Graves' disease had resolved and remain normal one yr later. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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]

The utilization of en bloc renal allografts from small pediatric donors has been adopted as an effective strategy to expand the organ donor pool in adult recipients. Data in children are limited. The aim of our study is to describe the outcomes of en bloc renal transplants in children from our center. Medical records of children receiving pediatric en bloc renal transplants at our institution from January 2007 were abstracted. Data collected included recipient and donor demographics, operative technique and complications, and post-operative studies. Eight children received en bloc renal transplants at a median age of 17 yr; median follow-up was 0.9 yr. Donor body weight ranged from 4 to 22 kg. One kidney was lost to intra-operative thrombosis, while the other kidney from this en bloc graft remained viable. All grafts showed increased renal size at follow-up ultrasound. Surveillance biopsies showed glomerulomegaly in two patients. At last follow-up, the median e GFR was 130 mL/min/1.73 m2. The urinary protein to creatinine ratio was normal in four of seven patients. Our data suggest that in experienced centers, en bloc renal transplantation from young donors into pediatric recipients is effective. Long-term follow-up to monitor for complications, including hyperfiltration injury, is warranted. [ABSTRACT FROM AUTHOR]

Butani L, Gallay BJ. Acute humoral rejection in pediatric renal transplant recipients receiving steroid minimization immunosuppression. Abstract: SM protocols have increasingly gained acceptance owing to their favorable side effect profile with comparable cellular rejection rates. After encountering SM patients with AHR, we performed a case-control study to identify predictors associated with AHR in this cohort. Patients with (n = 4) and without (n = 19) biopsy proven AHR on a SM regimen were compared using the Student's t-tests. The median age at transplant was 13.8 yr. Compared to controls, the AHR cohort was older (15.9 vs. 12.1 yr, p = 0.01). Children with AHR had a lower mean tacrolimus trough level and were more likely to have a sub-therapeutic trough at six months (3.5 vs. 5.5 ng/mL, p = 0.05); mean MMF doses were lower at all times points except three months in the AHR group (not statistically significant). This occurred in spite of higher MPA trough levels at all study points in the AHR group (significant at 3 [p = 0.019] and 6 [p = 0.03] months). Children receiving a SM regimen have a lower safety net and may benefit from more intensive monitoring of tacrolimus exposure. MMF dose modifications based on MPA trough determinations should be resisted in the setting of SM. [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]

Steroid-minimization regimens have gained tremendous popularity for renal Tx in the recent past since they are associated with lower metabolic complications and other adverse effects related to long-term steroid exposure. Most such protocols have been restricted to low-risk recipients due to the concern for acute rejection with steroid-minimization. Herein, we report our experience in managing a child who received a positive flow cytometry cross-match living donor kidney transplant with low titer DSA and was successfully managed using a steroid-minimization drug regimen. The purpose of our report is to make pediatric transplant care providers aware of the feasibility of using a steroid-minimization immunosuppression regimen even in children who have traditionally been perceived to be at higher risk for immunologic complications, allowing successful avoidance of steroid toxicity. [ABSTRACT FROM AUTHOR]

The article discusses effective intervention strategies to prevent and treat dyslipidemia after renal transplantation. Discussed are steroid withdrawal and minimization protocols, the prevention of lipid abnormalities, the use of tacrolimus instead of cyclosporine A, the use of corticosteroids and calcineurin-inhibitors and the use of statins in improving the lipid profile in children and adolescents with familial hypercholesterolemia.

Little has been published on the rate of prehypertension (HBP) and hypertension (HTN) in children with hematologic malignancies. This study was preformed to determine the prevalence and predictors of HBP and HTN in newly diagnosed acute leukemia and lymphoma patients. Retrospectively, blood pressure (BP) values were followed from admission until normalization in 102 children. HBP and HTN were defined as either a systolic or diastolic value ≥ the 90th and 95th percentile BP measurement, respectively. HBP and HTN were identified in 68.6% and 52.9% of children prior to chemotherapy and 78.4% and 67.3% postchemotherapy, respectively. Mean time to BP normalization was 54days. Only ten children (15% of HTN patients) received antihypertensive therapy. Logistic regression determined that the only predictor for HBP and HTN was the estimated glomerular filtration rate (eGFR) at the time of admission—every 10ml/min per 1.73m2 increase led to a 16% and 14% decrease in the odds of postchemotherapy HBP ( p = 0.02) and HTN ( p = 0.03), respectively. A surprisingly high prevalence of BP abnormalities was identified and lower eGFR predicted HBP and HTN in children with newly diagnosed hematologic malignancies. Better recognition and serious consideration for treatment should be given to this cardiovascular abnormality. [ABSTRACT FROM AUTHOR]

This study investigated predictors of renal survival in children with Henoch-Schönlein purpura glomerulonephritis. Records of patients with Henoch-Schönlein purpura glomerulonephritis evaluated at our center, from 1953–1990, were reviewed. Data were abstracted from records of patients seen within 5 years. Others were mailed a questionnaire or contacted by telephone. Primary outcome measures were renal survival and presence of urinary abnormalities or hypertension. Of the 65 eligible patients with Henoch Schönlein purpura glomerulonephritis, follow-up data was obtainable for 8 1.5%. The median follow-up was 20 years. At last follow-up, 66% of patients had normal renal function and urinalyses, and 21% had progressed to end-stage renal disease, The only factor associated with the development of end-stage renal disease was the use of cytotoxic agents. There are no features at initial presentation that identify children at risk of disease progression. Close follow-up of all children with Henoch Schönlein purpura glomerulonephritis is warranted. [ABSTRACT FROM AUTHOR]

Because of its narrow therapeutic index, monitoring of drug exposure is recommended for tacrolimus (T). Limited data are available on kinetics of T in children after transplantation. Our study investigated the correlation between T trough and the area under the time-concentration curve (AUC) in pediatric renal transplant recipients and investigated the effect of steroids. Data on T troughs and two h and four h post-dose concentrations over the first post-transplant year in 20 transplant recipients from August 2001 to June 2005 were analyzed. Patients were analyzed in two groups based on their use of steroids. Although the overall correlation between the troughs and AUC was good (r = 0.85, Pearson test), during the first month the correlation was poor in the cohort receiving steroids (r = 0.5) compared with those on a steroid minimization regimen (r = 0.9). In 85% of patients there was a discrepancy between the trough and AUC leading to errors in dose adjustment. In conclusion, although the overall correlation between T trough and AUC is good, it is suboptimal in the first post-transplant month in children receiving steroids. Because of large variation in exposure, we recommend AUC monitoring for T. Prospective studies are needed to determine the impact of more accurate monitoring of T exposure on outcomes. [ABSTRACT FROM AUTHOR]

With longer graft and patient survival, recurrent disease is becoming recognized as an increasingly important contributor to long-term graft loss in renal transplant recipients. However, patients may present for the first time in end-stage renal disease (ESRD) leading to uncertainty as to their underlying diagnosis and the risk of recurrence. The purpose of this study was to describe the features of children who presented for the first time in ESRD and to determine the predictive value of investigations in differentiating diseases with and without a recurrence risk. From 7/99 to 11/04, 13 children presented to our center in ESRD. Their median age was 13.3 yr; 77% were male. The majority were hypertensive (77%) and oligoanuric (69%). All had proteinuria (median urine protein to creatinine ratio [Up/c] 7.0), and 92% had microhematuria. Only seven had small kidneys on ultrasound. All children underwent a serologic work-up and six (46%) were biopsied. Of the 13 children, seven had a glomerular disease; in five the diagnosis was made on biopsy, in one on serologic testing and one by family history. Of the remaining six children, three had non-glomerular diseases: obstructive uropathy in one and primary hyperoxaluria type 1 in two, and 3 had an unknown disease. When patients with glomerular diseases were compared with those with non-glomerular diseases, the two predictors for glomerular disease were a lower serum albumin (p = 0.004) and a higher serum bicarbonate level (p = 0.01). Comparing patients with and without a risk of recurrence, there were no differences between the two groups in any of their demographic, clinical, or biochemical parameters by analysis of variance (including serum albumin or proteinuria). In summary, the vast majority of children presenting in ESRD have hematuria and proteinuria, even with non-glomerular diseases. The significant overlap in clinical features between patients with and without a risk of recurrence emphasizes the need for all children presenting in ESRD to be evaluated extensively so that disease recurrence after transplantation can be anticipated or even prevented. [ABSTRACT FROM AUTHOR]

Hyperlipidemia is common after renal transplantation (Tx) and contributes to the increased cardiovascular morbidity seen in the post-transplant period. Limited data are available on the utility of the statins in children after renal Tx. This 12-month prospective study was undertaken to determine the efficacy of pravastatin in reducing dyslipidemia after renal Tx in children and to determine predictors of dyslipidemia after Tx. From August 2001 to April 2004, all 17 newly transplanted pediatric renal transplant recipients at our center were preemptively treated with pravastatin from the immediate post-transplant period. Fasting lipid profiles were obtained at 1, 3, 6 and 12 months after Tx. Trends in the lipid profile were analyzed using the repeated measures general linear model (GLM). A historical cohort of pediatric renal-transplant recipients not treated with pravastatin was used as the control population. The mixed effects GLM was used for multivariable logistic regression analyses to determine the independent effect of age, pretransplant cholesterol (Chol), body mass index (BMI), creatinine clearance (CrCl), and corticosteroid and tacrolimus doses on the development of dyslipidemia. The mean age of the children at Tx was 8.7 yr. The GLM analysis showed that with time, there was a significant decline in the total Chol, serum triglyceride (TG), LDL and also HDL-Chol (p-value <0.05 for each). Compared with the controls, the mean serum Chol was lower at all time points post-transplant in the treated patients. However, despite treatment, the prevalence of hypercholesterolemia increased from 31% pretransplant to 53% at 1-month, but declined thereafter to 6% at 3 and 6 months and 0% at 1 yr. Multivariable regression analyses showed the prednisone dose, pretransplant Chol and age to be the most important risk factors for the development of dyslipidemia. No child developed complications related to therapy. In summary, pravastatin is safe in the post-transplant period in children and reduces serum Chol, LDL-Chol and TG. An unexpected finding in our study was the decline in HDL-Chol after Tx. Whether the preemptive use of the statins will result in lower cardiovascular morbidity, especially considering the concomitant reduction in HDL-Chol remains to be determined. [ABSTRACT FROM AUTHOR]

Patients with chronic proteinuric nephropathies are at high risk of developing progressive renal insufficiency. There are limited controlled data on the efficacy of potentially toxic immunosuppressive therapies for many of these diseases such as immunoglobulin A nephropathy and idiopathic membranoproliferative glomerulonephritis. This limitation has not deterred healthcare providers from using such agents based on anecdotal experience. We report our experience taking care of three children with heavy proteinuria from chronic glomerular diseases. All were treated with a combination of angiotensin-converting enzyme inhibitors and angiotensin receptor blockers without concomitant immunosuppression. All went into complete remission soon after starting therapy, allowing corticosteroid avoidance. The purpose of this report is to make healthcare professionals more aware of the potential success that can be achieved with this relatively nontoxic drug regimen. Larger controlled clinical trials using this strategy are needed to better evaluate the efficacy and safety of this approach in children with glomerular diseases. [ABSTRACT FROM AUTHOR]

Pretransplant (Tx) inflammation is linked to adverse outcomes in adult Tx recipients but no such data exist for children. Our study evaluated the predictive value of three pre-Tx inflammatory markers: serum C-reactive protein (CRP), Neopterin (Neo) and interleukin (IL) 12, in determining outcome. Pre-Tx serum on 51 children (mean age 11 years) transplanted between 1985 and 2000 was analyzed. Data on other variables were abstracted from patient records. Primary end-points were graft survival and acute rejection (AR). Kaplan-Meier and log-rank statistics compared endpoints in patients at different quartiles for each marker. Cox regression analysis was used to determine the independent effect of the markers on the end-points. The mean CRP, Neo, and IL-12 were 1.3 mg/l, 1.78 ng/ml, and 123 pg/ml, respectively. The mean CRP, Neo, and IL-12 were not different between the patients with and without AR or graft loss ( P > 0.4 for all). Neither rejection-free survival nor graft survival was affected by CRP, Neo, or IL-12 quartiles. Cox-regression analysis demonstrated no predictive value of any marker on outcome. Unlike adults, a single pre-Tx determination of inflammatory markers was not predictive of AR or graft loss in children, indicating that the pathogenesis of AR may be different in children. [ABSTRACT FROM AUTHOR]

Idiopathic hypercalciuria is a common metabolic abnormality in children of all ages. There is evidence of an association of idiopathic hypercalciuria with nephrolithiasis, hematuria, and osteoporosis. However, much of this evidence is anecdotal and the precise role of hypercalciuria in the pathogenesis of these conditions is far from clear. Furthermore, the precise definition of idiopathic hypercalciuria has not yet been established. The methodologies for quantitating urinary calcium excretion have also not been standardized, adding another potential confounding factor to the accurate interpretation of urinary calcium excretion. Long-term studies on the natural history of unselected children with idiopathic hypercalciuria are needed to establish the true clinical significance of this condition. The focus of this review is to critically evaluate the methods currently being used to measure urinary calcium excretion in children and to assess the validity of existing criteria for diagnosing idiopathic hypercalciuria. [ABSTRACT FROM AUTHOR]

Abstract: Renal transplant (Tx) recipients frequently develop hypercholesterolemia. Pravastatin (P) has been shown to be effective in adult renal Tx recipients, not only in reducing serum cholesterol, but possibly also in decreasing graft rejection. However, there are no data on the use of P in children following renal transplantation. We conducted a retrospective case–control study evaluating the safety and efficacy of P (10–20 mg/day) in reducing hypercholesterolemia, when used pre-emptively in the post-Tx period in seven children, compared with an historical control (C) group of nine children who had not received P. The two groups were comparable with respect to their demographics and in their pretransplant serum cholesterol. Compared with the C group, the mean serum cholesterol in the P group was lower at 3 months (159 mg/dL vs. 225 mg/dL), 6 months (134 mg/dL vs. 200 mg/dL), 9 months (134 mg/dL vs. 209 mg/dL), and 12 months (125 mg/dL vs. 195 mg/dL) (p < 0.005 for all, Student's two-tailed t -test). At 1 month only 43% of the P group had hypercholesterolemia compared with 67% of the controls; by 12 months this difference was even more significant (0% in the P group vs. 45% in the C group). None of the treated patients developed any adverse reactions. This study demonstrates that the pre-emptive use of P in pediatric renal Tx recipients appears to be effective in significantly reducing serum cholesterol. Whether this effect will translate into improved allograft and patient survival in the long term cannot be predicted at present and will require additional studies to evaluate. [ABSTRACT FROM AUTHOR]

Discusses various devices used to measure blood pressure (BP) in children. Significance of BP measurements in children; Lack of uniformity in types of devices used to measure BP in children; Advantages of oscillometric devices over the conventional sphygmomanometry; Problems with oscillometric devices; Steps to ensure accuracy of BP measurements obtained by oscillometric devices.

The renal transplant (Tx) recipient is at risk for developing various complications including urolithiasis, the only manifestation of which may be hematuria. However, there are no data on the prevalence of microscopic hematuria in renal Tx recipients. The objective of our study was to determine the prevalence of microhematuria in our pediatric Tx patients and to investigate the causes of microhematuria. Records of all pediatric renal Tx recipients followed at our center from September 1999 to September 2000 were retrospectively reviewed; of the 21 patients, seven (33%) had persistent microscopic hematuria that was first noted 2.9 years post-Tx. Patients with and without hematuria had similar baseline characteristics. Only one patient had pre-existing hematuria that continued post-Tx. The etiology of hematuria in the other six patients was: recurrent IgA nephropathy (one patient), CMV nephritis (one patient), and unexplained (four patients). None had renal calculi or hypercalciuria. Three of the four patients with unexplained hematuria have chronic allograft nephropathy, and the fourth (original disease dysplasia) has hypocomplementemia. At their last follow-up, 5.3 years after onset of hematuria, all patients are alive with stable allograft function. In conclusion, microscopic hematuria is not uncommon in pediatric renal Tx recipients. While causes of post-Tx hematuria are diverse, stones are not commonly seen. Whether chronic allograft nephropathy per se can be implicated as a cause of hematuria remains to be determined. Renal biopsies should be considered at the onset of hematuria if proteinuria and/or deterioration in renal function are seen concomitantly, to look for recurrent or de novo glomerulonephritis. [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 article focuses on the reflection skill that should be modeled and taught to medical students so that they could practice and refine a reflective approach in their careers. It defines reflection which means to turn back or think back on experiences. It categorizes reflection to 3 domains which include clinical reasoning, scientific reflection and reflection .

About one-half of all children with systemic lupus erythematosus have clinical evidence of renal disease at initial presentation, such as proteinuria and acute renal failure. Herein, we report a case of a teenager who presented with end-stage renal disease (ESRD) of uncertain etiology, and who was subsequently determined to have lupus. The purpose of this report is to make health-care professionals aware of this unusual presentation of renal lupus, which has never been reported before. Children presenting in ESRD should be worked-up for autoimmune diseases since the discovery of such a disease process may impact future decision-making, especially with respect to subsequent renal transplantation. [ABSTRACT FROM AUTHOR]

Primary anti-phospholipid syndrome (APS) is perceived to be an uncommon disorder, infrequently recognized as a cause of renal disease in childhood. While renal involvement in APS classically manifests as thrombotic events, other renal diseases associated with APS have been reported in adults, including membranous nephropathy and minimal change disease. We report our experience of caring for a child who presented with acute anuric renal failure due to anti-neutrophilic cytoplasmic antibody-negative rapidly progressive glomerulonephritis (RPGN), with concomitant thrombotic microangiopathy (TMA). Recognition of the APS as a cause of the patient’s TMA facilitated institution of anticoagulation. Our patient’s renal failure did not improve and the patient remained dependent on dialysis until he was successfully transplanted. The purpose of our report is to make health-care professionals aware of the previously unreported association of pauci-immune RPGN and APS in children; early recognition of APS will allow initiation of anticoagulation to prevent recurrent thromboses and enable successful transplantation. [ABSTRACT FROM AUTHOR]

Hemolytic uremic syndrome (HUS) is a leading cause of acute renal failure (ARF) in children, and one for which treatment with peritoneal dialysis (PD) is often necessary. Between January 1982 and December 1996, 176 children received PD for ARF at St. Christopher’s Hospital for Children; 34 (19%) of whom had HUS. Of these 34, 7 (20%) developed pleural effusions (PE) while receiving PD, whereas none of the remaining 142 children with other causes of ARF did so. The mean age of the 7 affected children was 5.2 (range 0.4–17) years; none had heart failure or nephrotic syndrome, nor had any of them undergone thoracic surgery. PE were diagnosed by chest radiograph at an interval of 2 (range 1–3) days after starting PD. Thereafter, 4 (57%) patients were successfully maintained on a modified PD prescription; 2 others were converted to hemodialysis and 1 to continuous venovenous hemodiafiltration. Although PE are a known complication of PD, none of the patients so treated for non-HUS related ARF developed them. Whether they represent a purely mechanical complication of PD, or are in some way attributable to HUS itself, is not entirely clear. Regardless, when children with HUS require PD, physicians should monitor for the development of this potential complication to minimize the risk of serious respiratory compromise. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to the article "Child and parental perspectives of multidimensional quality of life outcomes after kidney transplantation," by S.J. Anthony and colleagues in the 2010 issue.

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]

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. [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]

A letter to the editor is presented in response to articles about minimal-change nephrotic syndrome which appeared in previous issues of this periodical.

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.

Presents a letter to the editor about remission of membranoproliferative nephritis with inhibition of Angiotensin Converting Enzyme (ACE) and Angiotensin II Receptor Blockers (ARB).

Focuses on the effects of dietary protein intake on the serum creatinine concentration (S&subcr;). Elevation in S&subcr; of healthy volunteers after beef ingestion; Validity of the methods used to measure S&subcr;; Impact of protein intake on patients with renal disease.

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]

AMR is increasingly being recognized as an important cause of renal allograft injury, contributing to significant morbidity and graft loss. There are few controlled trials and no well-established treatment guidelines for AMR in renal transplant recipients. We retrospectively reviewed the outcome of four pediatric renal transplant recipients on a steroid minimization immunosuppression protocol treated with bortezomib for elevated DSA and acute AMR from 2012 to 2013. All patients received four doses of bortezomib 1.3 mg/m2 given on days one, four, eight, and 11. All patients also received other treatments prior to bortezomib, which may have included rituximab, methylprednisolone, plasmapheresis, and/or IVIg. While bortezomib in addition to other therapies significantly decreased DSA titers, DSA remained very elevated months after treatment. All four patients had immediate improvement or stabilization of renal function but one eventually lost her graft. There were no adverse events related to bortezomib six months after treatment. [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]

Membranoproliferative glomerulonephritis, albeit uncommon, is associated with considerable morbidity and mortality in children. Corticosteroids are the mainstay of therapy for severe disease, although data supporting their use are limited. We report our experience in treating two children with nephrotic–nephritic syndrome from idiopathic membranoproliferative glomerulonephritis. Both children experienced a suboptimal response to prolonged courses of steroids and were started on tacrolimus as a steroid-sparing agent. Rapid and complete remission was achieved in both children after initiation of tacrolimus. The purpose of our report is to increase awareness of health care professionals to the potential benefits of this agent in inducing remission in children with severe membranoproliferative glomerulonephritis. [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]

Spontaneous epidural hemorrhage has been described in a variety of clinical scenarios, including chronic renal failure (CRF). During hemodialysis, patients with CRF rarely develop spinal or cranial epidural hematomas. Such hemorrhages have been attributed to intracranial pressure fluctuations during hemodialysis, heparin administration, uremic-platelet syndrome, or hypertension. Although the exact pathophysiology is not understood, this rare consequence of hemodialysis has been well documented in the literature. Hemorrhage in the absence of hemodialysis therapy in a CRF patient, however, has not been previously reported. We report a 16-year-old boy with no history of trauma who woke in the morning with severe headache and resultant neurological deterioration. He was found to have a large left tempero-parietal epidural hematoma and underwent urgent surgical evacuation. Postoperatively he developed a contralateral extra-axial hematoma that did not require surgical intervention. He recovered completely, with no significant neurological deficit. This unique presentation of spontaneous intracranial epidural hemorrhage in an adolescent not receiving hemodialysis highlights a rare, but serious, complication of CRF. [ABSTRACT FROM AUTHOR]

The article explores how educators can use questioning strategies to promote learning in a clinical setting. Topics discussed include a framework that matches questions to a learner's ability and suggestions for formulating questions to challenge learners and maintain supportive learning environment, an approach to effective questioning that considers the developmental stage of the learning and learning objectives that best fit the stage and the Dreyfus and Bloom model of skills acquisition.

In the contemporary era of potent immunosuppressive regimens, previously encountered signs of renal allograft rejection such as fever and hematuria are rarely encountered. We report a teenager with severe recurrent acute humoral and cellular rejection whose presenting feature was gross hematuria with the presence of blood clots in the urine. We want to highlight that severe rejection even in the setting of modern immunosuppressive drugs can present as gross hematuria. Contrary to conventional wisdom that gross hematuria with the presence of blood clots in the urine is indicative of pathology in the renal collecting system, a parenchymal disease process should also be considered in renal transplant recipients. [ABSTRACT FROM AUTHOR]