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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., (© 2023. 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 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: Permanent vascular access (PVA) is preferred for long-term hemodialysis. Arteriovenous fistulae (AVF) have the best patency and the lowest complication rates compared to arteriovenous grafts (AVG) and tunneled cuffed catheters (TCC). However, AVF need time to mature. This study aimed to investigate predictors of time to first cannulation for AVF in pediatric hemodialysis patients., Methods: Data on first AVF and AVG of patients at 20 pediatric dialysis centers were collected retrospectively, including demographics, clinical information, dialysis markers, and surgical data. Statistical modeling was used to investigate predictors of outcome., Results: First PVA was created in 117 children: 103 (88%) AVF and 14 (12%) AVG. Mean age at AVF creation was 15.0 ± 3.3 years. AVF successfully matured in 89 children (86.4%), and mean time to first cannulation was 3.6 ± 2.5 months. In a multivariable regression model, study center, age, duration of non-permanent vascular access (NPVA), and Kt/V at AVF creation predicted time to first cannulation, with study center as the strongest predictor (p < 0.01). Time to first cannulation decreased with increasing age (p = 0.03) and with increasing Kt/V (p = 0.01), and increased with duration of NPVA (p = 0.03). Secondary failure occurred in 10 AVF (11.8%). Time to first cannulation did not predict secondary failure (p = 0.29), but longer time to first cannulation tended towards longer secondary patency (p = 0.06)., Conclusions: Study center is the strongest predictor of time to first cannulation for AVF and deserves further investigation. Time to first cannulation is significantly shorter in older children, with more efficient dialysis treatments, and increases with longer NPVA duration.

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.

Cast nephropathy is the most common manifestation of renal injury in patients with multiple myeloma but is rarely reported in other conditions. We are reporting our experience in caring for a teenager with a metastatic neuroendocrine carcinoma who developed rapidly progressive kidney injury that advanced to end-stage renal disease. On renal biopsy extensive tubular necrosis and intratubular eosinophilic casts were noted. This previously unreported finding should prompt oncologists to closely monitor for such a complication in patients with secretory tumors. Whether early plasmapheresis could be of benefit, as has been tried in multiple myeloma, remains to be determined.

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.

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., (© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Background: Adults receiving preemptive renal transplants have better allograft survival. Our study investigated differences in graft and patient survival based on need for, and duration of, pretransplant dialysis in pediatric renal transplant recipients., Methods: Data on pediatric kidney transplants from January 1995 to December 2000 from the Organ Procurement and Transplantation Network were included. Multivariable Cox proportional hazards analysis was performed to determine the effect of pretransplant dialysis on graft and patient survival., Results: Of 3606 transplants, 28% were preemptive, 38% followed pretransplant hemodialysis (HD), and 34% peritoneal dialysis (PD). The 1-year acute rejection rate was lowest for the preemptive group (36%) compared with the HD (45.5%; P=0.0002) and PD (44.2%; P=0.0008) groups. On multivariable analysis, an increased relative risk of graft failure was seen with, among other variables, deceased donor transplantation and acute rejection within the first year, but not with pretransplant dialysis. When analyzed separately by donor source, pretransplant dialysis had no effect on graft survival for deceased donor graft recipients, whereas for living donor recipients, the use and duration of pretransplant HD adversely affected pediatric renal graft survival in a linear manner. No such effect was seen with pretransplant PD., Conclusions: There is a linear increase in the risk of graft failure with the use of and increasing duration of pretransplant HD for living donor grafts. This indicates another reason to minimize the need for and duration of pretransplant HD in children with chronic kidney disease.

As a result of the ongoing shortage in organ supply, en bloc renal transplantation from small donors has become more common for adult recipients with ESRD. However, because of concern for higher complication rates and sub-optimal outcomes, it is not being performed in every center, and data describing its use in pediatric recipients are even more limited. We retrospectively studied three patients who have undergone en bloc renal transplantation at our center. Median age at transplantation was 16.7 yr with a median follow-up of 1.2 yr. Donor age ranged from nine to 49 months with weight ranging from 10 to 22 kg. There were no post-operative thrombotic complications. All grafts showed increased renal size at follow-up by ultrasound. There was no clinical or histological rejection at last follow-up. To the best of our knowledge, this is the first report on the outcomes of en bloc kidney transplantation from pediatric donors into pediatric recipients. Based on our experience, albeit very limited, we feel that en bloc renal transplantation from young donors is an acceptable and safe procedure with low complication rates in pediatric recipients and should be given consideration to minimize wait times on the wait list and to improve quality of life., ((c) 2009 John Wiley & Sons A/S.)

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 81.5%. 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.

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.

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.

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 temperoparietal 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.

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.

Stem cell transplantation is one therapy employed in the management of children with high-risk solid tumors. However, this therapy is not without risk, having been associated with multiple end-organ toxicities. Both acute renal failure and chronic renal insufficiency have been reported in marrow transplant recipients, primarily in the context of the use of calcineurin inhibitors and radiation therapy. This report reviews our experience in managing an adolescent with metastatic Ewing's sarcoma who developed rapid progression to end-stage renal disease following a pretransplant conditioning regimen with high-dose carboplatinum. She had not received radiation or prior cisplatinum therapy. The possible reasons for the patient's highly unusual course and recommendations on ways to prevent this complication are discussed.

Children who develop end-stage renal disease (ESRD) as a result of obstructive uropathies require evaluation and treatment of associated bladder dysfunction to ensure a good outcome following renal transplantation. Bladder dynamics can often be optimized medically, although surgical intervention is occasionally necessary. For those patients who require bladder augmentation, the use of a dilated native ureter (ureterocystoplasty) is preferred to the more commonly used intestine or stomach (enterocystoplasty), which carry a higher risk of complications. Unfortunately, most patients do not have a suitable anatomy for ureterocystoplasty and, by necessity, intestine or stomach has to be utilized. Herein, we describe the successful application of ureterocystoplasty in the presence of ESRD and a solitary kidney prior to renal transplantation. We believe that owing to the many advantages of native urothelium, every effort should be made to use ureter and avoid the use of intestine.

Prolongation of the QTc interval, a risk factor for cardiac arrhythmias, has been observed in adult hemodialysis patients; there are few data on the QTc interval in children with chronic renal failure (CRF) and following renal transplantation (Tx). The purpose of our study was to determine the QTc interval in children with CRF and post renal Tx. Twenty children with CRF and 16 children with renal Tx who were followed at the University of California, Davis, underwent prospective EKG monitoring. The mean QTc interval in the CRF and post-Tx cohorts was normal at 407.9 ms and 408.2 ms, respectively. None of the children with CRF had QTc prolongation. Two Tx recipients had QTc prolongation; both had cardiac dilatation on echocardiography (ECHO). There was no correlation between the QTc interval and the creatinine clearance in either group. However, a significant correlation was noted between QTc prolongation and cardiac dilatation on ECHO in the Tx group (P=0.02, Fisher's exact test). In conclusion, QTc prolongation is uncommon in children with CRF and following Tx, in the absence of cardiac dilatation. However, caution is still needed before prescribing medications known to cause QTc prolongation.