A survey on nutritional management of critically ill children on continuous renal replacement therapy.

We conducted a survey of pediatric nephrologists to examine the knowledge and current practices of and identify challenges in the nutritional management of critically ill children during continuous renal replacement therapy (CRRT). Although it is known that there is a significant effect on nutrition during CRRT, there seems to be a lack of knowledge as well as variability in the practices of nutritional management in these patients, as indicated by our survey results. The heterogeneity of our survey results highlights the need to establish clinical practice guidelines and develop consensus around optimal nutritional management in pediatric patients requiring CRRT. The results as well as the known effects of CRRT on metabolism should be considered during the development of guidelines in critically ill children on CRRT. Our survey findings also highlight the need for further research in the assessment of nutrition, determination of energy needs and caloric dosing, specific nutrient needs, and management.

Conservative therapy is associated with worse clinical features and biochemical derangements than renal replacement therapy: a retrospective study in Kumasi, Ghana.

BACKGROUND: The incidence of end stage kidney disease (ESKD) is increasing in Ghana as with the rest of the world. This study compared the sociodemographic, diagnostic characteristics (clinical, biochemical and imaging) and clinical outcomes of ESKD patients who chose either renal replacement therapy (RRT) or conservative therapy as well as the factors that influenced their choice. METHODS: We retrospectively reviewed the records of 382 ESKD patient from 2006 to 2018. The data was collected from the Nephrology Clinic at the Komfo Anokye Teaching Hospital (KATH). Sociodemographic, diagnostic (clinical, biochemical and imaging) and therapeutic data were obtained, organized and analyzed with Statistical Package for the Social Sciences (SPSS). RESULTS: Of the 382 patients, 321 had conservative therapy whiles 61 had renal replacement therapy. The mean age of participants was 47.71 ± 16.10 years. Bipedal swelling (16.8%), fatigue (10.4%) and facial swelling (9.2%) were the major clinical features. Chronic glomerulonephritis (31.4%), hypertension (30.3%) and diabetes mellitus nephropathy (28.2%) were the most frequent predisposing conditions. Nifedipine (82.0%), bisoprolol (32.8%), aspirin (19.7%), ranitidine (26.2%), metformin (13.1%) and lasix (78.7%) were commonly used by the RRT patients than their conservative therapy counterparts. Compared to their RRT counterparts, patients on conservative therapy were more on irbesartan/lisinopril (57.9%) and sodium hydro carbonate (NaHCO3) (52.0%). Diastolic blood pressure (DBP) (p = 0.047), uremic gastritis (p = 0.007), anaemia, uraemia, haematuria and hyperkalaemia (p < 0.001) were more common in conservative therapy patients than RRT patients with RRT patients showing better corticomedullary differentiation (38.1% vs. 27.7%, p < 0.001) and normal echotexture (15.0% vs. 11.6%, p = 0.005). Age, gender, occupation and duration of illness were significantly associated with the decision to opt for conservative therapy. CONCLUSION: Patients on conservative therapy have worse clinical outcomes than their RRT counterparts. Early referrals to nephrologist as well as subsidized RRT should be targeted.

Serum micronutrient levels in critically ill patients receiving continuous renal replacement therapy: A prospective, observational study.

BACKGROUND: Acute kidney injury (AKI) treated with continuous renal replacement therapy (CRRT) may deplete micronutrient levels. Patients are also at risk for micronutrient depletion due to underlying illness(s), poor nutrient intake prior to intensive care unit (ICU) admission and/or increased requirements. We determined vitamin and trace element status before, during and after CRRT in critically ill patients. METHODS: This prospective observational study performed in mixed medical and surgical ICU patients. Serial serum vitamin B6 and vitamin C concentrations were measured by HPLC and folic acid by ECLIA. Serum chromium, copper, selenium, and zinc were measured using ICP-MS. Serum ceruloplasmin was measured by the Erel method. RESULTS: Fifty adult ICU patients with AKI were recruited. The median APACHE II score on ICU admission was high at 24.0 (6.0-33.0). The median days on CRRT was 2.0 (2.0-4.0) days. At baseline (within 10-15 minutes of CRRT initiation), serum vitamin C, selenium and zinc were below normal. Serum vitamin B6 levels at 72 hours on CRRT were significantly lower than at 24 hours (p = 0.011). Serum vitamin C values fell significantly at 24 and 72 hours during CRRT (p = 0.030 and p = 0.001), respectively, and remained low 24 and 48 hours after CRRT was stopped (p = 0.021). At baseline and during CRRT, 96% of participants had at least two or more micronutrient levels below the normal range. CONCLUSION: Serum vitamin C, selenium and zinc concentrations were below the normal range at baseline. CRRT was associated with a significant further decrease in levels of vitamin C, selenium and zinc.

CRRT-associated ketoacidosis: A series of 5 cases.

Continuous renal replacement therapy (CRRT) is a dialysis modality used in critically ill patients with acute kidney injury (AKI). Although most dialysate and replacement fluids are dextrose-containing, CRRT-associated hypophosphatemia sometimes warrants the use of phosphorus-containing solutions which are dextrose free. The other less commonly used dextrose-free dialysate solutions are certain formulations of Prismasol and Prismasate. As glucose is a small molecule, which is readily cleared with dialysis, use of these solutions can result in increased caloric loss, net glucose deficit, and shifting of the metabolic pathway towards gluconeogenesis and ketogenesis. Starvation ketosis is usually a benign entity, however when combined with factors such as stress of critical illness, can produce metabolic acidosis which at times can be severe. We describe five patients who developed worsening metabolic acidosis despite adequate clearance from CRRT and were diagnosed with CRRT-associated ketoacidosis. Administration of dextrose-containing fluids or tube feeds promptly resulted in resolution of ketonemia and acidosis. Recognition of this entity is of great importance as the reflexive reaction to increase the prescribed dose of CRRT to improve the acidosis, in fact worsens the problem.

Terapia de reemplazo renal continua. Presentación de un caso / Continuous renal replacement therapy. Case report

RESUMEN La insuficiencia renal aguda es definida como la pérdida de función del riñón ocasionada por diversas causas, entre ellas infección e ingesta de fármacos. Esta entidad tiene alta morbilidad y mortalidad en las unidades de cuidados críticos. El tratamiento de la misma va desde la propia protección renal hasta la sustitución artificial de las funciones del riñón lesionado. En la actualidad la terapia de reemplazo renal continua se ha utilizado como soporte renal, y ofrece mayor estabilidad clínica a los pacientes más inestables. En esta revisión se comentan conceptos, indicaciones y los más recientes estudios que validan el uso de esta terapéutica, así como el método de programación que se utilizó en un paciente con diagnóstico de una leptospirosis icterohemorrágica (síndrome de Weil), que estuvo en shock séptico con disfunción multiorgánica, donde se empleó esta terapia con resultados satisfactorios (AU).

ABSTRACT Acute kidney failure is defined as the loss of kidney function caused by various causes, including infection and drug intake. This entity has high morbidity and mortality in critical care units. Treatment ranges from renal protection to artificial replacement of the functions of the injured kidney. Currently, continuous renal replacement therapy has been used as renal support, and offers greater clinical stability to the most unstable patients. In this review, authors discuss concepts, indications and the most recent studies that validate the use of this therapeutic, as well as the programming method that was used in a patient with diagnosis of icteric-hemorrhagic leptospirosis (Weil syndrome), who was in septic shock with multiorgan dysfunction, where this therapy was used with satisfactory results (AU).

The Therapeutic Strategies for Uremic Toxins Control in Chronic Kidney Disease.

Uremic toxins (UTs) are mainly produced by protein metabolized by the intestinal microbiota and converted in the liver or by mitochondria or other enzymes. The accumulation of UTs can damage the intestinal barrier integrity and cause vascular damage and progressive kidney damage. Together, these factors lead to metabolic imbalances, which in turn increase oxidative stress and inflammation and then produce uremia that affects many organs and causes diseases including renal fibrosis, vascular disease, and renal osteodystrophy. This article is based on the theory of the intestinal-renal axis, from bench to bedside, and it discusses nonextracorporeal therapies for UTs, which are classified into three categories: medication, diet and supplement therapy, and complementary and alternative medicine (CAM) and other therapies. The effects of medications such as AST-120 and meclofenamate are described. Diet and supplement therapies include plant-based diet, very low-protein diet, probiotics, prebiotics, synbiotics, and nutraceuticals. The research status of Chinese herbal medicine is discussed for CAM and other therapies. This review can provide some treatment recommendations for the reduction of UTs in patients with chronic kidney disease.

Acute oxalate nephropathy due to high vitamin C doses and exocrine pancreatic insufficiency.

Oxalate kidney injury can manifest as oxalate nephropathy or nephrolithiasis and present as acute kidney injury or even as end-stage renal disease. There are several known causes for acute oxalate nephropathy; however, the combination of exocrine pancreatic insufficiency with overconsumption of vitamin C has not been described before. In this case, a man in his early 80s presented with anorexia and extreme fatigue for 1 week. He had a history of myalgic encephalomyelitis, also known as chronic fatigue syndrome, for which he took several supplements, including high doses of vitamin C. Furthermore, several years ago, he was diagnosed elsewhere with exocrine pancreatic insufficiency. On admission, acute kidney injury was diagnosed. The kidney biopsy showed oxalate nephropathy as the cause. We diagnosed acute oxalate nephropathy due to high vitamin C doses and exocrine pancreatic insufficiency. Within 14 days, his kidney function got worse and he required renal replacement therapy.

Transition Between Different Renal Replacement Modalities: Gaps in Knowledge and Care-The Integrated Research Initiative.

Patients with end-stage kidney disease (ESKD) have different options to replace the function of their failing kidneys. The "integrated care" model considers treatment pathways rather than individual renal replacement therapy (RRT) techniques. In such a paradigm, the optimal strategy to plan and enact transitions between the different modalities is very relevant, but so far, only limited data on transitions have been published. Perspectives of patients, caregivers, and health professionals on the process of transitioning are even less well documented. Available literature suggests that poor coordination causes significant morbidity and mortality.This review briefly provides the background, development, and scope of the INTErnational Group Research Assessing Transition Effects in Dialysis (INTEGRATED) initiative. We summarize the literature on the transition between different RRT modalities. Further, we present an international research plan to quantify the epidemiology and to assess the qualitative aspects of transition between different modalities.

Characteristics and Outcomes of Patients With Systemic Sclerosis (Scleroderma) Requiring Renal Replacement Therapy in Europe: Results From the ERA-EDTA Registry.

RATIONALE & OBJECTIVE: Data for outcomes of patients with end-stage renal disease (ESRD) secondary to systemic sclerosis (scleroderma) requiring renal replacement therapy (RRT) are limited. We examined the incidence and prevalence of ESRD due to scleroderma in Europe and the outcomes among these patients following initiation of RRT. STUDY DESIGN: Registry study of incidence and prevalence and a matched cohort study of clinical outcomes. SETTING & PARTICIPANTS: Patients represented in any of 19 renal registries that provided data to the European Renal Association-European Dialysis and Transplant Association (ERA-EDTA) Registry between 2002 and 2013. PREDICTOR: Scleroderma as the identified cause of ESRD. OUTCOMES: Incidence and prevalence of ESRD from scleroderma. Recovery from RRT dependence, patient survival after ESRD, and graft survival after kidney transplantation. ANALYTICAL APPROACH: Incidence and prevalence were calculated using population data from the European Union and standardized to population characteristics in 2005. Patient and graft survival were compared with 2 age- and sex-matched control groups without scleroderma: (1) diabetes mellitus as the cause of ESRD and (2) conditions other than diabetes mellitus as the cause of ESRD. Survival analyses were performed using Kaplan-Meier analysis and Cox regression. RESULTS: 342 patients with scleroderma (0.14% of all incident RRT patients) were included. Between 2002 and 2013, the range of adjusted annual incidence and prevalence rates of RRT for ESRD due to scleroderma were 0.11 to 0.26 and 0.73 to 0.95 per million population, respectively. Recovery of independent kidney function was greatest in the scleroderma group (7.6% vs 0.7% in diabetes mellitus and 2.0% in other primary kidney diseases control group patients, both P<0.001), though time required to achieve recovery was longer. The 5-year survival probability from day 91 of RRT among patients with scleroderma was 38.9% (95% CI, 32.0%-45.8%), whereas 5-year posttransplantation patient survival and 5-year allograft survival were 88.2% (95% CI, 75.3%-94.6%) and 72.4% (95% CI, 55.0%-84.0%), respectively. Adjusted mortality from day 91 on RRT was higher among patients with scleroderma than observed in both control groups (HRs of 1.25 [95% CI, 1.05-1.48] and 2.00 [95% CI, 1.69-2.39]). In contrast, patient and graft survival after kidney transplantation did not differ between patients with scleroderma and control groups. LIMITATIONS: No data for extrarenal manifestations, treatment, or recurrence. CONCLUSIONS: Survival of patients with scleroderma who receive dialysis for more than 90 days was worse than for those with other causes of ESRD. Patient survival after transplantation was similar to that observed among patients with ESRD due to other conditions. Patients with scleroderma had a higher rate of recovery from RRT dependence than controls.

Metabolic Support of the Patient on Continuous Renal Replacement Therapy.

Continuous renal replacement therapy (CRRT) is the modality of choice in critically ill patients with hemodynamic instability requiring renal replacement therapy. The goal of this review is to discuss an overview of CRRT types, components, and important considerations for nutrition support provision. Evidence basis for guidelines and our recommendations are reviewed. Nutrition support-related implications include the possibility of calorie gain with citrate-based anticoagulation, calorie loss with glucose-free replacement fluids and dialysate, and significant amino acid losses in effluent. We challenge nutrition support clinicians to develop a keen understanding of the specific CRRT modalities that are employed in their intensive care units and to be able to determine how the CRRT prescription may impact a patient's nutrition support prescription.

Clinical outcomes and healthcare use associated with optimal ESRD starts.

OBJECTIVES: To assess the association between optimal end-stage renal disease (ESRD) starts and clinical and utilization outcomes in an integrated healthcare delivery system. STUDY DESIGN: Retrospective observational cohort study in 6 regions of an integrated healthcare delivery system, 2011-2013. METHODS: Propensity score techniques were used to match 1826 patients who experienced an optimal start of renal replacement therapy (initial therapy of hemodialysis via an arteriovenous fistula or graft, peritoneal dialysis, or pre-emptive transplant) to 1826 patients who experienced a nonoptimal start (hemodialysis via a central venous catheter). Outcomes included 12-month rates of sepsis, mortality, and utilization (inpatient stays, total inpatient days, emergency department visits, and outpatient visits to primary care and specialty care). RESULTS: Optimal starts were associated with a 65% reduction in sepsis (odds ratio, 0.35; 95% CI, 0.29-0.42) and a 56% reduction in 12-month mortality (hazard ratio, 0.44; 95% CI, 0.36-0.53). Optimal starts were also associated with lower utilization, except for nephrology visits. Large utilization differences were observed for total inpatient days (9.4 for optimal starts vs 27.5 for nonoptimal starts; relative rate [RR], 0.45; 95% CI, 0.38-0.52) and outpatient visits for specialty care other than nephrology or vascular surgery (12.5 vs 18.3, respectively; RR, 0.62; 95% CI, 0.53-0.74). CONCLUSIONS: Compared with patients with nonoptimal starts, patients with optimal ESRD starts have lower morbidity and mortality and less use of inpatient and outpatient care. Late-stage chronic kidney disease and ESRD care in an integrated system may be associated with greater benefits than those previously reported in the literature.

[Ajuste de fármacos y nutrición en terapias de reemplazo renal continuo].

Del 6 al 23% de los pacientes con lesión renal aguda (LRA) en unidades de cuidados intensivos (UCI) requieren apoyo renal, siendo la terapia continua una modalidad de alta frecuencia de uso en el paciente críticamente enfermo. Si bien el objetivo general de las terapias de reemplazo renal continuo (TRRC) es restablecer el equilibrio hídrico y ácido-base, junto con la eliminación de toxinas urémicas e inflamatorias relacionadas con la pérdida de depuración renal y la disfunción multiorgánica; reconocemos como efecto colateral la depuración (K) no deseado de moléculas y sustancias deseadas en la recuperación del paciente crítico, como pueden ser antimicrobianos y nutrientes. La sepsis es la causa más frecuente de LRA en la UCI y en este contexto la terapia antimicrobiana adecuadamente seleccionada y a la dosis correcta es la médica terapéutica más importante. De la misma manera, es indispensable garantizar el adecuado apoyo nutricional en este grupo poblacional. Proponemos en esta revisión una aproximación teórica y práctica para seleccionar el tratamiento farmacológico de antimicrobianos y el apoyo nutricional en el paciente en TRRC.Six to 23% of patients with acute kidney injury (AKI) in intensive care units (ICU) require renal support. Continuous renal replacement therapies (CRRT) have become the modality of choice in critical care. Although the aim of CRRT is to restore the water and acid-base balance, together with the removal of uremic and inflammatory toxins related to the loss of renal clearance and multi-organ dysfunction; we recognize as a side effect the unwanted clearance of molecules and substances desired for the recovery of the critically ill patient such as antimicrobials and nutrients. Sepsis is the most frequent cause of AKI in the ICU and, in this context, the appropriate selection of antimicrobial therapy, and at the correct dose, is one of the most important decisions; it is also essential to guarantee the adequate nutritional support in this population. We propose in this review a theoretical and practical approach to address the pharmacological management of antimicrobials and nutritional support in the patient in CRRT.

Elimination of Doripenem during Dialysis and Pharmacokinetic Evaluation of Posthemodialysis Dosing for Patients Undergoing Intermittent Renal Replacement Therapy.

Doripenem is a broad-spectrum parenteral carbapenem with enhanced activity against Pseudomonas aeruginosa and Enterobacteriaceae Current dosing regimens recommend the administration of 0.25 to 0.5 g once daily in patients undergoing intermittent renal replacement therapy. As patients are usually dialyzed thrice weekly, we aimed to investigate a 1-g posthemodialysis regimen, thus reducing treatment costs and enhancing patient compliance. A second objective of this trial was to describe the pharmacokinetics of intradialytic doripenem. Ten oliguric or anuric patients in need of intermittent renal replacement therapy were included in this trial. All patients suffered from a septic episode. The mean hemofilter clearance was 123.46 ± 42.03 ml/min, and the total body clearance between hemodialysis sessions was 16.79 ± 6.02 ml/min. The average prehemodialysis trough concentration was 2.4 ± 1.3 mg/liter, while the EUCAST resistance breakpoint for Enterobacteriaceae is set at 2 mg/liter. The interpatient variability was considerably higher than the intrapatient variability. Apart from one patient who suffered an allergic reaction, doripenem was tolerated well by all patients. Our data indicate that posthemodialysis administration of 1 g of doripenem results in sufficient plasma levels in anuric but not oliguric patients during the entire dosing interval. (This trial was registered with EudraCT under registration no. 2009-018010-18 and at ClinicalTrials.gov under registration no. NCT02018939.).

Pharmacokinetics of piperacillin in critically ill patients with acute kidney injury receiving sustained low-efficiency diafiltration.

Background: Piperacillin is a ß-lactam penicillin antibiotic commonly used for the empirical therapy of sepsis and other hospital-acquired infections. However, knowledge regarding the effect of sustained low-efficiency diafiltration (SLED-f), a technique increasingly being used in ICUs, on piperacillin pharmacokinetics (PK) and dosing in critically ill patients is lacking. Objectives: To describe the PK of piperacillin during SLED-f and compare the results with those reported for other forms of renal replacement therapies. Methods: Serial blood samples were collected at pre- and post-filter ports within the SLED-f circuit during SLED-f in one session and from an arterial catheter during sampling without SLED-f. Piperacillin concentrations were measured using a validated chromatography method. Non-compartmental PK analysis of the data was performed. Results: The median clearance and area under the concentration-time curve during SLED-f were 6 L/h and 532 mg·h/L, respectively. Fifty-eight percent of piperacillin was cleared by a single SLED-f session (6 h) compared with previous reports of 30%-45% clearance by a 3.5 h intermittent haemodialysis session. Clearance, half-life and area under the concentration-time curve during SLED-f obtained from this study were comparable with those reported in the post-dilution mode of continuous veno-venous haemodiafiltration studies. Conclusions: As it can be challenging to accurately predict when SLED-f will be initiated in the critically ill, a maintenance dose of at least 4 g every 12 h with at least a 2 g replacement dose post-SLED-f would be a practical approach to piperacillin dosing in ICU patients with anuria receiving SLED-f with a duration similar to the current study.

Prevention of hypophosphatemia during continuous renal replacement therapy-An overlooked problem.

Hypophosphatemia is a common and potentially serious complication occurring during continuous renal replacement therapy (CRRT). Phosphate supplementation is required in the vast majority of patients undergoing CRRT, particularly beyond the first 48 hours. Supplementation can be provided either as a standalone oral or parenteral treatment or as an additive to CRRT solutions. Each approach has advantages and disadvantages, and clinicians must weigh the individual factors most relevant in their practice setting. Currently there are no consensus protocols for phosphate replacement in CRRT, and many centers replete phosphate in response to hypophosphatemia as opposed to pre-emptively. Repletion protocols have also been challenged in recent years by shortages in injectable phosphate solutions. More recently a commercially available phosphate-containing CRRT solution was approved in the United States, but there has been limited clinical experience with this product. In this review, we present recommendations for phosphate repletion in CRRT to prevent hypophosphatemia, and describe our experience using phosphate-containing CRRT solutions.

Changes in co-morbidity pattern in patients starting renal replacement therapy in Europe-data from the ERA-EDTA Registry.

Background: Patients starting renal replacement therapy (RRT) for end-stage renal disease often present with one or more co-morbidities. This study explored the prevalence of co-morbidities in patients who started RRT in Europe during the period from 2005 to 2014. Methods: Using data from patients aged 20 years or older from all 11 national or regional registries providing co-morbidity data to the European Renal Association - European Dialysis and Transplant Association Registry, we examined the prevalence of the following co-morbidities: diabetes mellitus (DM) (primary renal disease and/or co-morbidity), ischaemic heart disease (IHD), congestive heart failure (CHF), peripheral vascular disease (PVD), cerebrovascular disease (CVD) and malignancy. Results: Overall, 70% of 7578 patients who initiated RRT in 2014 presented with at least one co-morbidity: 39.0% presented with DM, 25.0% with IHD, 22.3% with CHF, 17.7% with PVD, 16.4% with malignancy and 15.5% with CVD. These percentages differed substantially between countries. Co-morbidities were more common in men than in women, in older patients than in younger patients, and in patients on haemodialysis at Day 91 when compared with patients on peritoneal dialysis. Between 2005 and 2014 the prevalence of DM and malignancy increased over time, whereas the prevalence of IHD and PVD declined. Conclusions: More than two-thirds of patients initiating RRT in Europe have at least one co-morbidity. With the rising age at the start of RRT over the last decade, there have been changes in the co-morbidity pattern: the prevalence of cardiovascular co-morbidities decreased, while the prevalence of DM and malignancy increased.

Treatment of severe leptospirosis with therapeutic plasma exchange in a pediatric patient.

Ekinci F, Yildizdas RD, Horoz ÖÖ, Alabaz D, Tolunay I, Petmezci E. Treatment of severe leptospirosis with therapeutic plasma exchange in a pediatric patient. Turk J Pediatr 2018; 60: 566-570. Leptospirosis is a common zoonotic disease caused by spirochetes of the genus Leptospira. Although it is mostly a tropical disease, some case reports have been published from temperate regions of the world. The disease presents with a wide spectrum; from asymptomatic self limited disease to a fatal illness characterized by multi-organ involvement. An 8-year-old girl presented with a 5-day history of fever, myalgia, fatigue, vomiting and diarrhea. She developed anuria, hypotension and became unconscious one day after admission and was referred to our pediatric intensive care unit for further evaluation and treatment. Initial physical examination revealed fever, jaundice, diffuse petecchiae on whole body, hepatomegaly and severe hypotension. Laboratory investigations showed elevated liver enzymes and bilirubin levels, elevated creatinine and creatine kinase levels and trombocytopenia. The diagnosis of Leptospirosis was detected by rapid IgM test and confirmed by microscopic agglutination test later. She was treated with mechanical ventilation, wide spectrum antibiotics, positive inotropic agents and penicillin G plus two days of continuous renal replacement therapy and five sessions of therapeutic plasma exchange performed daily. She recovered completely and was transferred to the pediatric ward on the 14th day of hospitalization. The exact role of therapeutic plasma exchange has not been well documented yet, it seems to have benefical effects on clinical and laboratory findings and survival as we observed in our patient and learned from experiences in adult patients presented as case reports.

Outcomes of renal replacement therapy in boys with prune belly syndrome: findings from the ESPN/ERA-EDTA Registry.

BACKGROUND: As outcome data for prune belly syndrome (PBS) complicated by end-stage renal disease are scarce, we analyzed characteristics and outcomes of children with PBS using the European Society for Pediatric Nephrology/European Renal Association-European Dialysis and Transplant Association (ESPN/ERA-EDTA) Registry data. METHODS: Data were available for 88 male PBS patients aged <20 years who started renal replacement therapy (RRT) between 1990 and 2013 in 35 European countries. Patient characteristics, survival, and transplantation outcomes were compared with those of male patients requiring RRT due to congenital obstructive uropathy (COU) and renal hypoplasia or dysplasia (RHD). RESULTS: Median age at onset of RRT in PBS was lower [7.0; interquartile range (IQR) 0.9-12.2 years] than in COU (9.6; IQR: 3.0-14.1 years) and RHD (9.4; IQR: 2.7-14.2 years). Unadjusted 10-year patient survival was 85% for PBS, 94% for COU, and 91% for RHD. After adjustment for country, period, and age, PBS mortality was similar to that of RHD but higher compared with COU [hazard ratio (HR) 1.96, 95% confidence interval (CI) 1.03-3.74]. Seventy-four PBS patients (84%) received a first kidney transplant after a median time on dialysis of 8.4 (IQR 0.0-21.1) months. Outcomes with respect to time on dialysis before transplantation, chance of receiving a first transplant within 2 years after commencing RRT, and death-censored, adjusted risk of graft loss were similar for all groups. CONCLUSIONS: This study in the largest cohort of male patients with PBS receiving RRT to date demonstrates that outcomes are comparable with other congenital anomalies of the kidney and urinary tract, except for a slightly higher mortality risk compared with patients with COU.