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Management of acute liver failure (ALF) and acute on chronic liver failure (ACLF) in the pediatric population can be challenging. Kidney manifestations of liver failure, such as hepatorenal syndrome (HRS) and acute kidney injury (AKI), are increasingly prevalent and may portend a poor prognosis. The overall incidence of AKI in children with ALF has not been well-established, partially due to the difficulty of precisely estimating kidney function in these patients. The true incidence of AKI in pediatric patients may still be underestimated due to decreased creatinine production in patients with advanced liver dysfunction and those with critical conditions including shock and cardiovascular compromise with poor kidney perfusion. Current treatment for kidney dysfunction secondary to liver failure include conservative management, intravenous fluids, and kidney replacement therapy (KRT). Despite the paucity of evidence-based recommendations concerning the application of KRT in children with kidney dysfunction in the setting of ALF, expert clinical opinions have been evaluated regarding the optimal modalities and timing of KRT, dialysis/replacement solutions, blood and dialysate flow rates and dialysis dose, and anticoagulation methods., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Raina, Sethi, Filler, Menon, Mittal, Khooblall, Khooblall, Chakraborty, Adnani, Vijayvargiya, Teo, Bhatt, Koh, Mourani, de Sousa Tavares, Alhasan, Forbes, Dhaliwal, Raghunathan, Broering, Sultana, Montini, Brophy, McCulloch, Bunchman, Yap, Topalglu and Díaz-González de Ferris.)

The objective of this study was to inform the pediatric nephrologists of recent advances in acute kidney injury (AKI) epidemiology, pathophysiology, novel biomarkers, diagnostic tools, and management modalities. Studies were identified from PubMed, EMBASE, and Google Scholar for topics relevant to AKI. The bibliographies of relevant studies were also reviewed for potential articles. Pediatric (0-18 years) articles from 2000 to May 2020 in the English language were included. For epidemiological outcomes analysis, a meta-analysis on data regarding AKI incidence, mortality, and proportion of kidney replacement therapy was performed and an overall pooled estimate was calculated using the random-effects model. Other sections were created highlighting pathophysiology, novel biomarkers, changing definitions of AKI, evolving tools for AKI diagnosis, and various management modalities. AKI is a common condition seen in hospitalized children and the diagnosis and management have shown to be quite a challenge. However, new standardized definitions, advancements in diagnostic tools, and the development of novel management modalities have led to increased survival benefits in children with AKI. IMPACT: This review highlights the recent innovations in the field of AKI, especially in regard to epidemiology, pathophysiology, novel biomarkers, diagnostic tools, and management modalities., (© 2021. The Author(s), under exclusive licence to the International Pediatric Research Foundation, Inc.)

Background: Acute kidney injury (AKI) is a significant problem in neonates, but the evidence is sparse. Neonatal AKI is an independent risk factor for increased mortality and prolonged hospital stay. There are stark differences in the epidemiology of AKI in neonates amongst the developing and the developed world. Increased prevalence of neonatal sepsis, lack of awareness about neonatal AKI and poor access to pediatric nephrologists add to the improper management of neonatal AKI in the developing countries. Methods: This study is a multicentric, national, prospective cohort study [The Indian iconic Neonatal Kidney Educational Registry (TINKER)] conducted in level 2-3 NICUs in 11 centers across India. We have enrolled nearly 2,000 neonates over the study period. Neonates (≤ 28 days) who were admitted in NICU and those who received intravenous (IV) fluids for at least 48 h for hydration and/or nutrition have been included. Data collection included: (1) baseline demographics (2) daily physiologic and laboratory parameters (3) discharge data. KDIGO workgroup AKI definition modified for neonates was used for defining AKI. Data entry was carried out by individual participating centers using a web-based database (akiregistry.org). De-identified data has been maintained and handled by the principal investigator (PI). This collaboration plans to disseminate data through peer-reviewed publications and through presentations at educational conferences. Conclusions: The purpose of this study is to create the first prospective neonatal all-cause AKI data repository and describe the incidence of neonatal AKI in NICUs in the country and determine the risk factors as well as the outcomes of such neonates-both short-term and long-term outcomes. This will eventually spur therapeutic advancements, facilitate decipherment of epidemiological trends, risk factors as well as outcomes and identify disparities in management across the nation., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Agrawal, Wazir, Sethi, Tibrewal, Dhir, Bajaj, Gupta, Mirgunde, Sahoo, Balachandran, Afzal, Shrivastava, Bagla, Krishnegowda, Konapur, Soni, Kolukula, Jangid, Bunchman and Raina.)

Introduction: The slow increase in use of telemedicine began to expand rapidly, along with reimbursement changes, during the coronavirus disease-2019 (COVID-19) pandemic. Standardized protocols for these services are lacking but are needed for effective and equitable health care. In this study, we queried pediatric nephrologists and their patients about their telemedicine experiences during the pandemic., Methods: Surveys that were in compliance with the Health Insurance Portability and Accountability Act were deployed online to patients and physicians., Results: We collected survey responses from 400 patients and 197 pediatric nephrologists. Patients reported positive experiences with telemedicine visits as it was logistically easier than in-person visits. Patients also felt that the quality of their visits were equivalent to what they would receive in person. Physicians used a wide variety of online systems to conduct synchronous telemedicine with Zoom (23%), EPIC (9%), Doxy.me (7%), services not specified (37%), or a mix of local or smaller services (24%). Most physicians' concerns were related to technological issues and the ability to procure physical exams and/or laboratory results., Conclusions: There is a paucity of published trials on telemedicine services in pediatric nephrology. Virtual care was feasible and acceptable for patients, caregivers, and providers during the COVID-19 pandemic., (© 2021 International Society of Nephrology. Published by Elsevier Inc.)

Pediatric acute kidney injury (AKI) is a frequently missed complication. AKI has a significant impact on both short- and long-term outcomes in children. Within the last decade, there have been major landmark developments in this field of critical care pediatric nephrology. The topic was searched by two independent researchers using Google Scholar and PubMed and related studies published in the last 10 years. The terms used for the search were 'pediatric acute kidney injury,' 'pediatric acute renal failure,' 'pediatric dialysis,' 'biomarkers,' 'nephrotoxins,' 'nephrotoxicity in children,' and 'pediatric critical care nephrology.' We found that AKI is common in critically ill neonates and children. Among the various definitions, the Kidney Disease: Improving Global Outcomes (KDIGO) definition is most commonly used. In addition, it is imperative to risk stratify sick children at admission in the hospital to predict AKI and worse outcomes as this aids in early management. There are now major landmark trials that describe the epidemiology, prevention, and management guidelines in this field and health care professionals need to be aware they should diagnose AKI early. Overall, this review highlights the landmark studies in the last decade and shows that early diagnosis and management of AKI in 'at risk' children can improve outcomes.

The recent worldwide pandemic of COVID-19 has had a detrimental worldwide impact on people of all ages. Although data from China and the United States indicate that pediatric cases often have a mild course and are less severe in comparison to adults, there have been several cases of kidney failure and multisystem inflammatory syndrome reported. As such, we believe that the world should be prepared if the severity of cases begins to further increase within the pediatric population. Therefore, we provide here a position paper centered on emergency preparation with resource allocation for critical COVID-19 cases within the pediatric population, specifically where renal conditions worsen due to the onset of AKI., (Copyright © 2020 Raina, Chakraborty, Sethi and Bunchman.)

Background: Focal segmental glomerulosclerosis (FSGS) causes end stage renal disease (ESRD) in significant proportion of patients worldwide. Primary FSGS carries poor prognosis and management of FSGS patients, refractory to standard treatments or resistant to steroids, remains a major challenge. Lipoprotein apheresis is a therapeutic approach for drug resistant primary FSGS and post-renal transplant primary FSGS recurrence. Objectives: To examine the safety and probable benefit at 1, 3, 6, 12, and 24-months following completion of apheresis treatment using Liposorber® LA-15 system in patients with nephrotic syndrome (NS), due to refractory primary FSGS or primary FSGS associated NS, in post renal transplant children. Material and Methods: Prospective, multicenter, single-arm intervention study using Liposorber® LA-15 system. Patients ≤21 years old with drug resistant or drug intolerant NS secondary to primary FSGS with glomerular filtration rate (GFR) ≥60 ml/min/1.73 m 2 or post renal transplant patients ≤21 years old with primary FSGS associated NS were included in the study. Each patient had 12 dextran-sulfate plasma adsorption lipoprotein apheresis sessions over a period of 9 weeks. All patients were followed up at 1, 3, 6, 12, and 24-months following completion of treatment. Results: Of 17 patients enrolled, six were excluded from the outcome analysis (protocol deviations). Of the remaining 11 patients, all but one have completed apheresis treatments. Three patients were lost to follow-up immediately after completion of apheresis and excluded from outcome analysis. At one-month follow-up, 1 of 7 patients (14.3%) attained partial remission of NS while 2 of 4 subjects (50%) and 2 of 3 subjects (66.7%) had partial/complete remission at 3- and 6-months follow-up, respectively. One of two patients followed up for 12 months had complete remission and one patient had partial remission of NS after 24 months. Improved or stable eGFR was noted in all patients over the follow-up period. Conclusion: The results of our multicenter study showed improvement in the response rates to steroid or immunosuppressive therapy and induced complete or partial remission of proteinuria in some of the patients with drug resistant primary FSGS. The main limitation of our study is the small number of subjects and high dropout rate., (Copyright © 2019 Raina, Krishnappa, Sanchez-Kazi, Quiroga, Twombley, Mathias, Lo, Chakraborty, Mahesh, Steinke, Bunchman and Zaritsky.)

Objectives: To present recommendations and supporting literature for RBC transfusions in critically ill children supported with extracorporeal membrane oxygenation, ventricular assist devices, or renal replacement therapy., Design: Consensus conference series of international, multidisciplinary experts in RBC transfusion management of critically ill children., Methods: The panel of 38 experts developed evidence-based, and when evidence was lacking, expert-based clinical recommendations as well as research priorities for RBC transfusions in critically ill children. The extracorporeal membrane oxygenation/ventricular assist device/renal replacement therapy subgroup included six experts. We conducted electronic searches of the PubMed, EMBASE, and Cochrane Library databases from 1980 to May 2017, using medical subject heading terms and text words to define concepts of RBC transfusion, extracorporeal membrane oxygenation, ventricular assist device, and renal replacement therapy. We used a standardized data extraction form to construct evidence tables and graded the evidence using the Grading of Recommendations Assessment, Development, and Evaluation system. Recommendations developed and supporting literature were reviewed and scored by all panel members. Agreement was obtained using the Research and Development/UCLA Appropriateness Method., Results: For inpatients requiring extracorporeal membrane oxygenation, ventricular assist device, or renal replacement therapy support, there was expert agreement (> 80%) on five good practice statements aimed to improve accuracy and uniform reporting of RBC transfusion data in pediatric extracorporeal membrane oxygenation, ventricular assist device, and renal replacement therapy studies and quality improvement projects; four clinical recommendations of physiologic metrics and biomarkers of oxygen delivery, in addition to hemoglobin concentration, to guide RBC transfusion, acknowledging insufficient evidence to recommend specific RBC transfusion strategies; and eight research recommendations., Conclusions: Further research surrounding indications, risks, benefits, and alternatives to RBC transfusion in children on extracorporeal devices is clearly needed. Using a structured literature review and grading process, the Transfusion and Anemia Expertise Initiative panel concluded that there is currently insufficient evidence to recommend specific RBC transfusion variables in children requiring extracorporeal membrane oxygenation, ventricular assist device, or renal replacement therapy support.

Objectives: To date, there are no published guidelines to direct RBC transfusion decision-making specifically for critically ill children. We present the recommendations from the Pediatric Critical Care Transfusion and Anemia Expertise Initiative., Design: Consensus conference series of multidisciplinary, international experts in RBC transfusion management of critically ill children., Setting: Not applicable., Intervention: None., Subjects: Children with, or children at risk for, critical illness who receive or are at risk for receiving a RBC transfusion., Methods: A panel of 38 content and four methodology experts met over the course of 2 years to develop evidence-based, and when evidence lacking, expert consensus-based recommendations regarding decision-making for RBC transfusion management and research priorities for transfusion in critically ill children. The experts focused on nine specific populations of critically ill children: general, respiratory failure, nonhemorrhagic shock, nonlife-threatening bleeding or hemorrhagic shock, acute brain injury, acquired/congenital heart disease, sickle cell/oncology/transplant, extracorporeal membrane oxygenation/ventricular assist/ renal replacement support, and alternative processing. Data to formulate evidence-based and expert consensus recommendations were selected based on searches of PubMed, EMBASE, and Cochrane Library from 1980 to May 2017. Agreement was obtained using the Research and Development/UCLA Appropriateness Method. Results were summarized using the Grading of Recommendations Assessment, Development, and Evaluation method., Measurements and Results: The Transfusion and Anemia Expertise Initiative consensus conference developed and reached consensus on a total of 102 recommendations (57 clinical [20 evidence based, 37 expert consensus], 45 research recommendations). All final recommendations met agreement, defined a priori as greater than 80%. A decision tree to aid clinicians was created based on the clinical recommendations., Conclusions: The Transfusion and Anemia Expertise Initiative recommendations provide important clinical guidance and applicable tools to avoid unnecessary RBC transfusions. Research recommendations identify areas of focus for future investigation to improve outcomes and safety for RBC transfusion.

Hypothesis: Acute kidney injury (AKI) is a common cause of morbidity and mortality worldwide, with a pediatric incidence ranging from 19.3% to 24.1%. Treatment of pediatric AKI is a source of debate in varying geographical regions. Currently CRRT is the treatment for pediatric AKI, but limitations due to cost and accessibility force use of adult equipment and other therapeutic options such as peritoneal dialysis (PD) and hemodialysis (HD). It was hypothesized that more cost-effective measures would likely be used in developing countries due to lesser resource availability., Methods: A 26-question internet-based survey was distributed to 650 pediatric Nephrologists. There was a response rate of 34.3% (223 responses). The survey was distributed via pedneph and pcrrt email servers, inquiring about demographics, technology, resources, pediatric-specific supplies, and preference in renal replacement therapy (RRT) in pediatric AKI. The main method of analysis was to compare responses about treatments between nephrologists in developed countries and nephrologists in developing countries using difference-of-proportions tests., Results: PD was available in all centers surveyed, while HD was available in 85.1% and 54.1% (p = 0.00), CRRT was available in 60% and 33.3% (p = 0.001), and SLED was available in 20% and 25% (p = 0.45) centers of developed and developing world respectively. In developing countries, 68.5% (p = 0.000) of physicians preferred PD to costlier therapies, while in developed countries it was found that physicians favored HD (72%, p = 0.00) or CRRT (24%, p = 0.041) in infants., Conclusions: Lack of availability of resources, trained physicians and funds often preclude standards of care in developing countries, and there is much development needed in terms of meeting higher global standards for treating pediatric AKI patients. PD remains the main modality of choice for treatment of AKI in infants in developing world.

Acute kidney injury (AKI) in critically ill children is frequently a component of the multiple organ failure syndrome. It occurs within the framework of the severe catabolic phase determined by critical illness and is intensified by metabolic derangements. Nutritional support is a must for these children to improve outcomes. Meeting the special nutritional needs of these children often requires nutritional supplementation by either the enteral or the parenteral route. Since critically ill children with AKI comprise a heterogeneous group of subjects with varying nutrient needs, nutritional requirements should be frequently reassessed, individualized and carefully integrated with renal replacement therapy. This article is a state-of-the-art review of nutrition in critically ill children with AKI.

Despite abnormal clotting, circuits clot during continuous renal replacement therapy (CRRT) in children with acute liver failure (ALF). We report our experience. All children with ALF needing CRRT were studied over 2 years. Patient and circuit factors associated with circuit use were evaluated. Thirty-one children in liver failure (median age 7.4 years) underwent CRRT, of which 17 (54.8%) died. A total of 98 filtration episodes were used. The smallest access catheter was 6.5 Fr, while the largest was 13.5 Fr. The most common filter used was HFO7 (63%). Mean duration (SD) of circuit use was 33.13(30.83) hours. Of the 98 filtration episodes, circuits blocked in 25, whereas the access catheter blocked in 25. Fifty-two circuits were changed electively for a variety of reasons. Prostacyclin was the anticoagulant in 62 filtration episodes. The remaining filtration episodes had either no anticoagulation or heparin. The mean (SD) "downtime" was 5.13 (9.15) hours. We found a significant association between fresh frozen plasma (FFP) use with circuit blockade. Neither the duration of CRRT nor the "downtime" influenced mortality. The CRRT circuits blocked in children despite deranged clotting in liver disease. Circuits are changed for a variety of reasons other than clotting. The use of FFP reduces circuit life., (© 2014 The Authors. Therapeutic Apheresis and Dialysis © 2014 International Society for Apheresis.)

Introduction: Pediatric renal transplant patients may require native nephrectomy to avoid complications at the time of kidney transplantation. We have performed unilateral and bilateral transperitoneal pretransplant laparoscopic nephrectomies (PLNs), followed by living-related renal transplantation. The benefits of transperitoneal versus retroperitoneal dissection remain controversial., Subjects and Methods: A retrospective review of patients who underwent unilateral or bilateral transperitoneal PLNs between May 1, 2001 and April 30, 2009 was performed. A transperitoneal approach was used in all patients., Results: Eight patients underwent transperitoneal PLN. Four patients were female, and the average age was 91 months (range, 9-199 months). Five of the cases involved bilateral nephrectomy. Average operating times, including hemodialysis catheter placement and other procedures, was 4.93 hours (range, 4.25-5.97 hours) for bilateral PLN and 3.93 hours (range, 2.57-5.48 hours) for unilateral PLN. The average hospital stay was 5.9 days. All patients underwent successful renal transplantation following PLN without rejection at an average 2.3 years of follow-up., Conclusions: PLN appears to be an effective, safe method of removing potentially problematic, diseased kidneys prior to planned renal transplantation. A transperitoneal technique using four midline ports affords excellent access to both kidneys and, more importantly, allows for additional procedures to be completed simultaneously.

This article provides the bedside clinician an overview of the unique renal complications that are seen commonly in the pediatric intensive care unit. These sections are purposely succinct to give a quick guide to the clinician for the care of these children. We have identified four major areas that should result in discussion and cooperative care between intensive care physicians and nephrologists for the care of these children: (1) hypertension, (2) chronic kidney failure, (3) acute kidney injury, and (4) renal replacement therapy., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Objective: To report circuit characteristics and survival analysis in children weighing ≤10 kg enrolled in the Prospective Pediatric Continuous Renal Replacement Therapy (ppCRRT) Registry., Study Design: We conducted prospective cohort analysis of the ppCRRT Registry to: (1) evaluate survival differences in children ≤10 kg compared with other children; (2) determine demographic and clinical differences between surviving and non-surviving children ≤10 kg; and (3) describe continuous renal replacement therapy (CRRT) circuit characteristics differences in children ≤5 kg versus 5-10 kg., Results: The ppCRRT enrolled 84 children ≤10 kg between January 2001 and August 2005 from 13 US tertiary centers. Children ≤10 kg had lower survival rates than children >10 kg (36/84 [43%] versus 166/260 [64%]; P < .001). In children ≤10 kg, survivors were more likely to have fewer days in intensive care unit prior to CRRT, lower Pediatric Risk of Mortality 2 scores at intensive care unit admission and lower mean airway pressure (P(aw)), higher urine output, and lower percent fluid overload (FO) at CRRT initiation. Adjusted regression analysis revealed that Pediatric Risk of Mortality 2 scores, FO, and decreased urine output were associated with mortality. Compared with circuits from children 5-10 kg at CRRT initiation, circuits from children ≤5 kg more commonly used blood priming for initiation, heparin anticoagulation, and higher blood flows/effluent flows for body weight., Conclusion: Mortality is more common in children who are ≤10 kg at the time of CRRT initiation. Like other CRRT populations, urine output and FO at CRRT initiation are independently associated with mortality. CRRT prescription differs in small children., (Copyright © 2013 Mosby, Inc. All rights reserved.)

Background: The EXtracorporeal TReatments In Poisoning (EXTRIP) workgroup was formed to provide recommendations on the use of extracorporeal treatment (ECTR) in poisoning. To test and validate its methods, the workgroup reviewed data for thallium (Tl)., Methods: After an extensive search, the co-chairs reviewed the articles, extracted the data, summarized findings, and proposed structured voting statements following a predetermined format. A two-round modified Delphi method was chosen to reach a consensus on voting statements and RAND/UCLA Appropriateness Method to quantify disagreement. Blinded votes were compiled, returned, and discussed during a conference call. A second vote determined the final recommendations., Results: Forty-five articles met inclusion criteria. Only case reports and case series were identified, yielding a very low quality of evidence for all recommendations. Data on 74 patients, including 11 who died, were abstracted. The workgroup concluded that Tl is slightly dialyzable and made the following recommendations: ECTR is recommended in severe Tl poisoning (1D). ECTR is indicated if Tl exposure is highly suspected on the basis of history or clinical features (2D) or if the serum Tl concentration is >1.0 mg/L (2D). ECTR should be initiated as soon as possible, ideally within 24-48 hours of Tl exposure (1D), and be continued until the serum Tl concentration is <0.1 mg/L for a minimal duration of 72 hours (2D)., Conclusion: Despite Tl's low dialyzability and the limited evidence, the workgroup strongly recommended extracorporeal removal in the case of severe Tl poisoning.

Objective: Continuous renal replacement therapy is the most often implemented dialysis modality in the pediatric intensive care unit setting for patients with acute kidney injury. However, it also has a role in the management of patients with nonrenal indications such as clearance of drugs and intermediates of disordered cellular metabolism., Measurements and Methods: Using data from the multicenter Prospective Pediatric Continuous Renal Replacement Therapy Registry, we report a cohort of pediatric patients receiving continuous renal replacement therapy for nonrenal indications. Nonrenal indications were obtained from the combination of "other" category for continuous renal replacement therapy initiation and patient diagnosis (both primary and secondary). This cohort was further divided into three subgroups: inborn errors of metabolism, drug toxicity, and tumor lysis syndrome., Results: From 2000 to 2005, a total of 50 continuous renal replacement therapy events with nonrenal indications for therapy were included in the Prospective Pediatric Continuous Renal Replacement Therapy Registry. Indication-specific survival of the subgroups was 62% (inborn errors of metabolism), 82% (tumor lysis syndrome), and 95% (drug toxicity). The median small solute dose delivered among the subgroups ranged from 2125 to 8213 mL/1.73 m/hr, with 54%-59% receiving solely diffusion-based clearance as continuous venovenous hemodialysis. No association was established between survival and dose delivered, modality of continuous renal replacement therapy, or use of intermittent hemodialysis prior to continuous renal replacement therapy., Conclusions: Pediatric patients requiring continuous renal replacement therapy for nonrenal indications are a distinct cohort within the population receiving renal replacement therapy with little published experience of outcomes for this group. Survival within this cohort varies by indication for continuous renal replacement therapy and is not associated with continuous renal replacement therapy modality. Additionally, survival is not associated with small solute doses delivered within a cohort receiving >2000 mL/1.73 m/hr. Our data suggest metabolic control is established rapidly in pediatric patients and that acute detoxification may be provided with continuous renal replacement therapy for both the initial and maintenance phases of treatment using either convection or diffusion at appropriate doses.

Extracorporeal treatments (ECTRs), such as hemodialysis and hemoperfusion, are used in poisoning despite a lack of controlled human trials demonstrating efficacy. To provide uniform recommendations, the EXTRIP group was formed as an international collaboration among recognized experts from nephrology, clinical toxicology, critical care, or pharmacology and supported by over 30 professional societies. For every poison, the clinical benefit of ECTR is weighed against associated complications, alternative therapies, and costs. Rigorous methodology, using the AGREE instrument, was developed and ratified. Methods rely on evidence appraisal and, in the absence of robust studies, on a thorough and transparent process of consensus statements. Twenty-four poisons were chosen according to their frequency, available evidence, and relevance. A systematic literature search was performed in order to retrieve all original publications regardless of language. Data were extracted on a standardized instrument. Quality of the evidence was assessed by GRADE as: High = A, Moderate = B, Low = C, Very Low = D. For every poison, dialyzability was assessed and clinical effect of ECTR summarized. All pertinent documents were submitted to the workgroup with a list of statements for vote (general statement, indications, timing, ECTR choice). A modified Delphi method with two voting rounds was used, between which deliberation was required. Each statement was voted on a Likert scale (1-9) to establish the strength of recommendation. This approach will permit the production of the first important practice guidelines on this topic.

Although rare, renal replacement therapy (RRT) for the treatment of the metabolic, respiratory and hemodynamic complications of intoxications may be required. Understanding the natural clearance of the medications along with their volume of distribution, protein binding and molecular weight will help in understanding the benefit of commencing RRT. This information will aid in choosing the optimal forms of RRT in an urgent setting. Overdose of common pediatric medications are discussed with suggestions on the type of RRT within this educational review.

Objective: Megestrol acetate (MA) has been used to treat weight loss in pediatric patients with malignancies, cystic fibrosis and HIV/AIDS. We herein report our experience with MA in pediatric patients with chronic kidney disease (CKD)., Design: We conducted a retrospective cohort study. Charts were evaluated for clinical, treatment, and laboratory data at six time points: approximately 6 months prior to initiation of MA, at initiation and cessation of MA, and at 2-, 4-, and 8-month follow-up. Anthropometric measurements were corrected for age and sex by conversion to z scores., Setting: Division of Pediatric Nephrology, Helen DeVos Children's Hospital, Grand Rapids, MI., Patients: Pediatric patients (n = 25) with CKD and poor weight gain., Intervention: Patients were administered MA at initial and tapered doses of 14.4 ± 8.1 mg/kg/d and 10.1 ± 6.5 mg/kg/d, respectively, for 5.4 ± 6.3 months., Results: The study population (n = 25) was 60% male, 16% African American, 72% white, and 12% Hispanic with a mean ± SD age of 8.9 ± 5.4 years. Prior to MA therapy, patients demonstrated a decrease in BMI and poor weight gain. The treatment phase was associated with significant increases in BMI (P < .0001) and weight (P < .0001), which were well sustained at 8-month follow-up (P < 0.01 and P < 0.001, respectively). Patients demonstrated continued increases in height. A single patient exhibited physical adverse side effects (cushingoid features) associated with MA; otherwise, MA was well tolerated., Conclusions: MA appears to effectively improve weight gain in pediatric CKD patients with minimal adverse side effects and may therefore serve as a safe, short-term, nutritional strategy., (Copyright © 2010 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.)

Background: Low birth-weight infants are at risk for renal disease when renal insults occur in the neonatal period. Renal growth as measured by sonography over time is utilized by many nephrologists as predictors of future renal disease., Objective: To identify infants at risk by defining normal renal growth for the very premature infant., Materials and Methods: Renal growth was evaluated in 30 infants whose birth weight was 1,500 g or less and gestational age was <31 weeks. During a 2-month time period, three US measurements were taken (first week of life, age 28 days, and age 56 days or earlier if discharged). Infants were divided according to birth weight: the extremely low birth-weight group (ELBW) was <1,000 g (n = 14), and the very low birth-weight group (VLBW) was 1,000-1,500 g (n = 16)., Results: In both groups, the right and left renal lengths were similar. In the ELBW group, the initial mean length was 3.25 cm and grew to 4.16 cm, while the mean volume was 4.85 cm and grew to 10.39 cm. In the VLBW group the initial mean length was 3.69 cm and grew to 4.35 cm while the mean volume was 7.25 cm and grew to 11.83 cm., Conclusion: These data establish normal expected growth for future studies.

Objective: This study sought to evaluate the use of adult renal formulas in hyperkalemic infants with chronic kidney disease (CKD)., Design: This was a retrospective, single-center cohort study., Setting: This study took place at the Department of Pediatric Nephrology, Dialysis, and Transplantation at Helen DeVos Children's Hospital (Grand Rapids, MI)., Patients: Seven hyperkalemic infants (mean age, 6.9 months) comprised the study population: 29% with stage 3 CKD, 29% with stage 4 CKD, and 42% with stage 5 CKD., Intervention: Infants were empirically treated with adult renal formulas for an average duration of 9.6 months. Six of seven infants were started on breast milk or infant formula (Similac PM 60/40, Abbott Laboratories, Columbus, OH), but because of inadequate growth and hyperkalemia, were transitioned to adult renal formulas (Suplena, Abbott Laboratories, Columbus, OH; Nepro, Abbott Laboratories, Columbus, OH; and/or Renalcal, Nestle Nutrition, Minnetonka, MN). One infant received adult renal formula at birth., Main Outcome Measures: The outcome measures included amount of potassium delivered by infant and adult renal formulas, level of serum potassium, and anthropometric measurements adjusted for age and gender (z-scores)., Results: The transition from infant to adult renal formula resulted in a decrease in mean amount of potassium delivered by formula (from 2.6 to 1.0 mEq/kg/day, P < .001) and a decrease in mean serum potassium (from 5.1 to 4.0 mmol/L, P < .01). During treatment with adult renal formula, the infants demonstrated a significant increase in mean weight z-score (from -1.0 to 0.5, P < .01), height z-score (from -1.9 to -0.5, P < .01), and head-circumference z-score (from -1.5 to -1.0, P=.03). Adult renal formulas were well-tolerated., Conclusions: Hyperkalemic infants with CKD can be nutritionally managed on adult renal formula., (Copyright (c) 2010 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.)

Background: Paediatric patients with systemic lupus erythematosus (SLE) often have severe presentations including lupus nephritis (LN). Few paediatric studies have evaluated the anticardiolipin antibody (aCL) and renal histology. The purpose of this study was to evaluate clinicopathologic features, including aCL, short-term clinical and renal histologic outcomes of paediatric patients with new-onset SLE nephritis., Methods: We conducted a single centre, retrospective inception cohort study. Charts were reviewed at presentation (initial renal biopsy), 6-month (follow-up biopsy) and 12-month follow-up., Results: The population consisted of 21 patients (median age, 14.5 years): 19/21 were female, 6/21 African American, 3/21 Asian, 9/21 Caucasian and 3/21 Hispanic. At presentation, 19/21 had elevated aCL, 15/21 hypertensive, 12/21 nephrotic and 7/21 required haemodialysis (HD)-2/7 HD patients had thrombotic microangiopathy, 1/7 crescentic glomerulonephritis. Two patients had thromboembolism: both had aCL, were taking oral contraceptives and required HD, one was nephrotic and the other had elevated lupus anticoagulant. Initial biopsies revealed 6/21 ISN/RPS class II nephritis, 3/21 class III, 7/21 class IV and 5/21 class V. Treatment consisted of methylprednisolone, corticosteroids, cyclophosphamide or mycophenolate mofetil. Follow-up biopsies revealed 12/13 to have improved histology. Indication for a follow-up biopsy was severe illness at presentation. At 12-month follow-up, no patients were nephrotic (P < 0.001) or required HD (P < 0.001), and 3/14 had elevated aCL (P < 0.001)., Conclusion: Elevated aCL, hypertension, nephrotic syndrome and need for HD were common presentations among our paediatric SLE nephritis population. Renal histology and aCL were helpful in the therapeutic management.

Background: Critically ill children with hemodynamic instability and acute kidney injury often develop fluid overload. Continuous renal replacement therapy (CRRT) has emerged as a favored modality in the management of such children. This study investigated the association between fluid overload and mortality in children receiving CRRT., Study Design: Prospective observational study., Setting & Participants: 297 children from 13 centers across the United States participating in the Prospective Pediatric CRRT Registry., Predictor: Fluid overload from intensive care unit (ICU) admission to CRRT initiation, defined as a percentage equal to (fluid in [L] - fluid out [L])/(ICU admit weight [kg]) x 100%., Outcome & Measurements: The primary outcome was survival to pediatric ICU discharge. Data were collected regarding demographics, CRRT parameters, underlying disease process, and severity of illness., Results: 153 patients (51.5%) developed < 10% fluid overload, 51 patients (17.2%) developed 10%-20% fluid overload, and 93 patients (31.3%) developed > or = 20% fluid overload. Patients who developed > or = 20% fluid overload at CRRT initiation had significantly higher mortality (61/93; 65.6%) than those who had 10%-20% fluid overload (22/51; 43.1%) and those with < 10% fluid overload (45/153; 29.4%). The association between degree of fluid overload and mortality remained after adjusting for intergroup differences and severity of illness. The adjusted mortality OR was 1.03 (95% CI, 1.01-1.05), suggesting a 3% increase in mortality for each 1% increase in severity of fluid overload. When fluid overload was dichotomized to > or = 20% and < 20%, patients with > or = 20% fluid overload had an adjusted mortality OR of 8.5 (95% CI, 2.8-25.7)., Limitations: This was an observational study; interventions were not standardized. The relationship between fluid overload and mortality remains an association without definitive evidence of causality., Conclusions: Critically ill children who develop greater fluid overload before initiation of CRRT experience higher mortality than those with less fluid overload. Further goal-directed research is required to accurately define optimal fluid overload thresholds for initiation of CRRT., (Copyright 2010 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.)

Recent data suggest that elevated levels of uric acid (UA) might contribute to the progression of renal disease. Rasburicase, recombinant urate oxidase, is a highly safe and efficacious hypo-uricosuric agent for treatment of elevated UA levels from tumor lysis. We adopted the use of rasburicase for management of hyperuricemia in infants with acute kidney injury (AKI) and, herein, report our experience. We conducted a retrospective chart review of infants with hyperuricemia (UA > 8 mg/dl) secondary to AKI (serum creatinine > 1.5 mg/dl) treated with rasburicase. Seven infants (mean age 34 +/- 55 days, six male), with a mean weight of 3.2 +/- 1.2 kg, were identified. Rasburicase was administered intravenously as a single, onetime, bolus of 0.17 +/- 0.04 mg/kg body weight. Within 24 h, serum UA had decreased from 13.6 +/- 4.5 mg/dl to 0.9 +/- 0.6 mg/dl (P < 0.05), creatinine had decreased from 3.2 +/- 2.0 mg/dl to 2.0 +/- 1.2 mg/dl (P < 0.05), and urinary output had increased from 2.4 +/- 1.2 ml/kg per hour to 5.9 +/- 1.8 ml/kg per hour (P < 0.05). Continued improvements in UA, creatinine, and urinary output were observed in the week following administration of rasburicase, without rebound of the UA. We observed no treatment-related side effects. All patients demonstrated a normalization of uric acid level without need of renal replacement therapy. In conclusion, a single intravenously administered bolus of rasburicase appears to be a novel treatment for hyperuricemia in infants with AKI.

Renovascular hypertension from renal artery aneurysmal formation is a rare complication of fibromuscular dysplasia. Few data exist to direct the management of intrarenal artery aneurysms in pediatric patients. We report the presentation, diagnosis and management of renovascular hypertension and intrarenal aneurysmal disease in a preschool child.

Corticosteroid immunosuppression has permitted the development of successful allotransplantation; however, corticosteroids are associated significant post-transplant complications. To circumvent these problems, we implemented a protocol of rapid discontinuation of corticosteroids in 19 consecutive pediatric primary kidney transplant recipients. Mean age at time of transplant was 13.4 (+/-4.5) yr, 52.6% were male, 63.2% underwent living donor transplantation. All patients were administered Thymoglobulin [anti-thymocyte globulin (rabbit)] as induction immunosuppression with a rapid tapering dose of corticosteroids (total of five daily doses), and maintained on mycophenolate mofetil and tacrolimus. Two patients had immediate recurrence of primary disease (FSGS), requiring further corticosteroid therapy. Otherwise, remaining 17 patients were maintained off corticosteroids, with excellent graft function; mean baseline eGFR of 112 mL/min/1.73 m(2) (+/-19) at 28 months (+/-14) post-transplantation. There was 100% patient and rejection-free graft survival at 27 months (range 5-58 months) post-transplantation; 47% underwent renal transplant biopsy secondary to acute rise in serum creatinine with or without worsening hypertension. All biopsies had no evidence of acute rejection; 62.5% had findings consistent with tacrolimus toxicity. Renal transplantation utilizing a rapid discontinuation of corticosteroid protocol in pediatric patients appears to be safe and effective, without increasing the risk of acute rejection or graft loss.

Objective: Few published reports describe nutrition provision for critically ill children and young adults with acute kidney injury receiving continuous renal replacement therapy. The goals of this study were to describe feeding practices in pediatric continuous renal replacement therapy and to evaluate factors associated with over- and under-prescription of protein and calories., Design: Retrospective database study., Setting: Multicenter study in pediatric critical care units., Patients: Patients with acute kidney injury (estimated glomerular filtration rate < 75 mL/min/1.73 m at continuous renal replacement therapy initiation) enrolled in the Prospective Pediatric Continuous Renal Replacement Therapy Registry., Interventions: None., Measurements: Nutrition variables: initial and maximal protein (g/kg/day) and caloric (kcal/kg/day) prescription and predicted resting energy expenditure (kcal/kg/day). We determined factors predicting initial and maximal protein and caloric prescription by multivariate analysis., Results: One hundred ninety-five patients (median [interquartile range] age = 8.1 [12.8] yrs, 56.9% men) were studied. Mean protein and caloric prescriptions at continuous renal replacement therapy initiation were 1.3 +/- 1.5 g/kg/day (median, 1.0; range, 0-10) and 37 +/- 27 kcal/kg/day (median, 32; range, 0-107). Mean maximal protein and caloric prescriptions during continuous renal replacement therapy were 2.0 +/- 1.5 g/kg/day (median, 1.7; range, 0-12) and 48 +/- 32 kcal/kg/day (median, 43; range, 0-117). Thirty-four percent of patients were initially prescribed < 1 g/kg/day protein; 23% never attained > 1 g/kg/day protein prescription. By continuous renal replacement therapy day 5, median protein prescribed was > 2 g/kg/day. Protein prescription practices differed substantially between medical centers with 5 of 10 centers achieving maximal protein prescription of > 2 g/kg/day in > or = 40% of patients. Caloric prescription exceeded predicted resting energy expenditure by 30%-100%. Factors independently associated with maximal protein and caloric prescription while on continuous renal replacement therapy were younger age, initial protein and caloric prescription and number of continuous renal replacement therapy treatment days (p < 0.05)., Conclusions: Protein prescription in pediatric continuous renal replacement therapy may be inadequate. Inter-center variation exists with respect to nutrition prescription. Feeding practice standardization and research in pediatric acute kidney injury nutrition are essential to begin providing evidence-based feeding recommendations.

Children born with developmental disabilities (DD) are at risk for some common renal manifestations because of their underlying DD. These manifestations include renal stones, hypertension, and recurrent urinary tract infections. Children born with chronic kidney disease (CKD) are at risk for DD because of a myriad of issues related to their CKD, including late identification, malnourishment, and metabolic derangements. This article explores both populations from a nephrologic viewpoint.

Over the past two decades, the etiology and therapy of acute kidney injury (AKI) in children has changed. Historically, hemolytic uremic syndrome was the major cause of pediatric AKI, but advances in technology have meant that sepsis and deterioration of often unrecognized long-term organ dysfunction are now more common causes of pediatric AKI in the developed world. At the same time, major advances in renal replacement therapy in children have occurred as a result of improved strategies for vascular access, more-adaptable equipment, and better techniques and protocols. This Review outlines the etiology, incidence, diagnosis, and treatment--both dialytic and non-dialytic--of pediatric AKI.

Provision of renal replacement therapy to the critically ill child entails numerous technical and prescriptive considerations because pediatric nephrologists and intensive care physicians are faced with patients ranging in size from 2-kg neonates to 200-kg adolescents or young adults. The current article focuses on the common technical and prescriptive issues that inform the care of children with acute kidney injury.

Patients with acute kidney injury (AKI) often require initiation of renal replacement therapy (RRT). Currently, there is wide variation worldwide on the indications for and timing of initiation and discontinuation of RRT for AKI. Various parameters for metabolic, solute, and fluid control are generally used to guide the initiation and discontinuation of therapy; however, there are currently no standards in this field. Members of the recently established Acute Kidney Injury Network, representing key societies in critical care and nephrology along with additional experts in adult and pediatric AKI, participated in a 3-d conference in Vancouver in September 2006 to evaluate the available literature on this topic and draft consensus recommendations for research studies in this area. Key questions included the following: what are the indications for RRT, when should acute RRT support be initiated, and when should RRT be stopped? This report summarizes the available evidence and describes in detail the key questions, and some of the methods of answering them that will need to be addressed with the goal of standardizing the care of patients with AKI and improving outcomes.

Pediatric stem cell transplant (SCT) recipients commonly develop acute renal failure (ARF). We report the demographic and survival data of pediatric SCT patients enrolled in the Prospective Pediatric Continuous Renal Replacement Therapy (ppCRRT) Registry. Since 1 January 2001, 51/370 (13.8%) patients entered in the ppCRRT Registry had received a SCT. Median age was 13.63 (0.53-23.52) years. The primary reasons for the initiation of continuous renal replacement therapy (CRRT) were treatment of fluid overload (FO) and electrolyte imbalance (49%), FO only (39%), electrolyte imbalance only (8%) and other reasons (4%). The CRRT modalities included continuous veno-veno hemodialysis (CVVHD), 43%, continuous veno-veno hemofiltration (CVVH), 37% and continuous veno-veno hemodiafiltration (CVVHDF), 20%. Seventy-six percent had multi-organ dysfunction syndrome (MODS), 72% received ventilatory support and the mean FO was 12.41 +/- 3.70%. Forty-five percent of patients survived. Patients receiving convective therapies had better survival rates (59% vs 27%, P < 0.05). Patients requiring ventilatory support had worse survival (35% vs 71%, P < 0.05). Mean airway pressure (Paw) at the end of CRRT was lower in survivors (8.7 +/- 2.94 vs 25.76 +/- 2.03 mmH(2)O, P < 0.05). Development of high mean airway pressure in non-survivors is likely related to non-fluid injury, as it was not prevented by early and aggressive fluid management by CRRT therapy.

An infant with a suspected inborn metabolism error was treated with a metabolic cocktail of intravenous sodium phenylacetate (NaPh) and sodium benzoate (NaBz) for hyperammonemia. Sequential hemodialysis (HD) then hemofiltration (HF) was performed due to hyperammonemia. Dialytic and convective clearance (K; ml/min) of ammonia, NaPh, and NaBz was measured. The K of ammonia was 57 and 37 for HD and HF, respectively. The K of NaBz was 37 and 12 for HD and HF, respectively. The K of NaPh was 38 and 14 ml/min for HD and HF, respectively. Despite high clearance of both NaPh and NaBz by HD and HF, the hyperammonemia was corrected.