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Kidney replacement therapy (KRT) is used to provide supportive therapy for critically ill patients with severe acute kidney injury and various other non-renal indications. Modalities of KRT include continuous KRT (CKRT), intermittent hemodialysis (HD), and sustained low efficiency daily dialysis (SLED). However, circuit clotting is a major complication that has been investigated extensively. Extracorporeal circuit clotting can cause reduction in solute clearances and can cause blood loss, leading to an upsurge in treatment costs and a rise in workload intensity. In this educational review, we discuss the pathophysiology of the clotting cascade within an extracorporeal circuit and the use of various types of anticoagulant methods in various pediatric KRT modalities., Author(s): Rupesh Raina [sup.1] [sup.2] , Ronith Chakraborty [sup.1] [sup.2] , Andrew Davenport [sup.3] , Patrick Brophy [sup.4] , Sidharth Sethi [sup.5] , Mignon McCulloch [sup.6] , Timothy Bunchman [sup.7] [...]

Background and objectives Dialysis disequilibrium syndrome (DDS) is a rare neurological complication, most commonly affecting patients undergoing new initiation of hemodialysis (HD), but can also be seen in patients receiving chronic dialysis who miss regular treatments, patients having acute kidney injury (AKI), and in those treated with continuous kidney replacement therapy (CKRT) or peritoneal dialysis (PD). Although the pathogenesis is not well understood, DDS is likely a result of multiple physiological abnormalities. In this systematic review, we provide a synopsis of the data available on DDS that allow for a clear picture of its pathogenesis, preventive measures, and focus on effective management strategies. Methods We conducted a literature search on PubMed/Medline and Embase from January 1960 to January 2021. Studies were included if the patient developed DDS irrespective of age and gender. A summary table was used to summarize the data from individual studies and included study type, population group, age group, sample size, patient characteristics, blood and dialysate flow rate, and overall outcome. A descriptive analysis calculating the frequency of population size, symptoms, and various treatments was performed using R software version 3.1.0. Results A total of 49 studies (321 samples) were identified and analyzed. Out of the included 49 studies, a total of 48 studies reported the presence of DSS among patients (1 study reported based on number of dialysis and therefore was not considered for analysis). Among these 48 studies, 74.3% (226/304) patients were reported to have DSS. The most common symptoms were nausea (25.2%), headache (24.8%), vomiting (23.9%), muscle cramps (18.1%), affected level of consciousness (8.8%), confusion (4.4%), and seizure (4.9%) among the 226 DDS patients. Furthermore, 12 studies decided to switch from HD to alternative dialysis modalities including continuous venovenous hemofiltration/hemodiafiltration (CVVH/CVVHDF) or PD which reported no DDS symptoms. Conclusion Early recognition and timely prevention are crucial for DDS patients. We have provided comprehensive clinical practice points for pediatric, adolescent, and young adult populations. However, it is essential to recognize that DDS was reported more frequently in the early dialysis era, as there was a lack of advanced dialysis technology and limited resources., Author(s): Rupesh Raina [sup.1] [sup.2] [sup.3] , Andrew Davenport [sup.4] , Bradley Warady [sup.5] , Prabhav Vasistha [sup.1] , Sidharth Kumar Sethi [sup.6] , Ronith Chakraborty [sup.1] [sup.2] , Prajit [...]

Background and objectives COVID-19 is responsible for the 2019 novel coronavirus disease pandemic. Despite the vast research about the adult population, there has been little data collected on acute kidney injury (AKI) epidemiology, associated risk factors, treatments, and mortality in pediatric COVID-19 patients admitted to the ICU. AKI is a severe complication of COVID-19 among children and adolescents. Methods A comprehensive literature search was conducted in PubMed/MEDLINE and Cochrane Center Trials to find all published literature related to AKI in COVID-19 patients, including incidence and outcomes. Results Twenty-four studies reporting the outcomes of interest were included. Across all studies, the overall sample size of COVID positive children was 1,247 and the median age of this population was 9.1 years old. Among COVID positive pediatric patients, there was an AKI incidence of 30.51%, with only 0.56% of these patients receiving KRT. The mortality was 2.55% among all COVID positive pediatric patients. The incidence of multisystem inflammatory syndrome in children (MIS-C) among COVID positive patients was 74.29%. Conclusion AKI has shown to be a negative prognostic factor in adult patients with COVID-19 and now also in the pediatric cohort with high incidence and mortality rates. Additionally, our findings show a strong comparison in epidemiology between adult and pediatric COVID-19 patients; however, they need to be confirmed with additional data and studies. Graphical abstract, Author(s): Rupesh Raina [sup.1] [sup.2] [sup.3] , Ronith Chakraborty [sup.1] [sup.2] , Isabelle Mawby [sup.1] , Nirav Agarwal [sup.1] , Sidharth Sethi [sup.4] , Michael Forbes [sup.5] Author Affiliations: (1) [...]

Background There are similarities in hemodialysis (HD) between adults and children and also unique pediatric aspects. In this systematic review, we evaluated the existing HD literature, including vascular access, indications, parameters, and outcomes as a reflection on real-life HD practices. Methods Medline, Embase, CINAHL, Web of Science, and Cochrane Library were systematically searched for literature on HD in children (1-20 years). Two reviewers independently assessed the literature and data on indications; vascular access, outcomes, and specific parameters for HD were extracted. Results Fifty-four studies (8751 patients) were included in this review. Studies were stratified into age groups 1-5, 6-12, and 13-20 years based on median/mean age reported in the study, as well as era of publication (1990-2000, 2001-2010, and 2011-2019). Across all age groups, both arteriovenous fistulas and central venous catheters were utilized for vascular access. Congenital abnormalities and glomerulopathy were the most common HD indications. HD parameters including HD session duration, dialysate and blood flow rates, urea reduction ratio, and ultrafiltration were characterized for each age group, as well as common complications including catheter dysfunction and intradialytic hypotension. Median mortality rates were 23.3% (3.3), 7.6% (14.5), and 2.0% (3.0) in ages 1-5, 6-12, and 13-20 years, respectively. Median transplantation rates were 41.6% (38.3), 52.0% (32.0), and 21% (25.6) in ages 1-5, 6-12, and 13-20, respectively. Conclusion This comprehensive systematic review summarizes available literature on HD in children and young adults, including best vascular access, indications, technical aspects, and outcomes, and reflects on HD practices over the last three decades., Author(s): Rahul Chanchlani [sup.1] [sup.2] , Claire Young [sup.3] , Aisha Farooq [sup.3] , Stephanie Sanger [sup.3] , Sidharth Sethi [sup.4] , Ronith Chakraborty [sup.5] , Abhishek Tibrewal [sup.6] , [...]

Background: Pediatric sepsis is a significant public health issue. This condition is exacerbated by rising serum creatinine and inflammatory cytokines that lead to deleterious effects upon the body. The current standard of care involves the use of continuous kidney replacement therapy to remove harmful cytokines until the body returns to homeostasis. In order to promote faster clearance and reduced stay in the ICU, high-volume hemofiltration (HVHF) has shown promise. However, there is a paucity of studies to fully elucidate its benefits. Methods: A literature search was done using PubMed/ MEDLINE and Embase. The literature was reviewed by two independent reviewers, who independently assessed the quality of randomized controlled trials by using the Cochrane risk of bias tool for RCTs and Newcastle-Ottawa Scale (NOS) for assessing the quality of nonrandomized controlled trials. Data were combined from studies with a similar design. Results: The primary endpoint of all-cause mortality was found to be reduced by 40% across all of the pooled studies. For secondary endpoints, significant reductions of serum creatinine were found. Additionally, duration of ICU stays and treatment course was found to be significantly shorter in HVHF patients than the current standard of care. The rate of adverse effects was analyzed, and there was no difference in the proportion of patients developing hypokalemia, hyperkalemia, hypernatremia, or hyponatremia. The proportion of patients developing hyperglycemia was higher in patients undergoing HVHF, whereas the proportions of patients developing bleeding were significantly less in patients undergoing HVHF. One study reported a total number of adverse events between the two groups which were significantly lesser in patients undergoing HVHF. Conclusion: HVHF shows promise as a modality to treat pediatric patients with sepsis. In order to confirm the benefits of this modality, future studies need significantly more patients for analysis. [ABSTRACT FROM AUTHOR]

COVID‐19 has challenged the global healthcare system through rapid proliferation and lack of existing treatment resulting in over 180 million cases and 3.8 million deaths since December 2019. Although pediatric patients only comprise 1%–2% of diagnosed cases, their incidence of acute kidney injury ranges from 8.2% to 18.2% compared to 49% in adults. Severe infection, initiated by dysregulated host response, can lead to multiorgan failure. In this review, we focus on the use of various blood filters approved for use in pediatric kidney replacement therapy to mitigate adverse effects of severe illness. Therapeutic effects of these blood filters range from cytokine removal (CytoSorb, HA330, HCO/MCO), endotoxin removal (Toraymyxin, CPFA), both cytokine and endotoxin removal (oXiris), and nonspecific removal of proteins (PMMA) that have already been established and can be used to mitigate the various effects of the cytokine storm syndrome in COVID‐19. [ABSTRACT FROM AUTHOR]

Background and Objectives: The recent worldwide pandemic of COVID-19 has been a serious, multidimensional problem that has left a detrimental worldwide impact on individuals of all ages and several organ systems. The typical manifestation of kidney involvement is acute kidney injury (AKI); however, there is a lack of consensus data regarding AKI epidemiology in COVID-19. This systematic literature review aims to bridge this knowledge gap. Design, Setting, Participants, and Measurements: MEDLINE and Cochrane library were systematically searched for the literature related to AKI in COVID-19 patients of all ages. MedRxIV was searched for relevant unpublished manuscripts. Two reviewers independently assessed the literature on the incidence of AKI and mortality, extracting the need for kidney replacement therapy (KRT). Results: Sixty studies (n = 43,871 patients) were included in this review. The pooled incidence of AKI among COVID-19 patients was 19.45% (95% confidence intervals [95% CI]: 14.63–24.77%), while the pooled incidence of AKI COVID-19 patients requiring KRT was 39.04% (16.38–64.57%). The pooled proportion of COVID+ patients was significantly lower at 8.83% (5.64% to 12/66%). The overall mortality of COVID-19 patients was calculated to be 17.71% (95% CI: 11.49–24.93%), while the mortality among patients with AKI was higher at 54.24% (95% CI: 44.70–63.63%). Conclusion: This comprehensive systematic review summarizes the available literature pertaining to AKI epidemiology in COVID-19 patients and highlights the incidence, associated mortality, and the need for KRT in this susceptible population. [ABSTRACT FROM AUTHOR]

Background: Despite extensive research into acute kidney injury (AKI) in adults, research into the epidemiology, associated risk factors, treatment, and mortality of AKI in pediatric COVID-19 patients is understudied. Advancing understanding of this disease is crucial to further developing treatment and preventative care strategies to reduce morbidity and mortality. Methods: This is a retrospective analysis of 2,546 COVID-19 pediatric patients (age ≤ 21 years) who were admitted the ICU in North America. Analysis of the Virtual Pediatric Systems (VPS) COVID-19 database was conducted between January 1, 2020, and June 30, 2021. Results: Out of a total of 2,546 COVID positive pediatric patients, 10.8% (n = 274) were diagnosed with AKI. Significantly higher continuous and categorical outcomes in the AKI subset compared to the non-AKI cohort included: length of stay at the hospital (LOS) [9.04 (5.11–16.66) vs. 5.09 (2.58–9.94) days], Pediatric Index of Mortality (PIM) 2 probability of death [1.20 (0.86–3.83) vs. 0.96 (0.79–1.72)], PIM 3 probability of death [0.98 (0.72–2.93) vs. 0.78 (0.69–1.26)], mortality [crude OR (95% CI): 5.01 (2.89–8.70)], airway and respiratory support [1.63 (1.27–2.10)], cardio-respiratory support [3.57 (1.55–8.23)], kidney support [12.52 (5.30–29.58)], and vascular access [4.84 (3.70–6.32)]. Conclusions: This is one of the first large scale studies to analyze AKI among pediatric COVID-19 patients admitted to the ICU in North America. Although the course of the COVID-19 virus appears milder in the pediatric population, renal complications may result, increasing the risk of disease complication and mortality. [ABSTRACT FROM AUTHOR]

Kidney replacement therapy (KRT) is a common supportive treatment for renal dysfunction, especially acute kidney injury. However, critically ill or immunosuppressed patients with renal dysfunction often have dysfunction in other organs as well. To improve patient outcomes, clinicians began to initiate kidney replacement therapy in situations where nonrenal conditions may lead to acute kidney injury, such as septic shock, hematopoietic stem cell transplantation, veno‐occlusive renal disease, cardiopulmonary bypass, chemotherapy, tumor lysis syndrome, hyperammonemia, and various others. In this review, we discuss the use of various modes of kidney replacement therapy in treating renal and nonrenal complications to illustrate why kidney support therapy is a more appropriate terminology than kidney replacement therapy. [ABSTRACT FROM AUTHOR]

Kidney transplantation is the preferred treatment of end‐stage renal disease in children. However, time to transplant varies, making a well‐functioning long‐term vascular access essential for performing hemodialysis efficiently and without disruption until a kidney becomes available. However, establishing long‐term vascular access in pediatric patients can present distinct challenges due to this population's unique characteristics, such as smaller body size and lower‐diameter blood vessels. There are three main pediatric long‐term vascular access options, which include central venous catheters (CVC), arteriovenous fistula (AVF), and arteriovenous graft (AVG). CVC are currently the most widely used modality, although various studies and guidelines recommend AVF or AVG as the preferred option. Although AVF should be used whenever possible, it is crucial that clinicians consider factors such as patient size, physical exam findings, comorbidities, predicted duration of treatment to decide on the most optimal long‐term vascular access modality. This article reviews the three long‐term vascular access methods in children and the benefits and complications of each. [ABSTRACT FROM AUTHOR]

Focal segmental glomerulosclerosis (FSGS) is one of the most frequent and severe glomerular kidney disease with frequent progression to end-stage renal disease and a high rate of recurrence in renal transplantations. Due to intolerance or resistance to the current immunomodulatory treatments, the management of FSGS is a therapeutic challenge. Over the last few years, development in extracorporeal therapies has shown potential beneficial outcomes in drug-resistant and recurrent FSGS patients. Thus, this study reviews the current literature on the use of extracorporeal therapies, such as plasma exchange therapy, immunoadsorption, and low-density lipoprotein apheresis, for the treatment of FSGS in the pediatric population. [ABSTRACT FROM AUTHOR]

Background. In recent years, the use of adrenocorticotropic hormone (ACTH) therapy for treatment of proteinuria due to nephrotic syndrome (NS) has been heavily explored. ACTH therapy, which comes in the natural (H. P. Acthar Gel) or synthetic (tetracosactide) form, has resulted in remission in patients with immunosuppressive and steroid-resistant NS. However, the exact efficacy of ACTH therapy in the NS etiologies, such as membranous nephropathy (MN), focal segmental glomerulosclerosis (FSGS), minimal change disease (MCD), lupus nephritis (LN), IgA nephropathy (IgAN), and membranoproliferative glomerulonephritis (MPGN), has not been determined. Objective. This systematic review analyzed the published literature on ACTH therapy in various NS etiologies to determine its efficacy. Methods. A comprehensive search of MEDLINE, EMBASE, and Cochrane databases was conducted for articles through June 2019. An additional search was performed on clinicaltrials.gov to search for additional trials and cross reference the results of our database search. The literature which studied synthetic or natural ACTH treatment in patients with known etiologies of NS was included. Studies were excluded when they consisted of a single case report or did not analyze the lone effect of ACTH in NS. Results. The initial search yielded a total of 411 papers, and 22 papers were included. In 214 MN patients, there was an overall remission of 40% (85/214) and an overall remission of 43% (42/98) in FSGS patients. In other etiologies, there were overall remissions of 78% (11/14), 31% (5/16), 40% (16/40), and 62% (8/13) in MCD, LN, IgAN, and MPGN patients, respectively. Conclusion. ACTH showed benefits in proteinuria reduction across all etiologies of NS. However, more randomized controlled studies with larger population sets and longer follow-ups are imperative to establish causal benefits. New studies into its efficacy in children are also necessary. [ABSTRACT FROM AUTHOR]

The morbidity and mortality of adult and pediatric chronic kidney disease (CKD) and end-stage renal disease (ESRD) populations are mainly driven by cardiovascular disease (CVD). Improving CVD outcomes focuses on risk assessment of factors including diastolic blood pressure (DBP), systolic blood pressure (SBP), left ventricular mass index (LVMI), pulse pressure (PP), and pulse pressure index (PPi), which is calculated as PP/SBP. These markers are also proven predictors of CKD progression; however, their role in children has not been established. This study aims to evaluate the relationship between PP, PPi, ambulatory arterial stiffness index (AASI), and proteinuria with kidney function in pediatric CKD patients; it is a retrospective analysis of 620 patients (1-16 years) from the NIDDK Chronic Kidney Disease in Children (CKiD) registry. The authors analyzed data for three separate cohorts: an overall CKD as well as immunological versus non-immunological cause for CKD groups. An inverse relationship was found between SBP, DBP, and PP with iGFR and LVMI in the overall CKD group. Our immunological CKD subgroup showed significantly higher serum creatinine, SBP, DBP, and PP values with significantly lower serum albumin levels compared to the non-immunological group. There were no significant differences with iohexol-based glomerular filtration rate (iGFR), LVMI, PPi, or high-sensitivity C-reactive protein (hs-CRP) between the two groups. A subgroup analysis demonstrated that SBP, DBP, and PP all correlated significantly with LVMI in the immunological CKD patients but not the non-immunological subgroup. Additionally, AASI data in the overall CKD population were significantly correlated with PP, PPi, and DBP. This study is one of the first to correlate noninvasive measurements of vascular compliance including PP, PPi, and AASI with iGFR and LVMI in a pediatric CKD cohort. Improving our understanding of surrogate markers for early CVD is integral to improving the care of pediatric CKD population as these patients have yet to develop the hard end points of ESRD, heart failure, myocardial infarction, or stroke. [ABSTRACT FROM AUTHOR]

Chronic kidney disease and end-stage renal disease (ESRD) in children are major health concerns worldwide with increasing incidence and prevalence. Renal replacement therapies and kidney transplants have remarkably improved the management of patients with ESRD in both adult and pediatric populations. Kidney transplant has the best patient outcomes, but many a time it has a considerable waiting period. In the meantime, the majority of patients with pediatric ESRD are dependent on dialysis. The conventionally utilized hemodialysis regimen is the three times weekly, in-center hemodialysis. Many studies have demonstrated the unfavorable long-term morbidity associated with the conventional regimen. Intensified dialysis programs, which include extended nocturnal hemodialysis or short daily hemodialysis, are being increasingly advocated over the past two decades. In addition to having much better clinical outcomes as compared with the conventional regimen, the flexibility to provide dialysis at home serves as a great incentive. PubMed/Medline, Embase and Cochrane databases for literature on nocturnal home hemodialysis in children with ESRD were extensively searched. Contrary to the noticeable literature available on adult home hemodialysis, a small number of studies exist in the pediatric population. In this review, the benefits, implementation and associated barriers of nocturnal home hemodialysis in children were addressed. [ABSTRACT FROM AUTHOR]

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.)

Selective autophagy is a conserved subcellular process that maintains the health of eukaryotic cells by targeting damaged or toxic cytoplasmic components to the vacuole/lysosome for degradation. A key player in the initiation of selective autophagy in S. Cerevisiae (baker's yeast) is a large adapter protein called Atg11. Atg11 has multiple predicted coiled-coil domains and intrinsically disordered regions, is known to dimerize, and binds and organizes other essential components of the autophagosome formation machinery, including Atg1 and Atg9. We performed systematic directed mutagenesis on the coiled-coil 2 domain of Atg11 in order to map which residues were required for its structure and function. Using yeast-2-hybrid and coimmunoprecipitation, we found only three residues to be critical: I562, Y565, and I569. Mutation of any of these, but especially Y565, could interfere with Atg11 dimerization and block its interaction with Atg1 and Atg9, thereby inactivating selective autophagy., 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 Meyer, Winzeler, Taylor, Kilgore, Edicha, Chitwood, Spearin, Silvia, Chakraborty, Smith, Kennedy, Zois, Cawthon, Gilruth and Backues.)

Background/Introduction: Renal angina index (RAI) used to calculate and accurately predict risk for the development of acute kidney injury (AKI) has been heavily explored. AKI is traditionally diagnosed by an increase in serum creatinine (SCr) concentration or oliguria, both of which are neither specific nor sensitive, especially among children. An RAI score may be calculated by combining objective signs of kidney dysfunction (such as SCr) and patient context, such as AKI risk factors, thus potentially serving as a more accurate indicator for AKI. Objective: Due to the propitious and novel nature of RAI, this editorial commentary aims to analyze the current literature on RAI and determine how well RAI serves as a predictor of AKI outcomes. Method: A comprehensive literature search was conducted in PubMed/Medline and Google Scholar between January 2012 and July 2020. Literature included the prognostic aspect of early prediction of AKI in the pediatric and adult population via RAI. Results: The initial literature search included 149 studies, and a total of 10 studies reporting the outcomes of interest were included. The overall sample size across these studies was 11,026. The predictive ability of RAI had a pooled (95% CI) sensitivity of 79.21%, specificity of 73.22%, and negative predictive value of 94.83%. Conclusion: RAI shows benefit in the prediction of AKI among adult and pediatric populations. However, there is a lack of sufficient data, and further prospective studies are needed in pediatric populations to use RAI as a principal AKI indicator among clinicians., 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 Raina, Sethi, Mawby, Datla, Kumar, Agarwal, Tibrewal and Chakraborty.)

Renal cystic disease encompasses a large variety of illnesses with various phenotypic expressions that can manifest in utero, in infancy, and in childhood. These diseases may be unilateral or bilateral and present with single or multiple cysts. Various cystic diseases may also progress to chronic kidney disease (CKD), including kidney failure, and hepatic disease, thus potentially being life threatening. The prevalence and serious complications of CKD in the pediatric population make it vital that health care providers detect these conditions early and provide effective management. This installment of AJKD's Core Curriculum in Nephrology discusses various genetic and sporadic kidney cystic diseases, including multicystic dysplastic kidney, nephronophthisis, cystic dysplasia, hepatocyte nuclear factor 1-β (HNF1-β) nephropathy, Bardet-Biedl syndrome, Meckel-Gruber syndrome, Zellweger syndrome, calyceal diverticulum, autosomal recessive polycystic kidney disease (ARPKD), and autosomal dominant polycystic kidney disease (ADPKD). This article discusses the epidemiology, genetics and pathophysiology, diagnosis, presentation, and management for each of these renal cystic diseases, with particular attention to prenatal care and pregnancy counseling., (Copyright © 2021 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.)

Although the use of telemedicine in rural areas has increased steadily over the years, its use was rapidly implemented during the onset of the coronavirus disease 2019 (COVID-19) crisis. Due to this rapid implementation, there is a lack of standardized work flows to assess and treat for various nephrotic conditions, symptoms, treatment modalities, and transition processes in the pediatric population. To provide a foundation/suggestion for future standardized work flows, the authors of this report have developed standardized work flows using the Delphi method. These work flows were informed based on results from cross-sectional surveys directed to patients and providers. Most patients and providers were satisfied, 87% and 71%, respectively, with their telemedicine visits. Common issues that were raised with the use of telemedicine included difficulty procuring physical laboratory results and a lack of personal warmth during telemedicine visits. The work flows created based on these suggestions will both enhance safety in treating patients and allow for the best possible care., (© 2021 The Authors.)

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.

Hypertensive crisis can be a source of morbidity and mortality in the pediatric population. While the epidemiology has been difficult to pinpoint, it is well-known that secondary causes of pediatric hypertension contribute to a greater incidence of hypertensive crisis in pediatrics. Hypertensive crisis may manifest with non-specific symptoms as well as distinct and acute symptoms in the presence of end-organ damage. Hypertensive emergency, the form of hypertensive crisis with end-organ damage, may present with more severe symptoms and lead to permanent organ damage. Thus, it is crucial to evaluate any pediatric patient suspected of hypertensive emergency with a thorough workup while acutely treating the elevated blood pressure in a gradual manner. Management of hypertensive crisis is chosen based on the presence of end-organ damage and can range from fast-acting intravenous medication to oral medication for less severe cases. Treatment of such demands a careful balance between decreasing blood pressure in a gradual manner while preventing damage end-organ damage. In special situations, protocols have been established for treatment of hypertensive crisis, such as in the presence of endocrinologic neoplasms, monogenic causes of hypertension, renal diseases, and cardiac disease. With the advent of telehealth, clinicians are further able to extend their reach of care to emergency settings and aid emergency medical service (EMS) providers in real time. In addition, further updates on the evolving topic of hypertension in the pediatric population and novel drug development continues to improve outcomes and efficiency in diagnosis and management of hypertension and consequent hypertensive crisis., (Copyright © 2020 Raina, Mahajan, Sharma, Chakraborty, Mahajan, Sethi, Kapur and Kaelber.)

Acute kidney injury (AKI) in the pediatric population is a relatively common phenomenon. Specifically, AKI has been found in increasing numbers within the pediatric population following cardiac surgery, with up to 43% of pediatric patients developing AKI post-cardiac surgery. However, recent advances have allowed for the identification of risk factors. These can be divided into preoperative, intraoperative, and postoperative factors. Although the majority of pediatric patients developing AKI after cardiac surgery completely recover, this condition is associated with worse outcomes. These include fluid overload and increased mortality and result in longer hospital and intensive care unit stays. Detecting the presence of AKI has advanced; use of relatively novel biomarkers, including neutrophil gelatinase associated lipocalin, has shown promise in detecting more subtle changes in kidney function when compared to conventional methods. While a single, superior treatment has not been elucidated yet, novel functions of medications, including fenoldopam, theophylline and aminophylline, have been shown to have better outcomes for these patients. With the recent advances in identification of risk factors, outcomes, diagnosis, and management, the medical community can further explain the complexities of AKI in the pediatric population post-cardiac surgery.

Tuberous sclerosis complex (TSC) is a genetic condition caused by a mutation in either the TSC1 or TSC2 gene. Disruption of either of these genes leads to impaired production of hamartin or tuberin proteins, leading to the manifestation of skin lesions, tumors, and seizures. TSC can manifest in multiple organ systems with the cutaneous and renal systems being the most commonly affected. These manifestations can secondarily lead to the development of hypertension, chronic kidney disease, and neurocognitive declines. The renal pathologies most commonly seen in TSC are angiomyolipoma, renal cysts, and less commonly, oncocytomas. In this review, we highlight the current understanding on the renal manifestations of TSC along with current diagnosis and treatment guidelines., Competing Interests: None of the authors of this manuscript have any conflict of interests to disclose., (Copyright: Nair N et al.)

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.)

Cardiorenal syndrome (CRS) encompasses various disorders of the heart and kidneys; dysfunction of one organ leads to acute or chronic dysfunction of the other. It incorporates the intersection of heart-kidney interactions across several mediums, hemodynamically, through the alterations in neurohormonal markers, and increased venous and renal pressure, all of which are hallmarks of its clinical phenotypes. This article explores the epidemiology, pathology, classification and treatment of each type of CRS., Competing Interests: The authors have no conflict of interest to declare., (Copyright 2020, Raina et al.)

Background: Neonatal Acute Kidney Injury (AKI) occurs in 40-70% of critically ill newborn infants and is independently associated with increased morbidity and mortality. Understanding the practice patterns of physicians (neonatologists and pediatricians), caring for neonates in India is important to optimize care and outcomes in neonatal AKI. Aim: The aim of this study was to identify differences in physician's perception and practice variations of diagnosis, management, and follow-up of newborn infants with AKI in India. Methods: An online survey of neonatologists and pediatricians in India caring for newborn infants with AKI. Results: Out of 800 correspondents, 257 (135 neonatologists and 122 pediatricians) completed the survey, response rate being 32.1%. Resources available to the respondents included level III NICU (59%), neonatal surgery (60%), dialysis (11%), and extracorporeal membrane oxygenation (ECMO, 3%). Most respondents underestimated the risk of AKI due to various risk factors such as prematurity, asphyxia, sepsis, cardiac surgery, and medications. Less than half the respondents were aware of the AKIN or KDIGO criteria, which are the current standard criteria for defining neonatal AKI. Only half of the respondents were aware of the risk of CKD in preterm neonates and nearly half were unaware of the need to follow up with a pediatric nephrologist. Conclusions: Similar to other regions worldwide, there exists a knowledge gap in early recognition, optimal management and follow up of newborn infants with AKI amongst Indian physicians., (Copyright © 2020 Sethi, Agrawal, Wazir, Rohatgi, Iyengar, Chakraborty, Jain, Nair, Sinha, Chakrabarti, Kumar and Raina.)

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.)