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Background Autosomal dominant polycystic kidney disease (ADPKD) is among the most common inherited kidney diseases. Hypertension is a frequent cardiovascular manifestation, especially in adults, but elevated blood pressure is also found in children and adolescents. Acknowledgment of pediatric hypertension early is critical, as it can result in serious complications long-term if left undiagnosed. Objective We aim to identify the influence of hypertension on cardiovascular outcomes, mainly left ventricular hypertrophy, carotid intima media thickness, and pulse wave velocity. Methods We performed an extensive search on Medline, Embase, CINAHL, and Web of Science databases through March 2021. Original studies with a mix of retrospective, prospective, case-control studies, cross sectional studies, and observational studies were included in the review. There was no restriction on age group. Results The preliminary search yielded 545 articles with 15 articles included after inclusion and exclusion criteria. In this meta-analysis, LVMI (SMD: 3.47 (95% CI: 0.53-6.41)) and PWV (SMD: 1.72 (95% CI: 0.08-3.36)) were found to be significantly higher in adults with ADPKD compared to non-ADPKD; however, CIMT was not found to be significantly different. Also, LVMI was observed to be significantly higher among hypertensive adults with ADPKD (n = 56) as compared to adults without ADPKD (SMD: 1.43 (95% CI: 1.08-1.79)). Fewer pediatric studies were available with heterogeneity among patient populations and results. Conclusions Adult patients with ADPKD were found to have worse indicators of cardiovascular outcomes, including LVMI and PWV, as compared to non-ADPKD. This study demonstrates the importance of identifying and managing hypertension, especially early, in this population. Further research, particularly in younger patients, is necessary to further elucidate the relationship between hypertension in patients with ADPKD and cardiovascular disease. Registration number PROSPERO REGISTRATION: 343,013., Author(s): Rupesh Raina [sup.1] [sup.2] , Raghav Shah [sup.1] , Gordon Hong [sup.3] , Girish C. Bhatt [sup.4] , Brian Abboud [sup.3] , Rohit Jain [sup.1] , Rahul Chanchlani [sup.5] [...]

Point-of-care ultrasound (POCUS) has evolved in recent years in clinical practice, helping in early bedside diagnosis of important etiologies. Many medical schools and training programs are integrating POCUS into their curriculum. Especially with the technological advances of newer handheld ultrasound devices, POCUS has now become a component adjunct to clinical examination, in the clinic and bedside in critical care units. The diagnostic utility of POCUS lies both in early identification of critical kidney disease, and also extra-renal pathologies from a focused cardiac ultrasound, lung ultrasound, and integrated fluid assessment. There is a need to incorporate POCUS in training in pediatric nephrology and establish competency standard criteria. This review shall cover how POCUS helps in enhancing patient care in pediatric kidney disorders and critical children, and the recent advances., Author(s): Sidharth Kumar Sethi [sup.1] , Rupesh Raina [sup.2] , Abhilash Koratala [sup.3] , Afagh Hassanzadeh Rad [sup.4] , Ananya Vadhera [sup.5] , Hamidreza Badeli [sup.4] Author Affiliations: (1) grid.429252.a, [...]

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 Observing biomarkers that affect alternative pathway dysregulation components may be effective in obtaining a new and more rapid diagnostic portrayal of atypical hemolytic uremic syndrome. We have conducted a systematic review on the aHUS biomarkers: C3, C5a, C5b-9, factor B, complement factor B, H, and I, CH50, AH50, d-dimer, as well as anti-CFH antibodies. Methods An exhaustive literature search was conducted for aHUS patient population plasma/serum, collected/reported at the onset of diagnosis. A total of 60 studies were included with the data on 837 aHUS subjects, with at least one biomarker reported. Results The biomarkers C3 [mean (SD): 72.1 (35.0), median: 70.5 vs. reference range: 75-175 mg/dl, n = 752]; CH50 [28.3 (32.1), 24.3 vs. 30-75 U/ml, n = 63]; AH50 [27.6% (30.2%), 10% vs. [greater than or equal to] 46%, n = 23]; and CFB [13.1 (6.6), 12.4, vs. 15.2-42.3 mg/dl, n = 19] were lower among aHUS subjects as compared with the reference range. The biomarkers including C4 [mean (SD): 20.4 (9.5), median: 20.5 vs. reference range: 14-40 mg/dl, n = 343]; C4d [7.2 (6.5), 4.8 vs. [less than or equal to] 9.8 [mu]g/ml, n = 108]; CFH [40.2 (132.3), 24.5 vs. 23.6-43.1 mg/dl, n = 123 subjects]; and CFI [8.05 (5.01), 6.55 mg/dl vs. 4.4-18.1 mg/dl, n = 38] were all observed to be within the reference range among aHUS subjects. The biomarkers C5a [mean (SD): 54.9 (32.9), median: 48.8 vs. reference range: 10.6-26.3 mg/dl, n = 117]; C5b-9 [466.0 (401.4), 317 (186-569.7) vs. [less than or equal to] 250 ng/ml, n = 174]; Bb [2.6 (2.1), 1.9 vs. [less than or equal to] 1.6 [mu]g/ml, n = 77] and d-dimer [246 (65.05), 246 vs. < 2.2 ng/ml, 2, n = 2 subjects] were higher among patients with aHUS compared with the reference range. Conclusion If a comprehensive complement profile were built using our data, aHUS would be identified by low levels of C3, CH50, AH50, and CFB along with increased levels of C5a, C5b-9, Bb, anti-CFH autoantibodies, and d-dimer. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information., Author(s): Rupesh Raina [sup.1] [sup.2] , Sidharth K. Sethi [sup.3] , Marie-Agnès Dragon-Durey [sup.4] , Amrit Khooblall [sup.1] [sup.2] , Divya Sharma [sup.5] , Priyanka Khandelwal [sup.6] , Ron Shapiro [...]

Background AKI is an important complication post cardiac surgery in children. An early diagnosis can help in mitigating complications and allow for prognostication. Urinary albumin:creatinine ratio (ACR) as a biomarker can provide a cheaper and more accessible AKI risk assessment and prediction. There is a paucity of paediatric literature regarding its utility. Methods This was a prospective observational study, enrolling all children aged 1 month to 18 years, who underwent cardiac surgery, with use of cardiopulmonary bypass. Cohort was divided into groups 75.8 mg/g) predicted post-operative AKI after adjusting for clinical variables (adjusted RR, 11.71; 1.85-16.59). In the group aged 141.3 mg/g) predicted post-operative AKI in unadjusted analysis but not after adjusting for clinical variables (RR, 2.78; 0.70-6.65). For AKI risk prediction, AUC (95% CI) was highest after combining clinical model and pre-operative ACR for groups aged < 2 years [0.805 (0.713-0.896)] and [greater than or equal to] 2 years [0.872 (0.772-0.973)]. Conclusions This study provides evidence for use of albuminuria as a feasible biomarker in AKI prediction in children post cardiac surgery, especially when added to a clinical model. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information., Author(s): Arushi Nautiyal [sup.1] , Sidharth Kumar Sethi [sup.2] , Rajesh Sharma [sup.3] , Rupesh Raina [sup.4] , Abhishek Tibrewal [sup.4] , Romel Akole [sup.3] , Aditi Gupta [sup.5] , [...]

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 [...]

Acute kidney injury (AKI) has a significant impact on the short-term and long-term clinical outcomes of pediatric and neonatal patients, and it is imperative in these populations to mitigate the pathways leading to AKI and be prepared for early diagnosis and treatment intervention of established AKI. Recently, artificial intelligence (AI) has provided more advent predictive models for early detection/prediction of AKI utilizing machine learning (ML). By providing strong detail and evidence from risk scores and electronic alerts, this review outlines a comprehensive and holistic insight into the current state of AI in AKI in pediatric/neonatal patients. In the pediatric population, AI models including XGBoost, logistic regression, support vector machines, decision trees, naïve Bayes, and risk stratification scores (Renal Angina Index (RAI), Nephrotoxic Injury Negated by Just-in-time Action (NINJA)) have shown success in predicting AKI using variables like serum creatinine, urine output, and electronic health record (EHR) alerts. Similarly, in the neonatal population, using the "Baby NINJA" model showed a decrease in nephrotoxic medication exposure by 42%, the rate of AKI by 78%, and the number of days with AKI by 68%. Furthermore, the "STARZ" risk stratification AI model showed a predictive ability of AKI within 7 days of NICU admission of AUC 0.93 and AUC of 0.96 in the validation and derivation cohorts, respectively. Many studies have reported the superiority of using biomarkers to predict AKI in pediatric patients and neonates as well. Future directions include the application of AI along with biomarkers (NGAL, CysC, OPN, IL-18, B2M, etc.) in a Labelbox configuration to create a more robust and accurate model for predicting and detecting pediatric/neonatal AKI. [ABSTRACT FROM AUTHOR]

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] , [...]

An editorial highlights the pressing need for multidisciplinary teams in caring for children with atypical hemolytic uremic syndrome (aHUS). It emphasizes the challenges faced by patients and families due to the rarity and complexity of the disease, leading to delays in accurate diagnosis and treatment. It stresses the importance of prompt and thorough diagnosis, particularly given the urgent therapeutic implications of aHUS.

Background Intentional or unintentional ingestions among children and adolescents are common. There are a number of ingestions amenable to renal replacement therapy (RRT). Methods We systematically searched PubMed/Medline, Embase, and Cochrane databases for literature regarding drugs/intoxicants and treatment with RRT in pediatric populations. Two experts from the PCRRT (Pediatric Continuous Renal Replacement Therapy) workgroup assessed titles, abstracts, and full-text articles for extraction of data. The data from the literature search was shared with the PCRRT workgroup and two expert toxicologists, and expert panel recommendations were developed. Results and Conclusions We have presented the recommendations concerning the use of RRTs for treatment of intoxications with toxic alcohols, lithium, vancomycin, theophylline, barbiturates, metformin, carbamazepine, methotrexate, phenytoin, acetaminophen, salicylates, valproic acid, and aminoglycosides., Author(s): Rupesh Raina [sup.1] [sup.2] , Manpreet K Grewal [sup.2] , Martha Blackford [sup.3] , Jordan M. Symons [sup.4] , Michael J. G. Somers [sup.5] , Christoph Licht [sup.6] , [...]

Low-density lipoprotein (LDL) apheresis has been used increasingly in clinical practice for the treatment of renal diseases with nephrotic syndrome (NS), specifically focal segmental glomerulosclerosis (FSGS). Persistent hyperlipidemia for prolonged periods is nephrotoxic and leads to chronic progressive glomerular and tubulointerstitial injury. Effective management of hyperlipidemia with HMG-CoA reductase inhibitors or LDL apheresis in drug-resistant NS patients may prevent the progression of renal disease and, in some patients, resolution of NS symptoms. Available literature reveals beneficial effects of LDL apheresis for NS refractory to drug therapy. Here we update on the current understanding of lipid nephrotoxicity and application of LDL apheresis to prevent progression of renal diseases., Author(s): Rupesh Raina [sup.1] [sup.2] , Vinod Krishnappa [sup.2] [sup.3] Author Affiliations: (Aff1) grid.413473.6, 0000 0000 9013 1194, Department of Pediatric Nephrology, Akron Children's Hospital, , Akron, OH, USA (Aff2) [...]

Background: Diuretics are commonly used in neonatal AKI with the rationale to decrease positive fluid balance in critically sick neonates. The patterns of furosemide use vary among hospitals, which necessitates the need for a well-designed study. Methods: The TINKER (The Indian Iconic Neonatal Kidney Educational Registry) study provides a database, spanning 14 centres across India since August 2018. Admitted neonates (≤ 28 days) receiving intravenous fluids for at least 48 h were included. Neonatal KDIGO criteria were used for the AKI diagnosis. Detailed clinical and laboratory parameters were collected, including the indications of furosemide use, detailed dosing, and the duration of furosemide use (in days). Results: A total of 600 neonates with AKI were included. Furosemide was used in 8.8% of the neonates (53/600). Common indications of furosemide use were significant cardiac disease, fluid overload, oliguria, BPD, RDS, hypertension, and hyperkalemia. The odds of mortality was higher in neonates < 37 weeks gestational age with AKI who received furosemide compared to those who did not receive furosemide 3.78 [(1.60–8.94); p = 0.003; univariate analysis] and [3.30 (1.11–9.82); p = 0.03]; multivariate logistic regression]. Conclusions: In preterm neonates with AKI, mortality was independently associated with furosemide treatment. The furosemide usage rates were higher in neonates with associated co-morbidities, i.e. significant cardiac diseases or surgical interventions. Sicker babies needed more resuscitation at birth, and died early, and hence needed shorter furosemide courses. Thus, survival probability was higher in neonates treated with long furosemide courses vs. short courses. [ABSTRACT FROM AUTHOR]

Volume depletion is a common condition and a frequent cause of hospitalization in children. Proper assessment of the patient includes a detailed history and a thorough physical examination. Biochemical tests may be useful in selected cases. Understanding the pathophysiology of fluid balance is necessary for appropriate management. A clinical dehydration scale assessing more physical findings may help to determine dehydration severity. Most dehydrated children can be treated orally; however, intravenous therapy may be indicated in patients with severe volume depletion, in those who have failed oral therapy, or in children with altered consciousness or significant metabolic abnormalities. Proper management consists of restoring circulatory volume and electrolyte balance. In this paper, we review clinical aspects, diagnosis, and management of children with volume depletion. [ABSTRACT FROM AUTHOR]

Intradialytic hypotension (IDH) is a common adverse event resulting in premature interruption of hemodialysis, and consequently, inadequate fluid and solute removal. IDH occurs in response to the reduction in blood volume during ultrafiltration and subsequent poor compensatory mechanisms due to abnormal cardiac function or autonomic or baroreceptor failure. Pediatric patients are inherently at risk for IDH due to the added difficulty of determining and attaining an accurate dry weight. While frequent blood pressure monitoring, dialysate sodium profiling, ultrafiltration-guided blood volume monitoring, dialysate cooling, hemodiafiltration, and intradialytic mannitol and midodrine have been used to prevent IDH, they have not been extensively studied in pediatric population. Lack of large-scale studies on IDH in children makes it difficult to develop evidence-based management guidelines. Here, we aim to review IDH preventative strategies in the pediatric population and outlay recommendations from the Pediatric Continuous Renal Replacement Therapy (PCRRT) Workgroup. Without strong evidence in the literature, our recommendations from the expert panel reflect expert opinion and serve as a valuable guide., Author(s): Rupesh Raina [sup.1] [sup.2] , Stephanie Lam [sup.3] , Hershita Raheja [sup.4] , Vinod Krishnappa [sup.2] [sup.5] , Daljit Hothi [sup.6] , Andrew Davenport [sup.7] , Deepa Chand [sup.8] [...]

Background: Cardiovascular disease (CVD) is the most common cause of mortality in chronic kidney disease (CKD). Children with early-onset CKD arguably experience the greatest lifetime CVD burden. We utilized data from the Chronic Kidney Disease in Children Cohort Study (CKiD) to evaluate two pediatric CKD cohorts: congenital anomalies of the kidney and urinary tract (CAKUT) and cystic kidney disease for CVD risks and outcomes. Methods: CVD risk factors and outcomes including blood pressures, left ventricular hypertrophy (LVH), left ventricular mass index (LVMI), and ambulatory arterial stiffness index (AASI) scores were evaluated. Results: Forty-one patients in the cystic kidney disease group were compared to 294 patients in the CAKUT group. Cystic kidney disease patients had higher cystatin-C levels, despite similar iGFR. Systolic blood pressure (SBP) and diastolic blood pressure (DBP) indexes were higher in the CAKUT group, but a significantly higher proportion of cystic kidney disease patients was on anti-hypertensive medications. Cystic kidney disease patients had increased AASI scores and a higher incidence of LVH. Conclusions: This study provides a nuanced analysis of CVD risk factors and outcomes including AASI and LVH in two pediatric CKD cohorts. Cystic kidney disease patients had increased AASI scores, higher incidence of LVH, and higher rates of anti-hypertensive medication use which could imply a greater burden of CVD despite similar GFR. Our work suggests that additional mechanisms may contribute to vascular dysfunction in cystic kidney disease, and that these patients may need additional interventions to prevent the development of CVD. [ABSTRACT FROM AUTHOR]

Wide ranges of age and weight in pediatric patients makes renal replacement therapy (RRT) in acute kidney injury (AKI) challenging, particularly in the pediatric intensive care unit (PICU), wherein children are often hemodynamically unstable. Standard hemodialysis (HD) is difficult in this group of children and continuous veno-venous hemofiltration/dialysis (CVVH/D) has been the accepted modality in the developed world. Unfortunately, due to cost constraints, CVVH/D is often not available and peritoneal dialysis (PD) remains the common mode of RRT in resource-poor facilities. Acute PD has its drawbacks, and intermittent HD (IHD) done slowly over a prolonged period has been explored as an alternative. Various modes of slow sustained IHD have been described in the literature with the recently introduced term prolonged intermittent RRT (PIRRT) serving as an umbrella terminology for all of these modes. PIRRT has been widely accepted in adults with studies showing it to be as effective as CVVH/D but with an added advantage of being more cost-effective. Pediatric data, though scanty, has been promising. In this current review, we elaborate on the practical aspects of undertaking PIRRT in children as well as summarize its current status., Author(s): Rajiv Sinha [sup.1] , Sidharth Kumar Sethi [sup.2] , Timothy Bunchman [sup.3] , Valentine Lobo [sup.4] , Rupesh Raina [sup.5] Author Affiliations: (1) 0000 0004 1801 0469, grid.414710.7, Institute [...]

Objective To study the outcome of extremely low birth weight (ELBW) infants with a history of acute kidney injury (AKI). Method In a retrospective, case control study, medical records of all ELBW infants admitted to the neonatal intensive care unit (NICU) between Jan 2002 and Dec 2011 were reviewed. Medical records were reviewed for infants' demographics, blood pressure (BP) at NICU discharge and at [greater than or equal to]3 years, and estimated glomerular filtration rate (eGFR) at [greater than or equal to]2 years. Results During the study period, 222 patients met the inclusion criteria, of whom 10% (23 out of 222) had AKI stage 2 and 3, 39% (87 out of 222) had AKI stage 1, and the rest did not have AKI. At NICU discharge, there was a difference in diastolic BP (DBP) among infants who had AKI stages 2 and 3, those who had stage 1, and those who did not have AKI (53 ± 12 vs 46 ± 9 vs 46 ± 11 mmHg respectively; p = 0.007), and 11% (23 out of 209) had hypertension (HTN). Although there was a significant correlation between the rise in SCr and DBP at NICU discharge in infants with AKI (R = 0.304; p = 0.004), there was no difference in HTN between infants with and those without AKI. At [greater than or equal to]2 years of age, 4% (5 out of 120) across all groups had an eGFR < 90 ml/min/1.73m.sup.2 or chronic kidney disease (CKD). At [greater than or equal to]3 years of age, 5% (11 out of 222) had HTN. Conclusion At NICU discharge, infants with AKI stages 2 and 3 have a higher DBP than infants with stage 1 AKI and those who did not have AKI. However, there is no difference in the rate of HTN between the two groups. At [greater than or equal to]2 years ELBW infants are at risk for CKD independently of whether or not they develop neonatal AKI., Author(s): Syeda Maqsood [sup.1] , Nicholas Fung [sup.1] , Vikas Chowdhary [sup.1] , Rupesh Raina [sup.1] , Maroun J. Mhanna [sup.1] Author Affiliations: (1) 0000 0001 2164 3847grid.67105.35Department of Pediatrics, [...]

Author(s): Sidharth Kumar Sethi [sup.1] , Norma Maxvold [sup.2] , Timothy Bunchman [sup.2] , Pranaw Jha [sup.1] , Vijay Kher [sup.1] , Rupesh Raina [sup.3] Author Affiliations: (1) grid.429252.a, 0000000417644857, [...]

The article presents answers to a clinical quiz about the case of a child with chronic kidney disease and hepatic dysfunction.

Background: Primary hyperoxalurias (PHs) constitute rare disorders resulting in abnormal glyoxalate metabolism. PH-associated phenotypes range from progressive nephrocalcinosis and/or recurrent urolithiasis to early kidney failure. Methods: A retrospective study was conducted for patients with confirmed PH diagnoses from three tertiary centers in Saudi Arabia. Detailed clinical molecular diagnosis was performed for 25 affected individuals. Whole exome sequencing (WES)–based molecular diagnosis was performed for all affected individuals. Results: The male:female ratio was 52% male (n = 13) and 48% female (n = 12), and consanguinity was present in 88%. Nephrolithiasis and/or nephrocalcinosis were present in all patients. Kidney stones were present in 72%, nephrocalcinosis in 60%, hematuria in 32%, proteinuria in 16%, abdominal pain in 36%, developmental delay in 8%, and chronic kidney disease stage 5 (CKD stage 5) was observed in 28% of the patients. The most common PH disorder was type I caused by variants in the AGXT gene, accounting for 56%. The GRHPR gene variants were identified in 4 patients, 16% of the total cases. Seven patients did not reveal any associated variants. Missense variants were the most commonly observed variants (48%), followed by frame-shift duplication variants (28%). Conclusions: Characterization of the genetic and clinical aspects of PH in this unique population provides direction for improved patient management and further research. [ABSTRACT FROM AUTHOR]

Background: Acute kidney injury (AKI) is a common complication in patients with diabetic ketoacidosis (DKA) (incidence 35–77%). AKI evolution during DKA treatment/recovery is poorly understood. Our aim was to assess children with DKA for prevalence, short-term kidney outcomes, severity, and predictors of AKI development and resolution. Methods: This retrospective cohort study included children aged 2–14 years admitted with DKA between January 2016 and May 2020 in a Saudi tertiary care hospital. We defined AKI as an increase in serum creatinine of > 1.5 times baseline or > 3 mg/dL (26 mmol/L) within 48 h. Results: Of 213 patients admitted with DKA, 172 (80.75%) developed AKI: stage 1 in 83 (38.96%), stage 2 in 86 (40.37%), and stage 3 in 3 (1.4%). No patient required dialysis. Multivariate analysis showed an increased risk of developing AKI with male gender (OR = 2.85) and lower serum bicarbonate (OR = 0.83) when adjusted for initial heart rate, hematocrit, new onset diabetes, and recurrent AKI. The mean time to AKI resolution was 13.21 ± 6.78 h. Factors leading to prolonged recovery from AKI in linear regression analysis were older age (B coefficient = 0.44, p = 0.01), recurrent DKA episodes (B coefficient = 3.70, p value 0.003), increased acidosis severity (B coefficient = − 0.44, p = 0.04), increased time to anion gap normalization (B coefficient = 0.44, p = 0.019), and increased initial glucose (B coefficient = 0.01, p = 0.011). Conclusion: In our cohort, AKI is a common, but mostly transient complication in children presenting with DKA, and its severity is associated with longer intensive care stays and time for acidosis resolution. AKI was associated with male gender, and lower serum bicarbonate. Proper consideration of such risk factors is needed for AKI assessment and management in future DKA clinical practice guidelines. A higher resolution version of the Graphical abstract is available as Supplementary information [ABSTRACT FROM AUTHOR]

Author(s): Rupesh Raina [sup.1] [sup.2] , Sidharth Kumar Sethi [sup.3] , Timothy Bunchman [sup.4] Author Affiliations: (1) grid.413473.6, 0000 0000 9013 1194, Department of Nephrology, Akron Children's Hospital, , Akron, [...]

Author(s): Vivek Sharma [sup.1] , Sidharth Kumar Sethi [sup.2] , Rupesh Raina [sup.3] , Shyam Bansal [sup.2] , S. S. Baijal [sup.1] , Vijay Kher [sup.2] Author Affiliations: (1) grid.429252.a, [...]

Author(s): Vivek Sharma [sup.1] , Sidharth Kumar Sethi [sup.2] , Rupesh Raina [sup.3] , Shyam Bansal [sup.2] , S. S. Baijal [sup.1] , Vijay Kher [sup.2] Author Affiliations: (1) grid.429252.a, [...]

A quiz about the case of a 19-month-old girl with chronic kidney disease and hepatic dysfunction is presented.

The article presents answers to a clinical quiz about a rare complication of a pauci-immune crescentic glomerulonephritis in a child.