Publications by authors named "Rupesh Raina"

139 Publications

Post Cardiac Surgery.

Kidney Int Rep 2021 Jul 28;6(7):1850-1857. Epub 2021 Apr 28.

Department of Nephrology, Akron's Children Hospital, Akron, Ohio, USA.

Introduction: The long-term renal outcomes of survivors of pediatric acute kidney injury (AKI) are varied within the current literature, and we aim to establish long-term renal outcomes for pediatric patients after cardiac surgery. We studied long-term renal outcomes and markers of kidney injury in pediatric patients after congenital cardiac surgery.

Methods: In a prospective case-control observational study (the Renal Outcomes in Children with acute Kidney injury post cardiac Surgery [ROCKS] trial) we reviewed all children who underwent cardiac surgery on cardiopulmonary bypass (December 2010-2017).

Results: During the study period, 2035 patients underwent cardiac surgery, of whom 9.8% developed AKI postoperatively. Forty-four patients who had postoperative AKI had a long-term follow-up, met our inclusion criteria, and were compared with 49 control subjects. We conducted a univariate analysis of reported parameters. At a median follow-up of 41 months, the cases had significantly higher urine levels of neutrophil gelatinase-associated lipocalin (NGAL), interleukin-18 (IL-18), and kidney injury molecule-1 (KIM-1). The biomarkers remained higher after adjusting for the urine creatinine, and the ratio of urine KIM-1/urine creatinine was significantly higher among cases. None of the patients had proteinuria or hypertension on follow-up. The presence of AKI, AKI stage, and younger age were not associated with the occurrence of low glomerular filtration rate (GFR) at follow-up.

Conclusions: Urinary biomarker abnormalities persist years after a congenital cardiac surgery in children, who may have a low GFR on follow-up. The presence of AKI, AKI stage, and younger age at surgery are not associated with the occurrence of low GFR at follow-up. Children with a higher surgical complexity score have lower GFR on follow-up.
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http://dx.doi.org/10.1016/j.ekir.2021.04.018DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8258583PMC
July 2021

Incidence, Risk Factors, and Outcomes of Neonatal Acute Kidney Injury: Protocol of a Multicentric Prospective Cohort Study [The Indian Iconic Neonatal Kidney Educational Registry].

Front Pediatr 2021 9;9:690559. Epub 2021 Jul 9.

Akron's Children Hospital, Akron, OH, United States.

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. 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. 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.
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http://dx.doi.org/10.3389/fped.2021.690559DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8300429PMC
July 2021

Conversion to permanent vascular access is associated with improved markers of hemodialysis efficacy in children: Pediatric nephrology research consortium study.

Clin Nephrol 2021 Jun 30. Epub 2021 Jun 30.

Background And Objectives: Arteriovenous fistulae (AVF) and grafts (AVG) are preferred permanent vascular access (PVA) for chronic hemodialysis (HD) patients. Our objective was to examine the change in markers of HD efficacy after successful establishment of a PVA among children who started HD with a tunneled cuffed catheter (TCC).

Materials And Methods: Retrospective chart reviews were completed on patients from 20 pediatric dialysis centers. All patients used TCC prior to AVF/AVG, and each patient acted as his/her own control. Data on markers of HD efficacy (single-pool Kt/V, urea reduction ratio (URR), serum albumin and hematocrit (Hct)) were collected at the creation of AVF/AVG and for 2 years thereafter. Statistical methods included hypothesis testing and statistical modeling after adjusting for relevant demographic variables.

Results: First PVA was created in 98 individual children: 87 (89%) were AVF and 11 (11%) were AVG. The mean TCC vintage prior to AVF/AVG was 10.4 ± 17.3 months. At 1-year follow-up, Kt/V improved by 0.15 ± 0.06 (p = 0.02) and URR improved by 4.54 ± 1.17% (p < 0.0001). Furthermore, PVA was associated with improved serum albumin by 0.31 ± 0.07 g/dL (p < 0.0001) and Hct by 2.80 ± 0.65% (p < 0.0001) at 1 year. These HD efficacy markers remained statistically significant at 2nd-year follow-up. These observations were further supported by the adjusted models. Conversion to AVF was associated with statistically significant improvement in all four markers of HD efficacy at 1-year follow-up. This trend was not demonstrated for subjects who were converted to AVG.

Conclusion: Switching to PVA was associated with improved markers of HD efficacy, single-pool Kt/V, URR, serum albumin, and Hct. This improvement was mostly demonstrated at 1year and maintained for the 2nd year. The potential differential impact of the type of PVA on the trajectory of markers of HD efficacy should be further investigated.
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http://dx.doi.org/10.5414/CN110455DOI Listing
June 2021

Incidence and Outcomes of Acute Kidney Injury in COVID-19: A Systematic Review.

Blood Purif 2021 Jun 15:1-14. Epub 2021 Jun 15.

Division of Nephrology, Bone and Mineral Metabolism, University of Kentucky Medical Center, Lexington, Kentucky, USA.

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.
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http://dx.doi.org/10.1159/000514940DOI Listing
June 2021

Use of rituximab in paediatric nephrology.

Arch Dis Child 2021 Jun 10. Epub 2021 Jun 10.

Department of Paediatric Nephrology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK

Rituximab is a chimeric monoclonal antibody capable of depleting B cell populations by targeting the CD20 antigen expressed on the cell surface. Its use in oncology, initially in B cell lymphoma and post-transplant lymphoproliferative disorders, predates its current utility in various fields of medicine wherein it has become one of the safest and most effective antibody-based therapies. It was subsequently found to be effective for rheumatological conditions such as rheumatoid arthritis and antineutrophil cytoplasmic antibody-associated vasculitis. Over the past decade, rituximab has generated a lot of interest in nephrology and has become an emerging or accepted therapy for multiple renal conditions, including systemic lupus erythematosus, lupus nephritis, vasculitis, nephrotic syndrome and in different scenarios before and after kidney transplantation. This review outlines its current use in paediatric nephrology practice, focusing on the knowledge required for general paediatricians who may be caring for children prescribed this medication and reviewing them on a shared care basis.
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http://dx.doi.org/10.1136/archdischild-2020-321211DOI Listing
June 2021

Neonatal acute kidney injury risk stratification score: STARZ study.

Pediatr Res 2021 May 19. Epub 2021 May 19.

Akron's Children Hospital, Akron, OH, USA.

Background: Neonates admitted in the neonatal intensive care unit are vulnerable to acute kidney injury leading to worse outcomes. It is important to identify "at-risk" neonates for early preventive measures.

Methods: The study was a multicenter, national, prospective cohort study done in 11 centers in India. A multivariable logistic regression technique with step-wise backward elimination method was used, and a "Risk Prediction Scoring" was devised [the STARZ score].

Results: The neonates with admission in the NICU within <25.5 h of birth, requirement of positive pressure ventilation in the delivery room, <28 weeks gestational age, sepsis, significant cardiac disease, urine output <1.32 ml/kg/h or serum creatinine ≥0.98 mg/dl during the first 12 h post admission, use of nephrotoxic drugs, use of furosemide, or use of inotrope had a significantly higher risk of AKI at 7 days post admission in the multivariate logistic regression model. This scoring model had a sensitivity of 92.8%, specificity of 87.4% positive predictive value of 80.5%, negative predictive value of 95.6%, and accuracy of 89.4%.

Conclusions: The STARZ neonatal score serves to rapidly and quantitatively determine the risk of AKI in neonates admitted to the neonatal intensive care unit.

Impact: The STARZ neonatal score serves to rapidly and quantitatively determine the risk of AKI in neonates admitted to the neonatal intensive care unit. These neonates with a higher risk stratification score need intense monitoring and daily kidney function assessment. With this intensification of research in the field of AKI risk stratification prediction, there is hope that we will be able to decrease morbidity and mortality associated with AKI in this population.
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http://dx.doi.org/10.1038/s41390-021-01573-9DOI Listing
May 2021

AGREEing on clinical practice guidelines for idiopathic steroid-sensitive nephrotic syndrome in children.

Syst Rev 2021 05 10;10(1):144. Epub 2021 May 10.

Pediatrics Department, King Khalid University Hospital, Riyadh, Saudi Arabia.

Background: Nephrotic syndrome is the most common kidney disease in children worldwide. Our aim was to critically appraise the quality of recent Clinical Practice Guidelines (CPGs) for idiopathic steroid-sensitive nephrotic syndrome (SSNS) in children in addition to summarize and compare their recommendations.

Methods: Systematic review of CPGs. We identified clinical questions and eligibility criteria and searched and screened for CPGs using bibliographic and CPG databases. Each included CPG was assessed by four independent appraisers using the Appraisal of Guidelines for REsearch & Evaluation II (AGREE-II) instrument. We summarized the recommendations in a comparison practical table.

Results: Our search retrieved 282 citations, of which three CPGs were eligible and appraised: Kidney Disease: Improving Global Outcomes (KDIGO) 2012, Japan Society for Pediatric Nephrology (JSPN) 2014, and American Academy of Pediatrics (AAP) 2009. Among these, the overall assessment of two evidence-based CPGs scored > 70% (KDIGO and JSPN), which was consistent with their higher scores in the six domains of the AGREE II Instrument. In domain 3 (rigor of development), KDIGO, JSPN, and AAP scored 84%, 74%, and 41%, respectively. In domain 5 (applicability), they scored 22%, 16%, and 19%, respectively, and in domain 6 (editorial independence), they scored 94%, 65%, and 88%, respectively.

Conclusions: The methodological quality of the KDIGO CPG was superior, followed by JSPN and AAP CPGs with the relevant recommendations for use in practice.

Systematic Review Registration: The protocol was registered in the Center for Open Science (OSF) DOI: 10.17605/OSF.IO/6QTMD and in the International prospective register of systematic reviews PROSPERO 2020 CRD42020197511 .
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http://dx.doi.org/10.1186/s13643-021-01666-wDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8112064PMC
May 2021

Critical analysis of acute kidney injury in pediatric COVID-19 patients in the intensive care unit.

Pediatr Nephrol 2021 Apr 29. Epub 2021 Apr 29.

Director of Clinical Research and Outcomes Analysis, Department of Pediatrics, Akron Children's Hospital, Akron, OH, USA.

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.
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http://dx.doi.org/10.1007/s00467-021-05084-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8083920PMC
April 2021

Telemedicine for Pediatric Nephrology: Perspectives on COVID-19, Future Practices, and Work Flow Changes.

Kidney Med 2021 May-Jun;3(3):412-425. Epub 2021 Mar 28.

Department of Hematology Oncology, Akron Children's Hospital, Akron, OH.

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.
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http://dx.doi.org/10.1016/j.xkme.2021.01.007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8004477PMC
March 2021

Advances in pediatric acute kidney injury.

Pediatr Res 2021 Mar 17. Epub 2021 Mar 17.

Pediatric Nephrology & Transplantation, Children's Hospital of Richmond at VCU, Richmond, VA, USA.

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.
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http://dx.doi.org/10.1038/s41390-021-01452-3DOI Listing
March 2021

A Meta-Analysis of Extracorporeal Anticoagulants in Pediatric Continuous Kidney Replacement Therapy.

J Intensive Care Med 2021 Mar 10:885066621992751. Epub 2021 Mar 10.

Division of Critical Care Medicine, Summa Health, Akron, OH, USA.

Objective: Continuous kidney replacement therapy (CKRT) is the primary therapeutic modality utilized in hemodynamically unstable patients with severe acute kidney injury. As the circuit is extracorporeal, it poses an increased risk of blood clotting and circuit loss; frequent circuit losses affect the provider's ability to provide optimal treatment. The objective of this meta-analysis is to evaluate the safety and efficacy of the extracorporeal anticoagulants in the pediatric CKRT population.

Data Sources: We conducted a literature search on PubMed/Medline and Embase for relevant citations.

Study Selection: Studies were included if they involved patients under the age of 18 years undergoing CKRT, with the use of anticoagulation (heparin, citrate, or prostacyclin) as a part of therapy. Only English articles were included in the study.

Data Extraction: Initial search yielded 58 articles and a total of 24 articles were included and reviewed. A meta-analysis was performed focusing on the safety and effectiveness of regional citrate anticoagulation (RCA) vs unfractionated heparin (UFH) anticoagulants in children.

Data Synthesis: RCA had statistically significantly longer circuit life of 50.65 hours UFH of 42.10 hours. Two major adverse effects metabolic alkalosis and electrolyte imbalance seen more commonly in RCA compared to UFH. There was not a significant difference in the risk of systemic bleeding when comparing RCA . UFH.

Conclusion: RCA is the preferred anticoagulant over UFH due to its significantly longer circuit life, although vigilant circuit monitoring is required due to the increased risk of electrolyte disturbances. Prostacyclin was not included in the meta-analysis due to the lack of data in pediatric patients. Additional studies are needed to strengthen the study results further.
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http://dx.doi.org/10.1177/0885066621992751DOI Listing
March 2021

Pediatric acute kidney injury: new advances in the last decade.

Kidney Res Clin Pract 2021 Mar 3;40(1):40-51. Epub 2021 Mar 3.

Akron Nephrology Associates and Cleveland Clinic Akron General Medical Center, Akron, OH, USA.

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.
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http://dx.doi.org/10.23876/j.krcp.20.074DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8041642PMC
March 2021

The significance of anti-phospholipase receptor antibodies in a patient with membranous nephropathy.

Saudi J Kidney Dis Transpl 2020 Nov-Dec;31(6):1415-1419

Department of Nephrology, Akron Nephrology Associates/Cleveland Clinic Akron General; Department of Nephrology, Akron Children's Hospital, Akron, Ohio, USA.

Membranous nephropathy (MN) is the major cause of nephrotic syndrome in adults and may be secondary to systemic lupus erythematosus or malignancy in 25% of patients. Without any etiology, it is called primary MN, which is usually associated with phospholipase A2 (PLA2) receptor antibodies. Secondary MN can appear months before a secondary cause is identified. Here, we report a case of MN, that was found to be secondary to pancreatic adenocarcinoma and positive for PLA receptor antibodies.
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http://dx.doi.org/10.4103/1319-2442.308360DOI Listing
February 2021

Acute Kidney Injury Associated With Urinary Stone Disease in Children and Young Adults Presenting to a Pediatric Emergency Department.

Front Pediatr 2020 30;8:591520. Epub 2020 Nov 30.

Division of Nephrology, Akron Children's Hospital, Akron, OH, United States.

Acute kidney injury (AKI) due to urinary stone disease (USD) is rare in adults; AKI rates in children with USD may be higher, and emerging data links stones to chronic kidney disease (CKD) development in adults. This study is a retrospective analysis of USD patients at a single pediatric hospital system's emergency department (ED). Patients were initially identified by USD ICD codes; USD was then confirmed by imaging or physician documentation; patients had to have baseline creatinine (Cr) and Cr in the ED for comparison to be included. AKI was defined by Kidney Disease: Improving Global Outcomes (KDIGO), Acute Kidney Injury Network (AKIN), and Pediatric Risk, Injury, Failure, Loss, End Stage (pRIFLE). Of the 589 total visits, 264/589 (45%) had data to evaluate for AKI, 23% were AKI(+) and 77% were AKI(-). pRIFLE was most common (82%) and 18% were only positive by AKIN/KDIGO. AKI(+) were more likely to be younger (16.7 vs. 17.4 years, = 0.046) and more likely to present with vomiting {odds ratio [OR] [95% confidence interval (CI)]: 2.4 [1.4-4.3], = 0.002}; also, the proportion of AKI(+) was significantly higher in <18 vs. ≥18 years [26.9 vs. 15.5%, = 0.032, OR (95% CI): 2.0 (1.1-3.9)]. Urinary tract infection (UTI) and obstruction rates were similar between groups. AKI(+) patients had a significant OR <1 suggesting less risk of receiving non-steroidal anti-inflammatory drugs (NSAIDs); however, 51% of them did receive NSAIDs during their ED encounter. AKI(+) patients were more likely to require admission to the hospital (53 vs. 32%, = 0.001). We have demonstrated a novel association between USD-induced renal colic and AKI in a group of young adults and children. AKI(+) patients were younger and were more likely to present with vomiting. AKI(+) patients did not have higher rates of obstruction or UTI, and 51% of AKI(+) received NSAIDs.
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http://dx.doi.org/10.3389/fped.2020.591520DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7793999PMC
November 2020

Diagnosis and Management of Renal Cystic Disease of the Newborn: Core Curriculum 2021.

Am J Kidney Dis 2021 07 6;78(1):125-141. Epub 2021 Jan 6.

Department of Medicine, Nephrology, University Hospital Freiburg, Germany; Medizinische Genetik Mainz, Limbach Genetics, Mainz, Germany.

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.
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http://dx.doi.org/10.1053/j.ajkd.2020.10.021DOI Listing
July 2021

Diversity of Phenotype and Genetic Etiology of 23 Cystinuria Saudi Patients: A Retrospective Study.

Front Pediatr 2020 11;8:569389. Epub 2020 Nov 11.

King Abdullah International Medical Research Center, King Saud bin Abdulaziz University for Health Sciences, King Abdulaziz Medical City, Ministry of National Guard-Health Affairs, Riyadh, Saudi Arabia.

Cystinuria is an inborn error of metabolism that manifests with renal stones due to defective renal epithelial cell transport of cystine which resulted from pathogenic variants in the and/or genes. Among nephrolithiasis diseases, cystinuria is potentially treatable, and further stone formation may be preventable. We report 23 patients who were identified biochemically and genetically to have cystinuria showing the diversity of the phenotype of cystinuria and expanding the genotype by identifying a broad spectrum of mutations. This is a multicenter retrospective chart review, where clinical and biochemical data, genetic analysis and the progress of the disease were documented over five years at two centers from 2014 to 2019. Of 23 patients who were identified biochemically and/or genetically to have cystinuria, 14 (62%) were male. Thirteen patients were homozygous, and two were heterozygous for the gene. Seven were homozygous and one was compound heterozygous for the gene. We have detected 12 genetic variants including five novel variants. 1 gene variant c.1400 T > A (p.Met467Lys) is found in 38% of our cohort. Although 21 patients required surgical intervention, none developed ESRD. The number of stone episodes per year varied widely (median frequency of 0.45 stones/ per year, range between 0.06 and 78.2), with no significant difference in stone events per year between sexes ( = 0.73). Despite the high rate of consanguinity in Saudi Arabia, there was a broad spectrum of genetic variants. Most of our patients are homozygous recessive for genes with multiple generations affected which indicates early screening and prevention of disease in these families. Phenotypic heterogeneity is well documented in our cohort even with the same genotype and the first stone episode age was variable but most commonly seen in the first decade of life.
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http://dx.doi.org/10.3389/fped.2020.569389DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7686133PMC
November 2020

Hypertensive Crisis in Pediatric Patients: An Overview.

Front Pediatr 2020 20;8:588911. Epub 2020 Oct 20.

Departments of Pediatrics, Internal Medicine, Population and Quantitative Health Sciences, Center for Clinical Informatics Research and Education, Case Western Reserve University and Metro Health System, Cleveland, OH, United States.

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.
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http://dx.doi.org/10.3389/fped.2020.588911DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7606848PMC
October 2020

Evolution and change in paradigm of hemodialysis in children: a systematic review.

Pediatr Nephrol 2021 May 14;36(5):1255-1271. Epub 2020 Nov 14.

Akron Nephrology Associates/Cleveland Clinic Akron General, Akron, OH, USA.

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.
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http://dx.doi.org/10.1007/s00467-020-04821-yDOI Listing
May 2021

Challenges of long-term vascular access in pediatric hemodialysis: Recommendations for practitioners.

Hemodial Int 2021 01 18;25(1):3-11. Epub 2020 Oct 18.

Pediatric Nephrology & Pediatric Kidney Transplantation, Kidney and Urology Institute, Medanta, The Medicity Hospital, Gurgaon, India.

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.
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http://dx.doi.org/10.1111/hdi.12868DOI Listing
January 2021

A rare complication of pauci-immune crescentic glomerulonephritis in a child: Questions.

Pediatr Nephrol 2021 Jul 15;36(7):1947-1948. Epub 2020 Oct 15.

Kidney Institute, Medanta the Medicity, Haryana, 122001, Gurgaon, India.

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http://dx.doi.org/10.1007/s00467-020-04784-0DOI Listing
July 2021

A rare complication of pauci-immune crescentic glomerulonephritis in a child: Answers.

Pediatr Nephrol 2021 Jul 15;36(7):1949-1952. Epub 2020 Oct 15.

Kidney Institute, Medanta the Medicity, Gurgaon, Haryana, 122001, India.

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http://dx.doi.org/10.1007/s00467-020-04786-yDOI Listing
July 2021

Cerebral venous sinus thrombosis in pediatric nephrotic syndrome.

Kidney Int 2020 10;98(4):1056

Department of Nephrology, Akron Nephrology Associates at Cleveland Clinic Akron General Medical Center, Akron, Ohio, USA; Department of Pediatric Nephrology, Akron Children's Hospital, Akron, Ohio, USA. Electronic address:

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http://dx.doi.org/10.1016/j.kint.2020.03.014DOI Listing
October 2020

Renal Manifestations of Tuberous Sclerosis Complex.

J Kidney Cancer VHL 2020 27;7(3):5-19. Epub 2020 Aug 27.

Akron Nephrology Associates, Cleveland Clinic Akron General, Akron, OH, USA.

Tuberous sclerosis complex (TSC) is a genetic condition caused by a mutation in either the or 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.
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http://dx.doi.org/10.15586/jkcvhl.2020.131DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7478169PMC
August 2020

Changing the terminology from kidney replacement therapy to kidney support therapy.

Ther Apher Dial 2021 Aug 27;25(4):437-457. Epub 2020 Oct 27.

Department of Nephrology, Cleveland Clinic Akron General/Akron Nephrology Associates, Akron, Ohio, USA.

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.
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http://dx.doi.org/10.1111/1744-9987.13584DOI Listing
August 2021

Challenges of Vascular Access in the Pediatric Population.

Adv Chronic Kidney Dis 2020 05;27(3):268-275

Department of Nephrology, Cleveland Clinic Akron General/Akron Nephrology Associates, Akron, OH.

The incidence and prevalence of renal replacement therapy has continued to increase in the pediatric population. Recent data have shown that hemodialysis was the most frequently used dialysis modality, especially in pediatric ESRD patients (age 0-21 years). A well-functioning vascular access is required for effective hemodialysis and choosing the best vascular access option for pediatric patients can be difficult. Pediatric vascular options include arteriovenous fistula, arteriovenous graft, and central venous catheters (CVCs). There is a national initiative for fistula first-catheter last approach; however, CVCs have been reported as the most conventionally utilized vascular access option in pediatric patients. The use of CVCs should be minimized or avoided as they are associated with high risk of infections, thrombosis, and other complications. Thus, it is important for practitioners to plan appropriately in advance, practice good clinical judgment, and assure that the best vascular access is placed according to the patient's needs. Therefore, this article reviews the different types of pediatric vascular access and the associated benefits and potential complications of each.
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http://dx.doi.org/10.1053/j.ackd.2020.02.005DOI Listing
May 2020

Development of acute kidney injury following pediatric cardiac surgery.

Kidney Res Clin Pract 2020 Sep;39(3):259-268

Department of Nephrology, Cleveland Clinic Akron General Medical Center/Akron Nephrology Associates, Akron, OH, USA.

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.
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http://dx.doi.org/10.23876/j.krcp.20.053DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7530361PMC
September 2020

Kidney Replacement Therapy in COVID-19 Induced Kidney Failure and Septic Shock: A Pediatric Continuous Renal Replacement Therapy [PCRRT] Position on Emergency Preparedness With Resource Allocation.

Front Pediatr 2020 3;8:413. Epub 2020 Jul 3.

Pediatric Nephrology & Transplantation, Children's Hospital of Richmond, Virginia Commonwealth University, Richmond, VA, United States.

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.
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http://dx.doi.org/10.3389/fped.2020.00413DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7347905PMC
July 2020

Uncommon cause of fever in a child with steroid-dependent nephrotic syndrome.

Clin Nephrol Case Stud 2020 7;8:49-52. Epub 2020 Jul 7.

Akron General Medical Center, and.

Background: Children with nephrotic syndrome are vulnerable to developing infections due to a state of relative immunodeficiency, malnourishment, and use of immunosuppression. Case characteristics: We herein report the case of a 3-year-old child with steroid-dependent nephrotic syndrome who presented to us with fever of unknown origin.

Observation: The child was found to have an atypical mixed infection with mycoplasma and cytomegalovirus.

Outcome: The infection completely resolved with appropriate treatment and lowering of immunosuppression. Message: Persistently febrile pediatric patients, especially in the setting of recent immunosuppression and absence of otherwise-identified infectious pathogens, should be screened for atypical mixed infections.
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http://dx.doi.org/10.5414/CNCS110062DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7344764PMC
July 2020

An Overview of Rickets in Children.

Kidney Int Rep 2020 Jul 11;5(7):980-990. Epub 2020 Apr 11.

Akron Nephrology Associates/Cleveland Clinic Akron General, Akron, Ohio, USA.

Rickets is a common bone disease worldwide that is associated with disturbances in calcium and phosphate homeostasis and can lead to short stature and joint deformities. Rickets can be diagnosed based on history and physical examination, radiological features, and biochemical tests. It can be classified into 2 major groups based on phosphate or calcium levels: phosphopenic and calcipenic. Knowledge of categorization of the type of rickets is essential for prompt diagnosis and proper management. Nutritional rickets is a preventable disease through adequate intake of vitamin D through both dietary and sunlight exposure. There are other subtypes of rickets, such as vitamin D-dependent type 1 rickets and vitamin D-dependent type 2 rickets (due to defects in vitamin D metabolism), renal rickets (due to poor kidney function), and hypophosphatemic rickets (vitamin D-resistant rickets secondary to renal phosphate wasting wherein fibroblast growth factor-23 (FGF-23) often plays a major role), which requires closer monitoring and supplementation with activated vitamin D with or without phosphate supplements. An important development has been the introduction of burosumab, a human monoclonal antibody to FGF-23, which is approved for the treatment of X-linked hypophosphatemia among children 1 year and older.
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http://dx.doi.org/10.1016/j.ekir.2020.03.025DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7335963PMC
July 2020

ACTH Treatment for Management of Nephrotic Syndrome: A Systematic Review and Reappraisal.

Int J Nephrol 2020 4;2020:2597079. Epub 2020 Jun 4.

Akron Nephrology Associates/Cleveland Clinic Akron General, Akron, OH, USA.

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.
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http://dx.doi.org/10.1155/2020/2597079DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7292987PMC
June 2020
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