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Pediatric vasculitis is a complex group of disorders that commonly presents with multisystem involvement. Renal vasculitis can be isolated to the kidneys or can occur as part of a broader multiorgan vasculitis. Depending on severity, renal vasculitis may present as acute glomerulonephritis (AGN) often associated with hypertension and sometimes with a rapidly deteriorating clinical course. Prompt diagnosis and initiation of therapy are key to preserving kidney function and preventing long-term morbidity and mortality. This review focuses on the clinical presentation, diagnosis, and treatment objectives for common forms of renal vasculitis seen in pediatric patients., Competing Interests: Disclosure All the authors report no conflicts of interest in this work., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Objective: To evaluate the prevalence and factors associated with the risk of acute kidney injury (AKI) in pediatric patients with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and multisystem inflammatory syndrome in children (MIS-C). Study Design: We performed a retrospective chart review of 113 patients with SARS-CoV-2 infection with or without MIS-C admitted at Children's Hospital of Michigan (CHM) from March to August 2020. Patient demographic details, laboratory data, imaging studies, echocardiography reports, and treatment data were collected. Results: Of the 92 patients included in the final analysis, 22 (24%) developed AKI with 8/22 (36%) developing stage 3 AKI. The prevalence of AKI was much higher in patients with MIS-C 15/28 (54%) vs. those with acute SARS-CoV-2 infection 7/64 (11%), ( p < 0.001). Overall, when compared to patients without AKI, patients with AKI were older in age (11 vs. 6.5 years, p = 0.007), African American (86 vs. 58%, p = 0.028), had MIS-C diagnosis (68 vs. 19%, p < 0.001), required ICU admission (91 vs. 20%, p < 0.001), had cardiac dysfunction (63 vs. 16%, p < 0.001), required inotropic support (59 vs. 6%, p < 0.001) and had a greater elevation in inflammatory markers. In a multivariate analysis, requirement of inotropes [Odds Ratio (OR)-22.8, p < 0.001], African American race (OR-8.8, p = 0.023) and MIS-C diagnosis (OR-5.3, p = 0.013) were the most significant predictors for AKI. All patients had recovery of kidney function, and none required kidney replacement therapy. Conclusion: Children with acute SARS-CoV-2 infection and MIS-C are at risk for AKI, with the risk being significantly greater with MIS-C. The pathogenesis of AKI in acute SARS-CoV-2 infection appears to be a combination of both renal hypo-perfusion and direct renal parenchymal damage whereas in MIS-C, the renal injury appears to be predominantly pre-renal from cardiac dysfunction and capillary leak from a hyperinflammatory state. These factors should be considered by clinicians caring for these children with a special focus on renal protective strategies to aid in recovery and prevent additional injury to this high-risk subgroup., 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 Grewal, Gregory, Jain, Mohammad, Cashen, Ang, Thomas and Valentini.)

Hyperammonaemia in children can lead to grave consequences in the form of cerebral oedema, severe neurological impairment and even death. In infants and children, common causes of hyperammonaemia include urea cycle disorders or organic acidaemias. Few studies have assessed the role of extracorporeal therapies in the management of hyperammonaemia in neonates and children. Moreover, consensus guidelines are lacking for the use of non-kidney replacement therapy (NKRT) and kidney replacement therapies (KRTs, including peritoneal dialysis, continuous KRT, haemodialysis and hybrid therapy) to manage hyperammonaemia in neonates and children. Prompt treatment with KRT and/or NKRT, the choice of which depends on the ammonia concentrations and presenting symptoms of the patient, is crucial. This expert Consensus Statement presents recommendations for the management of hyperammonaemia requiring KRT in paediatric populations. Additional studies are required to strengthen these recommendations.

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., (© 2020 Wiley Periodicals, Inc.)

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.

Atypical hemolytic uremic syndrome (aHUS) is a chronic life threatening condition that arises from genetic abnormalities resulting in uncontrolled complement amplifying activity. The introduction of eculizumab, the humanized monoclonal antibody, has brought about a paradigm shift in the management of aHUS. However, there are many knowledge gaps, diagnostic issues, access and cost issues, and patient or physician challenges associated with the use of this agent. Limited data on the natural history of aHUS along with the underlying genetic mutations make it difficult to predict the relapses and thereby raising concerns about the appropriate duration and monitoring of treatment. In this review, we discuss the safety and efficacy of eculizumab in patients with aHUS and its associated challenges., Competing Interests: The authors report no conflicts of interest in this work., (© 2019 Raina et al.)