Clinical Practice Recommendations on Kidney Management in Methylmalonic Acidemia: an Expert Consensus Statement From ERKNet and MetabERN.

Methylmalonic acidemias (MMAs) are rare inherited metabolic diseases with multiorgan involvement. Chronic kidney disease (CKD) is a common complication, leading to kidney failure, dialysis, and kidney transplantation (KT). The objective of these guidelines was to develop clinical practice recommendations focusing on specific aspects of the kidney management of this disease.

Clinical Outcomes and Quality of Life of Patients Receiving Multi-Solid-Organ Transplants in Childhood Are Excellent: Results From a 20-Year Cohort Study.

Advances in medicine allow children with previously fatal conditions to survive longer and present as transplant candidates; some requiring multiple solid-organ transplants (MSOT). There is limited data on clinical outcomes and no data on quality of life (QoL). In this mixed methods cohort study clinical outcomes from the NHSBT registry were analysed for all patients who received a kidney and one other solid-organ transplant as a child between 2000 and 2021 in the UK.

Characteristics of Early Antibody Mediated Rejection in Antibody Incompatible Living Donor Kidney Transplantation.

Antibody incompatible transplantation (AIT) may be an only option for highly sensitized patients. Severe form of early antibody mediated rejection (AMR) adversely affects graft survival after AIT. The aim of this study was to identify individuals at risk of AMR.

3D printing: a useful tool for safe clinical practice in children with complex vasculature.

Background: 3D printing has been used in different medical contexts, although it is underutilised in paediatrics. We present the first use of 3D printing in the management of three paediatric patients with complex renovascular disease.

Methods: Patient-specific 3D models were produced from conventional 2D imaging and manufactured using 3D polyjet printing technology.

Cardiovascular outcomes improve in children with renovascular hypertension following endovascular and surgical interventions.

Background: Renovascular hypertension (RenoVH) is a cause of hypertension in children. A common cause of RenoVH is renal artery stenosis which acts by reducing blood supply to renal parenchyma and activating the renin-angiotensin-aldosterone axis, often leading to cardiac remodelling. This longitudinal observational study aims to describe occurrence of cardiovascular changes secondary to RenoVH and also any improvement in cardiac remodelling after successful endovascular and/or surgical intervention.

Histopathology of chironomids exposed to fly ash and microplastics as a new biomarker of ecotoxicological assessment.

In the last few decades, industrial pollution has gained extensive attention in terms of its effect on the aquatic environment. This imposes the need to develop sensitive biomarkers for early detection of pollutant toxicity in ecotoxicological assessment. The advantages of histopathological biomarkers are many, including quick reaction to the presence of contaminants, and the small number of individuals needed for efficient analysis.

The effects of early short-term insulin treatment vs. glimepiride on beta cell function in newly diagnosed type 2 diabetes with HbA1c above 9.

Background: Type 2 diabetes mellitus (T2D) is a complex metabolic impairment. Beta cell (BC) failure is the most challenging among its pathogenetic mechanisms. Recognizing reversible contributors to BC failure could guide individualized approach to early T2D treatment.

Associations of longitudinal height and weight with clinical outcomes in pediatric kidney replacement therapy: results from the ESPN/ERA Registry.

Background: Associations between anthropometric measures and patient outcomes in children are inconsistent and mainly based on data at kidney replacement therapy (KRT) initiation. We studied associations of height and body mass index (BMI) with access to kidney transplantation, graft failure, and death during childhood KRT.

Methods: We included patients < 20 years starting KRT in 33 European countries from 1995-2019 with height and weight data recorded to the ESPN/ERA Registry.

Safety, efficacy, and timing of transplantation(s) in propionic and methylmalonic aciduria.

Propionic (PA) and methylmalonic aciduria (MMA) share many clinical similarities, which include the risk of acute metabolic encephalopathies, and some long-term complications, such as optic neuropathy, pancreatic involvement, developmental disability, and similar management approaches, but they also represent distinct clinical and biochemical entities. In the severe forms of PA and MMA, most long-term complications cannot be prevented with conventional clinical management. Organ transplantation represents a form of partial enzyme replacement to improve the long-term outlook for these disorders.

Transplantation in paediatric patients with MMA requires multidisciplinary approach for achievement of good clinical outcomes.

Background: As modern medicine is advancing, younger, small, and more complex children are becoming multi-organ transplant candidates. This brings up new challenges in all aspects of their care.

Methods: We describe the first report of a small child receiving a simultaneous liver and kidney transplant and abdominal rectus sheath fascia transplant on the background of Williams syndrome and methylmalonic acidaemia.

Renal outcome and plasma methylmalonic acid levels after isolated or combined liver or kidney transplantation in patients with methylmalonic acidemia: A multicenter analysis.

Background: Methylmalonic acidemia (MMAemia) is characterized by accumulation of methylmalonic acid (MMA) in all body tissues. To minimize disease-related complications, isolated kidney (KTx), liver (LTx) or combined liver-kidney transplantation (LKTx) have been suggested. However, the impact of these different transplant strategies on outcome are unclear.

Presentation, treatment, and outcome of renovascular hypertension below 2 years of age.

Unlabelled: Renovascular hypertension in most cases requires endovascular treatment and/or surgery. This is technically much more difficult in small children and there is very limited published knowledge in this age group. We here present treatment and outcome of young children with renovascular hypertension at our institution.

Successful ABO and HLA incompatible kidney transplantation in children in the UK.

Background: There is increasing evidence of good short-term and medium-term outcomes of ABO incompatible (ABOi) and HLA incompatible (HLAi) kidney transplantation with pre-transplant positive crossmatches in paediatric practice. However, there remain concerns regarding the higher risks of infective complications and antibody-mediated rejections. The aim of our study is to show longer-term follow-up on all ABOi and HLAi paediatric kidney transplant recipients (pKTR) in the UK.

Adaptive neuro fuzzy selection of important factors for prediction of plasmons in silver nanorods.

The major goal of this study was to find predictors of plasmon positions in silver nanorod (NR) optical absorption spectra. The goal of this study is to use an adaptive neural fuzzy inference system to identify the various input parameters for longitudinal surface plasmon resonance (LSPR) and transverse surface plasmon resonance (TSP). A seed strategy has been used for preparation of the silver NRs.

Optimization of a plastic optical fiber based sensor for dye sensing coupled with an adapted neuro-fuzzy inference system.

In this study, estimation capacities and optimization of a dye concentration sensing model by an adapted neuro-fuzzy inference system (ANFIS) as well as central composite design coupled with response surface methodology using a plastic optical fiber (POF) based sensor were investigated. Various diameters of POF were used for sensing different concentrations of Remazol Black B (RBB), which acts as a sensing medium of the process. The efficiency of sensing was studied as a function of three independent variables: diameter of POF, concentration of RBB dye, and initial temperature of the solution.

Is Preemptive Kidney Transplantation Associated With Improved Outcomes when Compared to Non-preemptive Kidney Transplantation in Children? A Systematic Review and Meta-Analysis.

Preemptive kidney transplantation (PKT) is performed prior to dialysis initiation to avoid dialysis-related morbidity and mortality in children and adolescents. We undertook a systematic review to compare clinical outcomes in PKT versus kidney transplantation after dialysis initiation in paediatric patients. The bibliographic search identified studies that compared paediatric recipients of a first or subsequent, living or deceased donor PKT versus non-preemptive kidney transplant.

Essential Oils of Six Achillea Species: Chemical Profiles, Antimicrobial Potential and Toxicity toward Crustaceans.

This is the first comparative study on antimicrobial activity of essential oils (EOs) of six Achillea species (A. crithmifolia, A. distans, A.

Acute kidney injury in paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS) is not associated with progression to chronic kidney disease.

Background: Paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS) is a rare complication of SARS-CoV-2 associated with single or multiorgan dysfunction.

Objective: We aimed to evaluate the incidence of acute kidney injury (AKI) and risk factors for kidney dysfunction in PIMS-TS, with reporting of 6-month renal follow-up data. We also evaluated renal involvement between first and second waves of the SARS-CoV-2 pandemic in the UK, the latter attributed to the Alpha variant.

Psychological Concerns of Children Undergoing Kidney Transplantation During the Pandemic: Single-centre Experience.

Many paediatric kidney transplant programmes were closed during the COVID-19 pandemic, and due to the vulnerable nature of patients with end-stage kidney disease (ESKD), there were new concerns once these programmes reopened. We surveyed children and families who received a kidney transplant during the pandemic. We found that half of the participants felt scared and/or anxious about receiving a kidney transplant during the pandemic, and 2/8 participants were worried about catching COVID-19 during their recovery.

The potential use of microRNAs as a therapeutic strategy for SARS-CoV-2 infection.

Coronavirus disease 2019 (COVID-19) is caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). To date, there is no effective therapeutic approach for treating SARS-CoV-2 infections. MicroRNAs (miRNAs) have been recognized to target the viral genome directly or indirectly, thereby inhibiting viral replication.

Early relapse of atypical hemolytic uremic syndrome following ABO-incompatible living-related pediatric kidney re-transplant successfully treated with eculizumab.

Background: A 3-year-old girl with clinical features of atypical HUS (complement Factor I mutation inherited from an asymptomatic mother and Factor H autoantibodies) was treated with plasma exchange, progressed to kidney failure (KF) aged 4 years, and received an en bloc kidney DCD transplant aged 8 years with primary graft non-function necessitating transplant nephrectomy at the time of transplantation. She subsequently underwent re-transplantation from her father. This is a retrospective study of electronic patient records and medical notes.