IgA Nephropathy: Emerging Mechanisms of Disease.

Immunoglobulin A nephropathy (IgAN) is the most common primary glomerulonephritis reported across the world and is characterized by immunoglobulin A (IgA) dominant mesangial deposits, which are poorly -glycosylated. This deposition leads to a cascade of glomerular and tubulointerstitial inflammation and fibrosis, which can progress to chronic kidney disease. The variability in rate of progression reflects the many genetic and environmental factors that drive IgAN.

A systematic review of symptoms experienced by children and young people with kidney failure.

Background: Kidney failure at any age has a significant impact on quality of life (QoL) but the overall symptom burden for children and young people (CYP) is poorly described. Kidney failure has no cure and whilst transplantation is the preferred management option, it is not always possible, with patients requiring supportive care at the end of their lives.

Aim: To use the literature to understand the symptom burden for CYP with kidney failure who are approaching end-of-life.

Routine cardiac biomarkers for the prediction of incident major adverse cardiac events in patients with glomerulonephritis: a real-world analysis using a global federated database.

Rationale & Objective: Glomerulonephritis (GN) is a leading cause of chronic kidney disease (CKD). Major adverse cardiovascular events (MACE) are prolific in CKD. The risk of MACE in GN cohorts is multifactorial.

Themes associated with uncollected paediatric prescriptions at a large tertiary children's hospital: a retrospective, observational study.

Objective: This study aimed to assess the number of prescriptions that were uncollected by caregivers to identify any predisposing systemic themes that may act as barriers to children receiving medications.

Study Design And Setting: Data were retrospectively collected on uncollected prescriptions at a single, tertiary paediatric centre over a 2-month period. This included type and classification of the drug, prescriber specialty, the timing of prescription and the child's registered postcode.

The current use of proteomics and metabolomics in glomerulonephritis: a systematic literature review.

Background: Glomerulonephritis inherently leads to the development of chronic kidney disease. It is the second most common diagnosis in patients requiring renal replacement therapy in the United Kingdom. Metabolomics and proteomics can characterise, identify and quantify an individual's protein and metabolite make-up.

A Systematic Literature Review on the Use of Dried Biofluid Microsampling in Patients With Kidney Disease.

Background: Kidney disease is fairly unique due to the lack of symptoms associated with disease activity, and it is therefore dependent on biological monitoring. Dried biofluids, particularly dried capillary blood spots, are an accessible, easy-to-use technology that have seen increased utility in basic science research over the past decade. However, their use is yet to reach the kidney patient population clinically or in large-scale discovery science initiatives.

IgA vasculitis nephritis-outcomes in adult-onset disease.

Objectives: IgA vasculitis (IgAV) in adults has been relatively under-investigated. Since outcomes are worse in other forms of vasculitis with increasing age, we investigated the outcomes of IgAV comparing younger adults (18-34), middle aged adults (35-64) and elderly patients (≥64 years) focusing on kidney outcomes.

Methods: We identified patients with renal biopsy confirmed IgAV nephritis and collected data regarding clinical features and progression to end stage kidney disease (ESKD).

Urinary markers of the alternative and lectin complement pathway are increased in IgA vasculitis nephritis.

Background: IgA vasculitis (IgAV) is the most common form of childhood vasculitis. Nephritis (IgAVN) occurs in 50% of patients and 1-2% progress to chronic kidney disease stage 5. The pathophysiology of nephritis remains largely unknown, but recent evidence suggests that the complement system may be involved.

A case series on recurrent and persisting IgA vasculitis (Henoch Schonlein purpura) in children.

Background: IgA vasculitis (IgAV) is a small vessel vasculitis that is more common in childhood. Very limited evidence exists on patients who experience an atypical disease course. The aim of this study was to describe a cohort of children diagnosed with recurrent or persisting IgAV to identify any themes associated with their disease course and areas of unmet needs.

The readability of parent information leaflets in paediatric studies.

Background: Poor literacy can impact achieving optimal health outcomes. The aim of this project was to assess the readability of parent information leaflets (PILs).

Methods: A single-centre study using paediatric PILs.

Increased Urinary IgA in Paediatric IgA Vasculitis Nephritis.

IgA vasculitis (IgAV) is the most common form of paediatric vasculitis, with up to 50% of patients experiencing kidney inflammation. Much remains unknown about IgAV, but it is believed to arise due to galactose-deficient IgA1 promoting an auto-inflammatory response. This study assesses whether urinary IgA can be detected in children with IgAV to allow further evaluation of IgA1 and whether it has any relationship with nephritis.

Urinary complement proteins are increased in children with IgA vasculitis (Henoch-Schönlein purpura) nephritis.

Background: Children with immunoglobulin A vasculitis (IgAV Henoch-Schönlein purpura) frequently encounter nephritis (IgAV-N) with 1-2% risk of kidney failure. The pathophysiology of IgAV-N is not fully understood with speculation that complement may contribute. The aim of this study was to identify whether urinary complement proteins are increased in children with IgAV-N.

Urinary Protein Array Analysis to Identify Key Inflammatory Markers in Children with IgA Vasculitis Nephritis.

Chronic kidney disease is a recognised complication of immunoglobulin A vasculitis, (IgAV; formerly Henoch-Schonlein purpura-HSP). The pathophysiology of IgAV and why some patients develop significant renal involvement remains largely unknown. Identifying urinary inflammatory markers could direct targets for earlier intervention.

Vancomycin-associated acute kidney injury epidemiology in children: a systematic review.

Introduction: Vancomycin is a recognised cause of drug-induced acute kidney injury (AKI).

Objective: The aim of this systematic review was to summarise the incidence of, and the risk factors for, vancomycin-associated AKI (v-AKI) in children.

Design: A systematic search was performed in November 2020 on the search engines PubMed, Web of Science and Medline, using predefined search terms.

Renal manifestations of paediatric systemic lupus erythematosus.

Purpose Of Review: Systemic lupus erythematosus is a lifelong, multisystemic disease. Around a fifth of patients present during childhood. Children are recognized to have a more active disease course with more renal involvement (lupus nephritis) when compared with adults.

Anti-glomerular basement membrane disease in children: a brief overview.

Anti-glomerular basement membrane disease (Anti-GBM), previously known as Goodpasture syndrome, is an extremely rare cause of rapidly progressive glomerulonephritis and chronic kidney disease stage 5 (CKD5) in children. It is associated with acute pulmonary haemorrhage and it has a poor prognosis. It is classified as an autoimmune, small-vessel vasculitis caused by autoantibody formation against the alpha-3 chain in type IV collagen found in the glomerular basement membrane.

A systematic review of urine biomarkers in children with IgA vasculitis nephritis.

Background: Nephritis is a recognised complication of IgA vasculitis (IgAV, Henoch-Schönlein purpura) contributing to 1-2% of all chronic kidney disease (CKD) stage 5. Improved understanding may reduce irreversible damage in IgAV nephritis (IgAV-N).

Objective: The aim of this study was to perform a comprehensive systematic literature review to identify promising clinical and pre-clinical urine biomarkers in children with IgAV-N that could predict the presence of nephritis and/or determine its severity.