Developing Best Practice Recommendations for children with Inflammatory Kidney Diseases
Dr Oni is currently leading a national working group to undertake an exciting project to develop best evidence clinical practice recommendations on how to treat children and young people with inflammatory kidney diseases. This aims to improve the quality of care and reduce variation across the country through agreed standard treatment plans.
Standards of care for IgA Vasculitis have been produced and are awaiting sign off from the UK Kidney Association. These will help to transform the lives of children and young people, ensuring they get the best possible care and treatement.
Childhood Immunoglobulin A vasculitis (IgAV, previously known as Henoch Schonlein Purpura)
IgA Vasculitis (IgAV) is a condition that causes a skin rash of raised red/purple spots caused by inflammation, and although usually harmless in some cases it can affect organs, such as the kidneys. It is normally diagnosed via a clinical examination and kidney checks are performed. Immunoglobulin A vasculitis is the most common form of childhood vasculitis. Glomerulonephritis occurs in 30-50% of patients and it can cause long lasting renal damage. Understanding the scientific origin of this disease is important in order to develop new, more targeted treatments. We are currently looking at how the disease may originate from an abnormal production of immunoglobulin A (glycosylated versions) which is often co-located with complement products such as C3.
Childhood Lupus Nephritis – Renal Inflammation
We are investigating whether it is possible to predict a lupus renal flare earlier than using standard markers in clinical practice in the programme of work based on discovering 'Renal biomarkers in juvenile onset systemic lupus erythematosus'. Scientific investigation is continuing in the laboratory using immortalised renal cell lines to establish whether any of the specific biomarkers are arising directly from the kidney cells.
Through collaboration with Lupus UK and the UK JSLE Cohort Study group we have recently undertaken a patient awareness survey titled ‘Lupus and the Kidney’s’ to help us identify key targets for educating patients on the importance of renal health in lupus (CI: L .Oni).
Trials that the EATC4Children are involved in include:
Working towards a diagnostic test to enable stratification of children with IgA vasculitis (HSP)
This study, funded by Vasculitis UK, investigates proteomic techniques that are capable of identifying the abnormal immunoglobulin products (CI: L. Oni).
Preliminary investigation to study children with inflammatory renal diseases and the role of complement protein C5
This study is funded by the Alder Hey Children’s Kidney Fund and aims to develop laboratory techniques to measure complement products which could allow us to link these to clinical phenotypes in children with IgAV (CI: L. Oni).