Publications by authors named "P D Arkwright"
Receptor Interacting Serine/Threonine Kinase 1 (RIPK1) is widely expressed and integral to inflammatory and cell death responses. Autosomal recessive RIPK1-deficiency, due to biallelic loss of function mutations in RIPK1, is a rare inborn error of immunity (IEI) resulting in uncontrolled necroptosis, apoptosis and inflammation. Although hematopoietic stem cell transplantation (HSCT) has been suggested as a potential curative therapy, the extent to which disease may be driven by extra-hematopoietic effects of RIPK1-deficiency, which are non-amenable to HSCT, is not clear.
View Article and Find Full Text PDFBackground: CD4 T cells play essential roles in adaptive immunity. Distinct CD4 T-cell subsets-T1, T2, T17, T22, T follicular helper, and regulatory T cells-have been identified, and their contributions to host defense and immune regulation are increasingly well defined. IL-9-producing T9 cells were first described in 2008 and appear to play both protective and pathogenic roles in human immunity.
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- - C1q deficiency is a rare immune disorder that increases the risk of infections and autoimmune diseases similar to systemic lupus erythematosus (SLE), often leading to various health complications.
- - An international study involving 18 patients evaluated the outcomes of hematopoietic stem cell transplantation (HSCT) for C1q deficiency, showing a 71% overall survival rate and improvement in autoimmune symptoms for most patients.
- - The study highlights that patients with severe autoimmune symptoms have poorer survival rates, and specific antibodies were linked to different organ involvements, emphasizing the need for careful patient selection and risk assessment for HSCT as a treatment option.
Article Synopsis
- The study investigates how type 2 comorbidities like asthma, allergic rhinitis, and food allergies affect the treatment response to dupilumab in young children with atopic dermatitis.
- In a trial involving children aged 6 months to 5 years, results showed that those treated with dupilumab had significantly better outcomes compared to those on placebo, regardless of comorbidities.
- Overall, the treatment was found to be safe and effectively improved symptoms of atopic dermatitis in children, supporting its use in those with and without type 2 inflammatory conditions.
: The aetiology of inflammatory bowel disease (IBD), particularly if occurring early in childhood, is a diverse and patient-focused treatment that is required when standard therapy is ineffective. A clinical case report is presented of a child with very early-onset IBD (VEOIBD) and evidence of high serum IL-18 responding to anti-IL-18 immunotherapy. Detailed cytokine profiling was performed by ELISA and multiplex assay flow cytometry.
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