Nicholas J.A. Webb
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View article: Oral iptacopan therapy in patients with C3 glomerulopathy: a randomised, double-blind, parallel group, multicentre, placebo-controlled, phase 3 study
Oral iptacopan therapy in patients with C3 glomerulopathy: a randomised, double-blind, parallel group, multicentre, placebo-controlled, phase 3 study Open
Novartis Pharma.
View article: Polygenic Risk Scores and HLA Class II Variants are Biomarkers of Corticosteroid Response in Childhood Nephrotic Syndrome
Polygenic Risk Scores and HLA Class II Variants are Biomarkers of Corticosteroid Response in Childhood Nephrotic Syndrome Open
Introduction Nephrotic syndrome (NS), a common glomerular disease in children, is classified based on response to corticosteroid therapy as either steroid-sensitive nephrotic syndrome (SSNS), or steroid-resistant nephrotic syndrome (SRNS).…
View article: Quantifying association of early proteinuria and estimated glomerular filtration rate changes with long-term kidney failure in C3 glomerulopathy and immune-complex membranoproliferative glomerulonephritis using the United Kingdom RaDaR Registry
Quantifying association of early proteinuria and estimated glomerular filtration rate changes with long-term kidney failure in C3 glomerulopathy and immune-complex membranoproliferative glomerulonephritis using the United Kingdom RaDaR Registry Open
Overall, proteinuria a short time after diagnosis is strongly associated with long-term outcomes and a UPCR under 100 mg/mmol at one year is associated with a substantially lower kidney failure risk.
View article: The prevalence of immunoglobulin A nephropathy in the European Union and the impact of the COVID-19 pandemic: an estimation approach utilizing the kidney biopsy frequency
The prevalence of immunoglobulin A nephropathy in the European Union and the impact of the COVID-19 pandemic: an estimation approach utilizing the kidney biopsy frequency Open
Background Immunoglobulin A nephropathy (IgAN) is the most common type of primary glomerulonephritis, requiring a kidney biopsy for diagnosis. This study aimed to estimate the prevalence of primary IgAN within the European Union (EU) and i…
View article: Kidney Transplantation in Children and Adolescents With <scp>C3</scp> Glomerulopathy or Immune Complex Membranoproliferative Glomerulonephritis: An International Survey of Current Practice
Kidney Transplantation in Children and Adolescents With <span>C3</span> Glomerulopathy or Immune Complex Membranoproliferative Glomerulonephritis: An International Survey of Current Practice Open
Background Approximately 50% of patients with chronic kidney disease due to C3 glomerulopathy (C3G) or primary immune‐complex membranoproliferative glomerulonephritis (IC‐MPGN) will require dialysis and/or kidney transplantation (KTx) with…
View article: Factor B as a therapeutic target for the treatment of complement-mediated diseases
Factor B as a therapeutic target for the treatment of complement-mediated diseases Open
The complement system, consisting of three initiating pathways—classical, lectin and alternative, is an important part of innate immunity. Dysregulation of the complement system is implicated in the pathogenesis of several autoimmune and i…
View article: #277 Update to the long-term safety and efficacy of iptacopan in C3G: 33-month extension study data from patients enrolled in a phase 2 study
#277 Update to the long-term safety and efficacy of iptacopan in C3G: 33-month extension study data from patients enrolled in a phase 2 study Open
Background and Aims Complement 3 glomerulopathy (C3G), due to dysregulation of the alternative complement pathway (AP), is an ultra-rare primary glomerulonephritis. Iptacopan (LNP023) is an oral, proximal complement inhibitor that specific…
View article: WCN24-1146 SAFETY AND EFFICACY OF IPTACOPAN IN ADOLESCENT PATIENTS WITH IDIOPATHIC (PRIMARY) IMMUNE-COMPLEX-MEDIATED MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS (IC-MPGN)
WCN24-1146 SAFETY AND EFFICACY OF IPTACOPAN IN ADOLESCENT PATIENTS WITH IDIOPATHIC (PRIMARY) IMMUNE-COMPLEX-MEDIATED MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS (IC-MPGN) Open
IC-MPGN is an ultra-rare, fast-progressing complement-mediated kidney disease characterized by immunoglobulin deposits in the kidneys, which may be idiopathic (primary) or secondary to chronic infections, autoimmune disorders, or monoclona…
View article: Quantifying association of early proteinuria and eGFR changes with long-term kidney failure hazard in C3G and IC-MPGN
Quantifying association of early proteinuria and eGFR changes with long-term kidney failure hazard in C3G and IC-MPGN Open
Background C3 glomerulopathy (C3G) and immune-complex membranoproliferative glomerulonephritis (IC-MPGN) are rare disorders that frequently result in kidney failure over the long-term. At present, there are no disease-specific treatments a…
View article: Evaluating the Acceptability, Swallowability, and Palatability of Film-Coated Mini-Tablet Formulation in Young Children: Results from an Open-Label, Single-Dose, Cross-Over Study
Evaluating the Acceptability, Swallowability, and Palatability of Film-Coated Mini-Tablet Formulation in Young Children: Results from an Open-Label, Single-Dose, Cross-Over Study Open
Mini-tablets are advantageous over liquid formulations in overcoming challenges related to stability, taste, and dosage. This open-label, single-dose, cross-over study investigated the acceptability and safety of drug-free, film-coated min…
View article: The pediatric glucocorticoid toxicity index
The pediatric glucocorticoid toxicity index Open
To develop a Pediatric glucocorticoid toxicity index (pGTI), a standardized, weighted clinical outcome assessment that measures change in glucocorticoid (GC) toxicity over time. Fourteen physician experts from 7 subspecialties participated…
View article: Lower Leg Power and Grip Strength Are Associated With Increased Fall Injury Risk in Older Men: The Osteoporotic Fractures in Men Study
Lower Leg Power and Grip Strength Are Associated With Increased Fall Injury Risk in Older Men: The Osteoporotic Fractures in Men Study Open
Background Past research has not investigated both lower-extremity power and upper-extremity strength in the same fall injury study, particularly nonfracture fall injuries. Methods In the Osteoporotic Fractures in Men Study (baseline: N = …
View article: Expert guidance on the multidisciplinary management of cystinosis in adolescent and adult patients
Expert guidance on the multidisciplinary management of cystinosis in adolescent and adult patients Open
Cystinosis, a rare autosomal recessive lysosomal storage disorder, results in an abnormal accumulation of the amino acid cystine in multiple organs and tissues of the body. Renal symptoms typically develop in the first few months of life, …
View article: FC 132SHORT COURSE DAILY LOW-DOSE PREDNISOLONE AT THE TIME OF UPPER RESPIRATORY TRACT INFECTION (URTI) IN NON-SELECTED CHILDREN WITH RELAPSING STEROID SENSITIVE NEPHROTIC SYNDROME DOES NOT PREVENT URTI-RELATED RELAPSE: THE PREDNOS 2 TRIAL
FC 132SHORT COURSE DAILY LOW-DOSE PREDNISOLONE AT THE TIME OF UPPER RESPIRATORY TRACT INFECTION (URTI) IN NON-SELECTED CHILDREN WITH RELAPSING STEROID SENSITIVE NEPHROTIC SYNDROME DOES NOT PREVENT URTI-RELATED RELAPSE: THE PREDNOS 2 TRIAL Open
Background and Aims At least 80% of children with steroid sensitive nephrotic syndrome (SSNS) have relapses and many are triggered by upper respiratory tract infections (URTIs). Previous small studies (4 studies, 232 patients in total), mo…
View article: Steroid treatment for the first episode of childhood nephrotic syndrome: comparison of the 8 and 12 weeks regimen using an individual patient data meta-analysis
Steroid treatment for the first episode of childhood nephrotic syndrome: comparison of the 8 and 12 weeks regimen using an individual patient data meta-analysis Open
Steroids are the cornerstone of the treatment of childhood nephrotic syndrome. The optimal duration for the first episode remains a matter of debate. The aim of this study is to determine whether the 8 weeks International Study of Kidney D…
View article: Consultant-led triage of paediatric hospital referrals: a service evaluation
Consultant-led triage of paediatric hospital referrals: a service evaluation Open
Objective We established a paediatric demand management (PDM) service in our paediatric department in 2017. The aim of this consultant-delivered service is to manage referrals more efficiently by providing active triage of all referrals, d…
View article: Rare hereditary COL4A3/COL4A4 variants may be mistaken for familial focal segmental glomerulosclerosis
Rare hereditary COL4A3/COL4A4 variants may be mistaken for familial focal segmental glomerulosclerosis Open
Focal segmental glomerulosclerosis (FSGS) is a histological lesion with many causes including inherited genetic defects with significant proteinuria being the predominant clinical finding at presentation. Mutations in COL4A3 and COL4A4 are…
View article: Complement Inhibition with the C5 Blocker LFG316 in Severe COVID-19
Complement Inhibition with the C5 Blocker LFG316 in Severe COVID-19 Open
h e r , M o r g a n, M a t t , Wis e, M a t t P. a n d M o r g a n , B. P a ul 2 0 2 0. Co m pl e m e n t in hi bi tio n wi t h t h e C 5 bloc k e r LFG 3 1 6 in s e v e r e COVID-1 9. Am e ri c a n Jou r n al of R e s pi r a t o ry a n d …