IPNA clinical practice recommendations for the diagnosis and management of children with steroid-resistant nephrotic syndrome

A Trautmann, M Vivarelli, S Samuel, D Gipson… - Pediatric …, 2020 - Springer
Idiopathic nephrotic syndrome newly affects 1–3 per 100,000 children per year.
Approximately 85% of cases show complete remission of proteinuria following …

Childhood nephrotic syndrome

M Vivarelli, K Gibson, A Sinha, O Boyer - The Lancet, 2023 - thelancet.com
Idiopathic nephrotic syndrome is the most common glomerular disease in children.
Corticosteroids are the cornerstone of its treatment, and steroid response is the main …

[HTML][HTML] Diagnostic utility of exome sequencing for kidney disease

EE Groopman, M Marasa… - … England Journal of …, 2019 - Mass Medical Soc
Background Exome sequencing is emerging as a first-line diagnostic method in some
clinical disciplines, but its usefulness has yet to be examined for most constitutional …

Differentiating primary, genetic, and secondary FSGS in adults: a clinicopathologic approach

AS De Vriese, S Sethi, KA Nath… - Journal of the …, 2018 - journals.lww.com
FSGS describes a renal histologic lesion with diverse causes and pathogenicities that are
linked by podocyte injury and depletion. Subclasses of FSGS include primary, genetic, and …

Adeno-associated virus gene therapy prevents progression of kidney disease in genetic models of nephrotic syndrome

WY Ding, V Kuzmuk, S Hunter, A Lay… - Science Translational …, 2023 - science.org
Gene therapy for kidney diseases has proven challenging. Adeno-associated virus (AAV) is
used as a vector for gene therapy targeting other organs, with particular success …

Whole exome sequencing of patients with steroid-resistant nephrotic syndrome

JK Warejko, W Tan, A Daga, D Schapiro… - Clinical Journal of the …, 2018 - journals.lww.com
Results In 74 of 300 families (25%), we identified a causative mutation in one of 20 genes
known to cause steroid-resistant nephrotic syndrome. In 11 families (3.7%), we detected a …

Podocyte-targeted therapies—progress and future directions

K Meliambro, JC He, KN Campbell - Nature Reviews Nephrology, 2024 - nature.com
Podocytes are the key target cells for injury across the spectrum of primary and secondary
proteinuric kidney disorders, which account for up to 90% of cases of kidney failure …

A multi-institutional study found a possible role of anti-nephrin antibodies in post-transplant focal segmental glomerulosclerosis recurrence

Y Shirai, K Miura, K Ishizuka, T Ando, S Kanda… - Kidney International, 2024 - Elsevier
Possible roles of anti-nephrin antibodies in post-transplant recurrent focal segmental
glomerulosclerosis (FSGS) have been reported recently. To confirm these preliminary …

Whole-exome sequencing in adults with chronic kidney disease: a pilot study

S Lata, M Marasa, Y Li, DA Fasel… - Annals of internal …, 2018 - acpjournals.org
Background: The utility of whole-exome sequencing (WES) for the diagnosis and
management of adult-onset constitutional disorders has not been adequately studied …

Management of steroid-resistant nephrotic syndrome in children and adolescents

K Tullus, H Webb, A Bagga - The Lancet Child & Adolescent Health, 2018 - thelancet.com
More than 85% of children and adolescents (majority between 1–12 years old) with
idiopathic nephrotic syndrome show complete remission of proteinuria following daily …