Podocytopathies

JB Kopp, HJ Anders, K Susztak, MA Podestà… - Nature Reviews …, 2020 - nature.com
Podocytopathies are kidney diseases in which direct or indirect podocyte injury drives
proteinuria or nephrotic syndrome. In children and young adults, genetic variants in> 50 …

The Italian Society for Pediatric Nephrology (SINePe) consensus document on the management of nephrotic syndrome in children: Part I-Diagnosis and treatment of …

A Pasini, E Benetti, G Conti, L Ghio, M Lepore… - Italian journal of …, 2017 - Springer
This consensus document is aimed at providing an updated, multidisciplinary overview on
the diagnosis and treatment of pediatric nephrotic syndrome (NS) at first presentation. It is …

Mutations in six nephrosis genes delineate a pathogenic pathway amenable to treatment

S Ashraf, H Kudo, J Rao, A Kikuchi, E Widmeier… - Nature …, 2018 - nature.com
No efficient treatment exists for nephrotic syndrome (NS), a frequent cause of chronic kidney
disease. Here we show mutations in six different genes (MAGI2, TNS2, DLC1, CDK20 …

Evaluation of daily low-dose prednisolone during upper respiratory tract infection to prevent relapse in children with relapsing steroid-sensitive nephrotic syndrome …

MT Christian, NJA Webb, S Mehta, RL Woolley… - JAMA …, 2022 - jamanetwork.com
Importance In children with corticosteroid-sensitive nephrotic syndrome, many relapses are
triggered by upper respiratory tract infections. Four small studies found that administration of …

The extrafollicular B cell response is a hallmark of childhood idiopathic nephrotic syndrome

TA Al-Aubodah, L Aoudjit, G Pascale… - Nature …, 2023 - nature.com
The efficacy of the B cell-targeting drug rituximab (RTX) in childhood idiopathic nephrotic
syndrome (INS) suggests that B cells may be implicated in disease pathogenesis. However …

The role of the immune system in idiopathic nephrotic syndrome

A Hackl, SEDA Zed, P Diefenhardt, J Binz-Lotter… - Molecular and Cellular …, 2021 - Springer
Idiopathic nephrotic syndrome (INS) in children is characterized by massive proteinuria and
hypoalbuminemia and usually responds well to steroids. However, relapses are frequent …

Minimal change disease: a “two-hit” podocyte immune disorder?

M Shimada, C Araya, C Rivard, T Ishimoto… - Pediatric …, 2011 - Springer
Minimal change disease (MCD) is the most common nephrotic syndrome in children and is
commonly thought to be a T-cell disorder mediated by a circulating factor that alters …

Serum immunoglobulins and risk of infection: how low can you go?

DE Furst - Seminars in arthritis and rheumatism, 2009 - Elsevier
OBJECTIVES: To determine the relationship between the levels of serum immunoglobulin
(Ig) levels and risk of infection. The following 2 main questions are addressed:(1) At what …

Molecular mechanisms of proteinuria in minimal change disease

S Purohit, F Piani, FA Ordoñez… - Frontiers in …, 2021 - frontiersin.org
Minimal change disease (MCD) is the most common type of idiopathic nephrotic syndrome
in childhood and represents about 15% cases in adults. It is characterized by massive …

[HTML][HTML] Immunosuppressive burden and risk factors of infection in primary childhood nephrotic syndrome

K Alfakeekh, M Azar, B Al Sowailmi… - Journal of infection and …, 2019 - Elsevier
Introduction Patients with primary childhood nephrotic syndrome (PCNS) develop alterations
in their cellular and humoral immunity that predisposes them to the development of infection …