[HTML][HTML] 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 …

[HTML][HTML] 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 …

[HTML][HTML] KDIGO 2021 clinical practice guideline for the management of glomerular diseases

BH Rovin, SG Adler, J Barratt, F Bridoux… - Kidney …, 2021 - kidney-international.org
Glomerular disease, be it primary or secondary, occurring in the setting of systemic
autoimmune diseases, infections, drugs, or malignancy, affects individuals of all ages. In …

Idiopathic nephrotic syndrome in children

DG Noone, K Iijima, R Parekh - The Lancet, 2018 - thelancet.com
The incidence of idiopathic nephrotic syndrome (NS) is 1· 15–16· 9 per 100 000 children,
varying by ethnicity and region. The cause remains unknown but the pathogenesis of …

[HTML][HTML] Management and treatment of glomerular diseases (part 2): conclusions from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies …

BH Rovin, DJ Caster, DC Cattran, KL Gibson… - Kidney international, 2019 - Elsevier
In November 2017, the Kidney Disease: Improving Global Outcomes (KDIGO) initiative
brought a diverse panel of experts in glomerular diseases together to discuss the 2012 …

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 …

[HTML][HTML] Exploring the clinical and genetic spectrum of steroid resistant nephrotic syndrome: the PodoNet registry

A Trautmann, BS Lipska-Ziętkiewicz… - Frontiers in …, 2018 - frontiersin.org
Background: Steroid resistant nephrotic syndrome (SRNS) is a rare condition, accounting for
10–15% of all children with idiopathic nephrotic syndrome. SRNS can be caused by genetic …

[HTML][HTML] Oral Coenzyme Q10 supplementation leads to better preservation of kidney function in steroid-resistant nephrotic syndrome due to primary Coenzyme Q10 …

S Drovandi, BS Lipska-Ziętkiewicz, F Ozaltin, F Emma… - Kidney international, 2022 - Elsevier
Primary Coenzyme Q10 (CoQ10) deficiency is an ultra-rare disorder caused by defects in
genes involved in CoQ10 biosynthesis leading to multidrug-resistant nephrotic syndrome as …

[HTML][HTML] The important roles and molecular mechanisms of annexin A2 autoantibody in children with nephrotic syndrome

Q Ye, Y Zhang, J Zhuang, Y Bi, H Xu… - Annals of …, 2021 - ncbi.nlm.nih.gov
Background In recent years, B-cell dysfunction has been found to play an important role in
the pathogenesis of primary nephrotic syndrome (PNS). B cells play a pathogenic role by …

[HTML][HTML] Current understandings in treating children with steroid-resistant nephrotic syndrome

JM Lee, A Kronbichler, JI Shin, J Oh - Pediatric Nephrology, 2021 - Springer
Steroid-resistant nephrotic syndrome (SRNS) remains a challenge for paediatric
nephrologists. SRNS is viewed as a heterogeneous disease entity including immune-based …