Ofatumumab in post-transplantation recurrence of a pediatric steroid-resistant idiopathic nephrotic syndrome

2018 ◽  
Vol 22 (4) ◽  
pp. e13175 ◽  
Author(s):  
Josselin Bernard ◽  
Alexandra Bruel ◽  
Emma Allain-Launay ◽  
Jacques Dantal ◽  
Gwenaelle Roussey
Keyword(s):  
Nephrotic Syndrome ◽  
Idiopathic Nephrotic Syndrome ◽  
Post Transplantation ◽  
Steroid Resistant

scholarly journals Urinary Extracellular Vesicle Protein Profiles Discriminate Different Clinical Subgroups of Children with Idiopathic Nephrotic Syndrome

Diagnostics ◽  
2021 ◽  
Vol 11 (3) ◽  
pp. 456
Author(s):  
Lucia Santorelli ◽  
William Morello ◽  
Elisa Barigazzi ◽  
Giulia Capitoli ◽  
Chiara Tamburello ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Idiopathic Nephrotic Syndrome ◽  
Protein Profile ◽  
Therapy Response ◽  
Extracellular Vesicle ◽  
Distinct Pattern ◽  
Specific Patient ◽  
Steroid Resistant ◽  
Primary Glomerular Disease ◽  
Disease Signature

Idiopathic nephrotic syndrome (INS) is the most frequent primary glomerular disease in children, displaying high grade proteinuria and oedema. The mainstay of therapy are steroids, and patients are usually classified according to the treatment response (sensitive vs. resistant). The mechanisms involved in INS pathogenesis and treatment responsiveness have not yet been identified. In this context, the analysis of urinary extracellular vesicles (UEv) is interesting, since they represent a molecular snapshot of the parental cells, offering a “fingerprint” for monitoring their status. Therefore, the aim of this study is to verify the feasibility of using UEv of INS patients as indicators of therapy response and its prediction. UEv were isolated from the urine of pediatric patients in remission after therapy; they showed characteristic electrophoresis profiles that matched specific patient subgroups. We then built a statistical model to interpret objectively each patient UEv protein profile: in particular, steroid-resistant patients cluster together with a very distinct pattern from other INS patients and controls. In conclusion, the evaluation of the UEv protein profile looks promising in the investigation of INS, showing a disease signature that might predict clinical evolution.

Hodgkin's disease following steroid-resistant idiopathic nephrotic syndrome

1994 ◽  
Vol 8 (3) ◽  
pp. 395-396
Author(s):  
Georges Desch�nes ◽  
Christophe Allard ◽  
St�phane Benoit ◽  
Odile Lejars ◽  
Jean Pierre Lamagnere ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Hodgkin's Disease ◽  
Idiopathic Nephrotic Syndrome ◽  
Hodgkin’S Disease ◽  
Steroid Resistant

Urinary Proteome of Steroid-Sensitive and Steroid-Resistant Idiopathic Nephrotic Syndrome of Childhood

2006 ◽  
Vol 26 (3) ◽  
pp. 258-267 ◽  
Author(s):  
Robert P. Woroniecki ◽  
Tatyana N. Orlova ◽  
Natasha Mendelev ◽  
Ibrahim F. Shatat ◽  
Susan M. Hailpern ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Idiopathic Nephrotic Syndrome ◽  
Steroid Resistant ◽  
Urinary Proteome ◽  
Steroid Sensitive

scholarly journals WT1 mutations in steroid-resistant idiopathic nephrotic syndrome

2016 ◽  
Vol 27 (2) ◽  
pp. 417 ◽  
Author(s):  
OmP Mishra ◽  
ArunK Singh ◽  
Abhishek Abhinay ◽  
Gopeshwar Narayan ◽  
Rajniti Prasad ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Idiopathic Nephrotic Syndrome ◽  
Steroid Resistant

scholarly journals Apheresis Therapy for Steroid-Resistant Idiopathic Nephrotic Syndrome: Report on a Case Series

2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Hamza Naciri Bennani ◽  
Thomas Jouve ◽  
Johan Noble ◽  
Lionel Rostaing ◽  
Paolo Malvezzi ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Minimal Change Disease ◽  
Idiopathic Nephrotic Syndrome ◽  
Case Series ◽  
Calcineurin Inhibitors ◽  
Multidrug Resistant ◽  
Glomerular Permeability ◽  
Steroid Resistant ◽  
Double Filtration Plasmapheresis ◽  
Double Filtration

Idiopathic nephrotic syndrome (INS) represents 15%–30% of adulthood glomerulopathies. Corticosteroids have been the main treatment for decades and are effective in 70% of minimal-change disease patients and ~30% of focal segmental glomerulosclerosis patients. Multidrug-resistant (steroids, calcineurin-inhibitors, cyclophosphamide, mycophenolate-mofetil, rituximab) idiopathic nephrotic syndrome is a major therapeutic challenge in nephrology. Apheresis (double-filtration plasmapheresis or semi specific immunoadsorption) could act by eliminating the circulating factor (apolipoproteinA1b, solubleCD40L, suPAR) increasing glomerular permeability seen in INS. The aim of the study was to report the outcome of three patients with multidrug-resistant INS treated successfully with apheresis.

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