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 Management of idiopathic nephrotic syndrome in childhood

2004 ◽  
Vol 132 (9-10) ◽  
pp. 352-359 ◽  
Author(s):  
Amira Peco-Antic
Keyword(s):  
Nephrotic Syndrome ◽  
Idiopathic Nephrotic Syndrome ◽  
Alkylating Agents ◽  
Oral Prednisone ◽  
Symptomatic Treatment ◽  
Dietary Regimen ◽  
Steroid Resistant ◽  
Steroid Dependent ◽  
Frequent Relapses ◽  
Steroid Sensitive

The management of idiopathic nephrotic syndrome (INS) in children includes immunosuppressive and symptomatic treatment. The response to corticosteroid therapy is the best prognostic marker of the disease. The majority of children with INS (about 85%) are steroid-sensitive as they normalize proteinuria within 4 weeks of daily, oral prednisone administration. The most of steroid-sensitive patients (94%) has minimal change of nephrotic syndrome, while the majority (80.5%-94.4%) of those who are steroid-resistant has focal segmental glomerulosderosis or mesangioproliferative glomerulonephritis. Initial therapy of INS consists of 60 mg/m2/day prednisone daily for 4 weeks followed by 40 mg/m2 on alternate days for 4 weeks, thereafter decreasing alternate day therapy every month by 25% over the next 4 months. Thus, the overall duration of the initial cortico-steroids course is 6 months that may be significantly protective against the future development of frequent relapses. Approximately 30% of patients experience only one attack and are cured after the first course of therapy; 10-20% have only 3 or 4 steroid-responsive episodes before permanent cure; the remaining 40-50% of patients are frequent relapsers, or steroid-dependent. Standard relapse therapy consists of 60 mg/m2/ day prednisone until urine is protein free for at least 3 days, followed by 40 mg/m2 on alternate days for 4 weeks. The treatment of frequent-relapses and steroid-dependent INS includes several different regimens: maintenance (6 months) alternate steroid therapy just above steroid threshold (0.1-0.5 mg/kg/ 48h), levamisole, alkylating agents (cyclophosphamide or chlorambucil) or cyclosporine. The worse prognosis is expected in steroid-resistant patients who are the most difficult to treat. Renal biopsy should be performed in them. At present, there is no consensus on therapeutic regimen for steroid-resistant patients. The following immunosuppressive drugs have been used with varying success: cyclosporine, intravenous methyl prednisone pulses alone or combined with or followed by alkylating agents, plasma-exchange, and angiotensin-converting enzyme (ACE) inhibitors. Symptomatic treatment includes: 1) dietary regimen with normal protein intake and salt restriction, 2) calcium and vitamin D are prescribed with steroids, 3) diuretics should be used in case of severe edema, 4) infusion of albumin in case of severe hypovolemia, 5) treatment of hypertension, 6) anticoagulant therapy, and 7) prophylactic antibiotics in high-risk patients.

scholarly journals Predictive value of platelet indices in children with idiopathic nephrotic syndrome

2019 ◽  
Vol 6 (1) ◽  
pp. e04-e04
Author(s):  
Azar Nickavar ◽  
Sahar Sadr Moharerpour ◽  
Ehsan Abiry
Keyword(s):  
Nephrotic Syndrome ◽  
Idiopathic Nephrotic Syndrome ◽  
Steroid Resistance ◽  
Platelet Volume ◽  
Distribution Width ◽  
Steroid Resistant ◽  
Qualitative And Quantitative ◽  
Platelet Counts ◽  
Steroid Sensitive ◽  
Quantitative Changes

Introduction: Different biomarkers have been investigated for prognosis of patients with nephrotic syndrome. Qualitative and quantitative changes have been reported in platelets in these patients. Objectives: The aim of this study was to identify platelet abnormalities and their prognostic value of steroid response in children with idiopathic nephrotic syndrome. Patients and Methods: Platelet counts and indices (mean platelet volume [MPV], platelet distribution width [PDW] and platelet larger cell ratio [PLCR]) were evaluated and compared in 122 children with active nephrotic syndrome (64%; steroid resistant and 36%; steroid sensitive). Results: Mean age at diagnosis was 55 months (6 to169 months), and males outnumbered females (1.7/1). Steroid resistant patients had significantly higher platelet counts and lower PLCR, compared to steroid sensitive group. Regarding area under the ROC curve, low MPV, high PDW and low PLCR showed relatively acceptable correlation with steroid resistance. Conclusion: Increased platelet counts in addition to low PLCR are the suggestive indicators of steroid resistance in children with idiopathic nephrotic syndrome.

scholarly journals Predictive Value of Serum Interleukins in Children with Idiopathic Nephrotic Syndrome

2020 ◽  
Author(s):  
Azar Nickavar ◽  
Ehsan Valavi ◽  
Baranak Safaeian ◽  
Parisa Amoori ◽  
Mostafa Moosavian
Keyword(s):  
Nephrotic Syndrome ◽  
Serum Concentration ◽  
Inflammatory Cytokines ◽  
Predictive Value ◽  
Idiopathic Nephrotic Syndrome ◽  
Healthy Controls ◽  
Steroid Resistant ◽  
Acceptable Accuracy ◽  
Steroid Sensitive ◽  
Pro Inflammatory Cytokines

Pro-inflammatory cytokines have been suggested in the pathogenesis of idiopathic nephrotic syndrome (INS), with conflicting results. This study was performed to identify alteration of different serum interleukins (ILs) in children with INS, and their predictive value in response to steroid treatment. Three groups of children (27; steroid-sensitive INS, 21; steroid-resistant INS, and 19 healthy controls) with normal serum C3, negative serologic tests of hepatitis B virus (HBV), hepatitis C virus (HCV), human immune deficiency virus (HIV), and parasitic infections were included in this study. Serum concentrations of IL-1β, IL-2, IL-6, IL-8, IL-13, and IL-18 were measured, using quantitative colorimetric sandwich ELISA kits. Children with secondary nephrotic syndrome, inflammations, systemic disorders, and chronic kidney disease were excluded. The serum concentration of all ILs; except IL-13 and IL-18; was significantly higher in children with INS, compared with the healthy controls. Serum IL-2 had the highest sensitivity of (95.24%) in patients with INS. All of the serum ILs had acceptable accuracy in children with INS, compared with the control group. The serum concentration of IL-1β, IL-6, and IL-8 was significantly higher in children with steroid-sensitive nephrotic syndrome (SSNS), compared with steroid-resistant nephrotic syndrome (SRNS). All of these ILs had acceptable accuracy for the prediction of steroid response in patients with INS. Our findings suggested the pathogenic role of pro-inflammatory cytokines in children with INS, of which IL-1β, IL-6, and IL-8 were accurate biomarkers for the prediction of steroid response in these patients.

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.

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