Levamisole in Steroid-Sensitive Nephrotic Syndrome Children with Frequent Relapses and/or Steroid Dependency: Comparison of Daily and Every-Other-Day Usage

2004 ◽  
Vol 97 (4) ◽  
pp. c137-c141 ◽  
Author(s):  
Lin-Shien Fu ◽  
Chao-Yan Shien ◽  
Ching-Shiang Chi
Keyword(s):  
Nephrotic Syndrome ◽  
Steroid Dependency ◽  
Frequent Relapses ◽  
Steroid Sensitive Nephrotic Syndrome ◽  
Steroid Sensitive

scholarly journals Levamisole in Children with Idiopathic Nephrotic Syndrome: Clinical Efficacy and Pathophysiological Aspects

2019 ◽  
Vol 8 (6) ◽  
pp. 860 ◽  
Author(s):  
Anne K. Mühlig ◽  
Jun Young Lee ◽  
Markus J. Kemper ◽  
Andreas Kronbichler ◽  
Jae Won Yang ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Idiopathic Nephrotic Syndrome ◽  
Low Frequency ◽  
Glomerular Diseases ◽  
Steroid Dependency ◽  
Randomized Controlled ◽  
Steroid Dependent ◽  
Frequent Relapses ◽  
Steroid Sensitive Nephrotic Syndrome ◽  
Steroid Sensitive

Steroid sensitive nephrotic syndrome is one of the most common pediatric glomerular diseases. Unfortunately, it follows a relapsing and remitting course in the majority of cases, with 50% of all cases relapsing once or even more often. Most children with idiopathic nephrotic syndrome respond initially to steroid therapy, nevertheless repeated courses for patients with relapses induce significant steroid toxicity. Patients with frequent relapses or steroid dependency thus require alternative treatment, such as cyclophosphamide, cyclosporine, tacrolimus, mycophenolate mofetil, levamisole, or rituximab. To reduce the relapse rate, several drugs have been used. Among these, levamisole has been considered the least toxic and least expensive therapy. Several randomized controlled trials (RCT) showed that levamisole is effective in reducing the relapse risk in steroid sensitive forms of nephrotic syndrome with a low frequency of side effects. Levamisole is a synthetic imidazothiazole derivative with immune-modulatory properties. In this article, we review recent data from randomized trials and observational studies to assess the efficacy of levamisole in frequently relapsing nephrotic syndrome and steroid-dependent nephrotic syndrome.

scholarly journals Recent Update in The Management of Childhood Nephrotic Syndrome

2016 ◽  
Vol 34 (1) ◽  
pp. 26-32 ◽  
Author(s):  
Mohammad Imrul Kayes
Keyword(s):  
Nephrotic Syndrome ◽  
Calcineurin Inhibitors ◽  
Clinical Problem ◽  
Developed Countries ◽  
Optimal Care ◽  
Steroid Dependency ◽  
Steroid Dependent ◽  
Frequent Relapses ◽  
Steroid Sensitive

Nephrotic Syndrome (NS) is a common renal disease seen in children. Children who go into complete remission following treatment with corticosteroids are classified as having steroid sensitive NS. In developed countries over 80% of children with idiopathic NS have steroid sensitive disease. The exact pathogenesis of this condition remains elusive. Podocyte injury and proteinuria are the two main issues in the pathogenesis. Recent studies suggest release of cytokines by T-cells as well as a strong contribution of Bcell immunity. Genetic studies have reported human leucocyte antigen (HLA) class II antigens DR and DQ associations linked to steroid sensitive NS. Most children with steroid sensitive NS have multiple relapses and a significant percentage also develop steroid dependent NS. Diuretic- resistant edema also a clinical problem to manage these patients. These children receive multiple courses of steroids and are at high risk of developing steroid toxicity. Patient with frequent relapses who develop steroid dependency thus require alternative treatment. Steroid resistant NS considers when failure to response within 8 weeks of steroid therapy. Steroids sparing agents used include levamisole, cyclophosphamide, mycophenolate mofetil (MMF), calcineurin inhibitors (cyclosporine and tacrolimus), rituximab and vincristine; these agents have greatly reduced the adverse effects seen with long-term use of steroids; so therapy needs to be individualized to achieve optimal care of each child.J Bangladesh Coll Phys Surg 2016; 34(1): 26-32

scholarly journals Pediatric idiopathic steroid-sensitive nephrotic syndrome: diagnosis and therapy —short version of the updated German best practice guideline (S2e) — AWMF register no. 166-001, 6/2020

2021 ◽  
Author(s):  
Rasmus Ehren ◽  
Marcus R. Benz ◽  
Paul T. Brinkkötter ◽  
Jörg Dötsch ◽  
Wolfgang R. Eberl ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Working Group ◽  
Pediatric Nephrology ◽  
High Rate ◽  
Short Version ◽  
Published Evidence ◽  
Cochrane Reviews ◽  
Frequent Relapses ◽  
Steroid Sensitive Nephrotic Syndrome ◽  
Steroid Sensitive

AbstractIdiopathic nephrotic syndrome is the most frequent glomerular disease in children in most parts of the world. Children with steroid-sensitive nephrotic syndrome (SSNS) generally have a good prognosis regarding the maintenance of normal kidney function even in the case of frequent relapses. The course of SSNS is often complicated by a high rate of relapses and the associated side effects of repeated glucocorticoid (steroid) therapy. The following recommendations for the treatment of SSNS are based on the comprehensive consideration of published evidence by a working group of the German Society for Pediatric Nephrology (GPN) based on the systematic Cochrane reviews on SSNS and the guidelines of the KDIGO working group (Kidney Disease - Improving Global Outcomes).

scholarly journals Minimal Change Nephrotic Syndrome Relapse after 52 Years of Remission: A Case Report

2018 ◽  
Vol 8 (2) ◽  
pp. 178-181
Author(s):  
Hideaki Yamabe ◽  
Mitsuaki Kaizuka ◽  
Satoru Tsunoda ◽  
Michiko Shimada
Keyword(s):  
Nephrotic Syndrome ◽  
Rare Case ◽  
Minimal Change Nephrotic Syndrome ◽  
Minimal Change ◽  
Urinary Protein ◽  
Japanese Male ◽  
Frequent Relapses ◽  
Old Japanese ◽  
Steroid Sensitive Nephrotic Syndrome ◽  
Steroid Sensitive

Minimal change nephrotic syndrome (MCNS) is the most common cause of nephrotic syndrome in children and can also present in adults. Corticosteroids generally induce remission of MCNS, and relapses are common after reduction or discontinuation of corticosteroids. We experienced a rare case of steroid-sensitive MCNS where the patient relapsed after 52 years of remission. The patient was a 61-year-old Japanese male who visited our clinic for an edema of the lower extremities which had already persisted for a few days. Laboratory testing showed massive urinary protein and low serum total protein and albumin levels. Therefore, he was diagnosed with nephrotic syndrome. He had a history of nephrotic syndrome that initially developed when he was 5 years old. Although corticosteroids reduced the urinary protein level, frequent relapses occurred when their doses were reduced, or when they were discontinued. He had previously experienced a relapse when he was 9 years old. For his current condition, treatment with corticosteroids and diuretics for 1 week reduced his edema and proteinuria. We suspected that this is a case of MCNS and that the present event is a relapse. Thus, we concluded that this is a very rare case of steroid-sensitive nephrotic syndrome that relapsed after 52 years of remission.

scholarly journals Steroid treatment for the first episode of childhood nephrotic syndrome: comparison of the 8 and 12 weeks regimen using an individual patient data meta-analysis

2021 ◽  
Author(s):  
Anne M. Schijvens ◽  
Nynke Teeninga ◽  
Eiske M. Dorresteijn ◽  
Steven Teerenstra ◽  
Nicholas J. Webb ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Individual Patient Data ◽  
Meta Analysis ◽  
Steroid Treatment ◽  
First Episode ◽  
Childhood Nephrotic Syndrome ◽  
Steroid Sensitive Nephrotic Syndrome ◽  
Steroid Sensitive ◽  
First Relapse ◽  
Steroid Regimen

AbstractSteroids are the cornerstone of the treatment of childhood nephrotic syndrome. The optimal duration for the first episode remains a matter of debate. The aim of this study is to determine whether the 8 weeks International Study of Kidney Disease in Children (ISKDC) regimen is equally effective as the 12 weeks steroid regimen from the German society of pediatric nephrology (Arbeitsgemeinschaft für Pädiatrische Nephrologie [APN]). An individual patient data (IPD) meta-analysis of randomized controlled trials reporting on prednisolone treatment for a first episode of childhood nephrotic syndrome was conducted. European trials aimed at investigating the ISKDC and/or APN steroid regimen were selected. The lead investigators of the selected trials were requested to provide the IPD of the specific treatment groups. Four trials included European cohorts using dosing schedules according to the regimens studied. IPD of two trials were available. A significant difference was found in time to first relapse after cessation of steroid treatment between the 8 and 12 weeks treatment group with a median time to relapse of 29 and 63 days, respectively. Moreover, relapse rate ratios during total follow-up were 51% higher for the 8 weeks regimen. Finally, younger children have a significantly lower time to first relapse and frequently relapsing nephrotic syndrome.Conclusions: The results of this IPD meta-analysis suggest that the 8 weeks steroid regimen for a first episode of steroid-sensitive nephrotic syndrome may not be equally effective as the 12 weeks steroid regimen. Moreover, this study highlights the importance of using uniform definitions to enable accurate comparison and interpretation of trial results.Trial registration: Registration number: CRD42020199244, date of registration 16-08-2020 What is Known:• Steroids are the cornerstone of the treatment of childhood nephrotic syndrome, however the optimal duration for the first episode remains a matter of debate.• Currently, the 8 weeks ISKDC protocol and 12 weeks APN protocol are among the most frequently used protocols in Europe. What is New:• The 8 weeks steroid regimen for a first episode of steroid-sensitive nephrotic syndrome may not be equally effective as the 12 weeks steroid regimen for the treatment of a first episode of nephrotic syndrome.• Younger children have a significantly shorter time to first relapse and time to frequent relapsing nephrotic syndrome.

scholarly journals Distribution of serum adiponectin isoforms in pediatric patients with steroid-sensitive nephrotic syndrome

2021 ◽  
Author(s):  
Tetsuro Tamai ◽  
Kaori Kamijo ◽  
Yoshifusa Abe ◽  
Satoshi Hibino ◽  
Shunsuke Sakurai ◽  
...  
Keyword(s):  
Molecular Weight ◽  
Nephrotic Syndrome ◽  
Pediatric Patients ◽  
Serum Adiponectin ◽  
Total Serum ◽  
Control Subjects ◽  
Remission Period ◽  
Total Adiponectin ◽  
Steroid Sensitive Nephrotic Syndrome ◽  
Steroid Sensitive

Abstract Background Serum adiponectin circulates in three multimeric isoforms: high-molecular-weight (HMW), middle-molecular-weight (MMW), and low-molecular-weight (LMW) isoforms. Potential change in the circulating adiponectin levels in patients with nephrotic syndrome (NS) remain unknown. This study aimed to assess the levels of total adiponectin and the distribution of its isoforms in pediatric patients with NS. Methods We sequentially measured total adiponectin and each adiponectin isoform levels at the onset of NS, initial remission, and during the remission period of the disease in 31 NS patients. We also calculated the ratios of HMW (%HMW), MMW (%MMW), and LMW (%LMW) to total adiponectin incuding 51 control subjects. Results The median of total serum adiponectin levels in patients were 36.7, 36.7, and 20.2 μg/mL at the onset, at initial remission, and during the remission period of NS, respectively. These values were significantly higher than those in control subjects. The median values of %HMW, %MMW, and %LMW values were 56.9/27.0/14.1 at the onset, 62.0/21.8/13.4 at the initial remission, and 58.1/21.7/17.5 at during the remission period of NS, respectively. Compared with control subjects, %HMW at initial remission and %MMW at the onset were high, and the %LMW values at the onset and at initial remission were low. Conclusions In patients with NS, total serum adiponectin levels increase at the onset of the disease, and the ratio of adiponectin isoforms changes during the course of the disease. Further studies are needed to delineate the mechanisms between proteinuria and adiponectin isoforms change.

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