scholarly journals Lipoprotein (a) as a Predictor of Steroid Dependence in Paediatric Steroid Sensitive Nephrotic Syndrome

2021 ◽  
Author(s):  
Surupa Basu ◽  
Sushmita Banerjee ◽  
Pranab Roy ◽  
Apurba Ghosh
Keyword(s):  
Nephrotic Syndrome ◽  
First Episode ◽  
Urine Protein ◽  
Lipoprotein A ◽  
Steroid Dependence ◽  
Steroid Dependency ◽  
Steroid Dependent ◽  
Steroid Sensitive ◽  
One Year ◽  
Lipid Parameters

Introduction: Lipoprotein a {Lp(a)} increases in Nephrotic Syndrome (NS). Although the majority of paediatric NS are steroid sensitive, relpase and steroid dependence are commonly seen in this cases. Lp(a) is an LDL-like lipoprotein that consists of an LDL particle to which the glycoprotein apolipoprotein(a) {apo(a)} is attached. Aim: To evaluate the potential of Lp(a), measured on admission, for the prediction of relapse/steroid dependency. Materials and Methods: Children (n=36) with first episode NS were recruited in this prospective observational case-control study and followed up for one year. They were tested at presentation for Lp(a) (mg/dL) and standard tests such as haemoglobin, albumin, protein, cholesterol, triglyceride, and urine protein. Children received standard therapy for NS, and were followed for a period of one year from diagnosis to record days to initial remission, relapse episodes, steroid dependence etc. Patients were categorised as: no relapse (NR), Infrequent Relapse (IFR), frequent relapse (FR) and Steroid Dependent (SD) as per standard definitions. Fifteen healthy volunteers were also tested for lipid profile and Lp(a) levels. Results: Of 36 cases (median age 3 years, 19 males), there were 15NR, 7IFR, 2FR and 12SD. The mean Lp(a) of the NS group (165.2±120.4 mg/dL) was higher than controls (30.52±21.9 mg/dL) (p<0.0001). All the lipid parameters except HDL-cholesterol were significantly higher in the NS group. Within the NS group, Lp(a) showed significant correlation (Spearman-rho) with albumin (p=0.0062,r=0.47), but no correlation with lipid parameters or urine protein. Comparison of Lp(a)levels in the NS groups revealed that the SD patients had a high Lp(a)(222.0±115.7 mg/dL) compared to NR (129.7±120.1 mg/dL) (p=0.02). Conclusion: Concentration of plasma Lp(a) in patients with SDNS was higher compared to patients who did not suffer any relapse, and this concentration may serve as a marker for prediction of SDNS.

scholarly journals A study to assess the prevalence of hypertension in children with nephrotic syndrome

2019 ◽  
Vol 6 (6) ◽  
pp. 2340
Author(s):  
Manasa M. ◽  
Anitha S. Prabhu ◽  
Santosh Pai ◽  
Kiran Raj H.
Keyword(s):  
Nephrotic Syndrome ◽  
Clinical Feature ◽  
First Episode ◽  
Sodium Retention ◽  
Single Drug ◽  
Steroid Dependent ◽  
Co Morbidity ◽  
Study Population ◽  
Steroid Sensitive ◽  
Hypertension In Children

Background: Hypertension is been one of the most common co morbidity of this disease. It was mostly attributed to sodium retention, which is a major clinical feature of nephrotic syndrome. These mechanisms likely have a role in the development of hypertension in nephrotic syndrome, where hypertension may be difficult to control, and provide new therapeutic options for the management of blood pressure in the setting of nephrotic syndrome. Objective of study the prevalence of hypertension in children with NS and also the number of antihypertensive required to control it.Method: A Retrospective study of the hospital records of 100 children diagnosed with nephrotic syndrome admitted to Pediatric and Nephrology Ward at YMCH was accessed.Results: In our study 35 (35%) of them were Infrequent relapse nephrotic syndrome (IFNS) and 35(35%) were  Frequent relapse nephrotic syndrome (FRNS) ,while 30 cases (30%) were First episode nephrotic syndrome (FENS). 65 cases were steroid sensitive, while 28 and 7 of them were steroid dependent and resistant respectively. Of the 100 study population 54 of them had hypertension while 46 of them did not develop it .Of the 54 hypertensive nephrotic syndrome children, 15 of them (28.%) required three anti hypertensives to control the pressure, while 19 (35%) and 20 (37%) required single and dual anti hypertensives respectively.Conclusion: Prevalence of hypertension is increasing among the children with nephrotic syndrome. Its more prevalent among the male then female FRNS, SRNS and SDNS are more prone to develop hypertension and also they needed two or more antihypertensives to control the hypertension, whereas hypertension in SSNS could be managed with single drug.

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 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 Cyclophosphamide in frequent-relapsing or steroid-dependent nephrotic syndrome: Review of 38 patients

2016 ◽  
Vol 45 (1) ◽  
pp. 18
Author(s):  
Yulia Iriani ◽  
Taralan Tambunan ◽  
Sudigdo Sastroasmoro
Keyword(s):  
Nephrotic Syndrome ◽  
Cyclophosphamide Therapy ◽  
Steroid Dependent ◽  
Steroid Sensitive Nephrotic Syndrome ◽  
Steroid Sensitive ◽  
The Mean ◽  
One Year ◽  
Steroid Dependent Nephrotic Syndrome ◽  
Better Than

Background Steroid-sensitive nephrotic syndrome (SSNS) in chil-dren is characterized by relapsing courses in a substantial propor-tion of affected individuals. Children with frequent-relapsing neph-rotic syndrome (FRNS) or steroid-dependent nephrotic syndrome(SDNS) are at risk of severe steroid toxicity and need individual-ized treatment. Previous studies have elucidated that cyclophos-phamide (CPA) reduced the risk of relapses and increased thelength of subsequent remissions in children with relapsing SSNS.Methods This retrospective study evaluated 38 patients (26 FRNSand 12 SDNS) after cyclophosphamide therapy to elucidate theefficacy of CPA in FRNS or SDNS in the Department of Child Health,Cipto Mangunkusumo Hospital. All patients were treated with CPA(2 mg/kg per day) for 8 weeks, in combination with prednisone.Results The median (range) duration of follow up was 45 months(24-140 months) for FRNS and 29 months (24-63 months) forSDNS. The mean relapse rate one year prior to CPA therapy inFRNS and SDNS were 3.8 relapses/year (95%CI 3.4; 4.2) and 4.0relapses/year (95%CI 3.3; 4.7), which were reduced to 1.6 relapses/year (95% CI 1.1; 2.1) and 2.3 relapses/year (95%CI 1.5;3.2), re-spectively. The overall rate of cumulative sustained good response(complete remission or infrequent relapses) was 65% after 36months. Frequent relapsing versus steroid-dependent status wassignificantly correlated with rate of sustained good response after36 months (85% versus 15%) with OR=23 (95%CI 3.1;225.2).Conclusion The efficacy of cyclophosphamide therapy in themanagement of FRNS is better than in SDNS

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.

Short-Term Steroid Regimen for Adult Steroid-Sensitive Minimal Change Disease

2018 ◽  
Vol 49 (1) ◽  
pp. 54-63 ◽  
Author(s):  
Takaya Ozeki ◽  
Takayuki Katsuno ◽  
Hiroki Hayashi ◽  
Sawako Kato ◽  
Yoshinari Yasuda ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Minimal Change Disease ◽  
Minimal Change ◽  
First Episode ◽  
Short Term ◽  
Short Term Treatment ◽  
Steroid Dose ◽  
Steroid Sensitive ◽  
Steroid Regimen ◽  
Frequent Relapse

Background: In pediatric patients with steroid-sensitive nephrotic syndrome, recent trials have revealed that a 2-month, short-term steroid regimen is not inferior to an extended steroid course. However, the optimal duration of initial steroid therapy for adult steroid-sensitive minimal change disease (MCD) remains unclear. Objectives: The aim of present study was to evaluate the effectiveness of a 2-month, short-term steroid regimen in the treatment of adult steroid-sensitive MCD patients. Method: This was a prospective observational study. Adult patients with steroid-sensitive MCD (n = 35) who were initiated on a short-term steroid regimen between January 2015 and June 2016 were included. The details of the regimen are as follows: (1) prednisolone was administered at an initial dose of 0.8–1.0 mg/kg/day and continued for 4–6 weeks and (2) dosage was reduced to 0.5–0.6 mg/kg/alternate day and continued for 4 weeks. Control patients (n = 140), who were treated using conventional steroid administration, were selected from our previous adult MCD cohort. All patients fulfilled the following criteria: biopsy-proven MCD, age ≥20 years, first episode of nephrotic syndrome, and attainment of complete remission within 4 weeks. The following parameters of patients who received short-term treatment regimen and control patients were compared: any relapse and frequent relapse, adverse events caused by steroid treatment and cumulative steroid dose. Results: Throughout the observation period (median: 17.3 months), 24 (68.6%) patients in the short-term group developed at least one relapse. The short-term regimen showed earlier occurrence of any relapse than the conventional regimen (adjusted hazard ratio [aHR] 2.45; 95% CI 1.51–3.97; p < 0.001), but there was no difference in frequent relapse (aHR 1.31; 95% CI 0.43–3.99; p = 0.63). None of the patients showed any symptoms of adrenal insufficiency after discontinuation of corticosteroids. The cumulative steroid dose during the observational period was significantly lower in the short-term group than in the conventional group. Conclusions: The short-term steroid regimen may represent an effective treatment option that ensures lower steroid exposure when treating adult steroid-sensitive MCD patients.

scholarly journals Prediction of Relapse by Plasma Lipoprotein(a) Concentration in Children with Steroid-Sensitive Nephrotic Syndrome

Nephron ◽  
2002 ◽  
Vol 92 (4) ◽  
pp. 807-811 ◽  
Author(s):  
Yukihiko Kawasaki ◽  
Junzo Suzuki ◽  
Ruriko Nozawa ◽  
Shigeo Suzuki ◽  
Hitoshi Suzuki
Keyword(s):  
Nephrotic Syndrome ◽  
Plasma Lipoprotein ◽  
Lipoprotein A ◽  
Steroid Sensitive Nephrotic Syndrome ◽  
Steroid Sensitive ◽  
Prediction Of Relapse

Levamisole in steroid-sensitive nephrotic syndrome: usefulness in adult patients and laboratory insights into mechanisms of action via direct action on the kidney podocyte

2015 ◽  
Vol 128 (12) ◽  
pp. 883-893 ◽  
Author(s):  
Lulu Jiang ◽  
Ishita Dasgupta ◽  
Jenny A. Hurcombe ◽  
Heather F. Colyer ◽  
Peter W. Mathieson ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Mode Of Action ◽  
Molecular Mechanisms ◽  
Cell Model ◽  
Direct Action ◽  
Adult Patients ◽  
Molecular Evidence ◽  
Puromycin Aminonucleoside ◽  
Steroid Dependent ◽  
Steroid Sensitive

Minimal change nephropathy (MCN) is the third most common cause of primary nephrotic syndrome in adults. Most patients with MCN respond to corticosteroid therapy, but relapse is common. In children, steroid-dependent patients are often given alternative agents to spare the use of steroids and to avoid the cumulative steroid toxicity. In this respect, levamisole has shown promise due to its ability to effectively maintain remission in children with steroid-sensitive or steroid-dependent nephrotic syndrome. Despite clinical effectiveness, there is a complete lack of molecular evidence to explain its mode of action and there are no published reports on the use of this compound in adult patients. We studied the effectiveness of levamisole in a small cohort of adult patients and also tested the hypothesis that levamisole's mode of action is attributable to its direct effects on podocytes. In the clinic, we demonstrate that in our adult patients, cohort levamisole is generally well tolerated and clinically useful. Using conditionally immortalized human podocytes, we show that levamisole is able to induce expression of glucocorticoid receptor (GR) and to activate GR signalling. Furthermore, levamisole is able to protect against podocyte injury in a puromycin aminonucleoside (PAN)-treated cell model. In this model the effects of levamisole are blocked by the GR antagonist mifepristone (RU486), suggesting that GR signalling is a critical target of levamisole's action. These results indicate that levamisole is effective in nephrotic syndrome in adults, as well as in children, and point to molecular mechanisms for this drug's actions in podocyte diseases.

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