First Episode Of Nephrotic Syndrome Research Articles

Early Rituximab as an Add-On Therapy in Children With the Initial Episode of Nephrotic Syndrome

The approximately 70% 12-month relapse in children experiencing the initial episode of steroid-sensitive nephrotic syndrome (SSNS) is a significant concern, with over 50% developing frequent relapses or steroid-dependent nephrotic syndrome (FRNS/SDNS). There is a lack of strategies to reduce relapse after the onset. Whether early administration of rituximab, which effectively reduces relapses in FRNS/SDNS, may be a solution has not been evaluated. A prospective, multicenter, open-label, single-arm trial was conducted in China, with a 12-month follow-up. Children aged 1 to 18 years with the first episode of nephrotic syndrome (NS) were screened for eligibility. Proteinuria was evaluated daily using dipsticks. A dose of 375 mg/m2 of rituximab was intravenously infused within 1 week after achieving corticosteroid-induced remission. The main outcome was 12-month relapse-free survival. Out of the initially 66 children screened, 44 were enrolled and received rituximab, with all but 1 participant completing the 12-month follow-up. The median age at diagnosis was 4.3 years (interquartile range [IQR]: 3.4-5.9), and 33 (77%) of the participants were male. In the rituximab group, the 12-month relapse-free survival was significantly higher compared to historical controls (32 of 43 [74.4%] vs. 10 of 33 [30.3%]; P< 0.001; hazard ratio [HR], 3.76; 95% confidence interval [CI], 1.80-7.81). The post hoc analysis revealed a higher 24-month relapse-free survival and a lower incidence of FRNS/SDNS at the 12-month follow-up. Treatment with rituximab was well-tolerated. Our findings support that early administration of rituximab may be associated with a higher 12-month relapse-free survival and a reduced incidence of FRNS/SDNS in children experiencing the initial episode of SSNS.

Serum lipid profile among pediatric folk with idiopathic nephrotic syndrome: An Observational Study

Introduction: The most familiar kidney disease in children is nephrotic syndrome (NS), associated with dyslipidemia and causes various cardiovascular complications. Persistent hyperlipidemia can further raise difficulties of NS and relapse cases. Earlier diagnosis and appropriate intervention increase the possibility of saving other vital organs.&#x0D; Methods: An observational study was conducted with 85 children aged 2-15 years with a history of NS. The study group was divided into first NS and second-episode relapse cases. They were further subgrouped into Frequent Relapse NS (FRNS) and Infrequent Relapse NS (IFRNS). Fasting lipid profiles were performed among active sufferers with a follow-up after 4 weeks.&#x0D; Results: The male-to-female ratio was 1.93:1. In the first episode of NS, all lipid parameters were high in the acute phase and returned to normal during remission. In relapse cases, lipid parameters were significantly higher even in remission. Serum triglyceride and very low-density lipoprotein were significantly more elevated in the FRNS group than in the IFRNS. Low-density lipoprotein was increased considerably in FRNS. Mean high-density lipoprotein was also significantly lower in the FRNS group than in the IFRNS.&#x0D; Conclusion: Persistent hyperlipidemia is most typical among FRNS. Hyperlipidemia management is urgent to prevent among pediatric NS.&#x0D; Bangladesh Journal of Medical Science Vol. 23 No. 01 January’24 Page : 39-46

A Study of Dyslipidemia and Its Clinical Implications in Childhood Nephrotic Syndrome.

Nephrotic syndrome in children is characterized by dyslipidemia, which is an indirect risk factor for cardiovascular diseases, progressive glomerulosclerosis, and related complications. The objective is to determine the range of lipid profile abnormalities in relation to onset, relapse, and remission, as well as to determine if there is any relationship between dyslipidemia and the frequency of relapses. One hundred and two diagnosed cases of nephrotic syndrome in the age group of less than 12 years were included, out of which 64 patients belonged to the first episode of nephrotic syndrome and 38 patients were relapse cases. Steroid-resistant nephrotic syndrome cases or patients with a history of diabetes mellitus, hypothyroidism, familial hypercholesterolemia, children with chronic kidney disease, and edema due to other causes were excluded from the study. There was a statistically significant increase in lipid parameters except for high-density lipoprotein (HDL) cholesterol at the disease onset when compared to remission in cases of the first episode as well as relapse cases of nephrotic syndrome. There was a positive correlation between relapse frequency and dyslipidemia. Dyslipidaemia was also associated with low serum albumin, with a p-value <0.001, which is statistically significant. Dyslipidemia is significantly higher in relapse cases of nephrotic syndrome and remains higher even during remission. Dyslipidemia is a directly associated risk factor for atherosclerosis and coronary heart disease (CAD), along with progressive glomerulosclerosis. Early identification and treatment of hyperlipidemia is therefore justified so that along with longevity, we can also improve the quality of life of children suffering from nephrotic syndrome.

Risk factors for relapse in pediatric nephrotic syndrome in Ranchi.

The nephrotic syndrome (NS) is a common childhood illness characterized by massive proteinuria, hyperlipidemia, and hypoalbuminemia. It is a disease of relapse, and therefore, it is a major problem to manage cases with frequent relapses. Prediction and prevention of risk factors is the key to successful management of childhood NS. An understanding of the risk factors that determine the course is useful in taking decisions regarding therapy and enables counseling. Sample size of 100 children of age 1-12 years of age with steroid sensitive nephrotic syndrome over duration of 1 year from April 2020 to May 2021. Risk factors for relapse were presence of infection during initial attack and first relapse as well as less time interval between remission in first episode of nephrotic syndrome and first relapse.incresed risk was also associated with inadequate treatment duration and less serum albumin level and high cholesterol level.

Four hundred IU vs One thousand IU of vitamin D supplementation in first episode of nephrotic syndrome

Use of steroids in nephrotic children may lead to changes in bone mineral density and osteoporosis eventually affecting growth on a long term basis. We compared the proportionate changes in bone mineral content (BMC) and density(BMD), Vitamin D levels, Serum Calcium, phosphate and alkaline phosphatase levels in nephrotic children with the aim of giving high Vs low vitamin D doses (1000 IU Vs 400 IU) to two groups; group 1 (n=20) vs group 2 (n=20) respectively. The median BMC in group 1 increased from 11.53±3.48 g to 11.61±3.54 g after 1000 IU Vitamin D supplement and was statistically significant. However group 2 showed insignificant increases in BMC from 11.24±2.71 g to 11.25±2.67 g following 400IU Vitamin D. The change in BMD observed in group 1 from a mean of 0.426 to 0.429g/cm2 whereas in group 2 with 400 IU of vitamin D it didn’t show any significant change. The median vitamin D increased significantly in both groups; from 16.62±7.20 ng/ml to 27.45±6.47 ng/ml in group 1 while in group 2 from 18.72±8.07 ng/ml to 26.18±7.61 ng/ml which was statistically significant. The serum calcium levels normalized irrespective of 1000 IU or 400 IU of vitamin D supplementation. Changes in serum phosphate levels (decline from initial) were statistically significant however the changes in serum ALP were insignificant. We concluded that children supplemented with 1000 IU /day of vitamin D had better osteoprotection as compared to the other group.

Cholinesterase is a Potential Biomarker with High Accuracy for the Nephrotic Syndrome Diagnosis in Minors.

BackgroundSerum Cholinesterase (CHE) levels have been found to be elevated in individuals with nephrotic syndrome (NS); nevertheless, it is unknown whether CHE can serve as a biomarker for NS diagnosis and what its diagnostic relevance is for NS in minors.MethodsIn this study, 138 minors aged 1–17 years with NS were enrolled, including 101 patients with the first episode of NS and 37 patients with relapsing NS. One hundred and four minors suffering from nephritis and 109 healthy minors were included as control groups. The clinical information and laboratory data of all NS patients and the control group were obtained. Logistic regression, correlation analyses and receiver operator characteristic curve were used to examine the value of CHE for NS patients.ResultsCompared to patients diagnosed with nephritis and healthy minors in the control group, the serum CHE levels of total/first episode/relapsing NS patients were substantially higher (P < 0.05). The CHE was an independent risk predictor of total (adjusted odds ratio [OR] = 2.23, 95% confidence interval [CI]: 1.57–3.18)/first episode (adjusted OR = 4.02, 95% CI: 1.47–11.08)/relapsing (adjusted OR = 2.04, 95% CI: 1.42–2.93) NS, and was positively correlated with total cholesterol in total/first episode/relapsing NS patients, respectively. The optimal cutoff for total/first episode/relapsing NS all was 11 KU/L, but the diagnostic accuracy in first episode NS (area under the curve [AUC] = 0.96, 95% CI: 0.94–0.98) was higher than the total NS (AUC = 0.93, 95% CI: 0.91–0.96) and relapsing NS (AUC = 0.85, 95% CI: 0.78–0.92).ConclusionCHE is a possible biomarker for NS and has good diagnostic accuracy for NS in minors, particularly for the first episode of NS in minors.

Interventions for minimal change disease in adults with nephrotic syndrome.

Interventions for minimal change disease in adults with nephrotic syndrome.

Time to remission in first episode of Idiopathic Nephrotic Syndrome in children with response to Levamisole in subsequent relapses

Background: Idiopathic nephrotic syndrome (NS) is the most common cause for NS in children. While it responds to steroids in most instances, relapses are also common. In frequently relapsing NS, though agents such as levamisole and cyclophosphamide are commonly used as steroid sparing agents, the response is often unpredictable. In this study, we aimed to determine the association, if any, between time to remission in the first episode of steroid sensitive nephrotic syndrome and the response to levamisole in the subsequent relapses. Materials and methods: The records of the children with steroid sensitive NS were studied. The children were grouped into two categories: group I, where the response to Prednisolone in the first episode occurred in less than 10 days of therapy, and group II, in whom the response to Prednisolone occurred between 10 and 28 days. The response to levamisole in the two groups was compared. Results: The records of 75 children (Males 48) were studied. Group I had 49 (65.3%) children, of whom, 42 children had frequent relapses and were treated with levamisole. 33(78.5%) children showed a good response to levamisole. In group II, all 26 (34.6%) children had frequent relapses and were treated with levamisole. Only 1 child (3.8%) showed a good response. There was a significant association of the time to remission in first episode of NS with the response to levamisole (p<0.001). Conclusion: Children with nephrotic syndrome showing a response to Prednisolone in less than 10 days are more likely to respond to levamisole, especially if the relapse rate is less than 3 in 6 months.

Severity of Vitamin D Deficiency in Children with Nephrotic Syndrome: A Study from Tertiary Care Center in Northern India.

In nephrotic syndrome (NS) due to podocytopathies, loss of vitamin D binding globulin along with albumin in urine leads to Vitamin D deficient state. We aimed to study the severity of vitamin D deficiency and its clinical correlation in children with NS. We performed a cross-sectional study at a tertiary care hospital in Northern India. Enrolment of children aged 1-18 years was done. Patient's detailed history, numbers of relapse, treatment details, and data regarding various immunomodulatory drugs treatment. Vitamin D level was estimated, and its status was further classified as deficiency <20 ng/mL and insufficiency 20-30 ng/mL as per Global Consensus Recommendations on evaluation, treatment, and prevention of vitamin D deficiency. Continuous variables were compared using tests such as unpaired t-test, Kruskal-Wallis test, and Wilcoxon rank sum test depending on the distribution of parameters. Categorical variables were compared using Chi-squared tests or Fisher's exact test. A total of 96 children with NS were screened, of which 77.1% had vitamin D deficiency. The mean serum vitamin D level was 14.393 ± 8.52 ng/mL. Among the 48 children of the first episode of NS 36 were deficient (36/48 = 75%). Whereas in the relapse category, 30 patients had infrequently relapsing NS (deficient 24/30 = 80%). Eleven children had frequently relapsing NS; among them, 10 were vitamin D deficient (10/11 = 90.9%), and there was a negative correlation between vitamin D level and duration of illness. Vitamin D deficiency is a common comorbidity in children with NS. Given the putative immunomodulatory property of vitamin D, this deficiency should be identified and treated routinely in all cases of NS.

Severe Acute Kidney Injury in a Child with Nephrotic Syndrome

Nephrotic syndrome (NS) may have complications like infection, thromboembolism, acute kidney injury (AKI) and hypovolemia in children. However, severe AKI with anuria is unusual and rarely reported. In this study, 9 year-old girl hospitalized with first episode of NS after recent antibiotic and ibuprofen use was presented. Despite albumin and furosemide, as she became anuric on the third day of hospitalization, hemodialysis was started and pulse methylprednizolone was given. Renal biopsy revealed minimal mesangial proliferation with widespread acute tubular injury findings. She was discharged at the 15th day with normal urine volume after 11 hemodialysis sessions. After six weeks of oral prednisolone, complete remission in NS was achieved. Acute tubular necrosis was the underlying diagnosis for anuric AKI in the setting of NS. Heavy proteinuria leading to tubular obstruction, presence of decreased effective intravascular volume due to severe hypoalbuminemia, and ibuprofen use may have caused acute tubular necrosis.

Evaluation of Acute Kidney Injury and its Risk Factors in Children Admitted with Diagnosis of Nephrotic Syndrome

Abstract Background and Aim: Acute Kidney Injury (AKI) is an important complication of Nephrotic Syndrome (NS) associated with adverse outcome. The frequency of AKI has increased to almost double in the last decade. To determine the incidence of AKI, risk factors and its association with outcome in hospitalized children with NS. Methods – All children aged 1-18 years with diagnosis of NS from 01 November 2018 to 31 May 2020 were enrolled for the study. AKI was diagnosed using the KIDIGO 2012 guidelines and classified according to pediatric RIFLE definition. Results –The mean age of children in this cohort was 4.7± 2.8 years. Complications were observed in 67% cases only. Among all complications the most frequent complication was anemia (25%). The incidence of AKI in hospitalized children with NS was 18.6%. According to pRIFLE criteria of AKI; 11.6% of children met Stage 1 (risk) criteria, 4.6% met Stage 2 (injury) criteria and 2.3% met Stage 3 (failure) respectively. Among all NS children, 53% received nephrotoxic drugs during the hospital stay. On applying multivariate logistic regression analysis, only male gender, associated anemia and Vancomycin use in NS patients came out to be significant independent risk factor for acute kidney injury. Conclusions: AKI is more frequent in first episode of NS rather than in any type of relapses. Although, nephrotoxic drug and male gender are known independent risk factors for development of AKI, associated anemia is till yet not considered as independent risk factor for AKI in children with NS.

ROLE OF MONTELUKAST IN PREVENTING RELAPSE IN CHILDHOOD IDIOPATHIC NEPHROTIC SYNDROME: A QUASI-EXPERIMENTAL STUDY

OBJECTIVE: To study the role of montelukast in preventing relapses in childhood idiopathic steroid sensitive nephrotic syndrome (SSNS).&nbsp;&nbsp; METHODS: This quasi-experimental, open label study was conducted at Department of Paediatric Nephrology, The Children’s Hospital and The Institute of Child Health, Multan, Pakistan. Total of 106 patients with first-episode of nephrotic syndrome, aged 1-10 years were enrolled from February-May 2019 and non-randomly assigned to Group-A &amp; Group-B (53 patients in each group). All patients in both groups were started on prednisolone 2mg/kg single morning dose for 4-weeks as per institutional protocol. Group-A (Cases) also received montelukast 5mg daily at bedtime for at least 12 months while Group-B (control) was not given montelukast. All patients were followed up for one year till May 2020 to monitor the response to treatment and evidence of relapse. Ten patients were excluded from final analysis due to loss to follow-up (n=3; 2 in Group-A and 1 in Group-B) or showing steroids resistance (n=7; 3 in Group-A and 4 in Group-B). RESULTS: Out of 96 children (48 each in Group-A &amp; B), 58 (60.4%) were males. Mean age was 4.7±1.9 years and most of children (n=64; 66.7%), were aged between 2-6 years.&nbsp; Overall relapse rate of 35.4% (n=34/98) was observed, with 9/48 (18.8%) patients in Group-A compared to 25/48 (52.1%) patients in Group-B (p&lt;0.001). Out of 34 patients with relapse, 20 (58.8%) were males and 14 (41.2%) were females. CONCLUSION: Montelukast showed significant efficacy in preventing relapse rate in children with SSNS during one year of follow-up.

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

Steroids 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 16-08-2020What 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.

Practice variations in the management of childhood nephrotic syndrome in the Netherlands

Nephrotic syndrome in childhood is a common entity in the field of pediatric nephrology. The optimal treatment of children with nephrotic syndrome is often debated. Previously conducted studies have shown significant variability in nephrotic syndrome management, especially in the choice of steroid-sparing drugs. In the Netherlands, a practice guideline on the management of childhood nephrotic syndrome has been available since 2010. The aim of this study was to identify practice variations and opportunities to improve clinical practice of childhood nephrotic syndrome in the Netherlands. A digital structured survey among Dutch pediatricians and pediatric nephrologists was performed, including questions regarding the initial treatment, relapse treatment, kidney biopsy, additional immunosuppressive treatment, and supportive care. Among the 51 responses, uniformity was seen in the management of a first presentation and first relapse. Wide variation was found in the tapering of steroids after alternate day dosing. Most pediatricians and pediatric nephrologists (83%) would perform a kidney biopsy in case of steroid-resistant nephrotic syndrome, whereas for frequent relapsing and steroid-dependent nephrotic syndrome this was 22% and 41%, respectively. Variation was reported in the steroid-sparing treatment. Finally, significant differences were present in the supportive treatment of nephrotic syndrome.Conclusion: Substantial variation was present in the management of nephrotic syndrome in the Netherlands. Differences were identified in steroid tapering, use of steroid coverage during stress, choice of steroid-sparing agents, and biopsy practice. To promote guideline adherence and reduce practice variation, factors driving this variation should be assessed and resolved.What is Known:• National and international guidelines are available to guide the management of childhood nephrotic syndrome.• Several aspects of the management of childhood nephrotic syndrome, including the choice of steroid-sparing drugs and biopsy practice, are controversial and often debated among physicians.What is New:• Significant practice variation is present in the management of childhood nephrotic syndrome in the Netherlands, especially in the treatment of FRNS, SDNS, and SRNS.• The recommendation on the steroid treatment of a first episode of nephrotic syndrome in the KDIGO guideline leaves room for interpretation and is likely the cause of substantial differences in steroid-tapering practices among Dutch pediatricians and pediatric nephrologists.

Comparison of clinical and lab profile between steroid sensitive and steroid resistant nephrotic syndrome at onset of disease and evaluating predictors for developing steroid resistance in nephrotic syndrome

Background: Nephrotic syndrome is a notable chronic disease in children. The objective of this study was to compare the clinical and lab profile between steroid sensitive nephrotic syndrome and steroid resistant nephrotic syndrome at the onset of disease. Certain parameters were tested if they could be significate predictors of developing steroid resistance at the onset of first episode of nephrotic syndrome.Methods: Retrospective observation study done children 1-12 years diagnosed with nephrotic syndrome in Sri Ramachandra Medical College and Hospital, Department of Paediatrics, Chennai. Sample size 150. Period of study Jan 2013- Dec 2015. Variables considered were age at onset, sex, parental consanguinity with essential lab parameters done at the onset of nephrotic syndrome proteinuria, pyuria, microscopic hematuria, urine protein creatinine ratio, serum creatinine, serum triglycerides and serum albumin. Children less than 1 year of age, cases with secondary causes of nephrotic syndrome and steroid dependant nephrotic syndrome, children with incomplete records were not included in this study. 150 cases who fulfilled the study criteria were included in this study.Results: 75 cases of steroid sensitive nephrotic syndrome (SSNS) were compared with an equal number of steroid resistant nephrotic syndrome (SRNS). 85 children had onset of disease before 3 years of age and majority had 3+ proteinuria and males predominated in both the groups. The overall consanguinity rates were higher among SRNS group. Triglyceride level &gt;300 mg/dl predominated in SRNS group along with a higher severity of hypoalbuminemia when compared to SSNS group. None of the parameters tested were significant predictors of developing SRNS subsequently.Conclusions: Comparing steroid sensitive with steroid resistance nephrotic syndrome, no lab parameter could identify the risk of a child developing steroid resistance subsequently. This could be a field of interest in future studies that could predict the development of steroid resistance at the onset of first episode of nephrotic syndrome itself.

Interventions for idiopathic steroid-resistant nephrotic syndrome in children.

Interventions for idiopathic steroid-resistant nephrotic syndrome in children.

SAT-313 EFFECT OF NORMALIZATION OF 25 (OH) VITAMIN D LEVELS ON FREQUENCY OF RELAPSES IN FIRST YEAR OF CHILDHOOD NEPHROTIC SYNDROME A NON RANDOMIZED CONTROLLED TRIAL

Low 25 (OH) Vitamin D levels have been described in childhood nephrotic syndrome (NS) due to nutritional deficiency and loss of vitamin D binding proteins in the urine. This study was done to detect the prevalence of 25(OH) D deficiency in children with the first attack of NS and to evaluate the impact of normalizing 25(OH) D levels on the number and frequency of relapses in the first year following the initial attack of NS. Children presenting with first episode of NS were treated with standard steroid regime for 12 weeks. Steroid sensitivity (SSNS) was assessed at the end of 4 weeks of daily therapy. SSNS were included in the study. 25(OH) Vitamin D was estimated. Levels =/>30 ng/ml were considered adequate. Oral cholecalciferol 60,000 units daily for 10 days was given if levels were < 10ng/ml and 60,000 units daily for 5 days for levels >10ng/ml but < 30ng/ml. Levels were estimated again at 12 weeks and if still low, a similar second course was given. Patients not willing to test 25(OH) D were followed up as control. All patients received daily maintenance cholecalciferol (1000 IU/day) and calcium supplements and were followed up for a period of one year. Time to first relapse, number of relapses in one year, number of relapses in the first and second six months and the number of infection associated relapses were studied. The sample size was calculated for alpha of 0.05 with power of 80% and relative risk of 0.4. The number required in each arm was 44. 97 children presented with the first attack of NS. At 4 weeks, 4 patients were excluded (2 steroid resistant; 2 lost to follow up). Of these, 45 who underwent estimation of serum 25(OH) D levels were included in the study group; 48 who refused evaluation were followed up as controls. 44 /45 patients (93.75%) in the study group had 25(OH) D levels < 30ng/ml at 4 weeks; 24 (54%) had deficiency (<10ng/ml) and 20 (44%) had insufficiency (10-30ng/ml). The mean level of 25(OH) D at 4 weeks was 11.11+/-7.023 ng/ml and at 12 weeks was 37.73+/- 18.38ng/ml. The two groups were comparable in age and sex. There was no significant difference between the two groups in time to first relapse, relapse rates, number of relapses in the first and second six months, total number of relapses or infection associated relapses. (Table 1) Table 1 Relapses in children with normalized 25(OH)D levels compared to standard therapyTabled 1ParameterStudy group (n=45) Mean and Standard deviationControl (n=48) Mean and Standard deviationSignificanceAge (years)4.50 +/- 2.693.80 +/- 2.750.057Males28 (68.3%)28 (52.2%)0.72Time to first relapse (months)8.38 +/5.079.53 +/- 5.960.428Relapse rate1.77 +/- 1.751.68+/- 1.870.809No of relapses in first 6 months0.39+/- 0.750.38+/- 0.680.923No of relapses in 6 months -1year0.76+/- 0.800.64+/-0.740.497Total relapses in first year1.20+/- 1.470.81+/-1.070.200No of Frequent relapsers10 (22.2%)11 (22.9%)0.052No of Infection associated relapses in first 6 months0.09 +/- 0.290.04+/- 0.200.374In next 6 months0.20+/- 0.460.21+/-0.380.700 Open table in a new tab Low 25(OH)D levels were found in 93% of children with SSNS. Early restoration of 25(OH) D levels to normal did not reduce the time to first relapse, the number of relapses in the first year, the number of frequent relapsers or infection associated relapses.

Plasma Free Homocysteine Levels in Children with Idiopathic Nephrotic Syndrome.

Altered metabolism of homocysteine in children with idiopathic nephrotic syndrome leads to raised plasma-free homocysteine levels. Elevated free homocysteine causes endothelial cell dysfunction and promotes early atherosclerosis and glomerulosclerosis. In this analytical study with a longitudinal follow-up, 29 children with first episode of nephrotic syndrome (FENS) aged 1–16 years along with 30 age andgender-matched healthy controls were enrolled. Plasma-free homocysteine was measured using high-performance liquid chromatography (HPLC). Other variables were measured using standard biochemical methods. The primary outcome measure was plasma-free homocysteine level in children with FENS and in controls. The secondary outcome measure was to observe the levels of plasma-free homocysteine in children with FENS at 12 weeks in remission and in steroid resistant states. Plasma-free homocysteine levels were significantly elevated in children with FENS at disease onset [Median (IQR) 2.170 (1.54–2.71); N = 29; P < 0.001], at 12 weeks of steroid-induced remission [Median (IQR) 1.946 (1.53–2.71); N = 22; P < 0.001], and in steroid-resistant states [Median (IQR) 2.262 (1.53–2.74); N = 7; P < 0.001] compared to controls. The levels did not decrease significantly at 12 weeks of steroid-induced remission compared to onset of nephrotic syndrome. Plasma-free homocysteine levels correlated positively with serum total cholesterol (P = 0.005; r = 0.362) and negatively with serum albumin (P = 0.032; r = 0.281). Plasma-free homocysteine levels are raised in children with FENS posing a risk of endothelial dysfunction which persists at least in short term. Long-term effects of raised plasma-free homocysteine needs to be studied.

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

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.

Initial treatment of steroid-sensitive idiopathic nephrotic syndrome in children with mycophenolate mofetil versus prednisone: protocol for a randomised, controlled, multicentre trial (INTENT study)

IntroductionIdiopathic nephrotic syndrome is the most common glomerular disease in childhood with an incidence of 1.8 cases per 100 000 children in Germany. The treatment of the first episode implies...