Nephrotic Syndrome Patients Research Articles

Kidney injury in systemic lupus erythematosus: Lack of correlation between clinical and histological data

BackgroundThe existence and type of renal involvement influences the prognosis of systemic lupus erythematosus and this information may be critical when it comes to taking appropriate therapeutic decisions. ObjectiveTo evaluate statistical correlations between clinical and histological data in patients with biopsied lupus nephropathy. MethodsReview of clinical information in adult kidney biopsy requests reported between 2002 and 2014 with a definitive clinical and histopathological diagnosis of renal involvement in systemic lupus erythematosus. Results134 cases (86% women), aged 15–59 years. Indication for renal biopsy: asymptomatic urinary abnormalities (30%), nephrotic proteinuria without hypoalbuminaemia (9%), nephrotic syndrome (19%), renal failure (40%) and two cases without clinical renal manifestations. The most common lesions were purely proliferative (68%). In patients with asymptomatic urinary abnormalities, 35% were class IV, 30% class III, 23% mixed, 10% class V and 2% class II. In subjects with nephrotic proteinuria, 75% were class IV, 17% mixed and 8% class III. In nephrotic syndrome patients, 46% were class IV, 27% class V, 19% mixed and 8% class III. In renal failure subjects, 67% were class IV, 22% mixed, 7% class III and 4% class V. These proportions were not statistically different. Although class IV showed the worst renal function, almost half (44%) of those without renal failure belonged to this class. ConclusionWe could not demonstrate a consistent clinical–pathological relationship that predicts patterns or severity of histological findings based on the clinical profile in patients with systemic lupus erythematosus and renal manifestations. These results highlight the importance of biopsy as a key diagnostic tool in this disease.

Screening of the LAMB2, WT1, NPHS1, and NPHS2 Genes in Pediatric Nephrotic Syndrome

Mutations in the NPHS1, NPHS2, LAMB2, and the WT1 genes are responsible for causing nephrotic syndrome (NS) in two third of the early onset cases. This study was carried out to assess the frequencies of mutations in these genes in a cohort of pediatric NS patients. A total of 64 pediatric familial or sporadic SRNS cases were recruited. Among these, 74% had a disease onset of up to 3 years of age. We found one homozygous frameshift mutation in the NPHS1 gene in one CNS case and two homozygous mutations in the NPHS2 gene. Six mutations in four cases in the LAMB2 gene were also identified. No mutation was detected in the WT1 gene in isolated SRNS cases. LAMB2 gene missense mutations were segregating in NS cases with no extra-renal abnormalities. Analysis of the population genomic data (1000 genome and gnomAD databases) for the prevalence estimation revealed that NS is more prevalent than previously determined from clinical cohorts especially in Asian population compared with overall world populations (prevalence worldwide was 1in 189036 and in South-Asian was 1in 56689). Our results reiterated a low prevalence of mutations in the NPHS1, NPHS2, LAMB2, and WT1 genes in the studied population from Pakistan as compared to some European population that showed a high prevalence of mutations in these genes. This is a comprehensive screening of the genes causing early onset NS in sporadic and familial NS cases suggesting a more systematic and robust approach for mutation identification in all the 45 disease-causing genes in NS in our population is required.

Nephronectin (NPNT) and the prediction of nephrotic syndrome response to steroid treatment.

Steroid-resistant nephrotic syndrome represents about 10-20% of pediatrics' nephrotic syndrome. The regeneration of glomerular barrier seems pivotal for cessation of proteinuria. Nephronectin (NPNT) plays a major role in nephrogenesis, signal transduction, and epithelial-mesenchymal interactions. This study aims to preliminary assess NPNT as potential noninvasive biomarker of glomerular regeneration and its ability to identify steroid resistance. In this case control study, 80 retrospectively selected patients with nephrotic syndrome were enrolled in addition to 40 healthy controls. Forty patients were steroid sensitive (SSNS) and the other 40 patients were steroid-resistant (SRNS), NPTN concentration was measured using ELISA and NPNT mRNA expression was assayed using real-time PCR. NPTN concentrations were significantly higher in SSNS than both SRNS and controls (The means were 4.64 ± 3.05, 0.69 ± 0.44, and 1.63 ± 0.59, respectively). Moreover, NPTN concentrations were significantly lower in SRNS than controls. NPTN was significantly overexpressed in SSNS compared to both SRNS and controls (the means were 10.82 ± 7.39, 1.19 ± 0.94, and 1.04 ± 0.10, respectively) with no statistically significant difference between SRNS and controls. ROC curves analysis showed that both NPNT expression and NPNT serum level are of promising diagnostic performance (ROCAUC 0.948 and 0.896, respectively). Regression analysis showed that both NPNT expression and NPNT serum level can be independent predictors of steroid resistance. The present study shows for the first time an enhanced expression of NPNT in steroid-sensitive nephrotic syndrome patients suggesting NPNT as a marker of glomerular regeneration. Also, serum NPNT can be a useful noninvasive biomarker of steroid resistance.

Induction prednisone dosing for childhood nephrotic syndrome: how low should we go?

Historically, children with nephrotic syndrome (NS) across British Columbia (BC), Canada have been cared for without formal standardization of induction prednisone dosing. We hypothesized that local historical practice variation in induction dosing was wide and that children treated with lower doses had worse relapsing outcomes. This retrospective cohort study included 92 NS patients from BC Children's Hospital (1990-2010). We excluded secondary causes of NS, age < 1year at diagnosis, steroid resistance, and incomplete induction due to early relapse. We explored cumulative induction dose and defined dosing quartiles. Relapsing outcomes above and below each quartile threshold were compared including total relapses in 2years, time to first relapse, and proportions developing frequently relapsing NS (FRNS) or starting a steroid-sparing agent (SSA). Cumulative prednisone was widely distributed with approximated median, 1st, and 3rd quartile doses of 2500, 2000, and 3000mg/m2 respectively. Doses ≤ 2000mg/m2 showed significantly higher relapses (4.2 vs 2.7), shorter time to first relapse (61 vs 175days), and higher SSA use (36 vs 14%) compared to higher doses. Doses ≤ 2500mg/m2 also showed significantly more relapses (3.9 vs 2.2), quicker first relapse (79 vs 208days), and higher FRNS (37 vs 17%) and SSA use (28 vs 11%). Relapsing outcomes lacked statistical difference in ≤ 3000 vs > 3000mg/m2 doses. Results strongly justify our development of a standardized, province-wide NS clinical pathway to reduce practice variation and minimize under-treatment. The lowest induction prednisone dosing threshold to minimize future relapsing risks is likely between 2000 and 2500mg/m2. Further prospective studies are warranted.

ABCB1 gene polymorphism in nephrotic syndrome

Nephrotic syndrome is the most prevalent glomerular disease in childhood. This study illustrates distribution of the most common three multidrug resistance gene 1 MDR1 (ABCB1) exonic polymorphisms G3435C, G2677 T/A, and C1236T in children with NS to assess their ability to be used as markers for response of NS to steroids. This study aimed to find association between ABCB1 gene polymorphisms and haplotypes and NS and if they can affect their response to steroids. ABCB1 gene polymorphisms in our cases were analyzed using polymerase chain reaction restriction fragment length polymorphism (PCR-RFLP). Our cases were divided into three groups: one group included 50 steroid sensitive nephrotic syndrome (SSNS) patients, another group included 100 steroid resistant nephrotic syndrome (SRNS) patients, and the third group for 100 age and sex-matched children (control group). ABCB1 C3435T TT, CT + TT genotypes, T allele; ABCB1 G2677A/T GT + GA, TT + AA, GT + GA + TT + AA genotypes, T + A allele frequencies were significantly higher in patients with NS compared to control group. Also, SRNS group showed significantly more frequencies of ABCB1 G2677A/T GT + GA, TT + AA, GT + GA + TT + AA genotypes, T + A allele than SSNS group. TTT + TAT, TTC + TAC haplotype of ABCB1 was also higher in NS patients compared to healthy control subjects. We found that the associations of ABCB1 G2677T/A and C3435T polymorphisms were observed in patients with NS. The ABCB1 G2677 T/A polymorphism was significantly increased in SRNS when compared to SSNS.

Effect of CYP3 A4, CYP3 A5 and ABCB1 gene polymorphisms on the clinical efficacy of tacrolimus in the treatment of nephrotic syndrome

BackgroundThe efficacy of tacrolimus (TAC) is variable in the treatment of nephrotic syndrome (NS), which might be related to genetic variation among patients. Therefore, we aim to investigate the effects of CYP3 A4, CYP3 A5 and ABCB1 gene polymorphisms on the clinical efficacy of TAC in the treatment of NS patients.Methods 100 NS patients were treated with TAC and prednisone and followed up for 3 months. Genotype differences (CYP3 A4*1G, CYP3 A5*3, ABCB1 1236C > T and ABCB1 2677G > T/A) were detected by Sanger sequencing. The clinical efficacy was evaluated by the 24 h urinary protein quantitation, albumin, renal function and the degree of edema. Multivariable logistic regression was used to analyze the effect of gene polymorphisms on the clinical efficacy of TAC.ResultsThere were 35 patients (35%) with complete remission, 43 patients (43%) with partial remission, 22 patients (22%) without remission, and no patients with recurrence. For CYP3A4, there were 56, 42, and 2 patients with *1/*1, *1/*1G and *1G/*1G genotype, respectively. For CYP3A5, there were 8, 36 and 56 cases with*1/*1, *1/*3 and *3/*3 genotype, respectively. For ABCB1 C1236T, there were 10, 44, and 46 cases with 1236CC, 1236CT and 1236TT genotype, respectively. For ABCB1 G2677 T/A, there were 13, 57, and 30 patients with 2677GG genotype, 2677GT/GA genotype and 2677TT/AA/TA genotype, respectively. The mutant allele frequencies of CYP3A4*1G, CYP3A5*3, ABCB1 C1236T and ABCB1 G2677 T/A were 23%, 74%, 68% and 58.5%, respectively. Results reveal that the gene polymorphisms of CYP3A4 and CYP3A5 and CCB do not affect the clinical efficacy of TAC. For ABCB1 C1236T,TT genotype can increase the effectiveness 12.085 times compared with CC and CT genotype (P = 0.018, OR = 12.085, 95%CI 1.535–95.148). For ABCB1 G2677 T/A, the clinical efficacy of patients with mutant genotype is 8.683 times than that of wild-type and heterozygous patients (P = 0.042, OR = 8.683, 95%CI 1.080–69.819). Overweight patients can improve the clinical efficacy by 15.838 times (P = 0.020, OR = 15.838, 95%CI1.550–161.788).ConclusionsABCB1 C1236T, ABCB1 G2677 T/A genotype and BMI are probably the factors influencing the clinical efficacy of TAC in treating patients with NS.

Urinary CD80 excretion is a predictor of good outcome in children with primary nephrotic syndrome.

The level of urinary cluster of differentiation 80 (uCD80) is elevated in most children with minimal change disease (MCD) as opposed to focal segmental glomerulosclerosis (FSGS) during the acute phase. The objective of this follow-up study was to evaluate whether uCD80 elevation is actually associated with MCD and whether it signals better prognosis. We evaluated uCD80 levels and a series of putative progression factors in a cohort of 64 patients with nephrotic syndrome (NS) seen between 2011 and 2016. We monitored progression of chronic kidney disease (CKD), assessed as a glomerular filtration rate of < 90ml/min/1.73m2 for at least 3months. Patients were classified according to uCD80 level and to the progression rate as calculated by Kaplan-Meier survival analysis and Cox's regression analysis. During a mean follow-up period of 4.8 ± 0.6 (range 3.5-6.0)years, 13 children (20%) evolved to at least CKD stage 2. The 64 patients with NS and normal baseline renal function were divided into two groups based on uCD80 excretion, i.e. below or above a defined cutoff (< or > 328.98ng/g creatinine). The predicted response to immunosuppression therapy was 34.5 and 100% in the low- and high-uCD80 excretion, respectively (p < 0.001). Progression to CKD was 41.4 vs. 2.9% in NS patients (p < 0.001). Using the Cox model, only uCD80 excretion (p = 0.013, relative risk 6.171) predicted progression to CKD. Urinary CD80 predicts progression and remission in children with NS. The use of uCD80 as a prognostic marker facilitates the identification of high-risk patients at an early stage and may lead to better treatment selection.

Pharmacology and pharmacogenetics of prednisone and prednisolone in patients with nephrotic syndrome

Nephrotic syndrome is one of the most common glomerular disorders in childhood. Glucocorticoids have been the cornerstone of the treatment of childhood nephrotic syndrome for several decades, as the majority of children achieves complete remission after prednisone or prednisolone treatment. Currently, treatment guidelines for the first manifestation and relapse of nephrotic syndrome are mostly standardized, while large inter-individual variation is present in the clinical course of disease and side effects of glucocorticoid treatment. This review describes the mechanisms of glucocorticoid action and clinical pharmacokinetics and pharmacodynamics of prednisone and prednisolone in nephrotic syndrome patients. However, these mechanisms do not account for the large inter-individual variability in the response to glucocorticoid treatment. Previous research has shown that genetic factors can have a major influence on the pharmacokinetic and dynamic profile of the individual patient. Therefore, pharmacogenetics may have a promising role in personalized medicine for patients with nephrotic syndrome. Currently, little is known about the impact of genetic polymorphisms on glucocorticoid response and steroid-related toxicities in children with nephrotic syndrome. Although the evidence is limited, the data summarized in this study do suggest a role for pharmacogenetics to improve individualization of glucocorticoid therapy. Therefore, studies in larger cohorts with nephrotic syndrome patients are necessary to draw final conclusions about the influence of genetic polymorphisms on the glucocorticoid response and steroid-related toxicities to ultimately implement pharmacogenetics in clinical practice.

GAMBARAN KADAR KOLESTEROL, ALBUMIN DAN SEDIMEN URIN PENDERITA ANAK SINDROMA NEFROTIK

Nephrotic syndrome (NS) is characterized by proteinuria, hypoalbuminemia accompanied by oedema and hypercholesterolemia.Nephrotic syndrome is an often relapsing disease (75%) and often the diagnosis is too late. This disease is 15 times greater in childrenthan in adult and the male to female ratio is 2:1. Laboratory examination is needed to rapidly detect and evaluate the progress of thedisease for treatment. To know the distribution of NS based on aged, gender, cholesterol, and albumin concentration and the urinesediment profile. The data in this retrospective descriptive study were collected from 56 patients with NS at the Wahidin SudirohusodoHospital, Makassar, in the period of January 2004 – June 2006. NS were found in 36 male patients (64.3%) and 20 female patients(35.7%). Cholesterol concentrations were 250 mg/dl in 50 patients (89.3%) and 250 mg/dl in 6 patients (10.7%). Albuminconcentration was 2.5 g/dl in 21 patients (37.5%) and &lt; 2.5 gr/dl in 35 patients (62.5%). In urine sediments, there were found in 23patients (41.1%) with positive proteinuria (+++), 51 patients (91.1%) with positive erythrocytes, 54 patients (96.4%) with positiveleucocytes, and 33 patients (58.9%) with positive cylinders such as rugged granular and fatty cost. More NS were found in male patientsin comparison to female, and many were aged + 6 years. Hypercholesterolemia, hypoalbuminemia, proteinuria, hematuria, leucocyturia,and positive cylinder (rugged granular and fatty cast) were found in the urine of most of the NS patients.

Correlation Between Single-Nucleotide Polymorphisms Within miR-30a and Related Target Genes and Risk or Prognosis of Nephrotic Syndrome.

This study was aimed to figure out the association of single-nucleotide polymorphisms (SNPs) within miR-30a and its downstream molecules (i.e., Notch1, Snail1, p53, CD73, and TET1) with susceptibility to and prognosis of nephrotic syndrome (NS). In the aggregate, 265 patients and 281 healthy controls were gathered, and related laboratory indicators were examined. The miR-30a, Notch1, Snail1, TET1, p53, and CD73 expressions were also evaluated by quantitative real-time polymerase chain reaction (qRT-PCR), immunohistochemistry, or enzyme-linked immunosorbent assay. Besides, the SNPs were genotyped by RT-PCR with aid of ABI-PRISM™ 377 DNA sequencing instrument. As a result, the NS patients were correlated with remarkably higher 24-h protein excretion, random urine protein/creatinine (UPCR), and serum creatinine, along with lower estimated glomerular filtration rate and serum albumin, when compared with normal subjects (p < 0.05). Furthermore, significant correlations were present between miR-30a expression and the expressions of Notch1 (rs = -0.350), p53 (rs = -0.339), CD73 (rs = -0.300), TET1 (rs = -0.249), and Snail1 (rs = -0.829) (all p < 0.05). The SNPs of miR-30a [i.e., rs2222722 (C>T)], Notch1 [i.e., rs3124599 (G>A), rs3124591 (C>T), and rs139994842 (G>A)], Snail1 [i.e., rs6020178 (T>C)], p53 [i.e., rs1042522 (C>G)], and CD73 [i.e., rs9444348 (G>A) and rs4431401 (T>C)] were significantly correlated with both differed NS risk and altered hormone sensitivity to NS (all p < 0.05). Moreover, haplotype AC of CD73 and haplotype ATG of Notch1 were the helpful factors against NS (p < 0.05), yet haplotype GT of CD73 functioned oppositely (p < 0.05). The haplotype AT of CD73 was beneficial to the NS patients for that the carriers could be treated with hormones without severe complications (p < 0.05). Conclusively, the SNPs situated within miR-30a and its downstream molecules (i.e., Notch1, Snail1, p53, CD73, and TET1) could become the promising biomarkers for both NS diagnosis and prediction of NS prognosis.

The Status of Oxidants and Antioxidants in Children with Nephrotic Syndrome

Background: The nephrotic syndrome (NS) resistance and relapse to treatment pose challenges in the management of NS. Several experimental studies on both animals and humans have assessed the association between NS and the balance between the oxidants and anti-oxidants. The study aims to compare the status of oxidants and anti-oxidants of NS patients between the massive proteinuria, the remission, the steroid resistance and the control groups.&#x0D; Methods: a cross-sectional design to assess the status of oxidants and antioxidants in children with the nephrotic syndrome. The eligible subjects were divided into four groups, the massive proteinuria group, the remission group, the steroid resistant group and the control group. The status of oxidants and anti-oxidant were evaluated with the Malondialdehyde (MDA) and the Total Antioxidant Status (TAS), respectively.&#x0D; Results: The highest mean MDA levels was observed in the steroid resistant group followed by the massive proteinuria group and the remission group. The mean MDA level of the proteinuria group (massive proteinuria and steroid-resistant) is higher than the remission group. The mean TAS levels in the remission group were higher than the massive proteinuria group, but the difference was not statistically significant. Moreover, the mean difference of SAT between the proteinuria group and without proteinuria was not statistically significant.&#x0D; Conclusions: The oxidative stress marker (MDA) was higher in the NS patients with proteinuria than the patients without proteinuria. The difference in the total anti-oxidant status in NS patients with massive proteinuria, remission and steroid resistance were not statistically significant.&#x0D; &#x0D; Keywords: Nephrotic syndrome, oxidative stress, anti oxidant

A diagnostic model for minimal change disease based on biological parameters.

BackgroundMinimal change disease (MCD) is a kind of nephrotic syndrome (NS). In this study, we aimed to establish a mathematical diagnostic model based on biological parameters to classify MCD.MethodsA total of 798 NS patients were divided into MCD group and control group. The comparison of biological indicators between two groups were performed with t-tests. Logistic regression was used to establish the diagnostic model, and the diagnostic value of the model was estimated using receiver operating characteristic (ROC) analysis.ResultsThirteen indicators including Anti-phospholipase A2 receptor (anti-PLA2R) (P = 0.000), Total protein (TP) (P = 0.000), Albumin (ALB) (P = 0.000), Direct bilirubin (DB) (P = 0.002), Creatinine (Cr) (P = 0.000), Total cholesterol (CH) (P = 0.000), Lactate dehydrogenase (LDH) (P = 0.007), High density lipoprotein cholesterol (HDL) (P = 0.000), Low density lipoprotein cholesterol (LDL) (P = 0.000), Thrombin time (TT) (P = 0.000), Plasma fibrinogen (FIB) (P = 0.000), Immunoglobulin A (IgA) (P = 0.008) and Complement 3 (C3) (P = 0.019) were significantly correlated with MCD. Furthermore, the area under ROC curves of CH, HDL, LDL, TT and FIB were more than 0.70. Logistic analysis demonstrated that CH and TT were risk factors for MCD. According to the ROC of “CH+TT”, the AUC was 0.827, with the sensitivity of 83.0% and the specificity of 69.8% (P = 0.000).ConclusionThe established diagnostic model with CH and TT could be used for classified diagnosis of MCD.

Nefrotik Sendromlu Çocuklarda Ağız Bulguları&amp;#775;

Objective: Nephrotic syndrome (NS) is caused by different disorders that damage the kidneys. This damage leads to the excessive release of protein into the urine. Due to alterations in mineral metabolism in young patients with nephrotic syndrome, dental development, periodontal tissues, and alveolar bone may be disturbed. The aim of this study was to assess the oral health status in children with nephrotic syndrome and compare the results with a control group. Material and Methods: The study was carried out at the University of Adnan Menderes Department of Pediatric Dentistry and Pediatric Nephrology. The study was approved by the Ethics Board Committee of the University Hospital. The study group consisted of 38 children with nephrotic syndrome and 52 healthy children were in the control group. Prepared forms to obtain descriptive information and dental examination findings were used for assessment. The statistical differences between the control and study groups were determined using chi-square and student's T test. Results: Enamel hypoplasia was present in one child in the study group. Mean dmft, DMFT, plaque, and gingival indices of the study group were 3.85, 3.17, 1.62±0.794 and 1.14±0.63 respectively. The control group's dmft, DMFT, plaque and gingival indices were 5.45, 3.73, 1.19±0.93 and 0.63±0.71. The means of dmfs, dmft, DMFS, DMFT indices were found to be lower in the study group compared to the control group. However, this difference was not statistically significant. Plaque index (PI) and gingival index (GI) values were determined to be higher in the study group than in the control group. While the difference between PI values was not statistically significant, the difference between GI values was found to be statistically significant. Conclusion: It was concluded that despite the poor oral hygiene in nephrotic syndrome patients, we found a low incidence of tooth decay. It assumed that immune suppressive drugs in this group of patients could have a negative impact on the oral health. However; the use of vitamin D and calcium may prevent the dental problems which arise from NS treatment.

Urinary Neutrophil Gelatinase- Associated Lipocalin in Children with Idiopathic Nephrotic Syndrome: A Biomarker to Differentiate Between Steroid Sensitive and Resistant Forms of Disease

Background: Objectives of the study were to find out the ability of urinary neutrophil gelatinase- associated lipocalin (NGAL) as a biomarker to differentiate between steroid resistant nephrotic syndrome (SRNS) and steroid sensitive nephrotic syndrome (SSNS), and also to observe variation in levels, if any, between focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD) in SRNS patients.Methods: The study included 63 patients of idiopathic nephrotic syndrome (19 SSNS in relapse, 19 SSNS in remission and 25 SRNS), aged 2.5-16 years, along with 17 controls. Urinary NGAL was measured by ELISA and the values were normalised with urinary creatinine.Results: Median urinary NGAL/creatinine level was significantly raised in SRNS as compared to SSNS relapse, SSNS remission and controls (p 18.3 ng/mg had sensitivity of 84%, specificity of 97.4% and area under the curve of 0.935 (p< 0.001, 95% confidence interval 0.871-0.998) to differentiate SRNS from SSNS patients. Median urinary NGAL/creatinine value was significantly raised in children with FSGS as compared to MCD (p=0.003) in SRNS patients. It had significant positive correlations with duration of illness (r=0.342, p=0.006), urine protein creatinine ratio (r=0.594, p< 0.001) and negative correlation with serum albumin (r=0.470, p<0.001).Conclusion: Urinary NGAL/creatinine level correlated with activity of the disease and it can distinguish not only SRNS from SSNS but also FSGS and MCD histopathological sub-types of SRNS in children.

Are Boys at Risk of Low Immune Response to Recombinant Hepatitis B Virus Vaccine in Steroid Sensitive Nephrotic Syndrome

Introduction: This study was conducted to evaluate the immune response following vaccination with different doses and to compare the antigen specific antibody response to Hepatitis B Vaccine (HBVac) in Steroid Sensitive Nephrotic Syndrome (SSNS) patients between boys and girls. Materials and Methods : This prospective study was conducted in 30 SSNS children at Bangladesh Institute of Child Health, Dhaka, Bangladesh from July 2012 to June 2013.Thirty patients who had all features of Minimal Change Nephrotic Syndrome (MCNS) according to International Study for Kidney Diseases for Children (ISKDC) and received oral prednisolone every alternate day and were HBsAg negative were enrolled in the study. The patients were randomly assigned to one of the two treatment group to receive either 0.5 ml (10μg) or double dose 1ml (20μg) of HB vaccine in a 0-1-2month schedule. After excluding hepatitis B virus infection, the vaccine was administered at a standard dose in group-A (0.5 ml or 10 microgram) and at a double dose (1 ml or 20 microgram) in group-B. After one month of the last dose, the seroprotection rate was measured and compared among sexes. Results: The mean age of the participants in group-A was 5.81 ± 1.73 years with a boy to girl ratio of 9:6 and the mean age of the subjects in group-B was 5.65 ± 1.68 years with a boy to girl ratio of 8:7. The mean vaccine titer was 25.60 ±19.97 mIU/ml in group-A and 617.47 ±292.11 mIU/mlin group-B, with a significant difference (p<0.05) between the two groups. Irrespective of the dose, the mean vaccine titer was higher in girls (37.33 ± 19.45 mIU/ml) compared to boys (16.22 ± 14.81mIU/ml) and the difference was statistically significant in group-A. It was also observed that the mean vaccine titer was significantly higher in girls (743.00±252.34mIU/ml) compared to boys (394.88±246.63 mIU/ml) in group-B (p<0.05,t-test). Conclusions: The results of our study showed a reduced response to HB Vaccine boys with SSNS in comparison to girls. As the study size was small, single center study and time limited follow-up, we cannot draw any valid conclusions. Keywords : Immune deficiency; Sex differences; Steroid Sensitive Nephrotic Syndrome; Seroconversion.

Ionized calcium, 25-hydroxyvitamin D, and parathyroid hormone in children with steroid-sensitive nephrotic syndrome

Background The chief complications of nephrotic syndrome (NS) are infections and thromboembolic events. Others include hypovolemic crisis, acute renal failure, and hormonal and mineral alterations. Objective The aim was to study the level of serum ionized calcium during the active stage and after remission in steroid-sensitive nephrotic syndrome (SSNS) patients and 25-hydroxyvitamin D [25(OH)D] and parathyroid hormone (PTH) during the active stage of the disease. Patients and methods The study was conducted on two groups. Twenty children with the first episode of SSNS were investigated and compared with 20 healthy children as a control group. Serum ionized calcium, serum 25(OH)D, PTH, phosphorus, and alkaline phosphatase were measured during the active stage of the disease and serum ionized calcium was repeated after remission. Results Children with active SSNS had low ionized calcium, low-serum 25 (OH)D levels, with high PTH and high phosphorus and low alkaline phosphatase levels compared with the control. All patients had 25(OH)D deficiency, of which, 80% were severely deficient. Both serum ionized calcium and 25(OH)D had a significant negative correlation with PTH (r=−0.655, P=0.002 and r=−0.575, P=0.008, respectively). Serum ionized calcium was negatively correlated to spot protein/creatinine ratio in urine (r=− 0.565, P=0.009). Levels of serum ionized calcium during the active stage of the disease were markedly lower than that after remission. However, both were significantly lower than the control group. Conclusion Children with SSNS are at risk of vitamin D deficiency and hypocalcemia, therefore, further research will be needed to prove the need for vitamin D supplementation to prevent the occurrence of possible complications.

Efficacy of Traditional Chinese Medicine Regimen Jian Pi Qu Shi Formula for Refractory Patients with Idiopathic Membranous Nephropathy: A Retrospective Case-Series Study.

Background The treatment of adult refractory idiopathic membranous nephropathy with steroid and other immunosuppressant-resistant nephrotic syndromes can be a significant challenge. We evaluated the efficacy and safety of the traditional Chinese medicine Jian Pi Qu Shi Formula (JPQSF) as a promising regimen. MethodsWe analyzed 15 consecutive patients with biopsy-proven idiopathic membranous nephropathy who failed immunosuppressive therapy from October 2013 to January 2017. JPQSF was administered orally two times per day, respectively, in the morning and at night for 6 months. All patients had at least 1 year of follow-up. The primary endpoints included complete or partial remission. Secondary endpoints included change of clinical parameters and adverse events after 12 months of treatment. Results After 12 months, complete remission was achieved in 13.3% of patients and partial remission in 66.7%, yielding a response rate of 80%. Proteinuria, serum albumin, and cholesterol were improved significantly (P<0.001, P<0.001, and P<0.05, respectively). After 1 year of treatment, proteinuria (mean ± SD) decreased from 5.93 ± 2.54 g per 24 h to 1.99 ± 1.17 g per 24 h (P<0.001). No serious adverse events occurred during the observation. Conclusions JPQSF may be an alternative therapeutic option for steroid and general immunosuppressant-resistant membranous nephrotic syndrome patients, with a favorable safety profile. Larger and longer follow-up studies evaluating this regimen are warranted.

Refractory Oedema of Nephrotic Syndrome in a Resource Poor Setting: A Case Presentation

Background: Oedema, a constant feature of childhood nephrotic syndrome can be severe, enough to cause respiratory embarrassment. It can also be refractory to diuretic monotherapy. In such cases, combination of salt poor albumin (SPA) infusion and diuretics has remained the best treatment option. However, the cost of SPA has made it practically unavailable in resource-poor settings. It becomes a therapeutic dilemma when nephrotic syndrome patients of financially-constrained caregivers present with refractory anasarca in a resource-poor settings. Case review: We present a seven-year-old boy with relapsed steroid sensitive nephrotic syndrome who presented to Abia State University Teaching Hospital Aba, in respiratory distress with anasarca and a weight of 58 kilograms. SPA could not be accessed due to financial constraints. A decision to use fresh whole blood in the place of SPA, in combination with frusemide, achieved a lifesaving diuresis and regression of the anasarca. Conclusion: Whole blood is a good alternative for the treatment of refractory oedema in children with nephrotic syndrome in resource poor settings.

Study of Xanthine Oxidase Activity in Sera of Iraqi Children with Nephrotic Syndrome

Introduction: To investigate the relationship of xanthine oxidase activity with nephrotic syndrome disorder in children, and the optimization of the enzyme activity conditions in the disorder. Material and methods: Sera of children with nephrotic syndrome (NS) (60 samples) were obtained from Central Child Teaching Al Karama Hospital (CCTAH), from 2nd Mar. 2013 to 28th Feb. 2014. Sera of the patients were assayed for xanthine oxidase (XO) activity using colorimetric absorbance technique. The obtained results were compared with the enzyme activity of normal children (70 samples) as control. Results and conclusions: The results revealed a significant (P < 0.001) elevation in XO activity in serum of nephrotic syndrome (0.12 ± 0.06 IU/L) compared with that of normal subjects (0.05 ± 0.009 IU/L), showing an elevation of (70%) in XO activity (about 2/3) that of normal group. Factors influencing XO activities were also studied and showed that XO activity is a pH dependent. Significant elevation (P < 0.001) was found in uric acid level in sera of NS patients (497.52 ± 3.21 μmol/L) compared with that in normal group (298.12 ± 1.70 μmol/L). Elevation was found in urea level in sera of NS patient (10.69 ± 7.55 mmol/L) compared with that of normal group (4.57 ± 1.27 mmol/L). It was appeared, there is a role of XO activity in the pathogenesis of endothelial injury during glomerular lesion in NS and that was confirmed by comparing XO activity and other related conditions with the activity of normal volunteers.

The role of bioelectrical impedance analysis, NT-ProBNP and inferior vena cava sonography in the assessment of body fluid volume in children with nephrotic syndrome

BackgroundAssessment of volume status and differentiating “underfill” and “overfill” edema is essential in the management of patients with nephrotic syndrome (NS). ObjectivesOur aim was to evaluate the volume status of NS patients by using different methods and to investigate the utility of bioelectrical impedance analysis (BIA) in children with NS. MethodsThe hydration status of 19 patients with NS (before treatment of NS and at remission) and 25 healthy controls was assessed by multifrequency BIA, serum N-terminal-pro-brain natriuretic peptide (NT-proBNP) levels, inferior vena cava (IVC) diameter, left atrium diameter (LAD) and vasoactive hormones. ResultsRenin, aldosterone levels, IVC diameter and LAD were not statistically different between the groups. NT-proBNP values were statistically higher in the attack period compared to remission and the control group (p=0.005 for each). Total body water (TBW), overhydration (OH) and extracellular water (ECW) estimated by the BIA measurement in the attack group was significantly higher than that of the remission group and controls. There were no significant correlations among volume indicators in group I and group II. However, significant correlations were observed between NT-proBNP and TBW/BSA (p=0.008), ECW/BSA (p=0.003) and ECW/ICW (p=0.023) in the healthy group. TBW was found to be higher in patients with NS in association with increased ECW but without any change in ICW. NT-proBNP values were higher in patients during acute attack than during remission. ConclusionsOur findings support the lack of hypovolaemia in NS during acute attack. In addition, BIA is an easy-to-perform method for use in routine clinical practice to determine hydration status in patients with NS.