Steroid-responsive Nephrotic Syndrome Research Articles

Elevated urinary CD80 excretion in children with steroid-responsive nephrotic syndrome

Background: Idiopathic nephrotic syndrome (INS) is one of the most common glomerular diseases in children with different pathological types and different responses to corticosteroids. A definitive diagnosis is essential for planning the treatment and determining the prognosis of these patients and currently, kidney biopsy is the only method for definitive diagnosis. However, this is an invasive procedure. In addition, in some cases, the biopsy is contraindicated or tissue obtained on biopsy is insufficient and may not represent the underlying disease. According to the recent hypothesis about the role of circulating permeability factors in the pathogenesis of INS, urine protein analysis as a noninvasive method to determine the specific biomarkers of the disease is of great interest to nephrologists and can be useful. Methods: In this case − control study, we analyzed urinary CD80 (uCD80) levels in 51 patients with INS using a special CD80 enzyme-linked immunosorbent assay kit and were compared between different groups of patients. Results: The highest urine CD80/creatinine ratio was found in patients with active minimal change disease and steroid-responsive nephrotic syndrome in the relapse stage of the disease. Conclusion: A significant level of uCD80 is correlated with better renal function and a more favorable response to steroids in patients with INS. Therefore, it can be concluded that a high level of uCD80 is correlated with a good prognosis in these patients.

Subclinical hypothyroidism in children with idiopathic childhood nephrotic syndrome at a tertiary institution in South-West Nigeria.

Hypothyroidism in children with nephrotic syndrome (NS) is often attributed to prolonged loss of thyroxine binding globulin and thyroid hormones alongside protein in the urine. It has been historically associated with steroid-resistant NS alone. However, recent evidence supports the fact that subclinical hypothyroidism (SCH) does occur even in children with steroid responsive NS. Complications such as weight gain, hypercholesterolemia, delayed growth, delayed puberty, and depression could result from hypothyroidism and be erroneously attributed to NS, or the effect of steroid used in treatment. Incidentally salt intake, the major form of dietary iodine is often restricted in children with NS, possibly exacerbating any underlying hypothyroid state. The study aimed to determine the burden of SCH among our cohort of NS patients. A comparative cross-sectional study was designed to assess SCH [defined by high TSH (>6.0 mU/L and normal free T4 (0.8-2.0 ng/dl)] in hundred children with NS aged between one and fifteen years compared with hundred age and gender matched comparison group without NS. Blood and urine samples were collected to analyze thyroid function, serum albumin, serum protein and urinary protein. The prevalence of SCH was significantly higher in subjects with NS than their age, sex matched comparison group (12% vs. 2%, P = 0.006). The highest proportion (24.1%) of the children with NS who had SCH was found in the age range of 11-15 years and majority were females (19.4% vs. 7.8%, respectively, P = 0.086). The proportion of children with SCH were higher in those with steroid-resistant NS than those responsive to steroids (26.3% vs. 8.6% P = 0.033). The average values of serum albumin and protein were also significantly lower in children with SCH than those without (2.91 mg/dl ± 0.8 vs. 3.78 mg/dl ± 0.9 and 3.99 mg/dl ± 1.3 vs. 5.02 mg/dl ± 1.3, respectively, P < 0.005). Also, the average value of urinary protein was significantly higher in those with SCH than those without [94.29 mg/dl (42.3-101.0) vs. 69.19 mg/dL (31.2-108.2), respectively, P = 0.023]. Participants with steroid-resistant NS have almost three-folds odd of developing SCH compared to steroid sensitive subjects (AOR 2.901; 95% CI 1.831-4.012; P = 0.038). Screening of children for SCH with NS especially steroid-resistant NS and frequent relapsing steroid sensitive NS for hypothyroidism before complications arise is pertinent to their holistic management. This becomes even more imperative in our environment as iodine deficiency hypothyroidism is still prevalent in some parts of the country.

The CLVS1 H310Y Variant Associated with Steroid-Responsive Nephrotic Syndrome Affects Podocyte Function and Glomerular Filtration Barrier Integrity

The CLVS1 H310Y Variant Associated with Steroid-Responsive Nephrotic Syndrome Affects Podocyte Function and Glomerular Filtration Barrier Integrity

Role of urinary nitrites in predicting steroid responsiveness of nephrotic syndrome: a study conducted in tertiary care center

Background: Nephrotic Syndrome (NS) is a common chronic disorder, characterized by alterations of selective permeability at the glomerular capillary wall, resulting in its inability to restrict the urinary loss of protein. Urinary nitrite excretion serves as a useful investigation in differentiating between steroid responsive and steroid resistant nephrotic syndrome. The aim of the study was to assess the relation between urinary nitrite levels and steroid responsiveness in nephrotic syndrome in children.Methods: 76 children were enrolled in the study suffering with nephrotic syndrome of which 58 children were Steroid Sensitive (SSNS) and 18 were Steroid Resistant (SRNS). 25 children were enrolled as controls. The urinary nitrites were estimated in these subjects and the results were analyzed.Results: All the control subjects were tested negative for urinary nitrites. After achieving remission with steroids, out of 58 SSNS subjects’ 27 subjects tested positive for urinary nitrites, remaining 31 tested negatives for the same. Of the 18 SRNS subjects 1 subject tested positive for urinary nitrites remaining 17 subjects were tested negative for the same.Conclusions: The findings of present study suggest that urinary nitrite excretion is increased in patients with steroid responsive nephrotic syndrome. The urinary nitrite estimation has low NPV and high PPV in predicting steroid responsiveness.

Intracardiac Thrombus in Renovascular Hypertension: A Case Report

Thromboembolism is a rare complication of primary nephortic syndrome. Both venous and arterial thrombosis might occur in steroid responsive and steroid resistant nephrotic syndrome. This is the report of an infant with nephrotic syndrome and renovascular hypertension, complicated with asymptomatic intracardiac thrombus and managed appropriately with medical treatment.

Short courses of daily prednisolone during upper respiratory tract infections reduce relapse frequency in childhood nephrotic syndrome.

Relapses of childhood nephrotic syndrome (NS) are frequently precipitated by viral upper respiratory tract infections (URTIs). A review of the literature reveals that in patients with steroid-dependent NS on alternate day corticosteroids, a short course of daily corticosteroid therapy during the course of an URTI may reduce relapse frequency. To assess the effect of a short course of low-dose corticosteroid therapy during the course of an URTI on relapse frequency in patients with steroid-sensitive NS who have not been taking any treatment for a minimum period of 3months. A double-blind placebo-controlled crossover trial was conducted on 48 patients with idiopathic NS who had not been receiving corticosteroid therapy for a minimum of 3months. Patients were randomized into two groups. Group A received 5days of daily prednisolone at 0.5mg/kg at the onset of an URTI while group B received 5days of placebo. Both groups were followed up for 1year and the URTI-induced relapse frequency was noted. A crossover was performed during the next year, with group A receiving placebo and group B receiving prednisolone. Thirty-three patients completed the study. In the treatment group, 115 episodes of URTI led to 11 relapses while in the control group 101 episodes of URTI led to 25 relapses. There was no significant difference between the mean number of URTIs between the treatment and control groups. The treatment group had significantly less relapses compared to the control group (p = 0.014). Within the treatment group, 65.6% did not relapse, while the remainder had a single relapse. In contrast, only 40.6% of the control group remained in remission while 40.6% suffered a single relapse and 18.8% had two or more relapses. Prescribing a short course of daily corticosteroids during an URTI significantly reduces the frequency of URTI-induced relapse in patients with steroid-responsive NS who are off corticosteroid therapy.

Serum immunoglobulin E levels in children with idiopathic nephrotic syndrome

Background Children with idiopathic nephrotic syndrome (INS)have been known to have T-cell dysfunction and an impairment ofthe cytokine network that may alter glomerular permeability andthe glomerular filtration barrier. This disorder may contribute to thepresence of urinary protein loss in children with INS. The elevationof serum IgE levels has been noted in some cases, but its associa-tion with steroid-responsive nephrotic syndrome has not been fullyelucidated.Objective This study was done to investigate the association be-tween serum IgE levels prior to prednisone treatment in childrenwith INS and the outcome of treatment.Methods A prospective observational study has been conductedon 22 children with INS. Prednisone therapy was given with a doseof 60 mg/m 2 body surface area (BSA) for four weeks followed by asingle dose of 40 mg/m 2 BSA every other day for another fourweeks. This protocol was applied for steroid-responsive INS chil-dren. Children with steroid resistance were given oral cyclophos-phamide 2 mg/kg for eight weeks. IgE level measurements wereperformed prior to prednisone therapy and at remission. Data wereanalyzed using one-way ANOVA and multiple regression.Results Twenty-two children were enrolled in this study. High lev-els of serum IgE were found in 95.5% of children, with a mean of2002.5 (SD 2172.1) IU/ml. The serum IgE levels of INS childrenwith history of allergy were significantly higher than those of neph-rotic children without history of allergy (P&lt;0.05). However, therewas no significant correlation between the serum IgE levels andthe outcome of treatment in children with INS.Conclusion The high serum IgE levels in children with INS seemto be associated with humoral immune disorder and did not haveany association with the outcome of therapy. Even though the se-rum IgE levels were significantly higher in INS children with historyof allergy, other factors that may influence serum IgE levels mustbe considered

The Genetics of Nephrotic Syndrome.

Nephrotic syndrome (NS) is a common pediatric kidney disease and is defined as massive proteinuria, hypoalbuminemia, and edema. Dysfunction of the glomerular filtration barrier, which is made up of endothelial cells, glomerular basement membrane, and visceral epithelial cells known as podocytes, is evident in children with NS. While most children have steroid-responsive nephrotic syndrome (SSNS), approximately 20% have steroid-resistant nephrotic syndrome (SRNS) and are at risk for progressive kidney dysfunction. While the cause of SSNS is still not well understood, there has been an explosion of research into the genetic causes of SRNS in the past 15 years. More than 30 proteins regulating the function of the glomerular filtration barrier have been associated with SRNS including podocyte slit diaphragm proteins, podocyte actin cytoskeletal proteins, mitochondrial proteins, adhesion and glomerular basement membrane proteins, transcription factors, and others. A genetic cause of SRNS can be found in approximately 70% of infants presenting in the first 3 months of life and 50% of infants presenting between 4 and 12 months, with much lower likelihood for older patients. Identification of the underlying genetic etiology of SRNS is important in children because it allows for counseling of other family members who may be at risk, predicts risk of recurrent disease after kidney transplant, and predicts response to immunosuppressive therapy. Correlations between genetic mutation and clinical phenotype as well as genetic risk factors for SSNS and SRNS are reviewed in this article.

Cerebral venous thrombosis in child with nephrotic syndrome: A case report

Cerebral sinovenous thrombosis (CSVT) is a rare and under-diagnosed complication of childhood nephrotic syndrome. With the advent of magnetic resonance imaging and magnetic resonance venography scans, CSVT cases are now being diagnosed more frequently. We report case of a three-year-old male child, known case of steroid responsive nephrotic syndrome, who presented with a 3-week history of headache and sudden onset intermittent convergent squint. He was diagnosed to have CSVT on magnetic resonance imaging and magnetic resonance venography of the brain. Devastating cerebral infarction was probably prevented with early institution of low molecular weight heparin. Nevertheless, randomized placebo-controlled studies are necessary for assessing its efficacy and absence of risk in children.

La croissance staturale des enfants ayant un syndrome néphrotique cortico-sensible en Côte d’Ivoire

We realized one of the first observational studies in sub-Saharan Africa whose aim was to analyze the growth in steroid-responsive nephrotic syndrome. This was a retrospective study, involving 30 children followed for nephrotic syndrome in pediatric nephrology unit of the University Hospital of Yopougon (Abidjan) from 28.02.2005 to 19.12.2010. Each child was followed for two years and we are interested in demographic characteristics and the evolution of size. At the end of our study, it appears that: the mean age was 89.33 ± 43.41 months (7.44 years) with a sex ratio of 3,28. At the end of the two years of follow-up, patients had received a median dose of steroids 6151 ± 3832 mg/m2, 9111 ± 1376 mg/m2 and 8664 ± 5379 mg/m2, respectively, for a relapse or no, two and at least three relapses. Fourteen patients had received vitamin D therapy and calcium. At the end of follow-up, four children had growth retardation. An average gain in Z-score was noted at the end of follow-up in girls and boys respectively +0.06 and +0.36. The growth retardation observed in our study was not associated with different growth periods (P=0.116), gender (P=0.548) and the mean Z-scores observed at the end of follow-up was not significantly different between the sexes (P=0.26). Growth retardation observed was not related to the cumulative dose administered (P=0.15), number of relapse (P=1.000).

Resolution of Late Steroid-Responsive Nephrotic Syndrome in a Patient with Alport Syndrome Treated with Atorvastatin

Experimental and clinical studies have pointed out the lipid-induced renal damage, and statins may have pleiotropic effects on renoprotection. We reported a girl with X-linked Alport syndrome whose late steroid-responsive nephrotic syndrome (NS) was resolved by atorvastatin. She had been in a nephrotic condition despite of prednisone therapy 60 mg/day for 8 weeks. Renal biopsy dispicted extreme foamy appearance of tubular epithelial cells with detachment led to luminal obliteration. Atorvastatin was started on the ninth week of prednisone therapy due to severe hypercholesterolemia. Partial remission of NS was dramatically achieved with unchanged dosage of prednisone at the end of the twelfth week. Our case provides a pathology-based evidence to support the use of statins in profoundly hyperlipidemic patients with NS. In patients with NS and profound hyperlipidemia, early initiation of statin therapy is required in combination with immunosuppressive therapy.

Urinary protein selectivity in nephrotic syndrome and pregnancy: resurrection of a biomarker when renal biopsy is contraindicated.

Significant proteinuria in pregnancy can indicate the presence of serious conditions requiring investigation and treatment. The nephrotic syndrome in pregnancy presents a multitude of difficulties and is a relative contraindication of renal biopsy, particularly in the third trimester. We present a case of nephrotic syndrome of unknown cause presenting at 33 weeks of pregnancy. With renal biopsy contraindicated, we used the urine protein selectivity test, a largely discarded test predicting steroid-responsive nephrotic syndrome, to help inform the decision to give steroids. This led to a successful clinical outcome including the avoidance of neonatal ICU care for baby.

Does the NPHS1 c.349G&gt;A Polymorphism Affect Outcomes in FSGS Patients?

Background: Treatment of steroid-resistant nephrotic syndrome with a focal segmental glomerulosclerosis (FSGS) histological diagnosis can be challenging, particularly in the presence of ciclosporin resistance. The importance of mutations in genes encoding the structural proteins of podocytes has been recently recognized and may influence medical decision-making. The c.349G>A variant of the NPHS1 gene has been associated with a poorer prognosis in IgA nephropathy and nephrotic syndrome. Case Diagnosis/Treatment: To study the potential association of the NPHS1: c.349G>A sequence variant with renal disease, we analyzed five children in whom this variant had been identified and who all presented with nephrotic syndrome and the histological diagnosis of FSGS. Although this sequence variant was present in all cases, one patient had steroid-responsive nephrotic syndrome without relapse following standard initial therapy, three responded to a variety of treatments, and one patient was unresponsive. Conclusion: The NPHS1: c.349G>A sequence variant may either be independent of, or a modifying agent in, the progression of the disease, and does not preclude these and similar patients from appropriate treatment and entry into clinical trials. Larger trials are needed to evaluate the therapeutic response and long-term outcome of FSGS patients with different NPHS gene mutations. Keywords: Nephrotic syndrome, steroid-resistant, focal and segmental glomerulosclerosis, nephrin, ciclosporin, mycophenolate mofetil. Read more →

Thyroid functions in Egyptian children with steroid responsive nephrotic syndrome: Relation to oxidative stress

Background : The interactions between kidney and thyroid functions have been known for many years; oxidative damage has been proposed as one of the possible mechanism involved in the nephrotic syndrome (NS). This study was done to find out the thyroid function of nephrotic children during nephrosis and to compare any significant changes of thyroid function status during nephrosis and remission in relation to oxidative stress markers. Patients and Methods: The study included 60 patients with steroid responsive NS (SRNS) and 30 children with matched age and sex as control. Cases were divided into three groups as follow, Group A: Include 35 patients with SRNS in relapse. Group B: Include 25 patients with SRNS in remission for periods ranging from 3 to 9 months, and not receiving steroid therapy. Group C: Include 30 children with matched age and sex as control. Methods: A thorough history and examinations, total serum thyroxine and triiodothyronine (TT4 and TT3) as well as serum free T4 (FT4), thyroid-stimulating hormone (TSH), and assessment of malondialdehyde (MDA) and total antioxidant capacity (TAC) levels as an oxidative stress markers were measured in all studied groups. Results: Serum TSH was significantly higher in patients with SRNS in relapse in comparison with patients with SRNS in remission and with control group (P < 0.001 in both), we found a significant decrease in TT4 and FT4 and TT3 and FT3 (FT3) concentrations in patients with SRNS in relapse in comparison with both cases with SRNS in remission and control group (P < 0.001 in both). MDA levels were significantly elevated, while TAC level was significantly decreased in patients with SRNS in relapse in comparison with patients with SRNS in remission and with control group (P < 0.001 in both). There was a positive correlation between serum TSH and plasma MDA and a negative correlation with FT4. Conclusions: The development of hypothyroidism in children with SRNS is associated with alteration in oxidant and antioxidant status. The biochemical hypothyroid state in relapse phase is temporary and improves with remission.

Multi-drug resistance-1 gene polymorphisms in nephrotic syndrome: Impact on susceptibility and response to steroids

BackgroundRole of multidrug resistance-1 (MDR-1) gene polymorphisms has not been clarified in nephrotic syndrome (NS). Additionally, researchers studied several genetic polymorphisms to explain their influence on different patients' responses to steroid; however the data were inconsistent. Therefore, we aimed to investigate the association of MDR-1 gene polymorphisms [C1236T, G2677T/A, C3435T] and haplotypes with susceptibility to childhood nephrotic syndrome, and whether they influence steroid response. MethodsWe detected MDR-1 gene polymorphisms using polymerase chain reaction restriction fragment length polymorphism (PCR-RFLP) in 138 NS patients and 140 age and sex matched healthy children. ResultsThe frequencies of MDR1 G2677T/A GT, GA, TT+AA genotypes or T allele, MDR1 C3435T TT genotype, and T allele genotype frequencies were significantly increased in NS group. While no significant differences were observed in distributions of C1236T genotypes or allele between NS patients and healthy children. Moreover, steroid non-responder NS patients had significantly higher frequencies of MDR1 G2677T/A GT, GA, and TT+AA genotypes than steroid responsive NS patients. We observed also that NS patients with age less than 6years old had increased frequencies of MDR1 G2677T/A GT, GA, TT+AA genotypes or T allele MDR1 C3435T CT, TT genotypes and T allele. Interestingly the frequency of the TGC haplotype of MDR1 was lower in the initial steroid responders than in non-responders NS patients. On the contrary, there were no any association between the MDR1 haplotypes with NS susceptibility and they did not influence renal pathological findings. ConclusionOur data suggested that MDR1 C3435T or G2677T/A gene polymorphisms are risk factors of increased susceptibility, earlier onset of NS, and steroid resistance.

Association between childhood nephrotic syndrome and hemophagocytic lymphohistiocytosis

Hemophagocytic lymphohistiocytosis (HLH) is caused by an excessive activation of nonmalignant macrophages. Renal lesions have been described in association with, but always after, HLH diagnosis. We describe a previously healthy 26-month-old girl who presented originally with steroid-responsive nephrotic syndrome (NS), but after 4 months, on the first NS relapse, experienced numerous complications (many of them reported to accompany NS as single events). Clinical and laboratory signs of HLH evolved with time and led to deterioration of her condition and death, within 5 months of her original presentation. To our knowledge, this is the first report of NS antedating the presentation of HLH.

Serum adiponectin level in children with nephrotic syndrome in relation to right ventricular functions and metabolic profiles

Background: Adiponectin (ADPN), a protein hormone (cytokine), is exclusively expressed on and secreted from adipocytes. It is a particularly interesting compound because it may have a protective influence on the cardiovascular system. Objective: This study was designed to evaluate serum ADPN level, right ventricular functions and metabolic profiles among children with nephrotic syndrome (NS) and asses the correlations between ADPN as a protective hormone and each of right ventricular functions and metabolic profiles. Patients and Methods: A total of 47 children (28 boys; 19 girls) with steroid-responsive nephrotic syndrome (SRNS) were studied. They included two groups: Group A: included 25 children with SRNS in relapse. Group B: included 22 children with SRNS in remission for periods ranging from 3-9 months. A control group included 28 children with matched age and sex. Methods: Serum level of ADPN was estimated by ELISA and nitric oxide (NO) by chemical detection. In addition to total cholesterol, triglycerides, high-density lipoprotein (HDL), low density lipoprotein (LDL), total protein, and albumin by Enzymatic Colorimetric kits. Also, 24-hour urine samples were collected for detection of proteinuria. Electrocardiogram (ECG) and echocardiography measuring right ventricular wall functions were done. Results: Serum levels of ADPN and NO were significantly higher in children with SRNS in relapse in comparison with children with SRNS in remission or control group. Children with SRNS in relapse showed significantly higher levels of total cholesterol, triglycerides, LDL and proteinuria while significantly lower levels of total protein, albumin and HDL as compared with SRNS in remission or control group. Echocardiographic findings revealed that a significant decrease in right ventricular ejection fraction (RVEF %) and significant increase in right ventricular end diastolic diameter (RVEDD), right ventricular peak pressure (RVPP) and pulmonary artery pressure (PAP) were found in cases with SRNS in relapse in comparison to cases with SRNS in remission and control hypertrophy in relapsed cases. Finally, in children with SRNS in relapse it was found that serum ADPN level was significantly positively correlated with each of serum NO, total cholesterol, triglycerides, LDL, proteinuria, RVEDD, RVPP and PAP, while significantly negatively correlated with serum total protein, albumin and RVEF%. Conclusion: During relapse of SRNS, serum ADPN level is higher than its level in SRNS in remission. This higher level may represent a physiologic response to the altered metabolic profiles and right ventricular strain so as to minimize cardiovascular complications.

Platelet Functions and Coagulation Changes in Indian Children with Nephrotic Syndrome

Only little is known on the effect of the platelet function in the paediatric nephrotic syndrome. The earlier studies which had been done on hypercoagulability have mainly featured the secondary forms of the nephrotic syndrome and the data on the minimal change type of disease is limited. We therefore, made an effort to study the platelet functions and the coagulation profile in children with the nephrotic syndrome,to find the relationship between the steroid response and the coagulation profile, and to look for the correlation between thromboembolism and the hypercoagulable states. Twenty nine children with the steroid responsive nephrotic syndromewere studied to see the platelet aggregation and the coagulation parameters and their response to the steroid therapy. Doppler studies were done for the renal vein and the inferior vena cava (IVC) thrombus. It was seen that an increased aggregability of the platelets occurred with Adenosine diphosphate (ADP) and collagen (out of the four agonists, ADP, Collagen, Ristocetin and Arachidonic acid) which were used as agonists for the assay. We also observed that the Partial thromboplastin time (PTT) had become prolonged and a significant decline in the high values of the procoagulant proteins (Protein C and Protein S) was seen after the steroid therapy and when the children went into remission. These findings were suggestive of a reversibility of the changes in the steroid responsive nephrotic syndrome with the steroid therapy. One child was found to have thrombosis of the inferior vena cava (IVC) on Doppler studies, which resolved with treatment subsequently. An increased platelet aggregability contributes to the hypercoagulable states, that may increase the incidence of thrombosis in such patients. Although the incidence of such complications is very low, in a given child with the hypercoagulable states, Doppler may be used to look for any evidence of a latent thrombus and, an early intervention could be instituted. A change in the coagulation parameters points to the reversibility of the changes which are produced in a diseased state.

Adiponectin serum level among children with nephrotic syndrome in relation to right ventricular functions

Adiponectin (ADPN), a recently discovered protein hormone, is exclusively expressed on and secreted from adipocyte. ADPN is a particularly interesting compound because it may have a protective influence on the cardiovascular system. This study was a prospective hospital based study aiming to evaluate ADPN serum level among children with nephrotic syndrome (NS), right ventricular functions by echocardiography and finding any correlation between ADPN as a protective hormone and right ventricular functions. The study included 47 patients (28 boys and 19 girls) with steroid responsive nephrotic syndrome (SRNS). Cases included two groups: Group A – included 25 patients with SRNS in relapse. Group B – included 22 patients with SRNS in remission for periods ranging from 3 to 9 months and with no steroid therapy. In addition to control group which included 28 children with matched age and sex. A thorough history and full clinical examinations and echocardiography measuring right and left ventricular wall functions and other abnormalities if present were done. Blood samples were collected for measuring serum levels of total cholesterol, triglycerides, high density lipoprotein (HDL), low density lipoprotein (LDL), very low density lipoprotein (VLDL) by enzymatic colorimetric kits, in addition to ADPN by ELISA method. Serum ADPN level was significantly higher in patients with SRNS in relapse (33.4 ± 15.60 μg/ml) in comparison with patients with SRNS in remission (12.54 ± 8.76 μg/ml) and with control group (10.54 ± 6.43 μg/ml) with P value <0.001 for both, we found a significant decrease in right ventricular ejection fraction (RVEF %) in patients with SRNS in relapse (57.9 ± 4.5) in comparison to cases with SRNS in remission and control group (75.2 ± 3.1 and 76.56 ± 3.3), respectively. During relapse of SRNS, ADPN serum level is higher than its level in SRNS in remission and also, right ventricular strain occurs in relapsed SRNS may be due to haemodynamic overload. Good management of NS with wide scope of investigations aiming for detection and management of thrombolic and cardiovascular complications of NS is highly recommended.

The Clinical Characteristics of Steroid Responsive Nephrotic Syndrome of Children according to the Serum Immunoglobulin E Levels and Cytokines

PurposeThe nephrotic syndrome (NS) is characterized by the favorable response to glucocorticoid therapy and the development of NS may be associated with dysfunctional immune systems. In order to investigate the serum immunoglobulin E (IgE) levels and cytokines activity in pediatric NS, the total of 32 steroid responsive NS patients and 5 healthy controls were enrolled in this study.Materials and MethodsAll patients were divided into two groups according to the initial serum IgE levels, such as normal and high IgE group, and their clinical characteristics were evaluated. In addition, serum levels of interleukin (IL)-4, IL-5, IL-10 and transforming growth factor (TGF)-β were compared and correlated with serum albumin, proteinuria by means of disease severity, and cytokines.ResultsIn the high IgE group, the higher comorbidity of allergic diseases and relapsing rate, the longer duration of steroid therapy before initial remission, and the higher serum IL-4 and IL-5 levels were found. In all patients, initially higher serum levels of IL-4 and IL-5 declined to normal levels after steroid therapy, whereas the serum IL-10 levels showed no significant difference between nephrotic phase (heavy proteinuria) and remission phase (no proteinuria) of NS. The serum TGF-β levels of the nephrotic phase were significantly lower than those of remission phase or control group, and returned to normal control levels after steroid therapy.ConclusionThis study indicates that initial IgE level is associated with steroid responsiveness and disease severity, and cytokine activities may also be related to the pathogenesis of pediatric steroid responsive NS.