Primary Nephrotic Syndrome In Children Research Articles

Association of ACTN4 Gene Mutation with Primary Nephrotic Syndrome in Children in Guangxi Autonomous Region, China.

Objective: To investigate the association between ACTN4 gene mutation and primary nephrotic syndrome (PNS) in children in Guangxi Autonomous Region, China. Methods: The high-throughput sequencing technology was used to sequence ACTN4 gene in 155 children with PNS in Guangxi Autonomous Region in China, with 98 healthy children serving as controls. Twenty-three exon-specific capture probes targeting ACTN4 were designed and used to hybridize with the genomic DNA library. The targeted genomic region DNA fragments were enriched and sequenced. The protein levels of ACTN4 in both case and control groups were quantified using ELISA method. Results: Bioinformatics analysis revealed five unique ACTN4 mutations exclusively in patients with PNS, including c.1516G>A (p.G506S) on one exon in 2 patients, c.1442 + 10G>A at the splice site in 1 patient, c.1649A>G (p.D550G) on exon in 1 patient, c.2191-4G>A at the cleavage site in 2 patients, and c.2315C>T (p.A772V) on one exon in 1 patient. The c.1649A>G (p.D550G) and c.2315C>T (p.A772V) were identified from the same patient. Notably, c.1649A>G (p.D550G) represents a novel mutation in ACTN4. In addition, three other ACTN4 polymorphisms occurred in both case and control groups, including c.162 + 6C>T (1 patient in case group and 2 patients in control group), c.572 + 11G>A (1 patient in case group and 2 patients in control group), and c.2191-5C>T (4 patients in the case group and 3 patients in control group). The serum ACTN4 concentration in the case group was markedly higher, averaging 544.7 ng/mL (range: 264.6-952.6 ng/mL), compared with 241.20 ng/mL (range: 110.75-542.35 ng/mL) in the control group. Conclusion: Five ACTN4 polymorphisms were identified among children with PNS in Guangxi Autonomous Region, China, including the novel mutation c.1649A>G. The lower serum levels of α-actinin-4 in the case group suggest that this protein might play a protective role in PNS.

Primary nephrotic syndrome in children in Cape Town, South Africa

Background. Histopathological patterns of childhood primary nephrotic syndrome (PNS) and clinical response to steroids have beenassociated with certain race groups in parts of South Africa. However, there are no recent studies of childhood PNS in Cape Town.Objectives. To describe the demographics, histological subtypes and steroid response of patients with PNS who underwent kidney biopsiesat Red Cross War Memorial Children’s Hospital (RCWMCH) over a 10-year period.Methods. Details of patients with PNS who underwent kidney biopsies in the Paediatric Nephrology Department at RCWMCH between2006 and 2015 were retrospectively recorded.Results. A total of 103 patients were included in the study. Most patients were either of mixed race (42%) or black (36%), with a mean age of6.8 years and a male-to-female ratio of 1.19:1. The most identified histopathological subtype was mesangioproliferative glomerulonephritis(MesPGN; 60% (n/N=62/103)). Of the patients with focal segmental glomerulosclerosis (FSGS), MesPGN and minimal change disease(MCD) 45% (n/N=43/95) were steroid-resistant, and 54% (n/N=51/95) were steroid-sensitive. There was no significant associationbetween any race group and steroid response. Patients with FSGS were more likely to be black, while MCD was more common in mixed-race patients (p=0.04). There was no difference in the likelihood of being mixed race or black between patients with FSGS and MesPGN(p=0.472).Conclusion. MesPGN was the most common histopathological subtype found in our study. There was no significant association betweenrace and steroid response. Patients with FSGS were more likely to be black than mixed race when compared with MCD patients. Race wasnot otherwise significantly associated with any histopathological subtype

Mycophenolate mofetil in therapy of primary nephrotic syndrome in children

Idiopathic nephrotic syndrome is the most common glomerular disease in children, with a prevalence of 1.15–16.9 cases per 100,000 children per year worldwide. In some cases, nephrotic syndrome has a frequently relapsing course or dependence on steroid therapy is formed, which leads to the appointment of immunosuppressive therapy. So far, in clinical practice, there is no unambiguous approach among physicians to the management of patients with nephrotic syndrome, especially when it comes to the further choice of immunosuppressive therapy. Because of the serious side effects of long-term corticosteroid use, doctors prescribe steroid adjuvants to maintain remission and limit the cumulative effect of glucocorticosteroids. Among adjuvants, mycophenolate mofetil, with mycophenolic acid as the active ingredient, is believed to be the most preferred option due to fewer adverse events, acceptable tolerability and, at the same time, high efficacy. This article describes the advantages and features of the use of mycophenolic acid in clinical practice, provides data on pharmacodynamics and drug monitoring, and discusses issues of personalized medicine.

Effect and mechanism of Yupingfeng Powder on preventing recurrent respiratory tract infection in children with refractory nephrotic syndrome

Primary nephrotic syndrome in children is a group of clinical syndromes caused by the significant increase in the permeability of glomerular filtration membrane to plasma albumin and the loss of a large amount of albumin from urine due to various reasons. It is sensitive to hormones but easy to relapse, and may be dependent on hormones or resistant to drugs after repeated attacks, thus affecting the prognosis. Clinically, it is summarized as refractory nephrotic syndrome in children. Respiratory tract infection is one of the main factors causing the recurrence of refractory nephrotic syndrome in children. Yupingfeng Powder is the prescription of fuzheng Gubenjing, simple and effective, and it is widely used in clinic. Modern research confirmed that Yupingfeng can promote immunoglobulin IgA, IgG, IgE and monocyte macrophages, improve the immune function of the body, in recent years YuPingFeng alone or combined western medicine treatment of children repeated respiratory tract infection shows a unique curative effect and advantages, thus reducing the recurrence frequency of pediatric refractory nephrotic syndrome and frequency.

Retracted: Study on the Effect of Combination of Prednisone and Vitamin D in the Treatment of Primary Nephrotic Syndrome in Children.

[This retracts the article DOI: 10.1155/2021/7932721.].

Clinical assessment of moderate-dose glucocorticoid in the treatment of recurrence of primary nephrotic syndrome in children: a prospective randomized controlled trial

To study the clinical effect and adverse drug reactions of different doses of glucocorticoid (GC) in the treatment of children with recurrence of steroid-sensitive nephrotic syndrome (SSNS). A total of 67 children who were hospitalized and diagnosed with SSNS recurrence in the Department of Nephrology, Children's Hospital, Capital Institute of Pediatrics, from November 2017 to December 2019 were enrolled. They were randomly divided into a moderate-dose GC group (32 children) and a full-dose GC group (35 children). The two groups were compared in terms of urinary protein clearance, recurrence rate within 6 months, and incidence rate of GC-associated adverse reactions. There was no significant difference in the urinary protein clearance rate between the moderate-dose GC and full-dose GC groups (91% vs 94%, P>0.05). There was also no significant difference in the recurrence rate within 6 months between the two groups (41% vs 36%, P>0.05). At 6 months of follow-up, compared with the full-dose GC group, the moderate-dose GC group had a significantly lower cumulative dose of prednisone [(87±18) mg/kg vs (98±16) mg/kg, P=0.039] and a significantly lower proportion of children with an abnormal increase in body weight (6% vs 33%, P=0.045). The logistic regression analysis showed that prednisone dose ≥10 mg/alternate day at enrollment was a risk factor for recurrence within 6 months in children with SSNS (P=0.018). For children with SSNS recurrence, moderate-dose GC has similar effects to full-dose GC in the remission induction rate and the recurrence rate within 6 months, with a lower cumulative dose and fewer GC-associated adverse reactions within 6 months than full-dose GC.

Systematic Review of the Efficacy and Safety of Traditional Chinese Medicine Granules Associated with Hormone When Treating Primary Nephrotic Syndrome in Children.

Objective To systematically evaluate the efficacy and safety of traditional Chinese medicine (TCM) granules associated with hormones when treating primary nephrotic syndrome (PNS) in children. Methods Search online databases such as PubMed, EMBASE, ScienceDirect, Cochrane Library, China Knowledge Network Database (CNKI), China VIP Database, Wanfang Database, and China Biomedical Literature Database (CBM) to search for information on the use of hormone-related Chinese medicine granules in the treatment of children with PNS controlled trials. Retrieval time was limited to the period from the date the database was established to the present. Separately, two researchers gathered the data. Statistical software RevMan5.4 was adopted to estimate bias risk in accordance with the Cochrane Handbook 5.3 standard. Results Finally, 7 articles were selected with a total sample size of 487 cases. The infection rate, recurrence rate, and adverse reaction rate after treatment were analyzed by meta-analysis. The infection rate and recurrence rate in the study group were notably lower, and the difference was statistically significant (P < 0.05). However, the incidence of adverse reactions exhibited not notably different (P > 0.05). The levels of albumin and blood cholesterol after treatment indicated no statistical difference between the levels (P > 0.05). Meta-analysis was performed on the time to negative urine protein and the time to edema subsidence after treatment. The urine protein negative time and edema subsidence time of the study group were shorter after treatment, but the difference exhibited not notable (P > 0.05). Meta-analysis was performed on the dosage of glucocorticoids after treatment. The dosage of glucocorticoid in the study group was notably lower, and the difference was statistically significant (P < 0.05). The levels of T lymphocytes after treatment were analyzed by meta. T lymphoid level in the study group was notably better after treatment, and the difference was statistically significant (P < 0.05). Further subgroup analysis indicated that the levels of CD3+ and CD4+ in the study group were higher after treatment (P < 0.05), and there exhibited no statistical difference in the levels of CD8+, CD4/CD8+, and CD19 (P > 0.05). Immunoglobulin levels in the study group after treatment were notably better, and the difference was statistically significant (P < 0.05). Further subgroup analysis indicated that the levels of IgA, IgM, and IgG in the study group were notably higher after treatment, and the difference was statistically significant (P < 0.05). Conclusion Huai Qi Huang can reduce the recurrence rate of PNS children and the incidence of infection and the dosage of prednisone. A long-term application can improve the cellular and humoral immune function of children with PNS. It has high treatment safety and has no notable effect on plasma cholesterol levels, so it is suitable for clinical application.

Study of the Therapeutic Effects of Chinese Herbal Decoction Combined with Glucocorticoid in Treating Primary Nephrotic Syndrome in Children

Background To investigate the clinical effects of Chinese medicine decoction combined with glucocorticoid in treating children with primary nephrotic syndrome. Methods A total of 70 children with pediatric nephritis nephrotic syndrome treated at Weifang People's Hospital from January 2019 to December 2019 were randomly allocated to the therapy group and the control group, each with 35 cases. The control group was treated with conventional Western medicine, and the therapy group received Western medicine and Chinese medicine. After 12 weeks of treatment, the therapeutic effect of the two groups was compared. Results After receiving the treatment, the levels of urine protein (UPro), triglyceride, and cholesterol were significantly decreased in the two groups (p < 0.05), and these levels in the therapy group were much lower than those of the control group (p < 0.05). However, the level of albumin (ALB) was predominantly increased in the two groups after treatment (p < 0.05), and this level in the therapy group was much higher than that of the control group (p < 0.05). Moreover, the immune indicators, coagulation function, and recurrence rate were noticeably improved after treatment (p < 0.05), and the therapy group was better than the control group (p < 0.05). Furthermore, the comparison of renal function indexes, liver function indexes, and blood routine between the two groups showed no statistical significance in the incidence of adverse reactions between the two groups (p > 0.05). Conclusions For the treatment of refractory nephrotic syndrome in children, based on conventional shock therapy, the addition of traditional Chinese medicine (Liuwei Dihuang pill decoction) remedy can significantly improve the disease symptoms in children and improve the efficacy, and the incidence of adverse reactions is low.

Study on the Effect of Combination of Prednisone and Vitamin D in the Treatment of Primary Nephrotic Syndrome in Children.

Objective To study the effect of prednisone combined with vitamin D in the treatment of primary nephrotic syndrome in children. Method 73 cases of primary nephrotic syndrome admitted to the nephrology department of our hospital were randomly selected and retrospectively analyzed. 36 cases were treated with prednisone as the control group, and 37 cases were treated with prednisone combined with vitamin D as the observation group. The efficacy was compared after 3 months of continuous treatment. Result After 3 months of treatment, the blood calcium of the observation group was higher than that of the control group, PTH was lower than that of the control group, and 25-(OH)2D3 and 1,25-(OH)2D3 were higher than those of the control group (P < 0.05). After 1, 2, and 3 months of treatment in the observation group, Scr and 24-h urine protein quantification were lower than those in the control group and eGFR was higher than that in the control group (P < 0.05). CD4+ and CD4+/CD8+ were lower in the observation group than in the control group after 3 months of treatment (P < 0.05). The serum sTfR and TGF-β1 levels were lower in the observation group than in the control group after 3 months of treatment (P < 0.05). The total effective rate of the observation group was 83.78% after 3 months of combined treatment with prednisone and vitamin D, which was significantly higher than the total effective rate of the control group of 61.11% (P < 0.05). The incidence of nausea and vomiting, heartburn, headache, dry cough, hypercalcemia, and constipation during treatment in the observation group was not statistically different from that in the control group (P > 0.05). Conclusion Combined treatment of primary nephrotic syndrome in children with prednisone and vitamin D can more significantly improve the level of clinical indicators, improve renal function and immune function, and obtain more satisfactory efficacy, without significantly affecting the safety of treatment.

Efficacy and safety of Huaiqihuang granule as adjuvant treatment for primary nephrotic syndrome in children: a meta-analysis and systematic review.

Huaiqihuang (HQH) granule is a traditional Chinese herbal complex that has been used as an adjuvant treatment in clinics for the primary nephrotic syndrome (PNS) for many years. However, the effectiveness and safety of HQH have not been systematically discussed. This review aimed to evaluate the effectiveness and safety of HQH in paediatric patients with PNS. The following databases were searched from inception to Mar 2019: MEDLINE, Cochrane Library, EMBASE, CNKI, Wanfang Database, the Chinese Scientific Journal Databaseandthe Chinese biomedical literature service system. All the randomized controlled trials (RCTs) eligible for inclusion were included. The primary outcomes were relapse, infection, remission and adverse events. The secondary outcomes included serum immunoglobulin levels (IgA, IgG or IgM), T-lymphocyte subtype (CD3+ , CD4+ , CD8+ , CD4+ /CD8+), IL-10, TNF-α, TNF-γ, total cholesterol and time of proteinuria turning negative. Fourteen RCTs (885 patients) were identified. Treatment with HQH reduced the chance of relapse [relative risk (RR): 0.47; 95% CI: 0.34, 0.66; P < 0.001] and infections (RR: 0.47; 95% CI: 0.35, 0.62; P < 0.001). No significant difference was found in adverse events. HQH also increased the serum levels of IgA [weighted mean difference (WMD): 0.40; 95% CI: 0.20, 0.60; P < 0.001] and IgG (WMD: 1.58; 95% CI: 1.38-1.78; P < 0.001), as well as CD4+ [standard mean difference (SMD): 0.90; 95% CI: 0.12-1.68; P = 0.02], CD3+ (WMD: 4.04; 95% CI: 3.27-4.82; P < 0.001), and the CD4+/CD8+ratio (WMD: 0.31; 95% CI: 0.21-0.41; P < 0.001), but decreased the level of CD8+ cells (WMD: -3.39; 95% CI: -5.73-1.05; P = 0.004). No statistically significant difference was found in IgM (WMD: 0.05; 95% CI: -0.13, 0.24; P = 0.57). HQH could reduce the rate of relapse and the frequency of infection in children with PNS. No apparent adverse effects were found. Moreover, the beneficial influence of HQH may act through immunomodulation. Additional multi-center, large-sample, high-quality studies are needed to confirm the effectiveness and safety of HQH.

Relationship between TIM-3 gene polymorphisms and steroid-resistant primary nephrotic syndrome in children

Nephrotic syndrome, also known as nephrosis, is a collection of symptoms in medicine and urology caused by damage to the basement membrane of the kidney glomeruli and the kidneys excrete a large amount of protein. This experiment was carried out to investigate the association of three single nucleotide polymorphisms (SNPs) of T-cell immunoglobulin and mucin-domain-containing-3 (Tim-3) with childhood primary nephrotic syndrome (PNS) steroid response in Han Chinese. For this purpose, a total of 218 children with steroid-resistant PNS and 189 children with steroid-responsive PNS were enrolled in this case-control study. Three single nucleotide polymorphisms (SNPs) of the TIM-3 gene promoter region (rs4704853, rs1051746, and rs10053538) were analyzed by polymerase chain reaction (PCR) and restriction enzyme digestion. Results showed that there were 124 males and 94 females in the steroid-resistant PNS group and 114 males and 75 females in the steroid-responsive PNS group. The mean ages of the two groups were 7.9 years and 7.7 years, respectively. The distribution of alleles of Rs1051746 and Rs10053538 were significantly different between the steroid-resistant PNS group and the steroid-responsive PNS group (P-value = 0.047 and 0.012, respectively). The distribution of their genotypes was also significantly different between the steroid-resistant PNS group and the steroid-responsive PNS group (P-value = 0.044 and 0.010, respectively). Haplotype G-C-G was less frequent among steroid-resistant PNS children than the steroid-responsive PNS children (P = 0.015). There was no significant difference between the three SNPs of TIM-3 and the clinical features of these PNS children (P&gt;0.05). It concluded that this study provided evidence showing that the polymorphisms of Rs1051746 and Rs10053538 at the TIM-3 gene were related to childhood PNS steroid response. This result provided fundamental support for future studies on the role of TIM-3 in pathogenesis and therapy of childhood PNS.

OxLDL promotes podocyte migration by regulating CXCL16, ADAM10 and ACTN4.

Nephrotic syndrome (NS) is one of the most common causes of chronic kidney disease in the pediatric population. Hyperlipidemia is one of the main features of NS. The present study investigated the role of CXC motif chemokine ligand 16 (CXCL16) and ADAM metallopeptidase domain 10 (ADAM10) in oxidized low-density lipoprotein (oxLDL)-stimualted podocytes and the underlying mechanisms. CXCL16 and ADAM10 expression levels in oxLDL-treated podocytes were measured via reverse transcription-quantitative PCR and western blotting. Cell migration assays were conducted to assess the migration of oxLDL-treated podocytes. CXCL16 or ADAM10 overexpression and knockdown assays were conducted. The results indicated that oxLDL stimulation increased ADAM10 and CXCL16 expression levels, and enhanced podocyte migration compared with the control group. Moreover, CXCL16 and ADAM10 overexpression significantly increased podocyte migration and the expression of actinin-α4 (ACTN4) compared with the control groups. By contrast, CXCL16 and ADAM10 knockdown significantly reduced podocyte migration and the expression of ACTN4 compared with the control groups. The results suggested that oxLDL promoted podocyte migration by regulating CXCL16 and ADAM10 expression, as well as by modulating the actin cytoskeleton. Therefore, CXCL16 and ADAM10 may serve as novel therapeutic targets for primary nephrotic syndrome in children.

К вопросу о стероидной терапии первичного нефротического синдрома у детей

К вопросу о стероидной терапии первичного нефротического синдрома у детей

Adjuvant treatment with Yupingfeng formula for primary nephrotic syndrome in children: A PRISMA systematic review and meta-analysis of randomized controlled trials.

Yupingfeng formula (YPFF) has been prescribed as adjuvant treatment for pediatric patients with primary nephrotic syndrome (PNS) in China for years. However, the efficacy and adverse effects of these formulations are controversial. A systematic review and meta-analysis of randomized controlled trials (RCTs) were performed to evaluate the benefits and harms of YPFF in treating PNS in children. The MEDLINE, EMBASE, Cochrane Library, CNKI, VIP, WanFang, and CBM databases were searched for RCTs comparing therapies with and without YPFF for PNS from inception to May 13, 2017. Relative risk (RR) and 95% confidence intervals (CI) were expressed for dichotomous outcomes, and weighted mean difference (WMD) with 95% CI for continuous outcomes. Cochrane collaboration tool was used to evaluate the risk of bias of methodologies. Eight studies with 538 participants were identified. Treatment with YPFF significantly increased serum levels of IgA (WMD, 0.48, 95% CI, 0.40-0.56, P < .001), IgG (WMD, 3.36, 95% CI, 2.61-4.12, P < .001), CD4 T-lymphocytes (WMD, 3.35, 95% CI, 2.26-4.43, P < .001), but decreased the level of CD8 T-lymphocytes (WMD, -3.38, 95% CI -5.48 to -1.28, P = .002). YPFF also increased the rates of complete remission (RR: 1.35, 95% CI, 1.09-1.67, P = .005), and decreased the rates of relapse (RR: 0.57, 95% CI, 0.45-0.71, P < .001), and infection (RR: 0.72, 95% CI 0.62-0.83, P < .001). There was no significant difference in the level of IgM between the groups (WMD, 0.12, 95% CI -0.11-0.35, P = .322). YPFF could improve total remission rate and decrease the frequency of relapse and infection rate. The beneficial influence of YPFF may be associated with its immunomodulatory effects. More high-quality studies with larger sample sizes are needed to further identify its efficacy and safety.

Первичный нефротический синдром у детей. Перспективы персонализированной терапии

Первичный нефротический синдром у детей. Перспективы персонализированной терапии

Histopathological Features of Primary Nephrotic Syndrome in Children

Renal biopsy was performed on 28 out of 50 children with primary nephrotic syndrome encountered during the period January 1994 - December 1995. Light microscope (LM) and immunofluorescence microscope QM) examinations were performed on all biopsy specimens. LM examination indicated minimal changes (MC) in 13 cases (46.4%), focal segmental glomerulosclerosis (FSGS) in 10 (35.7%), membranous glomerulonephritis (MG) in 2 (7.1%), mesangial proliferative glomerulonephritis (MPG) in 7 (7.1 %), and membranoproliferative glomerulonephritis (MPGN) in 1 (3.6%). On IM examination, immunoglobulin deposit was not detected in any MC patients, whereas in FSGS, lgG, lgM, C3 and fibrinogen deposits were found. In the MG group, IgG deposition was detected in one case. In the MPG cases, depositions of lgA, IgG, lgM, C3 and fibrinogen were detected and in the case of MPGN, deposits of lgM and C3 were found. Regarding to response to steroid treatment in the MC group, there was a significant difference between the steroid sensitive and steroid insensitive (p&lt;0.05). For the FSGS abnormality in the steroid treatment of U1e insensitive patients, there was found significant difference with the steroid sensitive abnormality (p&lt;0.05). In conclusion, nephritic symptoms (hematuria, proteinuria, azothemia) are possibly the non minimal group and hence, it would be necessary to carry out renal biopsy to prove this.

An implication of relationship between tuberculosis and primary nephrotic syndrome.

There have been few observations on the concurrence of tuberculosis (TB) and primary nephrotic syndrome (PNS). We try to define their relationship from multiple aspects. Patients with PNS (n = 386), secondary nephrotic syndrome (SNS, n = 60), pneumonia (n = 196), and TB (n = 156) were enrolled. Through a novel evaluation system, the evidence of TB infection (clinical, laboratory, and radiographic evidence) was classified into 5 levels: lack of evidence (level 0), mild-to-moderate evidence (level 1-2), strong evidence (level 3-4). Additionally, whether TB infection was primary or secondary to long-term steroid therapy for PNS was recorded. Through the evaluation system, 42.24% (68/161) of PNS inpatients had evidence of TB level 1-4, more than those in SNS (23.33%, P = .788) or pneumonia (22.45%, P = .004); 9.32% (15/161) PNS inpatients had evidence of level 3-4, who should be considered as having TB; 13 of the 15 patients had TB before the onset of PNS; 61.75%(96/153) PNS inpatients were abnormal on chest imaging. In the TB group, 28.21% (44/156) patients had abnormal urinalysis, more than those in the pneumonia group (8.16%, 16/196, P&lt;0.001). TB-related symptoms were seldom seen in PNS inpatients (cough 26.1%, fever 6.8%; night sweats, fatigue, and weight loss were negative). Around 10% of PNS in children has an association with TB infection that preceded the onset of PNS.

Correlation between expressions of IgE and CD23 and primary nephrotic syndrome in children

Objective To explore the correlation between the expressions of IgE and CD23 and primary nephrotic syndrome in children.Methods 52 children with primary nephrotic syndrome and 13 healthy children were recruited into this study.The serum IgE concentrations were detected by immunoradiation assay.The proportion of CD23 positive cells were determined by flow cytometry analysis.Results The IgE concentration and the proportion of CD23+ cells in the children with primary nephrotic syndrome were significantly higher than those of the health counterparts (IgE:[452.5 ± 239.7] vs.[132.6 ± 105.5],P ＜ 0.01; CD23+ cells％:[16.6 ± 7.6] vs.[5.2 ± 3.9],P ＜ 0.05).The serum IgE concentration was obviously higher in the patients with steroid-resistant nephrotic syndrome (SRNS) than in the children with steroid-sensitive nephrotic syndrome (SSNS) before and after the treatment (before the treatment:[462.9 ± 321.2] vs.[382.1 ± 269.0],P ＜ 0.05; after the treatment:[246.7 ± 182.1] vs.[171.7 ± 107.9],P ＜ 0.05).The proportion of CD23+ cells was significantly enhanced in the SRNS children than in the SSNS children before and after the treatment (before the treatment:[19.3 ± 6.4] vs.[15.6 ± 7.7],P ＜ 0.05; after the treatment:[12.2 ± 8.5] vs.[6.1 ± 4.3],P ＜ 0.01).IgE concentration positively correlated with CD23+ proportion in the children with primary nephrotic syndrome (r=0.605,P ＜ 0.05).Conclusions Immune dysfunction was observed in children with primary nephrotic syndrome,as indicated by the abnormal expression of serum IgE concentration and CD23+ cell proportion.Compared to SSNS children,these disorders were more obvious in SRNS children befaore and after treatment.Therefore,a combined testing of serum IgE concentration and CD23+ cell proportion shows great significance in the diagnosis and prognosis evaluation in primary nephrotic syndrome in children. Key words: Primary nephrotic syndrome; children; IgE; CD23

Pathological profile of biopsied Egyptian children with primary nephrotic syndrome: 15-year single center experience

Primary nephrotic syndrome (PNS) in children is a common problem worldwide. The pathological pattern of PNS differs between countries. However, data on the pathological pattern of PNS in Egyptian children are scant. This study was conducted to determine indications and results of renal biopsy in Egyptian children with PNS from a single tertiary children's hospital. Medical records of all children with PNS aged 3 months-18 years who underwent renal biopsy from 1998 to 2012 at Mansoura University Children's Hospital were retrospectively reviewed. A total of 741 patients (441 males, 300 females) underwent 798 biopsies of which only four specimens were insufficient. Mean age at time of biopsy was 7.4 ± 3.6 years. The main indication for biopsy was steroid resistance (n = 354, 44.4%) followed by atypical PNS (n = 234, 29.3%) of which gross hematuria was the most common cause. Minimal change disease (MCD) and its variants were the most frequent pathology (n = 431, 54.3%) irrespective of biopsy indication, and incidence of focal segmental glomerulosclerosis was observed to be increasing over the years. This is the first large study on the pathological pattern of PNS in children from Egypt, and it shows that MCD is the most frequent underlying pathology and steroid resistance is the most frequent indication for biopsy.

Distribution of pathological finding in the children with nephrotic syndrome from Guangxi.

To identify the variations in pediatric renal biopsy pathology and clinicopathological features in Guangxi, China, in the past ten years, we studied retrospectively the kidney biopsies performed to evaluate the primary nephrotic syndrome (PNS) in 218 children at two main medical centers in Guangxi from January 1999 to January 2009. The major pathological finding was mesangial proliferative glomerulonephritis (48.2%), focal segmental glomerulosclerosis (16.5%), immunoglobulin A nephropathy (13.3%) and minimal change disease (11.0%). Patients with different pathological types yielded different response rates to glucocorticoids (P <0.001). There were statistical significant differences between prognosis for the different pathological types (P <0.05). The pathological characteristics of PNS in children were diverse and significant for guiding the grade of glucocorticoid response and predicting the prognosis of the PNS disease.