scholarly journals HLA-B60 and HLA-DR4 alleles in Javanese children with steroid-sensitive primary nephrotic syndrome

2016 ◽  
Vol 46 (6) ◽  
pp. 246
Author(s):  
M P Damanik
Keyword(s):  
Nephrotic Syndrome ◽  
Strong Association ◽  
Control Sample ◽  
Hla Class I ◽  
Control Group ◽  
Healthy Children ◽  
Primary Nephrotic Syndrome ◽  
Steroid Sensitive Nephrotic Syndrome ◽  
Steroid Sensitive ◽  
Sequence Specific Oligonucleotide Probe

Background Steroid-sensitive nephrotic syndrome (SSNS) ofchildren is associated with several human leucocyte antigen (HLA)class I and class II.Objective To investigate the association between HLA-B60 andHLA-DR4 alleles and primary nephrotic syndrome (PNS) inJavanese children.Methods A case control study was conducted on 47 Javanesechildren with PNS who were typed for HLA-B60 and HLA-DR4 al-leles, using DNA sequence specific oligonucleotide probe (SSOP)as control sample, 47 healthy children were also typed for thoseHLA antigens using the same technique.Results Compared with control group, children with PNS had higherfrequency of both HLA-B60 (23.32% vs 4.3%; OR=6.85 [CI=1.32-35.65]; P<0.01) and HLA-DR4 (40.0% vs 2.1%; OR=30.67 [CI:3.71-253.33]; P<0.0002). There was association between HLA and PNSwith SSNS in children.Conclusion The strong association between PNS and HLA anti-gen support the immunogenetic background of the disease, whichseems to be stronger in young children with SSNS.

scholarly journals The role of cell signaling molecules in the pathogenesis of glomerulonephritis in children

2021 ◽  
Vol 64 (2) ◽  
pp. 37-41
Author(s):  
Angela Ciuntu ◽  
Keyword(s):  
Nephrotic Syndrome ◽  
Cell Signaling ◽  
Clinical Manifestations ◽  
Signaling Molecules ◽  
Control Group ◽  
Healthy Children ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Steroid Sensitive Nephrotic Syndrome ◽  
Steroid Sensitive

Background: Cytokines are functional class of tiny proteins and glycoprotein and fundamentally they are monomers that function as soluble mediators in an autocrine or paracrine manner. Cytokines are produced by a number of cell types, predominantly leukocytes, and their targets implicate both immune and non-immune cells. Material and methods: This study was performed on 75 children with glomerulonephritis (GN), aged from 2 up to 17 years. There were 20 children with steroid-sensitive nephrotic syndrome (SSNS), 15 children with steroid-resistant nephrotic syndrome (SRNS), 20 children with chronic glomerulonephritis (CGN) nephrotic form and 20 children with CGN mixed form. This study was performed on patients experiencing disease relapse and clinical remission. The control group consisted of 20 healthy children. Results: The results of this study demonstrated increased levels of cell signaling molecules (IL-8, TNF-α, MCP-1, MIP-1α) in the urine during clinical manifestations, valuable result due to their major role in the immunopathogenic mechanism of proteinuria in nephrotic syndrome. Conclusions: Determination of urinary concentrations of cellular signaling molecules may be useful as a predictive non-invasive method for estimating disease activity, monitoring disease progression, differentiating steroid-sensitive nephrotic syndrome from steroid-resistant nephrotic syndrome, and assessing the effectiveness of treatment in children with different variants of GN

A genetic study of steroid-resistant nephrotic syndrome: relationship between polymorphism -173 G to C in the MIF gene and serum level MIF in children

2015 ◽  
Vol 7 (1) ◽  
pp. 102-107 ◽  
Author(s):  
O. R. Ramayani ◽  
N. Sekarwana ◽  
P. P. Trihono ◽  
A. H. Sadewa ◽  
A. Lelo
Keyword(s):  
Nephrotic Syndrome ◽  
Steroid Resistance ◽  
Enzyme Linked Immunosorbent Assay ◽  
Healthy Children ◽  
Single Nucleotide ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Increased Risk ◽  
Steroid Sensitive Nephrotic Syndrome ◽  
Steroid Sensitive

There is no satisfactory explanation as to why some nephrotic syndrome (NS) patients respond to glucocorticoids and others do not. The aim of this study was to investigate an association between single nucleotide polymorphism of the MIF gene -rs755622 and serum MIF concentrations in NS patients. During a period between November 2011 and September 2012, 120 consecutive children divided into three groups [healthy children, steroid-resistant nephrotic syndrome (SRNS) and steroid-sensitive nephrotic syndrome (SSNS)] were examined. Children were defined as healthy when they had a normal estimated glomerular filtration rate and spot urinary albumin creatinine ratio <150 μg/mg creatinine. SRNS was diagnosed in children who did not respond to the usual doses of steroids within 4 weeks of initiating treatment. SSNS patients were defined as those who had remission after usual doses of steroids. The genotype of -173 G to C polymorphism of the MIF gene was determined using polymerase chain reaction restriction fragment length polymorphism methods. Serum MIF concentration was measured using sandwich enzyme-linked immunosorbent assay. The allele frequency of the C allele was higher in SRNS compared with that of SSNS patients (P=0.025). There was a trend toward an association between genotypes and serum MIF disturbances. In conclusion, this study noted elevated circulating serum MIF levels and higher frequency of the C allele of the MIF gene in SRNS patients. The presence of the C allele implies an increased risk for steroid resistance.

scholarly journals Association of HLA class II and history of atopy and frequent relapse of childhood steroid-sensitive nephrotic syndrome

2007 ◽  
Vol 47 (2) ◽  
pp. 60 ◽  
Author(s):  
Dany Hilmanto
Keyword(s):  
Nephrotic Syndrome ◽  
Class Ii ◽  
Hla Class Ii ◽  
Healthy Children ◽  
Upper Respiratory Tract ◽  
Steroid Sensitive Nephrotic Syndrome ◽  
History Of ◽  
Steroid Sensitive ◽  
Polymerase Chain ◽  
Control Study

Background The association between HLA class II and frequentrelapse of nephrotic syndrome (FRNS) has been reported.Objective To identify the association between HLA class II,history of atopy, and upper respiratory tract infection (URTI)with FRNS.Methods This was a case control study conducted at theDepartment of Child Health, Hasan Sadikin Hospital Bandungand Cipto Mangunkusumo Hospital Jakarta from November 2002to October 2003 on children aged 1-14 years with FRNS. Thesubjects consisted of 40 FRNS and 84 healthy children. HLAclass II was typed by polymerase chain reaction-sequence specificoligonucleotide (PCR-SSO) in Leiden, the Netherlands. Theassociation between HLA class II and FRNS was expressed byodds ratio (OR). The association between such factors and FRNSwas analyzed by logistic regression.Results Atopy was higher in patients than that in controls(P=0.013). URTI did not differ in both groups (P=0.173). HLA-DRB1*03 and DRB1*04 (OR=4.43, P=0.03), DQB1*02(OR=3.43, P=0.00), and DQB1*04 (OR=12.06, P=0.01) weresignificantly higher among patients than those in controls whereasHLA-DRB1*12 (OR=0.34, P=0.02) and DQB1*0301p(OR=0.35, P=0.02) were significantly lower among patients thanthose in controls. Using logistic regression analysis, only HLA-DRB1*12, DQB1*02 and atopy took part in FRNS.Conclusion HLA-DRB1*12, DQB1*02, and atopy all togetherhave association with FRNS.

scholarly journals R229Q Polymorphism of NPHS2 Gene in Group of Iraqi Children with Steroid-Resistant Nephrotic Syndrome

2017 ◽  
Vol 2017 ◽  
pp. 1-5 ◽  
Author(s):  
Shatha Hussain Ali ◽  
Rasha Kasim Mohammed ◽  
Hussein Ali Saheb ◽  
Ban A. Abdulmajeed
Keyword(s):  
Nephrotic Syndrome ◽  
Molecular Study ◽  
Control Group ◽  
Steroid Resistant ◽  
Sequence Variations ◽  
Medical City ◽  
Steroid Sensitive Nephrotic Syndrome ◽  
Steroid Sensitive ◽  
And Control ◽  
Nphs2 Gene

Background. The polymorphism R229Q is one of the most commonly reported podocin sequence variations among steroid-resistant nephrotic syndromes (SRNS). Aim of the Study. We investigated the frequency and risk of this polymorphism among a group of Iraqi children with SRNS and steroid-sensitive nephrotic syndrome (SSNS). Patients and Methods. A prospective case control study which was conducted in Al-Imamein Al-Kadhimein Medical City, spanning the period from the 1st of April 2015 to 30th of November 2015. Study sample consisted of 54 children having NS, divided into 2 groups: patients group consisted of 27 children with SRNS, and control group involved 27 children with SSNS. Both were screened by real time polymerase chain reaction for R229Q in exon 5 of NPHS2 gene. Results. Molecular study showed R229Q polymorphism in 96.3% of SRNS and 100% of SSNS. There were no phenotypic or histologic characteristics of patients bearing homozygous R229Q polymorphism and the patients with heterozygous R229Q polymorphism. Conclusion. Polymorphism R229Q of NPHS2 gene is prevalent in Iraqi children with SRNS and SSNS. Further study needs to be done, for other exons and polymorphism of NPHS2 gene in those patients.

scholarly journals Molecular factors predicting steroid resistance in pediatric nephrotic syndrome

2021 ◽  
pp. 32-37
Author(s):  
Ie. A. Burlaka ◽  
I. V. Bagdasarova
Keyword(s):  
Nephrotic Syndrome ◽  
Steroid Resistance ◽  
Control Group ◽  
Activation Markers ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Steroid Sensitive Nephrotic Syndrome ◽  
Cellular Hypoxia ◽  
Steroid Sensitive ◽  
Nephrotic Children

Objectives: the objective of this paper was to study the levels of cellular hypoxia, apoptosis controlling factors in children with steroid-sensitive and steroid-resistant nephrotic syndrome. Background: patients with steroid-resistant nephrotic syndrome (SRNS) represent a challenging subset of patients with nephrotic syndrome who often fail standard immunosuppression and have a higher likelihood of progressing to end-stage renal disease. The search of the biochemical markers undergoing the steroid-resistance is under urgent need. Methods: an examination of kidney biopsies and blood of 56 patients (aged 10 to 15 years) with nephrotic syndrome was done. Conventional clinical investigations, immunohistochemistry, immunoblotting were used in this study. Results: patients with steroid-resistant nephrotic syndrome show an increased level of HIF-1 alfa (a marker of cellular hypoxia) as compared to the control group and children with steroid-sensitive nephrotic syndrome. Patients with steroid-resistant nephrotic syndrome show a down-regulation of anti-apoptotic marker BcL-xL as compared to the control group and children with steroid-sensitive nephrotic syndrome. Conclusion: hypoxia-induces disorders and apoptosis activation markers are considered to be included in the complex scheme predicting steroid-resistance in nephrotic children. 

scholarly journals Changes in clinicopathological features of primary nephrotic syndrome in children over a 10 years period: Research Paper

2020 ◽  
Author(s):  
Haiyan Guo ◽  
Qian Li ◽  
Lichun Yu ◽  
Yuan Chen ◽  
Jing Wang ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Clinical Manifestations ◽  
Clinicopathological Features ◽  
Pathological Features ◽  
Primary Nephrotic Syndrome ◽  
Common Chronic Disease ◽  
Steroid Sensitive Nephrotic Syndrome ◽  
Group A ◽  
Steroid Sensitive ◽  
Group B

Abstract Background Primary nephrotic syndrome (PNS) is a common chronic disease in childhood and results in heavy illness burden. The aim of this study is to investigate the change in the clinicopathological features of children with NS worldwide. Methods Children with PNS were divided into two groups according to the date of admission. Group A was admitted from January 1, 2006 to December 31, 2010 and group B from January 1, 2011 to December 31, 2015. Clinical manifestations and pathological features of the children in both groups were retrospectively analyzed. Results This study includes 1072 patients. There were 783 cases of steroid-sensitive nephrotic syndrome (SSNS), 241 cases of steroid-resistant nephrotic syndrome (SRNS), and 48 cases of unknown steroid effects. The incidence of complications in group B was significantly higher than that in group A, especially for infections. A total of 136 (12.96%) patients underwent renal biopsy. The most common lesions were minimal change disease (44.12%). The rate of mesangial proliferative glomerulonephritis (MsPGN) significantly decreased and that of membranous nephropathy (MN) increased over the years. A total of 292 (27.24%) patients were administered immunosuppressants. The administration of Tripterygium was decreased, whereas the using of Tacrolimus increased over the years. Conclusion There have been changes in the incidence of complications, pathological features, and immunosuppressants in children with PNS over 10 years.

scholarly journals Predictability of Urinary CD80 in the Relapse of Primary Nephrotic Syndrome

2017 ◽  
Vol 2017 ◽  
pp. 1-6 ◽  
Author(s):  
Juan Liao ◽  
Xiao-Chuan Wu ◽  
Qia Cheng ◽  
Can-Lin Li ◽  
Zhu-Wen Yi ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Urinary Protein ◽  
Control Group ◽  
Healthy Children ◽  
Creatinine Excretion ◽  
Urinary Creatinine ◽  
Primary Nephrotic Syndrome ◽  
Significant Difference ◽  
Relapse Group ◽  
Urinary Creatinine Excretion

Purpose. The current study is aimed at investigating whether urinary CD80 is reliable to predict the recurrence of pediatric PNS. Materials and Methods. A total of 128 children, 105 males and 23 females, were enrolled in this study. Urinary samples were collected from SSNS and SRNS patients and 25 healthy children as controls. Urinary CD80 was measured by ELISA and adjusted for urinary creatinine excretion. Results. Urinary CD80 in relapse stage of SSNS was significantly higher, and the urinary CD80 of paired relapse and remission stages of each SSNS patient were also significantly different. No significant difference was found between the urinary CD80 in SRNS relapse group, SRNS remission group, and the control group. Similarly, there was no significant difference between frequent SSNS and not frequent SSNS in remission group, as well as the relapse group. There is no correlation between urinary CD80 and 24-hour urinary protein. Conclusion. The increase of urinary CD80 was closely associated with the relapse of SSNS but was not related to the frequency of relapse. The urinary CD80 changes of concentration were reliable to predict the recurrence of SSNS. However, it cannot be used to predicate the frequent recurrence of PNS.

scholarly journals Steroid treatment for the first episode of childhood nephrotic syndrome: comparison of the 8 and 12 weeks regimen using an individual patient data meta-analysis

2021 ◽  
Author(s):  
Anne M. Schijvens ◽  
Nynke Teeninga ◽  
Eiske M. Dorresteijn ◽  
Steven Teerenstra ◽  
Nicholas J. Webb ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Individual Patient Data ◽  
Meta Analysis ◽  
Steroid Treatment ◽  
First Episode ◽  
Childhood Nephrotic Syndrome ◽  
Steroid Sensitive Nephrotic Syndrome ◽  
Steroid Sensitive ◽  
First Relapse ◽  
Steroid Regimen

AbstractSteroids are the cornerstone of the treatment of childhood nephrotic syndrome. The optimal duration for the first episode remains a matter of debate. The aim of this study is to determine whether the 8 weeks International Study of Kidney Disease in Children (ISKDC) regimen is equally effective as the 12 weeks steroid regimen from the German society of pediatric nephrology (Arbeitsgemeinschaft für Pädiatrische Nephrologie [APN]). An individual patient data (IPD) meta-analysis of randomized controlled trials reporting on prednisolone treatment for a first episode of childhood nephrotic syndrome was conducted. European trials aimed at investigating the ISKDC and/or APN steroid regimen were selected. The lead investigators of the selected trials were requested to provide the IPD of the specific treatment groups. Four trials included European cohorts using dosing schedules according to the regimens studied. IPD of two trials were available. A significant difference was found in time to first relapse after cessation of steroid treatment between the 8 and 12 weeks treatment group with a median time to relapse of 29 and 63 days, respectively. Moreover, relapse rate ratios during total follow-up were 51% higher for the 8 weeks regimen. Finally, younger children have a significantly lower time to first relapse and frequently relapsing nephrotic syndrome.Conclusions: The results of this IPD meta-analysis suggest that the 8 weeks steroid regimen for a first episode of steroid-sensitive nephrotic syndrome may not be equally effective as the 12 weeks steroid regimen. Moreover, this study highlights the importance of using uniform definitions to enable accurate comparison and interpretation of trial results.Trial registration: Registration number: CRD42020199244, date of registration 16-08-2020 What is Known:• Steroids are the cornerstone of the treatment of childhood nephrotic syndrome, however the optimal duration for the first episode remains a matter of debate.• Currently, the 8 weeks ISKDC protocol and 12 weeks APN protocol are among the most frequently used protocols in Europe. What is New:• The 8 weeks steroid regimen for a first episode of steroid-sensitive nephrotic syndrome may not be equally effective as the 12 weeks steroid regimen for the treatment of a first episode of nephrotic syndrome.• Younger children have a significantly shorter time to first relapse and time to frequent relapsing nephrotic syndrome.

scholarly journals Distribution of serum adiponectin isoforms in pediatric patients with steroid-sensitive nephrotic syndrome

2021 ◽  
Author(s):  
Tetsuro Tamai ◽  
Kaori Kamijo ◽  
Yoshifusa Abe ◽  
Satoshi Hibino ◽  
Shunsuke Sakurai ◽  
...  
Keyword(s):  
Molecular Weight ◽  
Nephrotic Syndrome ◽  
Pediatric Patients ◽  
Serum Adiponectin ◽  
Total Serum ◽  
Control Subjects ◽  
Remission Period ◽  
Total Adiponectin ◽  
Steroid Sensitive Nephrotic Syndrome ◽  
Steroid Sensitive

Abstract Background Serum adiponectin circulates in three multimeric isoforms: high-molecular-weight (HMW), middle-molecular-weight (MMW), and low-molecular-weight (LMW) isoforms. Potential change in the circulating adiponectin levels in patients with nephrotic syndrome (NS) remain unknown. This study aimed to assess the levels of total adiponectin and the distribution of its isoforms in pediatric patients with NS. Methods We sequentially measured total adiponectin and each adiponectin isoform levels at the onset of NS, initial remission, and during the remission period of the disease in 31 NS patients. We also calculated the ratios of HMW (%HMW), MMW (%MMW), and LMW (%LMW) to total adiponectin incuding 51 control subjects. Results The median of total serum adiponectin levels in patients were 36.7, 36.7, and 20.2 μg/mL at the onset, at initial remission, and during the remission period of NS, respectively. These values were significantly higher than those in control subjects. The median values of %HMW, %MMW, and %LMW values were 56.9/27.0/14.1 at the onset, 62.0/21.8/13.4 at the initial remission, and 58.1/21.7/17.5 at during the remission period of NS, respectively. Compared with control subjects, %HMW at initial remission and %MMW at the onset were high, and the %LMW values at the onset and at initial remission were low. Conclusions In patients with NS, total serum adiponectin levels increase at the onset of the disease, and the ratio of adiponectin isoforms changes during the course of the disease. Further studies are needed to delineate the mechanisms between proteinuria and adiponectin isoforms change.

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