Antibody Status in Children with Steroid-Sensitive Nephrotic SyndromeOct 02, Author: Thus, patients may be considered for steroid treatment prior to kidney biopsy if they meet all of the following criteria:. Kidney biopsy should be performed prior to any immunosuppressive treatment, including steroids, in patients mccns meet one or more of the following criteria:. Steroid sensitive mcns criteria are laboratory findings possibly indicative of secondary nephrotic syndrome or INS other than minimal change nephrotic syndrome MCNSsuch how does trenbolone e work steroid sensitive mcns following:. In these sensifive, histology guides treatment, and steroids may or may not be indicated depending on the underlying etiology. Children steroid sensitive mcns than 12 years require a kidney biopsy due to the rising incidence of focal segmental glomerulosclerosis FSGS and other causes of nephrosis in that age range.
Steroid Sensitive Nephrotic Syndrome in Children | OMICS International
The pathophysiology of hypogammaglobulinemia in nephrotic syndrome NS remains unknown. We evaluated the differences in the distribution of anti-bacterial antibodies and anti-viral antibodies, and those of immune antibodies and natural antibodies in steroid-sensitive NS.
We examined the antibody status of 18 children who had routine vaccinations. The levels of immnunoglobulin G IgG , the IgG subclasses, and the antibodies induced by vaccinations such as diphtheria-pertussis-tetanus and measles-mumpsrubella were analyzed in children with steroid-sensitive NS. The antibodies induced by bacterial antigens were depressed and the seropositivity of anti-viral antibodies tended to be lower than those of age-matched control children during the acute stage.
The depressed immune antibody status recovered rapidly in the remission stage of NS, despite corticosteroid treatment. IgG levels correlated positively with albumin levels, and all antibodies, including immune and natural antibodies, were depressed in the acute stage of NS. Our results suggest that hypogammaglobulinaemia in NS may be associated with intravascular homeostasis of oncotic pressure.
Nephrotic syndrome NS is characterised by proteinuria, hypoalbuminemia, hyperlipidemia, and generalized edema. Since children with minimal-change nephrotic syndrome MCNS have highly selective proteinuria, 4 , 5 as do those with congenital NS, 6 , 7 the urinary loss of IgG is negligible when compared to that of other glomerulopathies.
Thus, instead of the urinary excretion of IgG, other factors have been suggested to explain the hypogammaglobulinaemia of MCNS, such as the increased catabolism of IgG or reduced IgG synthesis. IgG is the most abundant of the immunoglobulins and constitutes approximately one third to one half of the proteins in human plasma. Total IgG is composed of four subclasses that differ in their biological functions. It has been proposed that antibodies directed against bacteria or viruses are distributed differently in the IgG subclasses; antibodies directed against viruses are classified in the IgG1 and IgG3 subclasses, and antibodies directed against bacterial agents in the IgG2 subclass.
In this study, to evaluate the differences in the distributions of immune antibodies and natural antibodies, and differences in the distributions of anti-bacterial antibodies and anti-viral antibodies, we examined the levels of the IgG subclasses and various antibodies induced by vaccinations in children with MCNS.
As almost all of the children with steroid-sensitive NS have pathohistologically minimal change lesions, we regard our subjects as having MCNS without conducing a renal biopsy. However, children with steroid-resistant NS or other biopsy-proven glomerulopathies were excluded from the study since these disorders have the possibility of having poorly selective proteinuria. The data was obtained at the initial presentation of each child and included serum levels of albumin, cholesterol, and immunoglobulins IgG, IgM, IgA, and IgE.
The urine protein selectivity index IgG to transferrin ratio and hour urine protein electrophoresis were evaluated in eight children. Sera of 20 age-matched control subjects were prepared from healthy children who had minor elective surgery in our hospital. In Korea, it is assumed that most children over 5 years of age have been infected with EBV.
The seropositivity of anti-viral and anti-pertussis antibodies was measured by commercial kits, and the anti-diphtheria IgG and antitetanus IgG were analysed by quantitative method. Antibodies directed against the bacterial antigens anti-diphtheria IgG, anti-tetanus IgG and anti-pertussis IgG were measured by enzyme-linked immunosorbent assay Im-muno-Biological Laboratory Inc.
The means of continuous variables the values for the IgG subclasses, anti-diphtheria, and anti-tetanus IgGs between the acute stage and the remission stage of NS were compared using the paired t-test, and using the Mann-Whitney U test between NS groups and the control group.
The mean age of the 18 nephrotic children was 6. The mean values for albumin, IgG, and total cholesterol in the nephrotic children were 1. These results indicate that the IgM and IgE levels were elevated compared to the normal reference ranges for age.
The values for IgG and the IgG subclasses at presentation and at remission in the children with NS and those of the control children mean age 6. The values for total IgG and the four IgG subclasses were all significantly reduced during the acute and remission stages compared to those of the control children.
However, the levels of IgG3 and IgG4 in the acute stage were not statistically different from those in the remission stage Table 1.
There are significant differences among the groups, except IgG3 and IgG4 levels between at acute stage and remission stage. The rates of seropositivity for anti-viral antibodies during the acute stage were lower than those of the control children, but there was no statistically significant difference overall antibodies Table 2. Of the anti-bacterial antibodies, the values for anti-diphtheria IgG, anti-tetanus IgG during the acute stage were significantly lower than the corresponding values during remission, and significantly lower than those of the control children.
However, there was no difference between those of the nephrotic children during remission and those of the control children. There are lower seropositivities in all viral antibodies at acute stage compared to control, but no statistically significant differences among three groups. Although the immunopathogenesis of various renal diseases that can induce NS differs according to the disease, dysgammaglobulinaemia low IgG, but high IgM and IgE levels is a common feature in all nephrotic patients, including those with congenital NS which is caused by genetic defects in the glomerular basement membrane.
Although many studies have focused on the increased catabolism of IgG, including urinary loss, 8 , 17 the highly selective proteinuria of MCNS and the correlation of IgG and albumin levels during the acute stage of MCNS do not support the urinary loss of IgG.
Since the clinical and laboratory characteristics of NS hypercholesterolaemia and hypogammaglobulinaemia result from the urinary loss of medium-sized proteins, mainly albumin, the compensatory mechanisms for this disturbance of protein homeostasis in NS may be induced as soon as the beginning of massive proteinuria.
Patients with NS and nephrotic animals may overproduce high-molecular weight proteins, such as lipoproteins, which cause hyperlipidemia and are protected from urinary loss for maintaining the oncotic pressure. IVIG has been extensively used as a treatment for immune-mediated diseases, including some renal diseases such as lupus nephritis, although nephrotoxicity can be a serious but rare complication.
Although the methods for distinction between the two antibodies are not yet discovered, it is believed that the majority of IgGs in plasma are the natural antibodies.
In the present study, we found that all IgG antibodies IgG subclasses were depressed including immune antibodies both anti-viral and anti-bacterial antibodies in acute stage of NS.
Hypogammaglobulinaemia in NS has been proposed to be a risk factor for bacterial infections, 3 but the data for increased risk of viral infections in nephrotic children are limited.
Although antibodies against pathogens are distributed in different IgG classes according to viruses or bacteria, 12 , 13 in this study the levels of all the IgG subclasses showed rather homogeneous depression during the acute stage of MCNS as with previous studies. The same pattern of seropositivity in anti-pertussis antibodies with depressed levels of other two bacterial antibodies was also observed Table 3.
Since we did not quantify the antiviral antibodies, further studies with larger samples are required to determine whether there is a difference in the degree of depression of anti-bacterial and anti-viral antibodies. Given the rapid recovery of antibody titers in remission and the important role of cell-mediated immunity for viral infections, the seroconversion of viral antibodies in the acute stage may not indicate a loss of immunity against the invading viruses.
It has been reported that the levels of IgG3 and IgG4 in the acute stage are not statistically different from those in the remission stage, 2 , 21 and we confirmed this finding in this study Table 1. Although the association between IgG subclasses and some glomerulopathies has been reported, 22 - 24 the dysgammaglobulinemia including the IgG subclasses, IgM and IgE and its clinical implications in NS during the acute and remission stages have remained unresolved.
Children with NS show a normal immune response to vaccines, including that for S. Together with hypogammaglobulinaemia, other factors such as immunosuppressant treatments with corticosteroids or cytotoxic drugs and the urinary loss of immune substances, including those that participate in phagocytosis e. In conclusion, we found that IgG levels correlated positively with albumin levels and the levels of all antibodies, immune antibodies and natural antibodies, were reduced during the acute stage of MCNS and recovered after the cessation of proteinuria.
Our results indirectly suggest that hypogammaglobulinaemia in NS may be associated with systemic protein homeostasis in vivo. Further studies are required to explain the hypogammaglobulinaemia of patients with NS. The authors have no financial conflicts of interest. National Center for Biotechnology Information , U.
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Find articles by Joon-Sung Lee. Yonsei University College of Medicine This article has been cited by other articles in PMC. Abstract Purpose The pathophysiology of hypogammaglobulinemia in nephrotic syndrome NS remains unknown. Materials and Methods We examined the antibody status of 18 children who had routine vaccinations. Conclusions IgG levels correlated positively with albumin levels, and all antibodies, including immune and natural antibodies, were depressed in the acute stage of NS.
Nephrotic syndrome, minimal change, hypogammaglobulinaemia, IgG, IgG subclasses, diphtheriapertussis-tetanus, measles-mumps-rubella, children, natural antibodies. Open in a separate window. Positive correlation between albumin and immunoglobulin G IgG values. Footnotes The authors have no financial conflicts of interest. Serum immunoglobulins in the nephrotic syndrome. A possible cause of minimal-change nephrotic syndrome. N Engl J Med. Serum levels of immunoglobulins and IgG subclasses in steroid sensitive nephrotic syndrome.
Peritonitis in childhood nephrotic syndrome: Am J Dis Child. Sodium dodecyl sulphate polyacrylamide gel electrophoresis patterns of proteinuria in various renal diseases of childhood. Sodium dodecyl sulphate polyacrylamide gel electrophoresis of urinary proteins in steroidresponsive and steroid-resistant nephrotic syndrome in children. Altered immunoglobulin status in congenital nephrotic syndrome. Selectivity of proteinuria in congenital nephrotic syndrome of the Finnish type. Hypogammaglobulinaemia in nephrotic rats is attributable to hypercatabolism of IgG.
Impaired IgG synthesis in patients with the nephrotic syndrome. Decreased serum transferrin concentration in children with the nephrotic syndrome: Correlation between serum albumin and IgG level in minimal change nephrotic syndrome.
J Korean Soc Pediatr Nephrol. Correlation between serum IgG-2 concentrations and the antibody response to bacterial polysaccharide antigens. Subclass distribution of rubella virus-specific immunoglobulin G. Immunomodulation of autoimmune and inflammatory diseases with intravenous immune globulin. Immunoglobulin G has a role for systemic protein modulation in vivo: Intravenous immunoglobulin in autoimmune and inflammatory diseases: Plasma IgG pool is not defended from urinary loss in nephrotic syndrome.
Plasma composition in the nephrotic syndrome. Kawasaki diasease may be a hyperimmune reaction of genetically susceptible children to variants of normal environmental flora. Intravenous immunoglobulin and the kidney--a two-edged sword. IgG subclasses in children with nephrotic syndrome.
Am J Clin Pathol. IgG subclasses in patients with membranoproliferative glomerulonephritis, membranous nephropathy, and lupus nephritis.