الفهرس 
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Abstract
Nephrotic syndrome (NS) results from excessive urinary loss of albumin and other plasma proteins of similar mass and present clinically as a syndrome complex of low serum albumin levels, edema, high blood lipid levels, and lipids in the urine.
It is the most common renal disorder in Africa and accounts for 40% of renal disorders in Egypt. Minimal changes NS (MCNS) constitutes 88% of the cases of NS. The glomerular diseases that cause nephrotic syndrome generally can be divided into primary and secondary etiologies.
Primary nephrotic syndrome (PNS), also knows as idiopathic nephrotic syndrome (INS) is associated with glomerular diseases intrinsic to the kidney and not related to systemic causes. A wide variety of glomerular lesions can be seen in INS.
The include MCNS, focal segmental glomeruloselerosis (FSGS), membranous nephropathy (MN), membranoproliferative glomerulonephritis (MPGN), diffuse meningeal proliferation and others.
INS is divided into steroid-sensitive (SSNS) and steroid-resistant nephrotic syndrome (SRNS) because response to steroid has a high correlation with histological subtype and prognosis. The landmark study of nephritic syndrome in children, the international study of kidney disease in children (ISKDC), found that the vast majority of preadolescent children with INS and MCNS on kidney biopsy.
In recent years, different studies have been performed in relation to NS and immunity.
These studies can be classified under 2 main categories, namely cellular immunity and humoral immunity shalboub was the first to postulate that MCNS could be produced by a systemic abnormality of T-cell function. The mechanisms by which T cells increase glomerular permeability have remained elusive. There is evidence that idiopathic MCNS may be due to a circulating factor released from activated T cells. Efforts have been made to identify this specific cytokine as well as to understand the mechanism (s) for the increased release of this factor.
In MCNS, serum IgG and IgA levels are reduced, whereas serum Igm level is elevated. It was postulated that the primary defect in idiopathic NS is the deficiency in the T cell function that mediated the switch from OGM synthesis to IgG synthesis found abnormal levels of immunoglobulin’s both in active and in remission stages of the disease, whereas elevated levels of circulating immune complexes were soon only in patients with relapse.
Similar to albumin, IgG is lost in the urine, its serum concentration in decreased, and the fractional rate of its catabolism is increased, suggesting that the kidney contributes to IgG catabolism in conditions of proteinuria. IgG synthesis responds in a variable fashion in the NS, and may be decreased, thus contributing to its reduced serum concentration. In contrast, the serum concentration of the high-concentration of a variety of high-molecular weight liver-derived proteins.
A study suggested that hypogrammaglobulinemia of steroid-sensitive NS (SSNS) is characterized by a different constitution of IgG subelasses. In relapse, a reduction of serum levels of IgG-1, IgG-3 occurs, while low concentrations of IgG-2 might be the explanation for hypogrammaglobulinema in remission of (SSNS).( . Markus et al., 2002)
Conclusion
Nephrotic syndrome is an immune-mediated kidney disease associated with T-cell dysfunction and secondary disturbance of B-cell that leads to changes in the levels of IgG and M.
Therefore, the estimation of IgG and IgM levels and their ratio as well as the correlation of these values with clinical responsiveness to steroid therapy, relapse, and dependency is important to monitor management strategies and to determine the outcome.
Infection is the major complication of nephrotic syndrome. Children in relapse are an increased risk of developing bacterial infections because of urinary losses of Ig and properdin factor B, defective cell-mediated immunity, immunosuppressive therapy, malnutrition, and edema acting as a potential culture medium for organisms.
Serum IgM and IgG levels have prognostic value in patients with nephrotic syndrome and can predict the outcome and the frequency of recurrence of disease. Thre is a close relation between the degree of albuminuria and the levels of serum IgG and M.
Recommendation
To improve the outcome of management of nephrotic syndrome we should:
1. Insist for proper history taking from the patient parents or relatives with proper documentation in the patients’ files .
2. Follow the examination sheet of nephrotic syndrome and write daily progressive notes of the patients.
3. Improve the facilities as needed instruments and materials for renal biopsy and other necessary investigations.
4. We should follow more strictly the guidelines for management of nephrotic syndrome regarding choice of treatment lines and dose and duration of treatment and first line withdrawal mood and duration .
5. We hope in the future that nephrology Unit in our hospital can deal with most cases.