الفهرس 
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Abstract
Summary
Nephrotic syndrome is one of the most common kidney diseases seen in children. It is a disorder characterized by severe proteinuria , hypoproteinemia , hyperlipidemia and generalized edema resulting from alterations of permeability at the glomerular capillary wall . MCD is by far the most common cause of nephrotic syndrome in children. It accounts for approximately 80-90% of cases.
Endothelin system is represented by three structurally similar endogenous 21-aminoacid peptides named ET-1, ET-2 and ET-3, that activate two G-protein-coupled receptors ETA and ETB and two activating proteases. ET-1 is the predominant isoform of three distinct isopeptides constitutively secreted by endothelial and other vascular cells and the most potent endogenous vasoconstrictor known.
According to recent studies, Significantly higher levels of Endothelin 1 are detected in the serum samples of subjects with SDNS and SRNS than in those with SSNS and controls.The aim of our study to investigate the relationship between endothelin 1 serum level and clinical features of primary nephrotic syndrome in children.
The current study was conducted in the pediatric Nephrology clinic in Beni Suef University Hospital and consists of 46 nephrotic children, aged 1–18years. These children were divided into three groups according to clinical phenotypes. group (A) included 20 steroid sensitive nephrotic syndrome cases (SSNS), group (B) included 12 steroid dependant nephrotic syndrome cases (SDNS) and group (C) included 14 steroid resistant nephrotic syndrome cases (SRNS).
We revided the cases(39) whome done renal biopsy in to 3 groups : MCD (31), FSGS (5) and MesPGN (3) according to renal pathology putting in mind that we considered patients with typical presentation of MCD under group of MCD.
All patients were subjected to the following: Verbal consent of parents, full history taking and thorough physical examination. Laboratory investigations including: Kidney function tests (serum urea, creatinine), serum albumin, serum cholesterol, serum Na-K, urinary protein/creatinine ratio and serum Endothelin 1 level.
As regards to serum Endothelin 1 in our study, we found that serum Endothelin 1 showed significantly higher concentration in SDNS and SRNS groups as compared to control group, (p=0.001,0.008) respectively.
Also there was a highly significant increase in serum endothelin in SDNS and SRNS groups as compared to SSNS group ,(p=0.001,0.048) respectively.
Serum endothelin was highly significant in SDNS group as compared to SRNS group, (p=0.040).
There were no significant differences found between SSNS group and control group as regarding serum endothelin , (P= 0.386).
Also in our study there were no statistically significant differences between different types of biopsies (MCD, MesPGN and FSGS) as regarding serum Endothelin,despite of higher level of serum endothelin among FSGS group than other groups.
In our study there was a significant positive correlation between S.Endothelin and cholesterol (r= 049, p= 0.001) and there was a significant negative correlation between S.Endothelin and serum albumin (r= -0.40, p= 0.006).
Also we found that serum endothelin 1 at a concentration of <4 ng/ml showed the best cutoff value where the sensitivity was 82.6% and the specificity was 75 % calculated by the ROC curve.
Finally, we concluded that, serum Endothelin 1 levels were significantly higher in patients with SDNS and SRNS than in patients with SSNS or in healthy controls.