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Introduction: Nephrotic syndrome (NS) is considered a very common kidney illness that affect children. It is known by leaking of proteins from the blood to the urine by the damaged glomeruli. It is traditionally defined by nephrotic-range proteinuria ( more than forty mg/ m2/hour or urine protein/creatinine ratio more than 200 mg/mL or 3 + protein on urine dipstick), hypoalbuminaemia ( less than 25 g/L) and edema [1]. Children affected by NS can be congenital. It usually present within the first three months of life, and in these pediatric population there is often a genetic mutation affecting either the podocyte or the glomerular basement membrane, though it rarely could be linked with congenital infections, like cytomegalovirus. Apart from the congenital form of nephrotic syndrome, many causes could lead to nephrotic syndrome, involving glomerular disorders, vasculitides, infections, toxins, malignancy, genetic mutations and, most importantly, unknown. Aim of work: In this review, we will discuss nephrotic syndrome. Methodology: We did a systematic search for nephrotic syndrome using PubMed search engine (http://www.ncbi.nlm.nih.gov/) and Google Scholar search engine (https://scholar.google.com). All relevant studies were retrieved and discussed. We only included full articles. Conclusions: Generally speaking, the prognosis for NS is considered very good, with less than five percent that develop rapidly progressing to end-stage renal disease. As mentioned, steroid resistance continues to be the most important risk factor for future development of CKD. Though there are significant adverse effects from chronic use of steroids and steroid-sparing medications, the current management plan are successful in putting the patient into remission across the spectrum of the illness. There continue to be a lot of unanswered questions, including (1) who develop NS and the etiology, (2) what could explain the individual variability in response to different management plan, and (3) what are the specific risk factors that lead to relapse. More research is required to study the causes and duration guideline for calcineurin inhibitors, mycophenolate mofetil and rituximab therapies. Finally, we need to develop new therapies for the management of steroid-resistant disease. Further work is needed to close the gaps in our understanding of the different causes and the diversity of the clinical course of this common and complex childhood disease. Key words: nephrotic syndrome, children, presentation, causes, management. |
