Defects of CRB2 Cause Steroid-Resistant Nephrotic Syndrome
نویسندگان
چکیده
منابع مشابه
Steroid Resistant Nephrotic Syndrome
Minimal Change disease (MCD) is the most common cause of Nephrotic Syndrome (NS) in children accounting for 70 to 90% of cases under the age of 10 years and 50% in older children.In adults MCD is found in 10 to 15% of cases with primary nephrotic syndrome. Most patients with MCD remit with steroids.1 Remission is defined as absence of proteinuria (urine albumin nil or trace on 3 conservative da...
متن کاملChildren with Steroid-resistant Nephrotic Syndrome: a Single-Center Study
Background and Aim: Steroid-resistant nephrotic syndrome (SRNS) accounts for 10%-20% of all cases of idiopathic nephrotic syndrome. These patients are at risk of developing end-stage renal disease. The aim of this study was to determine the demographic characteristics, renal biopsy findings, response to immunosuppressive treatment, and prognosis in pediatric patients with SRNS.Materials and Met...
متن کاملTreatment of steroid-resistant pediatric nephrotic syndrome
Children who suffer from steroid-resistant nephrotic syndrome (SRNS) require aggressive treatment to achieve remission. When intravenous high-dose methylprednisolone fails, calcineurin inhibitors, such as cyclosporine and tacrolimus, are used as the first line of treatment. A significant number of patients with SRNS progress to end-stage renal disease if remission is not achieved. For these chi...
متن کاملSteroid resistant nephrotic syndrome: role of histopathology.
This study was conducted to (1) see the histopathological distribution of different subtypes in steroid resistant nephrotic syndrome (SRNS) and (2) compare the clinical, biochemical parameters and outcome between Minimal Change Disease (MCD) with non-MCD subtypes in response to immunosuppressive therapy. A retrospective analysis was done of data on all biopsy proven children with idiopathic SRN...
متن کاملTreatment of steroid-resistant nephrotic syndrome.
Nephrotic syndrome is the most common glomerular disorder in children, and corticosteroids are the first choice of treatment. While the majority of children respond to corticosteroid therapy, a few do not enter remission after daily therapy for 1-2 months, hence showing steroid-resistance. Most of these children show focal and segmental glomerulosclerosis (FSGS) upon renal biopsy. Steroid resis...
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ژورنال
عنوان ژورنال: The American Journal of Human Genetics
سال: 2015
ISSN: 0002-9297
DOI: 10.1016/j.ajhg.2014.11.014