Steroid-resistant nephrotic syndrome: impact of genetic testing
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 کاملThe Genetic Basis of Steroid-resistant Nephrotic Syndrome
Nephrotic syndrome (NS) is characterized by massive proteinuria (over 40 mg/m2/day) leading to hypoalbuminemia and edema. The prevalence in the general population is estimated to be 16/100,000 children (1). Whereas most children with NS would achieve remission within 4 weeks of daily steroid therapy, 10-20% remain steroid-resistant (SRNS). At least 50% of patients with SRNS, presenting in child...
متن کاملClinical utility of genetic testing in children and adults with steroid-resistant nephrotic syndrome.
BACKGROUND AND OBJECTIVES The increasing number of podocyte-expressed genes implicated in steroid-resistant nephrotic syndrome (SRNS), the phenotypic variability, and the uncharacterized relative frequency of mutations in these genes in pediatric and adult patients with SRNS complicate their routine genetic analysis. Our aim was to compile the clinical and genetic data of eight podocyte genes a...
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...
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ژورنال
عنوان ژورنال: Annals of Saudi Medicine
سال: 2013
ISSN: 0256-4947,0975-4466
DOI: 10.5144/0256-4947.2013.533