WCN23-0155 Genetic Screening of the Childhood and Adolescence-onset of Steroid Resistant Nephrotic Syndrome
نویسندگان
چکیده
Steroid-resistant nephrotic syndrome (SRNS) is a clinically and genetically heterogeneous disorder caused by either genetic or immunological factors their combination. Approximately 30-40% of patients with SRNS make fast progression to end-stage renal disease (ESRD). represents the second leading cause ESRD in individuals under age 25 years worldwide. Patients typically exhibit focal segmental glomerulosclerosis (FSGS), characterized histological pattern injury affecting minority (focal) portion (segmental) glomeruli.
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 کاملRapid detection of monogenic causes of childhood-onset steroid-resistant nephrotic syndrome.
BACKGROUND AND OBJECTIVES In steroid-resistant nephrotic syndrome (SRNS), >21 single-gene causes are known. However, mutation analysis of all known SRNS genes is time and cost intensive. This report describes a new high-throughput method of mutation analysis using a PCR-based microfluidic technology that allows rapid simultaneous mutation analysis of 21 single-gene causes of SRNS in a large num...
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 کاملChildhood Idiopathic Steroid Resistant Nephrotic Syndrome, Different Drugs And Outcome.
BACKGROUND The management of steroid resistant nephrotic syndrome (SRNS) is quite difficult in paediatric patients. Not only the remission is difficult but also these patients are at risk of progression to end stage renal disease (ESRD). The goal of treatment is either to achieve complete remission or even partial remission as it is the most important predictor of disease outcome. METHODS Thi...
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...
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ژورنال
عنوان ژورنال: Kidney International Reports
سال: 2023
ISSN: ['2468-0249']
DOI: https://doi.org/10.1016/j.ekir.2023.02.544