نتایج جستجو برای: Steroid-resistant nephrotic syndrome
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corticosteroid resistant and dependent nephrotic syndrome in children is a challenge and there are some difficulties in treating such patients. we reviewed the current studies that evaluated therapeutic role of a relatively new immunosuppressive drug “rituximab” in reducing proteinuria and reduction of relapse rate in less than 16 year old patients with non-responsive or steroid dependent nephr...
Thromboembolism is a rare complication of primary nephortic syndrome. Both venous and arterial thrombosis might occur in steroid responsive and steroid resistant nephrotic syndrome. This is the report of an infant with nephrotic syndrome and renovascular hypertension, complicated with asymptomatic intracardiac thrombus and managed appropriately with medical treatment.
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...
BACKGROUND AND OBJECTIVES Steroid-resistant nephrotic syndrome overwhelmingly progresses to ESRD. More than 30 monogenic genes have been identified to cause steroid-resistant nephrotic syndrome. We previously detected causative mutations using targeted panel sequencing in 30% of patients with steroid-resistant nephrotic syndrome. Panel sequencing has a number of limitations when compared with w...
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...
Human nephrotic syndrome has been related to mutations in glomerular proteins. Mutations in the NPHS2 gene that encodes podocin have been described as responsible for steroid-resistant nephrotic syndrome. It has been advised to test for NPHS2 mutations in parallel or before giving steroid treatment in nephrotic syndrome patients in order to avoid unnecessary therapy. We identified NPHS2 mutatio...
Background It is important to find a predictor for the course of Idiopathic Nephrotic Syndrome for better planning of treatment. Renal cortical echogenicity in ultrasonography has been postulated as a marker for the course of renal disease. The present study aimed at evaluating the relationship between renal cortical echogenicity and the course...
Of 231 children with nephrotic syndrome, 87% were steroid sensitive and 13% steroid resistant. Of patients with steroid sensitive nephrotic syndrome, 38.8% were non-relapsers, 34.8% frequent relapsers and 26.4% infrequent relapsers. Among those with steroid resistant nephrotic syndrome, 37.5% had focal segmental glomerulosclerosis with a high mortality rate of 44%
Mutations in the Wilms' tumor gene, WT1, can lead to syndromic steroid-resistant nephrotic syndrome and isolated steroid-resistant nephrotic syndrome. WT1 mutations have been identified in the majority of children with Denys-Drash or Frasier syndrome. WT1 mutations have not previously been identified in boys with sporadic isolated steroid-resistant nephrotic syndrome, but, recently, four boys w...
Schimke immune-osseous dysplasia (SIOD) is a rare autosomal recessive disorder presented with specific facial features, skeletal dysplasia, steroid resistance nephrotic syndrome (SRNS) and cellular immune insufficiency. This is a SIOD case reported from Iran. He was 5 years old boy when evaluated for proteinuria and short stature. In appearance, we detected hyperpigmented macules, kyphosc...
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