نتایج جستجو برای: congenital nephrotic syndrome
تعداد نتایج: 719259 فیلتر نتایج به سال:
Some cases of nephrotic syndrome in focal and segmental glomerulosclerosis (FSGS) are associated with a circulating factor, the FSGS permeability factor (FSPF). Galactose has a high affinity for FSPF, and experimental data suggest that it could reduce its activity. We describe the case of a 48-year-old male with a nephrotic syndrome found to be resistant to corticosteroids, immunosuppression an...
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...
Congenital nephrotic syndrome (CNS) is an inherited disease that caused by gene mutations and autosomal-recessive mechanism. In most cases, it associated with genetically mediated defects in the components of glomerular filtration barrier, especially nephrine podocytes, a typical histological picture diffuse mesenteric sclerosis or focal-segmental glomerulosclerosis. According to genetic resear...
BACKGROUND Nephrotic syndrome (NS) is pathological condition characterized by heavy proteinuria. Our study investigates hypothesis that change in cell proliferation of proximal tubules influences primary cilia structure and function and promotes cystogenesis in congenital nephrotic syndrome of the Finnish type (CNF) and focal segmental glomerulosclerosis (FSGS). METHODS CNF kidneys were analy...
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