نتایج جستجو برای: congenital nephrotic syndrome
تعداد نتایج: 719259 فیلتر نتایج به سال:
Background: Nephrotic syndrome is a kidney disorder that is identified by signs of nephrosis, severe proteinuria, hypoalbuminemia, and edema. It is a component of glomerulonephrosis, in which different degrees of proteinuria may occur. The complications of this syndrome may include blood clots, infections, and high blood pressure. Essentially, decreased protein through the kidneys (proteinuria)...
PURPOSE Nephrotic syndrome is a common chronic illness encountered during childhood. Infections have been identified as a cause of nephrotic syndrome. The aim of this study was to evaluate the association between enteroviral infection and nephrotic syndrome. METHODS A nationwide retrospective cohort study was conducted by analyzing data from the National Health Insurance Research Database in ...
BACKGROUND AND METHODS The NPHS1gene was analysed in different five Japanese patients with congenital nephrotic syndrome (CNS) from the patients in a previous report (Sako M, Nakanishi K, Obana M et al. Analysis of NPHS1, NPHS2, ACTN4, and WT1 in Japanese patients with congenital nephrotic syndrome. Kidney Int 2005; 67: 1248-1255) that suggested that the mutation of NPHS1 was not a major cause ...
BACKGROUND Nephrotic syndrome represents a condition in pediatric nephrology typified by a relapsing and remitting course, proteinuria and the presence of edema. The PROMIS measures have previously been studied and validated in cross-sectional studies of children with nephrotic syndrome. This study was designed to longitudinally validate the PROMIS measures in pediatric nephrotic syndrome. ME...
Nephrin, an important component of the podocyte filtration slit diaphragm, plays a key role in the maintenance of glomerular permselectivity. Mutations in nephrin lead to proteinuria and congenital nephrotic syndrome. Nephrin undergoes posttranslational modifications in the endoplasmic reticulum (ER) prior to export to the plasma membrane. We examined the effects of human nephrin disease-associ...
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...
CD2-associated protein (CD2AP) is an adapter protein associating with several membrane proteins, including nephrin, mutated in congenital nephrotic syndrome of the Finnish type, and polycystin-2, mutated in type 2 autosomal dominant polycystic kidney disease. Both proteins have critical roles in the maintenance of the integrity of the nephrons. Previous studies have suggested a role for CD2AP i...
N Engl J Med 2006;354:1387-401. Copyright © 2006 Massachusetts Medical Society. The inherited forms of proteinuria comprise a heterogeneous group of rare renal diseases in which glomerular dysfunction and proteinuria are prominent. Despite the rarity of hereditary proteinuria syndromes, genetic, biochemical, and structural studies of these diseases have made important contributions to our knowl...
There is an urgent need to develop alternative to kidney biopsy in children to diagnose nephrotic syndrome. Increased apoptosis plays a central role in the development of nephrotic syndrome. The aim of this study was to evaluate the blood levels of two markers of apoptosis, Fas-ligand (Fas-l) and granzyme-b in children with nephrotic syndrome. Thirty children with biopsy-proven nephrotic syndro...
The effect and outcome of pregnancy in patients with chronic idiopathic glomerulonephritis was studied. The study population consisted of 98 patients. All patients were divided into 8 groups according to presence, absence or coexistence of 3 general risk factors such as hypertension, renal failure and nephrotic syndrome. The adverse effect of renal disease and risk factors on the outcome of pre...
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