nephrotic syndrome

Several case reports suggest that rituximab (RTX) could be effective in steroid-dependent nephrotic syndrome, but RTX efficacy has not yet been studied in a series of patients...

The aim of this study was to establish the efficacy and safety of rituximab in refractory nephrotic syndrome (NS)...

..Patients with Denys-Drash syndrome present with early nephrotic syndrome with diffuse mesangial sclerosis progressing rapidly to end-stage renal failure, male pseudohermaphroditism, ..

..findings, treatment responses and progression to end-stage renal disease in childhood idiopathic nephrotic syndrome (NS). 227 children diagnosed with idiopathic NS were included in the study...

..or sialidase-treated rats, which show a dramatic loss of foot processes, comparable to that seen in the nephrotic syndrome. Thus NHERF2 appears to function as a scaffold protein linking PC to ezrin and the actin cytoskeleton...

The response to steroid therapy is used to characterize the idiopathic nephrotic syndrome (INS) of childhood as either steroid-sensitive (SSNS) or steroid-resistant (SRNS), a classification with a better prognostic capability than renal ..

To examine ocular findings in Japanese children with nephrotic syndrome who receive prolonged corticosteroid treatment.

A 16-year-old patient with steroid-dependent nephrotic syndrome with more than 35 relapses developed severe relapsing idiopathic thrombocytopenic purpura (ITP)...

..childhood conditions, this study aimed to identify recent time trends and ethnic patterns of childhood nephrotic syndrome. A population-based cohort of children (0-15 years) diagnosed according to strict criteria with nephrotic ..

..genotypes of Turkish children with primary focal segmental glomerulosclerosis (FSGS) and steroid-sensitive nephrotic syndrome (SSNS)...

..D) allele in the angiotensin-converting enzyme (ACE) gene has been reported as a probable genetic risk factor for idiopathic nephrotic syndrome (INS), but its role in determining resistance to steroid therapy remains to be evaluated.

Ethnicity and age play important roles in the epidemiology of idiopathic nephrotic syndrome (INS) in children...

Idiopathic nephrotic syndrome is a common renal disease in children. ACE gene insertion/deletion (I/D) polymorphism has been studied as a predictor of clinical response to steroid therapy.

Several genes have been implicated in genetic forms of nephrotic syndrome occurring in children...

..None of the patients treated with rituximab achieved remission in proteinuria, and one patient experienced early graft loss. In these four adult renal transplant patients with recurrent FSGS, rituximab failed to diminish proteinuria...

Circulating permeability factors may be important in idiopathic nephrotic syndrome (INS) including focal segmental glomerulosclerosis (FSGS) and in recurrence after renal transplantation...

Hypercholesterolemia is a consistent feature of the nephrotic syndrome. However, the mechanisms underlying this perturbation are unclear...

..We have investigated the association of ACE gene I/D polymorphism with the clinical presentation of idiopathic nephrotic syndrome (INS) in Kuwaiti children.

..from a consanguineous marriage were progressive myoclonic epilepsy without intellectual impairment and a nephrotic syndrome with a strong accumulation of C1q in capillary loops and mesangium of kidney...

..Because his original disease was nephrotic syndrome (focal segmental glomerulosclerosis, or FSGS) and electron microscopic examination of the renal biopsy ..

Congenital nephrotic syndrome of the Finnish type (NPHS1) is an autosomal-recessive disorder, characterized by massive proteinuria in utero and nephrosis at birth...

Long-term outcome of idiopathic steroid-resistant nephrotic syndrome was retrospectively studied in 78 children in eight centers for the past 20 years. Median age at onset was 4.4 years (1.1-15.0 years) and the gender ratio was 1.4...

Acute renal failure (ARF) is an uncommon but alarming complication of idiopathic nephrotic syndrome. The renal failure could be secondary to causes evident from the history and evaluation, such as severe intravascular volume depletion, ..

..the frequency and severity of the behavioral effects of high-dose oral steroid therapy in children with nephrotic syndrome. We conducted a prospective assessment of the behavior of 12 children using a standardized psychological ..

..This study examines three girls with steroid-resistant nephrotic syndrome related to mutations in the WT1 gene, but with normal 46, XX karyotype and normal female phenotype...

..at the slit diaphragm and expressed by the podocyte, were found to be causative in congenital/familial nephrotic syndrome (NS), but their role in acquired NS remains unclear...

..3, 3, and 3.5 g/dl; and urinary protein excretion 3, 8, and 1.5 g/day, respectively. Treatment with azathioprine in addition to colchicine could ameliorate the nephrotic syndrome and control the attacks very effectively in these cases.

The aim of the present study was to estimate the total antioxidant status (TAS) and main antioxidant enzyme activity in children with nephrotic syndrome (NS).

Minimal-change disease (MCD) is the most common cause of nephrotic syndrome (NS) and is characterized only by minor morphological alterations in podocytes...

Rituximab has been used over the last decade as a rescue therapy for refractory cases of nephrotic syndrome (NS)...

We describe an uncommon pediatric finding of unilateral renal artery stenosis, which presented as nephrotic syndrome, hypertension, failure to thrive, and hyponatremia...

Idiopathic NS (nephrotic syndrome) is characterized by massive proteinuria, due to a leak in the glomerular barrier to proteins...

We describe a 68-year-old man with necrotizing glomerulonephritis who presented with nephrotic syndrome accompanied by pulmonary cryptococcosis. He developed rheumatoid arthritis in July 1999 and was treated with low-dose prednisolone...

Cyclosporine A (CsA) has been widely used in children with steroid dependent and steroid resistant nephrotic syndrome (NS) because of its efficacy in relieving these patients from systemic side effects of steroids...

To determine the histological picture and outcome of treatment in cases of childhood nephrotic syndrome who needed renal biopsy.

b>Nephrotic syndrome is a recurrent or chronic disorder characterized by heavy proteinuria, hypoalbuminemia and edema...

..Herein, we describe a patient with severe relapse of nephrotic syndrome associated with bacteremic B. holmesii pneumonia.

The Nephrotic Syndrome is still a therapeutic and physiopathological challenge. The clinical response to systemic immunosuppression shows that an immunoinflammatory disorder supports the nephrotic syndrome...

Steroid-resistant nephrotic syndrome (SRNS) is a frequent cause of end-stage renal failure...

..6 vs 5.7 vs 8.5 years; P=0.01), also observed in the non-FSGS children (2.2 vs 3.9 vs 4.9 years; P=0.02). In contradistinction to non-FSGS children, there was a marked increase in steroid resistance with FSGS (43% vs 62% vs 86%; P=0.03)...

To determine the sequential therapy of childhood nephrotic syndrome (NS) with presumed minimal change nephropathy using the evidence from clinical trials.

Idiopathic nephrotic syndrome of childhood is thought to be associated with T lymphocyte dysfunction often triggered by viral infections, with the production of circulating factor(s) resulting in proteinuria...

..This review provides an update of podocyte functions with respect to novel podocyte-specific proteins and also focuses on the dynamic interaction between the actin cytoskeleton of podocytes, their cell surface receptors and the GBM...

Children with steroid-resistant nephrotic syndrome (SRNS) are at risk of developing renal failure...

Congenital nephrotic syndrome of the Finnish type (CNF or NPHS1) is an autosomal recessive kidney disorder resulting in severe proteinurea and renal dysfunction...

A case of infantile nephrotic syndrome (NS) with advanced membranoproliferative glomerulonephritis (MPGN), type I, and bilateral congenital glaucoma, is presented...

Some cases of nephrotic syndrome in focal and segmental glomerulosclerosis (FSGS) are associated with a circulating factor, the FSGS permeability factor (FSPF)...

Based on observations in 110 children with nephrotic syndrome (NS) and data from the literature, existing concepts on the pathogenesis of edema formation in the NS have been modified...

..would effectively induce a more rapid remission in young children with idiopathic steroid-resistant nephrotic syndrome (SRNS). A retrospective analysis was conducted of 11 consecutive SRNS patients (mean age 3.6 +/- 1...

Steroid resistant nephrotic syndrome (SRNS) remains a challenge facing pediatric nephrologists. The underlying histopathology usually affects the course of the disease and the response to treatment...

Steroid-resistant nephrotic syndrome (SRNS) is uncommon in children, but often leads to ESRD. We report our experience with SRNS and its treatments and outcomes.

The therapeutic management of pediatric idiopathic nephrotic syndrome is still a challenge due to the large number of potentially effective pharmacological alternatives and the insufficient scientific evidence available...

Achieving remission in children with steroid-resistant nephrotic syndrome (SRNS) could be difficult. Many immunosuppressive drugs are used with variable success rates...

The mechanism responsible for proteinuria in non-genetic idiopathic nephrotic syndrome (iNS) is unknown. Animal models suggest an effect of free radicals on podocytes, and indirect evidence in humans confirm this implication...

Mutations in NPHS1, which encodes nephrin, are the main causes of congenital nephrotic syndrome (CNS) in Finnish patients, whereas mutations in NPHS2, which encodes podocin, are typically responsible for childhood-onset steroid-resistant ..

INTRODUCTION. Childhood idiopathic nephrotic syndrome is characterized by frequent relapsing courses or steroid dependency. Levamisole is a popular drug for treatment of these patients...

Minimal change nephrotic syndrome (MCNS) in children is frequently associated with allergy and immunoglobulin E production...

..the changes in serum insulin like growth factor-I (IGF-I) and IGF binding proteins (IGFBPs) in children with nephrotic syndrome (NS) and the effect of glucocorticoid on serum IGF-I and IGFBPs...

In recent years, it has been proposed that nephrotic syndrome is a consequence of an imbalance between oxidant and anti-oxidant activity...

The association of malignancy with nephrotic syndrome and renal histopathologic abnormalities is well documented. We report a case of intravascular lymphomatosis (IVL) presenting as nephrotic syndrome which was diagnosed by renal biopsy...

We report on the isolation of a factor secreted by peripheral blood mononuclear cells from patients with idiopathic minimal lesion nephrotic syndrome (IMLNS) in relapse and its effect on proteinuria and podocyte morphology in the rat.

Cyclosporine (CsA) has been used in steroid-resistant idiopathic nephrotic syndrome (INS) in many previous studies.

To assess the effect of cyclosporine A (CyA) on the level of vascular endothelial growth factor (VEGF) in the plasma and urine of nephrotic syndrome children.

Children with nephrotic syndrome (NS) are usually treated with long-term low dose alternate day prednisolone with or without glucocorticoid sparing therapy, such as levamisole or ciclosporin, to maintain remission...

..However, the efficacy of this regimen in children with idiopathic frequent-relapsing nephrotic syndrome (NS) remains controversial.

Children with frequently relapsing nephrotic syndrome (FRNS) often develop adverse effects from prednisone. Attempts to induce long-term remission in such patients have had varying levels of success...

..Eight children presented with nephrotic syndrome, while one had nephrotic proteinuria and renal insufficiency that progressed to end-stage renal failure...

..drug than the conventional twice daily administration dosing used for the treatment of childhood idiopathic nephrotic syndrome (INS)...

Recurrence of nephrotic syndrome after renal transplantation leads to graft loss within 1 year in 50-80% of patients who do not receive any specific treatment...

..The study group comprised of 22 consecutive children with steroid-resistant nephrotic syndrome (SRNS) who were studied prospectively. TAC was initiated with a dose of 0...

Cyclosporin A (CsA) is an effective treatment for frequently relapsing steroid-dependent nephrotic syndrome (FR-SDNS), but its use can be complicated by renal toxicity and a high incidence of relapses after withdrawal...

..The correlation between the age of lupus onset, impaired renal function at the beginning of the disease, anemia, proteinuria, and poor renal outcome was not found...

Idiopathic nephrotic syndrome (iNS) with resistance or dependence to steroids is a common disease in children but in spite of an increasing clinical impact its pathogenesis is unknown...

We investigated the efficacy of oral mizoribine pulse therapy (mizoribine-pulse) for cyclosporin (CyA)-dependent, steroid-resistant nephrotic syndrome (SRNS) and frequently relapsing, steroid-dependent nephrotic syndrome (FR-SDNS).

..efficacy of cyclosporin A (CyA) for treating severe Henoch-Schönlein nephritis (HSN), seven patients with nephrotic syndrome, aged 3.9-13.8 years (mean 6.5 years), were analyzed retrospectively. Mean follow-up times were 5...

We report a Caucasian boy of Italian descent with congenital nephrotic syndrome of the Finnish type (NPHS1, CNF, MIM 256300) who developed recurrence of proteinuria and hypoalbuminemia on the seventh post-operative day following living ..

Previous studies of the Arbeitsgemeinschaft für Pädiatrische Nephrologie in children with steroid-sensitive nephrotic syndrome have shown that the length of initial prednisone therapy has an impact on the subsequent relapse rate...

..study was to assess the serum concentration of high-sensitivity C-reactive protein (hs-CRP) in children with nephrotic syndrome (NS) treated with prednisone and cyclosporine A (CyA)...

Cyclosporine A (CsA) has relieved children with steroid-dependent nephrotic syndrome (NS) from steroid toxicity...

BACKGROUND AND PURPOSE: Steroid treatment is a mainstay in the management of nephrotic syndrome, but has the potential to impair growth and development of children...

Given the variable response of steroid-resistant nephrotic syndrome (SRNS) to treatment with cyclosporin (CsA), it may be inappropriate to expose all SR patients to additional immunosuppression...

..single dose of oral mizoribine (MZB) pulse therapy two times per week for children with frequently relapsing nephrotic syndrome (FRNS)...

..4+/-42.4 months. The mean follow-up duration was 98.2+/-63.3 months. Eighty-five patients presented with nephrotic syndrome and seven presented with asymptomatic proteinuria...

Idiopathic membranous nephropathy (MN) is a rare cause of asymptomatic proteinuria (AP) or nephrotic syndrome (NS) in childhood...

..The aim of our present research was to determine FBP-1 activity in urine as an indicator of damage to renal proximal tubules in children with idiopathic nephrotic syndrome (INS).

To evaluate the efficacy of FK506 in the treatment of children with nephrotic syndrome of different underlying pathology.

To evaluate the efficacy of oral mizoribine (MZB) pulse therapy given twice a week for frequently relapsing steroid-dependent nephrotic syndrome (FR-SDNS).

The variation in time required to obtain cessation of proteinuria in children with nephrotic syndrome (NS) represents one aspect of the variations shown by these children in response to glucocorticoid (GC) treatment...

..This study aimed to compare these therapies and renal outcome over a long-term period for MPGN...

A retrospective study was made on 37 children with idiopathic nephrotic syndrome (INS). At the beginning, all patients were steroid sensitive but received more than one steroid course (median 4)...

To investigate the genetic polymorphism of angiotensin-converting enzyme (ACE) insertion/deletion (I/D) in children with idiopathic nephrotic syndrome (INS), as well as its relationship with patient's clinical response to steroid therapy.

..Three children (2 years, 3 years, and 6 years of age) with secondary steroid-resistant nephrotic syndrome (NS) were included, as they remained resistant to 30 days of treatment with prednisone (60 mg/m(2) per day), ..

To determine the efficacy of three different treatment protocols in steroid resistant idiopathic nephrotic syndrome, (SRINS).

Membranous nephropathy is the most common cause of idiopathic nephrotic syndrome in adults. The frequency of secondary forms varies from 20 to 30 per cent...

Cyclosporin (Cs-A) is an effective treatment for difficult cases of nephrotic syndrome (NS), but its use can be complicated by renal toxicity and a high incidence of relapses after withdrawal...

..METHODS: Here we studied kidneys from patients with congenital nephrotic syndrome of the Finnish type (CNF), a human model disease of isolated proteinuria...

BACKGROUND: Many children with steroid-sensitive nephrotic syndrome (SSNS) relapse frequently and receive immunosuppressive agents...

..Kidney Int 58:1219-1227, 2000. Fogo A: Nephrotic syndrome: molecular and genetic basis. Nephron 85:8-13, 2000...

..misfolded protein disorders such as nephrogenic diabetes insipidus, primary hyperoxaluria type 1, congenital nephrotic syndrome, and other lysosomal storage diseases such as Gaucher disease and Fabry disease...

..Project Narrative: Relevance. Diseases resulting in the nephrotic syndrome such as diabetes mellitus and other diseases of the glomerulus frequently cause progressive kidney damage ..

..Project Narrative: Relevance. Diseases resulting in the nephrotic syndrome such as diabetes mellitus and other diseases of the glomerulus frequently cause progressive kidney damage ..

..Project Narrative: Relevance. Diseases resulting in the nephrotic syndrome such as diabetes mellitus and other diseases of the glomerulus frequently cause progressive kidney damage ..

DESCRIPTION: States of pathological sodium retention and edema formation such as congestive heart failure, nephrotic syndrome, and cirrhosis of the liver are characterized by renal resistance to the natriuretic action of atrial ..

..Null mutations cause Pierson syndrome (congenital nephrotic syndrome with ocular and nervous system abnormalities), whereas some missense mutations cause isolated congenital ..

..Null mutations cause Pierson syndrome (congenital nephrotic syndrome with ocular and nervous system abnormalities), whereas some missense mutations cause isolated congenital ..

..Null mutations cause Pierson syndrome (congenital nephrotic syndrome with ocular and nervous system abnormalities), whereas some missense mutations cause isolated congenital ..

b>Nephrotic syndrome (NS) is one of the most common kidney diseases seen in children...

..the kidney and subsequently abate to low or undetectable levels when patients first present with full-blown nephrotic syndrome is supported by similar observation in Heymann nephritis, a rat model of MGN...

..Significant progress has been made to identify the genetic causes of familial forms of renal disease (nephrotic syndrome). However, no genetic variants have been found to be causally linked to common forms of renal disease...

..Significant progress has been made to identify the genetic causes of familial forms of renal disease (nephrotic syndrome). However, no genetic variants have been found to be causally linked to common forms of renal disease...

..COX2 inhibitors in the kidney have been demonstrated to decrease proteinuria in subjects with refractory nephrotic syndrome. In this study, we propose to extend the animal and human data suggesting a potential beneficial effect of ..

..We have made the novel finding that cathepsin L enzyme is present within the cytoplasm of podocytes during nephrotic syndrome and cleaves CD2AP, an important SD protein...

..We have made the novel finding that cathepsin L enzyme is present within the cytoplasm of podocytes during nephrotic syndrome and cleaves CD2AP, an important SD protein...

..Childhood nephrotic syndrome (NS) usually responds to GC and remains in remission as long as high-dose GC therapy is continued...

..We have made the novel finding that cathepsin L enzyme is present within the cytoplasm of podocytes during nephrotic syndrome and cleaves CD2AP, an important SD protein...