Permeability factors in focal segmental glomerulosclerosis
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Glomerulosclerosis
In 37 patients of nephrotic syndrome, serum protein levels, protein fractions and urinary levels of proteins and their fractions were determined. The findings of serum levels of proteins and their fractions were compared with an equal number of age and sex matched controls. Twenty three patients showed selective and 14 non-selective proteinuria. Most of the patients with selective proteinuria showed good response to steroids therapy while those with non-selective proteinuria did not respond.
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Objective To investigate the relationship between serum IgG and proteinuria in children with nephrotic syndrome.Methods Biostatistics was used to study the relationship between the serum level of IgG and the quantitative changes of urine protein in 46 pediatric patients with simple primary nephrotic syndrome which had not been treated with steroid therapy.Results No linear regression,nor correlation was found between the serum IgG and the quantity of urine protein(P0.05).Conclusion Although lowered IgG level may be seen in some nephrotic children,the present investigation revealed no linear regression or correlation betweem serum IgG and proteinuria,suggesting that heavy proteinuria may not be the main cause of the lowering of serum IgG in children with nephrotic syndrome.
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Nephrotic syndrome is one of the most common childhood kidney diseases. It is mostly found in the age group of 2 to 8 years. Around 10%–15% of nephrotic syndrome cases are non-responders of steroid treatment (SRNS). Angiotensin converting enzyme (ACE) (I/D) gene association studies are important for detecting kidney disease and herein we assessed the association of ACE (I/D) polymorphism with nephrotic syndrome in South Indian children. We recruited 260 nephrotic syndrome (162 boys and 98 girls) and 218 (140 boys and 78 girls) control subjects. ACE I/D polymorphism was analyzed by PCR using genotype allele specific primers. In ACE (I/D), we did not find significant association for the ungrouped data of nephrotic syndrome children and the control subjects. Kidney biopsies were done in 86 nephrotic syndrome cases (minimal change disease, n = 51; focal segmental glomerulosclerosis, n = 27; diffuse mesangial proliferation, n = 8). We segregated them into the minimal change disease / focal segmental glomerulosclerosis groups and observed that the ACE 'D' allele was identified with borderline significance in cases of focal segmental glomerulosclerosis and the 'Ⅰ' allele was assessed as having very weak association in cases of minimal change disease. 'Ⅱ' genotype was weakly associated with minimal change disease. Gender specific analysis revealed weak association of 'ID' genotype with female nephrotic syndrome in females. Dominant expression of DD genotype was observed in males with nephrotic syndrome. Our finding indicated that ACE (I/D) has moderate association with focal segmental glomerulosclerosis. However, due to the limited number of biopsy proven focal segmental glomerulosclerosis subjects enrolled, further studies are required to confirm these results.
Gene polymorphism
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Background and aim of work: Idiopathic nephrotic syndrome (INS) is a common and important chronic kidney disease in childhood. Steroid response pattern and the underlying histopathological spectrum of INS show variabilities in the different geographical regions. This work aimed to highlight the clinical profile, steroid response pattern and histopathological spectrum of INS in our locality in Sohag, Upper Egypt. Patients and Methods: A prospective study included 77 children with INS aged from 1-15 years followed for at least one year, diagnosed and treated at our pediatric nephrology unit in the period from January 2010 to December 2014 was done.The patients' demographic features, clinical profile, steroid response pattern and the underlying histopathological spectrum were studied. Results: Total included patients were 77 children. The mean age at disease onset was 4.87±2.94 (range 1.5-15) years. Male/female ratio was 1.6/1. Steroid was given to all patients, 79.2% of patients had steroid sensitive nephrotic syndrome (SSNS), while 20.8% had steroid resistant nephrotic syndrome (SRNS). Out of 61 steroid sensitive patients, about 54% developed either steroid dependent nephrotic syndrome (SDNS) or frequently relapsing nephrotic syndrome (FRNS). Biopsy was done in 33.8% of patients and revealed focal segmental glomerulosclerosis (FSGS) in 42%, minimal change disease (MCD) in 39% and mesangio-proliferative glomerulonephritis (MesPGN) in 19% of patients. End stage renal disease (ESRD) developed in 1 patient with SRNS and FSGS. Conclusion: About eighty percent of our patients were steroid sensitive but more than half of them had SDNS or FRNS. The main histopathological pattern seen in renal biopsies was FSGS
Minimal change disease
Nephrology
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Minimal change disease
Membranous Nephropathy
Nephrology
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Focal segmental glomerulosclerosis (FSGS) is a glomerular disease defined by a characteristic histologic pattern that occurs either as a primary kidney disease (primary FSGS) or as a result of a systemic illness (secondary FSGS). Proteinuria, often in the nephrotic range, is the hallmark of FSGS. The occurrence of nephrotic syndrome after an insect sting is rarely reported in the literature. We present a case of nephrotic syndrome with focal segmental glomerulosclerosis with a glomerular tip lesion developing after an insect bite.A 51-year-old Caucasian female was bitten by an insect on her left leg, which immediately became swollen. Generalized edema developed and she was admitted for further investigations. Urinary 24-h protein excretion was 7 g. Percutaneous renal biopsy was performed and showed focal segmental glomerulosclerosis of the tip variant. Nephrotic syndrome was steroid-resistant, and when we added cyclophosphamide for 8 weeks complete remission was achieved. There was no relapse of the disease during the 2-month follow-up.This report demonstrates the useful role of cyclophosphamide in the treatment of steroid-resistant nephrotic syndrome due to FSGS with glomerular tip lesion. A causal relationship between the insect bite and the nephrotic syndrome is suggested and an immune response could be responsible for the nephrotic syndrome.
Minimal change disease
Glomerulosclerosis
Nephrosis
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Methods for detection of proteinuria and its causes are reviewed. Recommendations are given concerning the diagnostic procedures after detection of pathological proteinuria. The nephrotic syndrome is discussed mainly under the aspect of predictability of the histo-pathological form of the glomerular lesions from clinical and biochemical parameters.
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The treatment of primary nephrotic syndrome such as minimal change nephropathy, membranous nephropathy, and focal segmental glomerulosclerosis nephropathy remains challenging. Whilst most cases of idiopathic nephrotic syndrome respond to steroid therapy and experience a limited number of relapses prior to complete remission, some cases suffer from frequent relapses and become steroid dependent or are primarily steroid resistant. Treatment options are limited to immunosuppressive drugs with significant side effect profiles. New modalities targeting novel pathways in the pathogenesis of nephrotic syndrome are actively sought. Here we report the case of a patient with steroid dependent focal segmental glomerulosclerosis (FSGS) nephrotic syndrome with a favourable response to a novel proteasome inhibitor saquinavir.
Membranous Nephropathy
Minimal change disease
Pathogenesis
Glomerulosclerosis
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In the light of the theory stated in Canon of Internal Medicine and other related theories expounded by specialists of past ages, this article proposes that proteinuria has an analogy with the refined nutritious substance leaking from the spleen and kidney in traditional Chinese medicine. The pathological change, zheng-differentiation and treatment of proteinuria in nephrotic syndrome are also discussed. Through years of his clinical experience, the author recommends some kinds of herbal medicines effective for treating proteinuria. This article, therefore, provides not only a theoretical basis, but also clinical experience for the treatment of proteinuria in nephrotic syndrome.
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The mechanism of proteinuria in nephrotic syndrome is unknown. The plasma behaviour of platelet-activating factor (ng/ml) in nephrotic and normal people has been evaluated.A total of 21 patients with nephrotic proteinuria due to glomerular pathology and 20 subjects as normal control people have been studied. Plasma PAF level is evaluated by ((125)I) RIA Kit (Du Pont NEN).Patients with glomerular proteinuria appeared to have a significant increase (p<0.05) plasma PAF bioactivity: 116.28+/-49.6 ng/ml versus 41.4+/-14.9 ng/ml of normal subjects.The study shows that PAF may be involved in the mechanism of genesis of human glomerular proteinuria.
Platelet-activating factor
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