Management of Idiopathic Nephrotic Syndrome in Adults: Minimal Change Disease and Focal Segmental Glomerulosclerosis

2009 ◽  
pp. 147-157
Author(s):  
Alain Meyrier
Keyword(s):  
Nephrotic Syndrome ◽  
Focal Segmental Glomerulosclerosis ◽  
Minimal Change Disease ◽  
Idiopathic Nephrotic Syndrome ◽  
Minimal Change ◽  
Segmental Glomerulosclerosis

scholarly journals Impact of Τh1 and Τh2 cytokines in the progression of idiopathic nephrotic syndrome due to focal segmental glomerulosclerosis and minimal change disease

2016 ◽  
Vol 6 (3) ◽  
pp. 187-195 ◽  
Author(s):  
Maria Stangou ◽  
Μichael Spartalis ◽  
Dimitra-Vasilia Daikidou ◽  
Theodora Kouloukourgiotou ◽  
Erasmia Sampani ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Focal Segmental Glomerulosclerosis ◽  
Minimal Change Disease ◽  
Idiopathic Nephrotic Syndrome ◽  
Minimal Change ◽  
Segmental Glomerulosclerosis

scholarly journals Clinical manifestations of focal segmental glomerulosclerosis in Japan from the Japan Renal Biopsy Registry: age stratification and comparison with minimal change disease

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Takaya Ozeki ◽  
Shoichi Maruyama ◽  
Toshiyuki Imasawa ◽  
Takehiko Kawaguchi ◽  
Hiroshi Kitamura ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Renal Biopsy ◽  
Focal Segmental Glomerulosclerosis ◽  
Clinical Diagnosis ◽  
Clinical Characteristics ◽  
Minimal Change Disease ◽  
Multivariable Analysis ◽  
Laboratory Data ◽  
Minimal Change ◽  
Segmental Glomerulosclerosis

AbstractFocal segmental glomerulosclerosis (FSGS) is a serious condition leading to kidney failure. We aimed to investigate the clinical characteristics of FSGS and its differences compared with minimal change disease (MCD) using cross-sectional data from the Japan Renal Biopsy Registry. In Analysis 1, primary FSGS (n = 996) were stratified by age into three groups: pediatric (< 18 years), adult (18–64 years), and elderly (≥ 65 years), and clinical characteristics were compared. Clinical diagnosis of nephrotic syndrome (NS) was given to 73.5% (97/132) of the pediatric, 41.2% (256/622) of the adult, and 65.7% (159/242) of the elderly group. In Analysis 2, primary FSGS (n = 306) and MCD (n = 1303) whose clinical diagnosis was nephrotic syndrome (NS) and laboratory data were consistent with NS, were enrolled. Logistic regression analysis was conducted to elucidate the variables which can distinguish FSGS from MCD. On multivariable analysis, higher systolic blood pressure, higher serum albumin, lower eGFR, and presence of hematuria associated with FSGS. In Japanese nationwide registry, primary FSGS patients aged 18–64 years showed lower rate of NS than those in other ages. Among primary nephrotic cases, FSGS showed distinct clinical features from MCD.

scholarly journals RENAL BIOPSY IN PAEDIATRIC POPULATION

1969 ◽  
Vol 3 (2) ◽  
pp. 314-317
Author(s):  
AHMAD ZEB KHAN ◽  
RIAZ GUL ◽  
AZIZ AHMAD
Keyword(s):  
Nephrotic Syndrome ◽  
Renal Biopsy ◽  
Focal Segmental Glomerulosclerosis ◽  
Minimal Change Disease ◽  
Paediatric Population ◽  
Minimal Change ◽  
Segmental Glomerulosclerosis ◽  
Renal Biopsies ◽  
A Prospective Study ◽  
Nephrotic Range Proteinuria

OBJECTIVE: To find out the pattern of glomerulopathies in paediatric population, undergoing renalbiopsy at Khyber Teaching Hospital, Peshawar.METHODS: This was a prospective study carried out at the department of Nephrology at Khyber TeachingHospital, Peshawar from June 2010 till June 2012. Ultrasound guided percutaneous renal biopsies werecarried out in patients with the finding of; 1 ) Nephrotic range proteinuria in children. 2) Non-Nephroticrange proteinuria with evidence of hypertension / haematuria / deranged renal function or active sedimentson urine microscopy. 3) Steroid resistant nephrotic syndrome in children (patients not responding to steroidin eight weeks time) and 4) Children with nephrotic syndrome who were not tolerant of steroid therapy orwere considered for immunosuppressive drugs.RESULT: A total of 155 renal biopsies were done. Out of these 90 were male patients and 65 were females.The most common histopathological lesion among children population was minimal change disease(42.66%) followed by focal segmental glomerulosclerosis (25.33%) and membranous GN (16.0%). Weobserved that nephrotic range proteinuria was most prevalent in minimal change disease and membranousGN followed by focal segmental glomerulosclerosis. While non-nephrotic range proteinuria was mostlyseen in patients with membranoprolifirative GN.CONCLUSION: In paediatric population, minimal change disease is the most common encounteredglomerulopathy, followed by focal segmental glomerulosclerosisand membranous GN.KEY WORDS: Nephrotic syndrome, Renal biopsy, Proteinuria, Glomerulopathy

scholarly journals P0338CRUMBS HOMOLOG2-EZRIN SIGNALING INDUCED PODOCYTE INJURY, LEADING TO MINIMAL-CHANGE DISEASE(MCD) AND FOCAL SEGMENTAL GLOMERULOSCLEROSIS(FSGS)

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Ichiro Hada
Keyword(s):  
Electron Microscopy ◽  
Nephrotic Syndrome ◽  
Cell Line ◽  
Focal Segmental Glomerulosclerosis ◽  
Minimal Change Disease ◽  
Immunofluorescence Microscopy ◽  
Minimal Change ◽  
Podocyte Injury ◽  
Heavy Proteinuria ◽  
Foot Process

Abstract Background and Aims The etiology and cellular pathogenesis of podocyte injury leading to minimal-change disease (MCD) and focal segmental glomerulosclerosis (FSGS) remain largely obscure. Genetic mutation of crumbs homolog 2 (CRB2) is a cause of congenital nephrotic syndrome. Type-1 transmembrane proteins including CRB2 transduce outside-in signals that are involved in various cellular events including changes in the cytoskeletal network. The aim of the present study is to determine whether alteration of CRB2-mediated signaling in podocytes causes MCD and FSGS. Method Mice were immunized with a partial recombinant protein including the extracellular part of mouse CRB2. Urinalysis was obtained, and the kidney was subjected to histopathology. Kidney samples were also subjected to immunofluorescence microscopy and glomerular isolation to determine whether activation of the ezrin/radixin/moesin (ERM) family of cross-linkers between plasma membrane proteins and the actin cytoskeleton is involved in the pathogenesis of this nephrotic model. A CRB2-expressing mouse podocyte cell line was generated and incubated with anti-CRB2 antibody, and cell lysates were subjected to immunoblot analysis of ERM phosphorylation. The presence of anti-CRB2 antibody in the serum was determined by Western blot analysis. Results Apparent anti-CRB2 antibody was detected in the serum from 4 weeks onward. Immunized mice developed proteinuria at 4 weeks, which continued at least until 29 weeks. Mice developing extremely heavy proteinuria also developed hematuria from 18 weeks onward. Light microscopy revealed MCD in mice with proteinuria alone and FSGS in mice with heavy proteinuria and hematuria. Immunofluorescence microscopy revealed positive granular IgG staining in podocyte foot processes, but not complement C3. Electron microscopy and immuno-electron microscopy revealed alteration of actin organization associated with prominent foot process effacement. Strong phosphorylation of ezrin was observed in the glomerulus from the proteinuric stage and in the cellular lysates from the CRB2-expressing podocyte cell line incubated with anti-CRB2 antibody. Conclusion The current data revealed that binding of anti-CRB2 antibody to the extracellular domain of CRB2 on the podocyte foot process activated the ezrin-cytoskeleton network, leading to podocyte injury. Our data also indicated that signaling by this one molecular can induce two different phenotypes of glomerular injury: MCD and FSGS. In our model, the signaling was activated by anti-CRB2 antibody, but in patients with nephrotic syndrome the CRB2 ligands remain unknown. Therefore, it will be important to identify the soluble factors interacting with CRB2, which may be novel factors contributing to the pathogenesis of MCD and FSGS.

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