scholarly journals Consolidation Treatment and Long-Term Prognosis of Rituximab in Minimal Change Disease and Focal Segmental Glomerular Sclerosis

2021 ◽  
Vol Volume 15 ◽  
pp. 1945-1953
Author(s):  
Li Lin ◽  
Weiming Wang ◽  
YiFan Wu ◽  
JingYuan Xie ◽  
Xiao Li ◽  
...  
Keyword(s):  
Minimal Change Disease ◽  
Minimal Change ◽  
Glomerular Sclerosis ◽  
Consolidation Treatment ◽  
Focal Segmental Glomerular Sclerosis ◽  
Long Term Prognosis ◽  
Segmental Glomerular Sclerosis

Idiopathic nephrotic syndrome

2018 ◽  
Author(s):  
Patrick Niaudet ◽  
Alain Meyrier
Keyword(s):  
Nephrotic Syndrome ◽  
Light Microscopy ◽  
Minimal Change Disease ◽  
Idiopathic Nephrotic Syndrome ◽  
Minimal Change ◽  
Glomerular Sclerosis ◽  
Focal Segmental Glomerular Sclerosis ◽  
Diffuse Mesangial Proliferation ◽  
Segmental Glomerular Sclerosis ◽  
Visceral Epithelial Cell

Idiopathic nephrotic syndrome is defined by the combination of massive proteinuria, hypoalbuminaemia, hyperlipidaemia, and oedema, and of non-specific histological abnormalities of the glomeruli. Light microscopy may disclose minimal change disease, diffuse mesangial proliferation, or focal segmental glomerular sclerosis (FSGS). The two main causes of idiopathic nephrotic syndrome are characterized histologically. On electron microscopy the glomerular capillaries show a fusion of visceral epithelial cell (podocyte) foot processes and with the exception of some variants no significant deposits of immunoglobulins or complement by immunofluorescence. In a majority of children only minimal changes are seen on light microscopy. These children are referred to as having ‘minimal change disease’. In adults with idiopathic nephrotic syndrome, lesions of FSGS are more frequent.

Long-Term Evaluation of Children with Nephrotic Syndrome and Focal Segmental Glomerular Sclerosis

Nephron ◽  
1983 ◽  
Vol 35 (4) ◽  
pp. 225-231 ◽  
Author(s):  
Amir Tejani ◽  
Anthony D. Nicastri ◽  
Dilip Sen ◽  
C.K. Chen ◽  
Kishore Phadke ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Glomerular Sclerosis ◽  
Focal Segmental Glomerular Sclerosis ◽  
Term Evaluation ◽  
Segmental Glomerular Sclerosis

scholarly journals A point mutation of mitochondrial genes in diabetes and deafness with focal segmental glomerular sclerosis

2021 ◽  
Vol 1 (1) ◽  
pp. 55-58
Author(s):  
Jin Zhang ◽  
Yan Wang ◽  
Yonggui Wu
Keyword(s):  
Clinical Symptoms ◽  
Histological Finding ◽  
Mitochondrial Genes ◽  
Term Treatment ◽  
Glomerular Sclerosis ◽  
Focal Segmental Glomerular Sclerosis ◽  
Long Term Treatment ◽  
History Of ◽  
Segmental Glomerular Sclerosis

Abstract Deafness, diabetes and proteinuria are typically understood to be an uncommon combination. Here, we reported a 26-year-old woman with a history of persistent deafness, diabetes mellitus, and proteinuria. The diagnosis mainly depends on clinical symptoms, but the cause of the disease should be examined. The histological finding in renal biopsy showed secondary focal segmental glomerular sclerosis (FSGS), but not classic diabetic nephropathy. Further pathogeny was found. Subsequently, a 3243A>G mutation in the mitochondrial DNA was found. Thus, the diagnosis of maternally inherited deafness and diabetes (MIDD) was considered. Ineffective and unnecessary immunosuppression can be avoided through timely diagnosis. Long-term treatment of CoQ10 can be useful in MIDD patients.

scholarly journals Terapia della sindrome nefrosica idiopatica: ruolo delle tecniche aferetiche

2013 ◽  
Vol 25 (4_suppl) ◽  
pp. S41-S45
Author(s):  
Luigi Moriconi
Keyword(s):  
Nephrotic Syndrome ◽  
Idiopathic Nephrotic Syndrome ◽  
Sono State ◽  
Minimal Change ◽  
Glomerular Sclerosis ◽  
Minimal Change Nephropathy ◽  
Focal Segmental Glomerular Sclerosis ◽  
Segmental Glomerular Sclerosis ◽  
State Identificate

La Sindrome Nefrosica Idiopatica (Idiopathic Nephrotic Syndrome, INS) ricorre essenzialmente in presenza di due glomerulopatie: la MCN (Minimal Change Nephropathy) e la FSGS (Focal Segmental Glomerular Sclerosis). La prima ha un decorso più benigno ed è più frequente nei bambini, mentre la seconda ha un decorso più severo, può portare a Insufficienza Renale Cronica Terminale e può re-cidivare nel trapianto. Soprattutto per la FSGS sono state identificate possibili eziologie virali o genetiche, oltre a forme secondarie in corso di altre malattie, per cui non è semplice classificare queste glomerulopatie. Le forme ricorrenti nel rene trapiantato costituiscono un gruppo più omogeneo. I fattori che sembrano essere comuni alla MCN e alla FSGS, anche se maggiormente espressi e studiati nella seconda, sono la lesione glomerulare caratterizzante a carico dei podociti, e il frequente riscontro di sostanze circolanti, definite fattori di permeabilità (PFs), capaci di indurre proteinuria. Corticosteroidi e Immunosoppressori sono la terapia standard della INS. Tuttavia, la presenza di casi farmaco-resistenti e l'identificazione di alcuni PFs circolanti hanno consentito di utilizzare nuove terapie dirette a bloccare la sintesi o l'azione di queste molecole e hanno fornito un ulteriore razionale alla loro rimozione mediante plasmaferesi convenzionale (PEX) o aferesi selettiva.

MO289IS THERE A ROLE OF INTERSTITIAL CHANGES ON THERAPY OUTCOME AMONG PATIENTS WITH FOCAL-SEGMENTAL GLOMERULAR SCLEROSIS AND MINIMAL CHANGE DISEASE?

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Aleksandar Jankovic ◽  
Petar Djuric ◽  
Ana Bulatovic ◽  
Dragana Markovic ◽  
Snezana Pešić ◽  
...  
Keyword(s):  
Complete Remission ◽  
Partial Remission ◽  
Minimal Change Disease ◽  
Minimal Change ◽  
Therapy Outcome ◽  
Glomerular Sclerosis ◽  
First Line Therapy ◽  
Focal Segmental Glomerular Sclerosis ◽  
Significant Difference ◽  
Immunosuppressive Protocol

Abstract Background and Aims It is well-known that interstitial changes in patients with focal-segmental glomerulosclerosis (FSGS) and minimal change disease (MCD) are linked with disease chronicity and progression. The aim of this study was to analyze is there any connection between interstitial changes and outcome after “first line” therapy in patients with FSGS/MCD. Method From 2014 till 2019, biopsy proven diagnosis of FSGS/MCD was established in 40 patients. Interstitial changes were classified in three groups as following: 0-w/o changes; 1-mild changes; 2-severe changes. Patients with nephrotic syndrome (No=29) were treated with prednisolone (1mg/kg) and after six months we have registered therapy outcome as: CR-complete remission; PR-partial remission; EX-death; NO-no effect. Results Among treated patients (age 50.4±15.3 years, 15 men), CR was achieved in 10 patients (34.5%) and 6 out of these 10 (60%) had no interstitial changes. Partial remission was observed in 11 patients (37.9%), in 4 patients (13.8%) therapy did not have any effect, and 4 patients (13.8%) deceased (table 1). All patients in EX and NO group had interstitial changes. There were no significant difference in age, gender, proteinuria, albuminaemia, creatinine and glycaemia levels between groups except for hemoglobin levels that were significantly lower in EX group than in others (∑ 15.144; p=0.002) and urea levels that were significantly higher in EX group (∑ 138.057; p=0.024). Conclusion Patients with FSGS/MCD respond well on standard immunosuppressive protocol, particularly in absence of interstitial changes what may increase the chance for achieving complete remission.

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