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 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.

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.

Therapeutic Response and Long-Term Renal Outcomes in Childhood Idiopathic Steroid-Resistant Nephrotic Syndrome: A Single-Center Study

Nephron ◽  
2021 ◽  
pp. 1-8
Author(s):  
Jiwon Jung ◽  
Joo Hoon Lee ◽  
Young Seo Park
Keyword(s):  
Renal Function ◽  
Nephrotic Syndrome ◽  
Therapeutic Response ◽  
Glomerular Sclerosis ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Renal Outcomes ◽  
Focal Segmental Glomerular Sclerosis

<b><i>Purpose:</i></b> We aimed to evaluate therapeutic response and long-term renal outcomes of childhood idiopathic steroid-resistant nephrotic syndrome (iSRNS). <b><i>Methods:</i></b> We retrospectively reviewed treatment regimens, especially calcineurin inhibitor (CNI), pathologic diagnoses, and long-term renal outcomes of iSRNS patients for 30 years. <b><i>Results:</i></b> Of 516 patients with idiopathic NS, 52 (10.1%) had iSRNS. Renal biopsies from 48 patients showed minimal change disease (MCD) in 23 (47.9%), focal segmental glomerulosclerosis in 24 (50.0%), and mesangioproliferative glomerulonephritis in 1 (2.1%). The median follow-up period was 66.5 (range, 4–275) months, and 90.4% of them were treated with a CNI. CNI induced remission in 70.2% within 50.4 ± 43.5 days. Of the patients with MCD and focal segmental glomerular sclerosis (FSGS), 86.4% (19/22) and 45.0% (9/20) (<i>p =</i> 0.005) responded to CNI, respectively. Mean time until remission after using CNI was longer with FSGS (90.4 ± 54.0 days) than with MCD (29.6 ± 26.3 days) (<i>p =</i> 0.010). CNI-responsive patients with FSGS or MCD showed preserved renal function, and CNI nonresponsive MCD patients also showed preserved renal function during follow-up. However, end-stage renal disease (ESRD) progressed in 8 out of 11 patients with FSGS nonresponsive to the CNI for an average of 44.9 ± 18.4 months after diagnosis. <b><i>Conclusion:</i></b> Different response rates and times for remission were achieved with the CNI according to the pathology of iSRNS. All MCD patients regardless of CNI response and all CNI-responsive patients with FSGS showed excellent renal outcomes, while almost all FSGS patients nonresponsive to CNI eventually progressed to ESRD.

Mycosis fungoides with focal segmental glomerular sclerosis and nephrotic syndrome

1998 ◽  
Vol 38 (2) ◽  
pp. 301-305 ◽  
Author(s):  
Jennifer Clay Cather ◽  
Clotilde Jackow ◽  
James Yegge ◽  
Frederick Hagemeister ◽  
Madeleine Duvic
Keyword(s):  
Nephrotic Syndrome ◽  
Mycosis Fungoides ◽  
Glomerular Sclerosis ◽  
Focal Segmental Glomerular Sclerosis ◽  
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.

Focal and segmental glomerular sclerosis

2009 ◽  
pp. 215-260 ◽  
Author(s):  
Francesco Scolari ◽  
Claudio Ponticelli
Keyword(s):  
Nephrotic Syndrome ◽  
Signs And Symptoms ◽  
Response To Treatment ◽  
Glomerular Sclerosis ◽  
Term Outcome ◽  
Glomerular Lesion ◽  
Stage Renal Disease ◽  
End Stage ◽  
Segmental Glomerular Sclerosis

Focal and segmental glomerular sclerosis (FSGS) is a glomerular lesion which is associated with distinctive clinical features. Because it may be pathogenetically heterogeneous, it is not yet appropriate to call it a disease, yet its discovery in a renal biopsy in a patient with the nephrotic syndrome does have important connotations with respect to response to treatment and to long-term outcome. By light microscopy the characteristic finding is segmental areas of sclerosis (and hyalinosis) involving only some glomeruli. Patients with the FSGS lesion typically have proteinuria which is usually in the nephrotic range (〉3.5g/d in an adult), accompanied by the typical constellation of signs and symptoms of the nephrotic syndrome and arterial hypertension. A minority of patients may have only asymptomatic proteinuria and these patients usually do not progress to end-stage renal disease (ESRD) but the natural course of FSGS is ominous in most patients with nephrotic syndrome. However, numerous observational studies have shown that about 50–70% of patients may respond completely or partially to prolonged glucocorticoid therapy or other ‘immunosuppressive’ treatments and thus have a fair outcome in the long term.

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