Focal and segmental glomerular sclerosis

Author(s):  
Francesco Scolari ◽  
Claudio Ponticelli

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.

1996 ◽  
Vol 16 (1_suppl) ◽  
pp. 505-509 ◽  
Author(s):  
Timothy E. Bunchman

The proper treatment of an infant with end-stage renal disease depends upon a number of factors including parental willingness to take on the task, experience of the health-care team, local and regional resources, and society's willingness to accept this support as a standard of care. Whereas the abilityto keep infants aliveon peritoneal dialysis (PD) is obtainable, it is not without physical, financial, as well as emotional cost. In order for a family to agree to take on such a task, an understanding of the risks and long-term prognosis should be offered. This “informed consent” is difficult to obtain in such a highly charged situation when emotions often dictate choice independently of logic. Long-term outcome of infants on PD has improved over time, yet is still fraught with complications. Options of treatment or nontreatment are explored.


2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Hirota Kida ◽  
Shungo Hikoso ◽  
Akihiro Sunaga ◽  
Oeun Bolrathanak ◽  
Takayuki Kojima ◽  
...  

Abstract Background and Aims End-stage renal disease (ESRD) patients frequently have the coronary artery disease. However, the short- and long-term outcome of ESRD patients with acute myocardial infarction (AMI) is little known. The aim of this study was to clarify it. Method Using the database of the Osaka Acute Coronary Insufficiency Study (OACIS), 8702 consecutive AMI patients (male: 75.2%, mean age: 66.9±12.2yrs) from 2002 to 2013 were analyzed. We classified these patients into two groups, those with ESRD [ESRD group (n=271)] and without ESRD [No-ESRD group (n=8431)] and examined in-hospital or long-term all-cause mortality. ESRD was defined as eGFR<15ml/min/1.73m2. Results ESRD group had higher frequency of diabetes (59.3% vs 37.8%, p<0.01), hypertension (90.1% vs 63.3%, p<0.01), Killip class≧2 (40.1% vs 21%, p<0.01), multi-vessel disease (69.3% vs 50.8%, p<0.01), and lower frequency of peak CK>3000 (21.7% vs 32.4%, p<0.01) than No-ESRD group. Mean follow-up period was 1041±721 days. In hospital mortality of ESRD group was 27% and No-ESRD group 7.2%. In patients who discharged alive (8027 patients), 1-year mortality of ESRD group was 12.2% and No-ESRD group 3.3%, 3-year mortality of ESRD group was 29.3% and No-ESRD group 8.7%. Kaplan-Meier analysis revealed that the all-cause mortality (log-rank p<0.01) was significantly higher in ESRD group than No-ESRD group. In ESRD patients who discharged alive (203patients), Cox univariate analysis after multiple imputation revealed that peak CK>3000 was significantly associated with an increased risk of mortality (Hazard ratio 2.67, 95% confidence interval 1.18to 6.07, p=0.031). Conclusion In patients with AMI, ESRD was significantly associated with worse short- and long-term outcome, suggesting that careful treatment might be required in ESRD patients with AMI, especially had peak CK>3000.


Nephron ◽  
1983 ◽  
Vol 35 (4) ◽  
pp. 225-231 ◽  
Author(s):  
Amir Tejani ◽  
Anthony D. Nicastri ◽  
Dilip Sen ◽  
C.K. Chen ◽  
Kishore Phadke ◽  
...  

2020 ◽  
Vol 75 (11) ◽  
pp. 2117
Author(s):  
Takuya Ogami ◽  
Eric Zimmermann ◽  
Roger Zhu ◽  
Yuming Ning ◽  
Paul A. Kurlansky ◽  
...  

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Chiara Salviani ◽  
Mattia Zappa ◽  
Guido Jeannin ◽  
Mario Gaggiotti ◽  
Regina Tardanico ◽  
...  

Abstract Background and Aims The recent decades have witnessed significant changes in the epidemiology and clinical course of infection-related glomerulonephritis (IRGN). We analyzed the clinicopathological features and long-term outcome of adult patients with biopsy-proven IRGN followed in a large Italian referral centre. Method We included patients with biopsy-proven IRGN diagnosed from 2000 to 2018. Clinical and laboratory findings, histological features, possible risk factors and therapy were assessed for both renal and patient outcome. Results Forty-one patients met the inclusion criteria (male:female ratio 3:1, mean age 61±16 years). Smoke habit (47.2%), alcoholism (30.6%), and diabetes (27.5%) were the most common risk factors. The most frequently identified sites of infection were skin, lung and heart (Table 1). Staphylococci spp. accounted for 76.5% of positive cultures (Figure 1). Hypocomplementaemia emerged in 48.5% of cases. The most frequent histologic patterns were diffuse proliferative (56.0%) and membranoproliferative (29.3%) glomerulonephritis. Haemodialysis was required by 22.5% of patients at inception. Two thirds of patients developed chronic kidney disease; half of them reached end-stage renal disease (ESRD). By multivariate analysis ESRD was associated with diabetes (HR 13.7; 95% CI, 1.6-121.0; p=0.018), crescents (HR 25.2; 95% CI, 2.7-235.7; p=0.005), and interstitial fibrosis (HR 31.0; 95% CI, 3.3-287.3; p=0.003). Male gender (HR 12.7; 95% CI, 10.8-14.6; p=0.008) hypertension (HR 40.8; 95% CI, 38.6-43.1; p=0.001), gross haematuria (HR 11.8; 95% CI, 9.4-14.2; p=0.047), need for haemodialysis at onset (HR 16.3; 95% CI, 14.7-17.8; p<0.0001), and interstitial infiltrate (HR 13.3; 95% CI, 11.6-15.0; p=0.003) significantly affected survival. Corticosteroids did not affect prognosis. Conclusion While traditionally considered a “benign” disease with a favorable course in children, IRGN is a potentially severe disease in adults, particularly when a background of major comorbidities and older age are present. A significant proportion of patients does not recover renal function, with a remarkable risk of ESRD.


2013 ◽  
Vol 35 (3) ◽  
pp. 191-199 ◽  
Author(s):  
Alberto Zagury ◽  
Anne Louise de Oliveira ◽  
Jose Augusto Araujo Montalvão ◽  
Regina Helena Leite Novaes ◽  
Vinicius Martins de Sá ◽  
...  

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