Focal segmental glomerulosclerosis (FSGS) is a histological pattern of glomerular injury, rather than a single disease, that is caused by diverse clinicopathological entities with different mechanisms of injury with the podocyte as the principal target of lesion, leading to the characteristic sclerotic lesions in parts (i.e., focal) of some (i.e., segmental) glomeruli. The lesion of FSGS has shown an increasing prevalence over the past few decades and is considered the most common glomerular cause leading to ESKD. Primary FSGS, which usually presents with nephrotic syndrome, is thought to be caused by circulating permeability factors that have a main role in podocyte foot process effacement. Secondary forms of FSGS include maladaptive FSGS secondary to glomerular hyperfiltration such as in obesity or in cases of loss in nephron mass, virus-associated FSGS, and drugassociated FSGS that can result in direct podocyte injury. Genetic FSGS is increasingly been recognized and a careful evaluation of patients with atypical primary or secondary FSGS should be performed to exclude genetic causes. Unlike primary FSGS, secondary and genetic forms of FSGS do not respond to immunosuppression and tend not to recur after kidney transplantation. Distinguishing primary FSGS from secondary and genetic causes has a prognostic significance and is crucial for an appropriate management. In this review, we examine the pathogenesis, clinical approach to distinguish between the different causes, and current recommendations in the management of FSGS.
Chronic kidney disease (CKD) is a silent and poorly known killer. The current concept of CKD is relatively young and uptake by the public, physicians and health authorities is not widespread. Physicians still mix up CKD with chronic kidney insufficiency or failure, For the wider public and health authorities, CKD evokes kidney replacement therapy (KRT). In Spain, the prevalence of KRT is 0.13%. Thus, health authorities may consider CKD a non-issue: very few persons eventually need KRT and, for those in whom kidneys fail, the problem is “solved” by dialysis or kidney transplantation. However, KRT is the tip of the iceberg in the burden of CKD. The main burden of CKD is accelerated aging and premature death. The cut-off points for kidney function and kidney damage indexes that define CKD also mark an increased risk for all-cause premature death. CKD is the most prevalent risk factor for lethal COVID-19 and the factor that most increases the risk of death in COVID-19, after old age. Men and women undergoing KRT still have an annual mortality which is 10- o 100-fold higher than similar age peers, and life expectancy is shortened by around 40 years for young persons on dialysis and by 15 years for young persons with a functioning kidney graft. CKD is expected to become the fifth global cause of death by 2040 and the second cause of death in Spain before the end of the century, a time when 1 in 4 Spaniards will have CKD. However, by 2022, CKD will become the only top-15 global predicted cause of death that is not supported by a dedicated well-funded CIBER network research structure in Spain. Realizing the underestimation of the CKD burden of disease by health authorities, the Decade of the Kidney initiative for 2020-2030 was launched by the American Association of Kidney Patients (AAKP) and the European Kidney Health Alliance (EKHA). Leading Spanish kidney researchers grouped in the kidney collaborative research network REDINREN have now applied for the RICORS call of collaborative research in Spain with the support of the Spanish Society of Nephrology, ALCER and ONT: RICORS2040 aims to prevent the dire predictions for the global 2040 burden of CKD from becoming true.
The aim of this study was to determine the prevalence of platelet autoantibodies (PAA) in patients with systemic lupus erythematosus (SLE) and its correlation with clinical and other laboratory manifestations of the disease, as well as to evaluate the influence of platelet count and disease activity on the result of the test for PAA. Ninety SLE patients, 29 with thrombocytopenia, were evaluated. The presence of PAA was determined using the direct and indirect platelet suspension immunofluorescence test. A total of 166 PAA determinations were performed in the 90 patients upon entry into the study. Fifty-six of the 90 patients (62%) with SLE were positive for PAA. There were no statistically significant correlations between the presence of PAA and the different disease manifestations except for thrombocytopenia (P = 0.0005). The presence of PAA in the same patient was significantly associated with current thrombocytopenia and disease activity. It was concluded that the prevalence of PAA in SLE patients is high. All SLE patients with thrombocytopenia had PAA, although some patients with PAA had a normal platelet count. The presence of PAA in SLE patients is not synonymous with thrombocytopenia but should instead be regarded as one of the multiple serological abnormalities which may develop in these patients.
Our results show that the highest levels of TNFR1 are independently associated with progression of renal disease and death in type 2 DN. The renin angiotensin blockers have no effect on these inflammatory markers.
Background Kidney replacement therapy (KRT) confers the highest risk of death from COVID-19. However, most data refer to the early pandemic waves. Whole year analysis in comparison with prior secular trends are scarce. Methods We present the 2020 REMER Madrid KRT registry, corresponding to the Spanish Region hardest hit by COVID-19. Results In 2020, KRT incidence decreased 12% versus 2019 while KRT prevalence decreased (−1.75%) for the first time since records began and the number of kidney transplants (KT) decreased by 16%. Mortality on KRT was 10.2% (34% higher than the mean for 2008–2019). The 2019 to 2020 increase in mortality was larger for KT (+68%) than for HD (+24%) or PD (+38%). The most common cause of death was infection (n = 419, 48% of deaths), followed by cardiovascular (200, 23%). Deaths from infection increased by 167% year over year and accounted for 95% of excess deaths in 2020 over 2019. COVID-19 was the most common cause of death (68% of infection deaths, 33% of total deaths). The bulk of COVID-19 deaths (209/285, 73%) occurred during the first COVID-19 wave, which roughly accounted for the increased mortality in 2020. Being a KT recipient was an independent risk factor for COVID-19 death. Conclusions COVID-19 negatively impacted the incidence and prevalence of KRT, but the increase in KRT deaths was localized to the first wave of the pandemic. The increased annual mortality argues against COVID-19 accelerating death of patients with short life expectancy and the temporal pattern of COVID-19 mortality suggests that appropriate healthcare may improve outcomes.
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