Focal segmental glomerulosclerosis: challenges in definitions, pathogenesis and management

Focal segmental glomerulosclerosis (FSGS) is a well-defined pattern of glomerular injury identifiable on renal biopsy using light microscopy. FSGS is not a single entity and much information is needed to make a proper evaluation in each subject with the condition to identify the cause, prognosticate, and inform treatment choices. Categories of information required include: clinical presentation, responsiveness to steroids, pathological subtype, genetic background, and evidence for other adaptive, viral, and toxic causes. Primary FSGS describes a cohort of conditions identified by exclusion of known contributory causes, but does not represent a single entity. Clinical manifestations and outcomes of FSGS vary widely; they include asymptomatic proteinuria, cases of spontaneous remission, steroid-sensitive nephrotic syndrome, and nephrotic syndrome resistant to immune modulating therapy progressing to end-stage renal disease with recurrence after transplantation. Although immune modulating therapy (based notably on corticosteroids and calcineurin inhibitors) are widely used in primary FSGS, robust evidence of their efficacy remains scant

El Banco de España elige ALEPH y la British Library ILS, ambos productos de ExLibris

Sección: Noticias. Noticias externasEl Banco de España ha sustituido durante 2003 el programa de gestión de bibliotecas DOBIS/LIBIS que usaba hasta el momento por el sistema ALEPH 500. Es el quinto banco central europeo que opta por Aleph (otros son los bancos centrales de Holanda, Italia y Luxemburgo).N

IgA nephropathy at two score and one

On May 26–28, 2009, an international symposium on IgA nephropathy was convened in Stresa, Italy, as a Satellite Symposium of the World Congress of Nephrology held in Milan. This meeting was attended by a large number of scientists and clinicians working in the field of IgA nephropathy. The oral and poster presentations (over 70) ranged from very fundamental structural biology to clinical management. This article attempts to summarize the main findings of the meeting and to put forth some new perspectives and hypotheses regarding human IgA nephropathy on the 41st anniversary of its original description by Berger and Hinglais in 1968

Training nephrologists from developing countries: does it have a positive impact?

In the past 25 years the International Society of Nephrology has sponsored 545 physicians from 83 developing countries to undertake nephrology training in renal units in the developed world. Data collected biennially from past fellows have demonstrated a very positive impact of the program on individual trainees and their home institutions. Many of the trainees have gone on to leadership positions in their home institutions, countries, and regions. Increasingly, fellowships are undertaken in selected developed centers within the fellow's own region, which increases the relevance and utility of the training to the fellow and the fellow's home institution, and lessens the risk of ‘brain drain’

Mesangial IgA1 in IgA nephropathy exhibits aberrant O-glycosylation: Observations in three patients

Mesangial IgA1 in IgA nephropathy exhibits aberrant O-glycosylation: Observations in three patients.BackgroundIn IgA nephropathy (IgAN), circulating IgA1 molecules display an abnormal pattern of O-glycosylation. This abnormality may potentially contribute to mesangial IgA1 deposition, but this is unproven because the O-glycosylation of mesangial IgA1 has not been analyzed.MethodsIgA1 was eluted from glomeruli isolated from the kidneys of three IgAN patients obtained after nephrectomy or at postmortem. Serum from these patients, other patients with IgAN, and controls was subjected to the same treatment as the glomerular eluates. The O-glycosylation of eluted and serum IgA1 was measured by lectin binding using an enzyme-linked immunosorbent assay-based system.ResultsIn all three cases, the lectin binding of IgA1 eluted from the glomeruli of IgAN patients was markedly higher than that of the serum IgA1 of the same individual, and also all but one of a series of serum IgA1 samples from other patients and controls.ConclusionsThe higher lectin binding of glomerular compared with serum IgA1 suggests that O-glycosylated IgA1 molecules abnormally and selectively deposit in the kidney. These results provide the first evidence that mesangial IgA1 is abnormally O-glycosylated, and support a direct role for abnormal IgA1 O-glycosylation in the mechanism of mesangial IgA deposition in IgAN

Three-Year Clinical Outcomes of the First South Asian Prospective Longitudinal Observational IgA Nephropathy Cohort

INTRODUCTION: Glomerular Research And Clinical Experiments—IgA Nephropathy in Indians (GRACE-IgANI) is the first prospective South Asian IgA nephropathy (IgAN) cohort with prespecified objectives, protocolized longitudinal follow-up, and extensive biosample collection. The baseline risk scores predicted high risk of kidney disease progression. METHODS: A total of 195 of 201 patients (97%) completed 3-year follow-up in September 2020. All patients received optimized supportive care, and those at high risk of progression were offered systemic corticosteroids. RESULTS: A total of 76 patients (76 of 193, 39.4%) had rapid progression in 3 years (≥5 ml/min per 1.73 m(2) decline in estimated glomerular filtration rate [eGFR] per year). A total of 72 patients (72 of 195, 36.9%) experienced the composite outcome (CO), defined as ≥50% fall in eGFR, eGFR < 15 ml/min per 1.73 m(2), commenced kidney replacement therapy or death, in 3 years. At each scheduled follow-up, achievement of proteinuria level < 1 g/d significantly delayed the time to the CO. The receiver operating characteristic curve of average annual decline in eGFR ≥ 5 ml/min per 1.73 m(2) had 86% sensitivity and 89% specificity for CO in 3 years and had good discrimination from 1 year onwards (area under the curve 0.8, SE 0.04, 95% CI 0.7–0.9, P < 0.0001). The significant predictors of CO by Cox proportional-hazards model were as follows: baseline MEST-T2 score (hazard ratio [HR] 3.3, 95% CI 1.7–6.5, P < 0.001), along with 24-hour urine protein level ≥ 1 g/d (HR 2.1, 95% CI 1.1–3.9, P = 0.02), eGFR < 60 ml/min per 1.73 m(2) (HR 2.9, 95% CI 1.1–7.6, P = 0.03), and rate of eGFR decline ≥ 5 ml/min per 1.73 m(2)/yr (HR 2.7, 95% CI 1.6–4.8, P < 0.001) all measured at 6 months. Mortality was 11 of 195 (5.6%). CONCLUSION: We identified longitudinal clinical variables measured at 6 months and ≥5 ml/min per 1.73 m(2) annual fall in eGFR after kidney biopsy as important predictors for composite outcome in addition to baseline histology

A multi-centre qualitative study exploring the experiences of UK South Asian and White Diabetic Patients referred for renal care

Background An exploration of renal complications of diabetes from the patient perspective is important for developing quality care through the diabetic renal disease care pathway. Methods Newly referred South Asian and White diabetic renal patients over 16 years were recruited from nephrology outpatient clinics in three UK centres - Luton, West London and Leicester – and their experiences of the diabetes and renal care recorded. A semi-structured qualitative interview was conducted with 48 patients. Interview transcripts were analysed thematically and comparisons made between the White and South Asian groups. Results 23 South Asian patients and 25 White patients were interviewed. Patient experience of diabetes ranged from a few months to 35 years with a mean time since diagnosis of 12.1 years and 17.1 years for the South Asian and White patients respectively. Confusion emerged as a response to referral shared by both groups. This sense of confusion was associated with reported lack of information at the time of referral, but also before referral. Language barriers exacerbated confusion for South Asian patients. Conclusions The diabetic renal patients who have been referred for specialist renal care and found the referral process confusing have poor of awareness of kidney complications of diabetes. Healthcare providers should be more aware of the ongoing information needs of long term diabetics as well as the context of any information exchange including language barriers

Epidemiology, baseline characteristics and risk of progression in the first South-Asian prospective longitudinal observational IgA nephropathy cohort

Introduction: Glomerular Research And Clinical Experiments-IgA Nephropathy in Indians (GRACE-IgANI) is the first prospective South Asian IgAN cohort with protocolized follow-up and extensive biosample collection. Here we report the baseline clinical, biochemical, and histopathologic characteristics of GRACE IgANI and calculate baseline risk of progression for the cohort. Methods: 201 incident adults with kidney biopsy-proven primary IgAN were recruited into GRACE-IgANI between March 2015 and September 2017. As of April 30, 2020, the cohort had completed a median followup of 30 months (interquartile range [IQR] 16-39). Results: The commonest clinical presentation in GRACE IgANI was hypertension, with or without proteinuria, and nephrotic-range proteinuria was present in 34%, despite Conclusions: The predicted risk of progression in this cohort was considerable. Over the next 5 years, we will dissect the pathogenic pathways that underlie this severe South Asian IgAN phenotype