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Noris M.,Clinical Research Center for Rare Diseases Aldo e Cele Dacco | Remuzzi G.,Unit of Nephrology and Dialysis
Nature reviews. Nephrology | Year: 2014

Haemolytic uraemic syndrome (HUS) is characterized by nonimmune haemolytic anaemia, thrombocytopenia and renal impairment-most incidents in childhood are caused by shiga toxin-producing bacteria. Atypical HUS (aHUS) accounts for 10% of cases and has a poor prognosis. About 60% of patients with aHUS have dysregulation of the alternative complement pathway (complement-mediated aHUS). The kidney is the main target organ, but other organs might also be affected. Cardiac complications occur in 3-10% of patients with complement-mediated aHUS, as a consequence of microangiopathic injury in the coronary microvasculature, and can cause sudden death. Emerging evidence also suggests that either thrombosis or stenosis of the medium and large arteries might complicate disease course, and such disorders occur even after renal function is lost. In this Perspectives article we discuss the impact of cardiovascular involvement in complement-mediated aHUS, the role of acute and chronic complement hyperactivation in such events and the implications for treatment. Source

Rattazzi M.,University of Padua | Bertacco E.,University of Padua | Del Vecchio A.,Unit of Nephrology and Dialysis | Puato M.,University of Padua | And 2 more authors.
Nephrology Dialysis Transplantation | Year: 2013

Several clinical studies reported an increased prevalence and accelerated progression of aortic valve calcification among patients with end-stage renal disease when compared with subjects with normal kidney function. Recently, mechanisms of calcific valve degeneration have been further elucidated and many of the pathways involved could be amplified in patients with decreased renal function. In particular, calcium-phosphate balance, MGP metabolism, OPG/RANK/RANKL triad, fetuin-A mineral complexes and FGF-23/Klotho axis have been shown to be impaired among patients with advanced chronic kidney disease and could play a role during vascular/valve calcification. The scope of the present review is to summarize the clinical data and the pathophysiological mechanisms potentially involved in the link between renal function decline and the progression of aortic valve disease. © The Author 2013. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. Source

Kumbhani D.J.,Cleveland Clinic | Bavry A.A.,University of Florida | Harvey J.E.,Cleveland Clinic | De Souza R.,Harvard University | And 3 more authors.
American Heart Journal | Year: 2011

Background: We sought to systematically evaluate whether percutaneous revascularization is associated with additional clinical benefit in patients with renal artery stenosis (RAS) as compared with medical management alone. Methods: We included randomized controlled trials that compared percutaneous revascularization in addition to medical therapy versus medical management alone in patients with RAS. Six trials with 1,208 patients were included. Results: At a mean follow-up of 29 months, there was no change in systolic blood pressure (weighted mean difference [WMD] = 1.20 mm Hg, 95% CI -1.18 to 3.58 mm Hg) or diastolic blood pressure (WMD = -1.60 mm Hg, 95% CI -4.22 to 1.02 mm Hg) from baseline in the percutaneous revascularization arm compared with the medical management arm. There was a reduction in the mean number of antihypertensive medications (WMD = -0.26, 95% CI -0.39 to -0.13, P < .001), but not serum creatinine (WMD = -0.14 mg/dL, 95% CI -0.29 to 0.007 mg/dL), in the percutaneous revascularization arm at the end of follow-up. Percutaneous revascularization was not associated with a significant difference in all-cause mortality (relative risk [RR] = 0.96, 95% CI 0.74-1.25), congestive heart failure (RR = 0.79, 95% CI 0.56-1.13), stroke (RR = 0.86, 95% CI 0.50-1.47), or worsening renal function (RR = 0.91, 95% CI 0.67-1.23) as compared with medical management. Conclusions: In patients with RAS, percutaneous renal revascularization in addition to medical therapy may result in a lower requirement for antihypertensive medications, but not with improvements in serum creatinine or clinical outcomes, as compared with medical management over an intermediate period of follow-up. Further studies are needed to identify the appropriate patient population most likely to benefit from its use. © 2011 Mosby, Inc. All rights reserved. Source

Ruggenenti P.,Clinical Research Center for Rare Diseases Aldo e Cele Dacco | Ruggiero B.,Clinical Research Center for Rare Diseases Aldo e Cele Dacco | Cravedi P.,Clinical Research Center for Rare Diseases Aldo e Cele Dacco | Vivarelli M.,Childrens Hospital Bambino Gesu | And 12 more authors.
Journal of the American Society of Nephrology | Year: 2014

The outcome of steroid-dependent or frequently relapsing nephrotic syndrome of minimal change disease (MCD), mesangial proliferative GN (MesGN), or FSGS may be poor and with major treatment toxicity. This academic,multicenter, off-on trial (ClinicalTrials.gov #NCT00981838) primarily evaluated the effects of rituximab therapy followed by immunosuppression withdrawal on disease recurrence in 10 children and 20 adults with MCD/MesGN (n=22) or FSGS who had suffered ≥2 recurrences over the previous year and were in steroidinduced remission for ≥1 month. Participants received one dose (n=28) or two doses of rituximab (375 mg/m2 intravenously).At 1 year, allpatientswere in remission: 18were treatment-free and 15 never relapsed.Compared with the year before rituximab treatment, total relapses decreased from 88 to 22 and the per-patient median number of relapses decreased from 2.5 (interquartile range [IQR], 2-4) to 0.5 (IQR, 0-1; P<0.001) during 1 year of follow-up. Reduction was significant across subgroups (children, adults,MCD/MesGN, and FSGS; P<0.01). After rituximab, the per-patient steroid maintenance median dose decreased from 0.27 mg/kg (IQR, 0.19-0.60) to 0mg/kg (IQR, 0-0.23) (P,0.001), and the median cumulative dose to achieve relapse remission decreased from 19.5 mg/kg (IQR, 13.0-29.2) to 0.5 mg/kg (IQR, 0-9.4) (P<0.001). Furthermore, the mean estimated GFR increased from 111.3±25.7 to 121.8±29.2ml/min per 1.73m2 (P=0.01),with the largest increases in children and in FSGS subgroups. The mean height z score slope stabilized in children (P<0.01). Treatment was well tolerated. Rituximab effectively and safely prevented recurrences and reduced the need for immunosuppression in steroiddependent or frequently relapsing nephrotic syndrome, and halted disease-associated growth deficit in children. Copyright © 2014 by the American Society of Nephrology. Source

Sharma S.K.,BP Koirala Institute of Health science | Zou H.,Southern Medical University | Togtokh A.,University of Mongolia | Ene-Iordache B.,Mario Negri Institute for Pharmacological Research | And 9 more authors.
American Journal of Kidney Diseases | Year: 2010

Background In 2007, the International Society of Nephrology funded the Kidney Disease Data Center database to house data from sponsored programs aimed at preventing chronic kidney disease and its complications in developing nations. This study compares baseline characteristics and burden of illness among participants from centers in China, Mongolia, and Nepal. An important secondary objective is to show the feasibility of screening for chronic kidney disease and its major risk factors in a diverse group of lower income settings. Study Design Cross-sectional screening study. Setting & Participants Participants from Nepal (n = 8,398), China (n = 1,999), and Mongolia (n = 997). Screening was open to the public for participants in China and Nepal; referral from a general practitioner was required for participants in Mongolia. Outcomes Estimated glomerular filtration rate (eGFR), proteinuria, hypertension, diabetes, obesity, cardiovascular risk. Measurement Demographic and clinical data were collected prospectively using a standard format. Blood and urine specimens were provided according to local protocol. Results Of 11,394 participants, decreased eGFR (<60 mL/min/1.73 m2) was present in 7.3%-14% of participants across centers; proteinuria (<1+) on dipstick (2.4%-10%), hypertension (26%-36%), diabetes (3%-8%), and obesity (body mass index <30 kg/m2; 2%-20%) were all common. Predicted 5-year cardiovascular risk <10% ranged from 20%-89%. Numbers needed to screen to detect a new case of eGFR <60 mL/min/1.73 m2, hypertension, or diabetes were 2.6 (95% CI, 2.5-2.7), 3.4 (95% CI, 3.1-3.7), and 4.7 (95% CI, 3.3-8.0) for Nepal, China, and Mongolia, respectively. Limitations May not be representative of the general population. Conclusions The acceptable diagnostic yield of abnormalities across these 3 diverse settings suggests that trials of targeted screening and intervention are feasible and warranted in such countries. © 2010 National Kidney Foundation, Inc. Source

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