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Background: IgA nephropathy (IgAN) is the most common glomerulonephritis worldwide. While studies have primarily focused on identifying risk factors for disease progression, very few data exist on the likelihood of achieving complete recovery from the disease., Methods: We conducted a single-center retrospective study on all consecutive patients with biopsy-proven IgAN diagnosed between 1986 and 2018 in our pediatric center. Biopsies were classified according to the MEST-C Oxford classification score. "Complete clinical remission" was defined as the absence of proteinuria, hematuria, and hypertension in patients with normal kidney function who had been off therapy for more than 2 years., Results: Overall, 153 patients with age at onset of 10.6 ± 4 years were enrolled in the study. Of these, 41 achieved "complete clinical remission." The estimated probability of complete clinical remission at 10 years was 43% (95%CI 33-54). However, seven patients relapsed within 10 years. Multivariable analysis showed that higher age at onset (HR 0.89, 95%CI 0.80-0.98, p = 0.017) and segmental glomerulosclerosis lesions (HR 0.28, 95%CI 0.10-0.79, p = 0.017) decreased significantly the chances of achieving complete clinical remission. Immunosuppressive therapy was not significantly associated with clinical outcomes., Conclusions: Approximately one-third of patients with pediatric-onset IgAN achieve prolonged remission, in particular, very young children at disease onset without sclerotic glomerular lesions. Longer term follow-up is needed to assess if these patients have achieved permanent remission., (© 2024. The Author(s), under exclusive licence to International Pediatric Nephrology Association.)

Background: Joubert syndrome (JS) is an inherited ciliopathy characterized by a complex midbrain-hindbrain malformation and multiorgan involvement. Renal disease, mainly juvenile nephronophthisis (NPH), was reported in 25-30% patients although only ∼18% had a confirmed diagnosis of chronic kidney disease (CKD). NPH often remains asymptomatic for many years, resulting in delayed diagnosis. The aim of the study was to identify a biomarker able to quantify the risk of progressive CKD in young children with JS., Methods: Renal features were investigated in 93 Italian patients, including biochemical tests, ultrasound and 1-deamino-8D-arginine vasopressin test in children with reduced basal urine osmolality. A subset of patients was followed-up over time., Results: At last examination, 27 of 93 subjects (29%) presented with CKD, ranging from isolated urinary concentration defect (UCD) to end-stage renal disease. Both normal and pathological urine osmolality levels remained stable over time, even when obtained at very early ages. Follow-up data showed that the probability of developing CKD can be modelled as a function of the urine osmolality value, exceeding 75% for levels <600 mOsm/kg H2O, and significantly increased in patients with an early diagnosis of isolated UCD., Conclusions: We conclude that the frequency of CKD in JS increases with age and is higher than previously reported. Urine osmolality represents an early sensitive quantitative biomarker of the risk of CKD progression., (© The Author(s) 2018. Published by Oxford University Press on behalf of ERA-EDTA.)

Background: There is a need for early identification of children with immunoglobulin A nephropathy (IgAN) at risk of progression of kidney disease., Methods: Data on 261 young patients [age <23 years; mean follow-up of 4.9 (range 2.5-8.1) years] enrolled in VALIGA, a study designed to validate the Oxford Classification of IgAN, were assessed. Renal biopsies were scored for the presence of mesangial hypercellularity (M1), endocapillary hypercellularity (E1), segmental glomerulosclerosis (S1), tubular atrophy/interstitial fibrosis (T1-2) (MEST score) and crescents (C1). Progression was assessed as end stage renal disease and/or a 50 % loss of estimated glomerular filtration rate (eGFR) (combined endpoint) as well as the rate of renal function decline (slope of eGFR). Cox regression and tree classification binary models were used and compared., Results: In this cohort of 261 subjects aged <23 years, Cox analysis validated the MEST M, S and T scores for predicting survival to the combined endpoint but failed to prove that these scores had predictive value in the sub-group of 174 children aged <18 years. The regression tree classification indicated that patients with M1 were at risk of developing higher time-averaged proteinuria (p < 0.0001) and the combined endpoint (p < 0.001). An initial proteinuria of ≥0.4 g/day/1.73 m 2 and an eGFR of <90 ml/min/1.73 m 2 were determined to be risk factors in subjects with M0. Children aged <16 years with M0 and well-preserved eGFR (>90 ml/min/1.73 m 2 ) at presentation had a significantly high probability of proteinuria remission during follow-up and a higher remission rate following treatment with corticosteroid and/or immunosuppressive therapy., Conclusion: This new statistical approach has identified clinical and histological risk factors associated with outcome in children and young adults with IgAN.

Objectives: Kidney size has been found to be correlated with anthropometric features and kidney function. Therefore, we postulate that if the conventionally measured renal sonographic parameters (pole-to-pole length, width, and parenchymal thickness) are taken according to standardized rules and corrected for body height, their association with kidney function could be strengthened, thus helping validate renal sonographic information for a better assessment of chronic kidney disease (CKD) status., Methods: This cross-sectional study included 72 stable adult patients with stage 1 to 4 CKD. Sonographic parameters were obtained from both kidneys and averaged, and the measurements obtained were further corrected for patients' body height. The glomerular filtration rate (GFR) was estimated by the Chronic Kidney Disease Epidemiology Collaboration equation., Results: Parenchymal thickness and renal length showed the highest correlation level with the GFR. This significant correlation, however, was greatly ameliorated by the correction for patients' body height (r = 0.537; P < .001; r = 0.510; P < .001, respectively). Of note, the product of these two parameters corrected for body height showed the best degree of correlation with the GFR (r = 0.560; P < .001), as confirmed by analysis of variance after subdivision of the population into CKD stage groups according to the GFR. Receiver operating characteristic curve analysis for discrimination of a GFR of less than 60 mL/min indentified the combined parameter as the one with the highest area under the curve (0.78; 95% confidence interval, 0.66-0.89), followed renal length corrected for height (area under the curve, 0.77; 95% confidence interval, 0.66-0.88)., Conclusions: Correction of renal sonographic parameters for body height strengthens the degree of the correlation of renal sonography with the GFR. The improved correlation with the GFR makes renal sonography a reliable tool for a more complete assessment of patients with CKD., (© 2015 by the American Institute of Ultrasound in Medicine.)

Background: Ramipril administered once daily is characterized by an attenuation of its pharmacological activity in the following 24 hours, whose effects on antiproteinuric activity have not yet been investigated., Methods: The antiproteinuric efficacy of Ramipril has been evaluated in a cross-over study in 20 patients with renal disease, proteinuria and hypertension (GFR50 mL / min, proteinuria <3 g / day; SBP/DBP 150/90 mmHg). Proteinuria was measured over 24 hours on three consecutive urine collections (morning, afternoon and night) in the absence of antiproteinuric drugs and after ten days of treatment with single morning administration of Ramipril 2.5 mg or Ramipril 10 mg., Results: At baseline: mean proteinuria was not significantlychanged over the course of the three urinary collections (88 7.2 mg/h in the morning of 80 10.5 mg/h in the afternoon and 81 10.1 mg/hr during the night). After Ramipril 2.5 mg/day: slight reduction in mean proteinuria, with no significant differences between collections (80 11 mg/h in the morning, 69 7.4 mg/h in the afternoon and 75 9.1 mg/h during the night). After Ramipril 10 mg/day: afternoon and night values of proteinuria were significantly reduced compared to baseline; noctural proteinuria was significantly lower than morning value (51 7.5 mg/h vs. 81 10 mg/h, p <0.05)., Conclusion: The antiproteinuric effectiveness of Ramipril tends to decrease significantly over the 24hours after a single daily administration. An increase and/or division of Ramipril dose might help to stabilize and to maximizeits antiproteinuric effectiveness.

Ageing is characterised by a decline in renal function and by a higher susceptibility to renal diseases. This has been defined by Fliser as the "myth of the inexorable decline of renal function with senescence" and is a consequence of multiple factors that predispose to renal damage. These include physiological factors that cannot be modified or treated, and pathological factors that can be treated and, sometimes, prevented; the former are represented by anatomic, molecular, and functional changes that physiologically occur during the ageing process; the latter-by acquired risk factors, whose incidence increases in the elderly, thus predisposing to or aggravating the renal damage. These include increased prevalence of age-related diseases, increased consumption of potentially nephrotoxic drugs, increased necessity of radiological procedures using iodinated contrast media, and increased necessity of major surgery. In this review we analyse these factors and their relevance in increasing the risk of renal damage in the elderly.

Background: Proteinuria is a common presentation of mesangioproliferative glomerulonephritis (MsPGN). No studies are available on the long-term effect of treatment by renin-angiotensin system (RAS) inhibitors on renal outcome in MsPGN patients. This study prospectively evaluates the effects of RAS inhibitors on renal outcome in patients with low risk MsPGN followed up for 10 years using historical patients with similar features at the time of presentation as untreated controls., Endpoints: decrease of basal proteinuria>20% and loss>20% of basal glomerular filtrate rate (GFR) at the end of first year of observation. The patients were re-evaluated bimonthly during the first year and every 6 months thereafter., Results: Twenty-five patients fulfilled the selection criteria. After one year follow-up 19 patients reached the endpoint of proteinuria and no patient reached the endpoint of GFR. No significant change in blood pressure levels (BP) and GFR was registered, by contrast daily proteinuria decreased significantly (p<0.001), falling by 29% at sixth month and 47% at the end of the follow-up. The historical control group consisted of 15 untreated patients seen between 1987 and 1992. The two-way analysis of variance for repeated measures showed greater values of GFR (p<0.001) and lower levels of daily proteinuria (p<0.001) in treated patients as compared to untreated controls., Conclusions: This 10-year follow-up study indicates that the early treatment with RAS inhibitors at low doses favourably influences the long-term renal outcome in proteinuric patients with MsPGN. Limitations were the small sample size and lack of randomization., (Copyright © 2011 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.)

Introduction: In the early years of life, the angular deviation of knee causes anxiety in parents and alert to paediatricians. The problem is often an expression of physiological development of the lower limbs and correction occurs spontaneously. A small number of children has a true pathology, which can lead to functional problems, modification of the knee's axis and muscular-skeletal disorders. We analyzed the prevalence of pathological forms of varus-valgus knee and their association with lower limb dysmetria and scoliotic attitude., Materials and Methods: We evaluated radiographies of the lower limbs of 60 calabrian children; we analyzed the mechanical axis of knee and articular spacing angles. We evaluated the X-ray of the spine with study of Cobb's angle and the gradient of the femoral head., Results: 61.6% of children had a deflection angle of the knee: 6.7% physiological varus and 28.3% physiological valgus, 8.3% pathological varus and 18.3% pathological valgus. Mechanical axis deviation was: +15.6 degrees with articular spacing angle of about +2.4 degrees in pathological varus and +1.95 degrees with articular spacing angle of about -2.09 degrees in pathological valgus. 15% of children had pathological deviation and femoral head dysmetria (7.7 mm). 10% of children had association between pathological valgus/varus knee, dysmetria and scoliotic attitude ( 8.3% sacral-lumbar scoliotic attitude with Cobb's angle <20 degrees )., Conclusions: Without alarm we must pay attention to varus/valgus knee, because probably it will be a physiological deviation. In the pathological condition, it is important to consider the association with the limbs dysmetria that can lead to irreversible scoliotic postural deviation, if ignored for a long time. Unlikely early diagnosis and a subsequent correction of these defects could assurance a good development of skeletal structure.

Background and Aim: Protein-Energy Wasting and inflammation are the principal risk factors of haemodialysis complications. We evaluated the reliability of a simple and non expensive test, the Prognostic Inflammatory and Nutritional Index (PINI), for regular screening of maintenance haemodialysis (MHD) patients in order to detect early onset of inflammation and malnutrition., Methods and Results: 121 adult patients on maintenance dialysis were followed up for 32 months and screened every 6 months for PINI, calculated as alpha1-Acid Glycoprotein (alpha1-AG)xC-Reactive Protein (CRP)/AlbuminxTransthyretin. PINI score < or =1 was considered normal. Patients were stratified according to their PINI score: 86 patients (71.66%) had a normal score, whereas 35 (28.33%) had PINI > or = 1. The latter also had higher CRP levels, despite no clinical evidence of inflammation at the time of enrolment. Survival in patients with normal PINI was similar to patients with normal CRP, while in patients with abnormal PINI it was significantly lower than in patients with low serum albumin (p<0.05) or elevated CRP (p<0.05). After follow-up, all surviving MHD patients with PINI > or = 1 had at least one cardiovascular event vs 2.5% of patients with PINI > or = 1., Conclusion: The assessment of PINI can reliably identify MHD patients at higher risk of mortality and morbidity even in the absence of overt Malnutrition-Inflammation Complex Syndrome (MICS). This simple test appears to be more sensitive and specific of the single components, and not expensive, so that it could be routinely used to identify patients with sub-clinical inflammation and/or malnutrition.

Background: Cooler temperature dialysate (TD) has gained in popularity in the treatment of hypotension during hemodialysis (HD). In this study we verified the hypothesis of an eventual involvement of cytokines., Methods: Four patients on regular HD underwent two 4-hour HD sessions once at 37 degrees C TD and once at 35 degrees C TD. The concentration of the cytokines (cyt) IL-1, IL-2, IL-8, IL-12 and tumor necrosis factor-alpha (TNF-alpha) was measured before the HD session initiation and after 20, 60, 120 and 240 minutes. Body temperature, weight, blood pressure and heart rate were registered at the same time points., Results: We found a higher blood pressure at 35 degrees C but no intradialytic differences in cyt concentration at 35 degrees C or 37 degrees C. The percentage changes of cyt from baseline were very slight, except for IL-8 which decreased by 40% both at 35 degrees C and 37 degrees C., Conclusions: These preliminary results suggest that cytokines do not seem play a relevant role in determining the favorable effects of cooler TD on blood pressure. Our study is preliminary and our results need to be confirmed by other studies.

Objective: Erythropoietin has been shown to have a protective effect in certain models of ischaemia-reperfusion, and in some cases the protection has been correlated with activation of signalling pathways known to play a role in cell survival and proliferation. We have studied whether erythropoietin would overcome direct toxic effects of hydrogen peroxide (H(2)O(2)) treatment to human renal proximal tubular (HK-2) cells., Materials and Methods: HK-2 cells were incubated with H(2)O(2) (2 mm) for 2 h with or without erythropoietin at concentrations of 100 and 400 U/ml, and cell viability/proliferation was assessed by chemical reduction of MTT. Changes in phosphorylation state of the kinases Akt, glycogen synthase kinase-3beta (GSK-3beta), mammalian target of rapamycin (mTOR) and extracellular signal-regulated kinase 1 and 2 (ERK1/ERK2) were also analysed., Results: Cells incubated with H(2)O(2) alone showed a significant decrease in viability, which did not significantly change by addition of erythropoietin at concentration of 100 U/ml, but was further reduced when concentration of erythropoietin was increased to 400 U/ml. Phosphorylation state of the kinases Akt, GSK-3beta, mTOR and ERK1/ERK2 of H(2)O(2)-treated HK-2 cells was slightly altered in the presence of erythropoietin at concentration of 100 U/ml, but was significantly less in the presence of erythropoietin at a concentration of 400 U/ml. Phosphorylation of forkhead transcription factor FKHRL1 was diminished in cells incubated with H(2)O(2) and erythropoietin at a concentration of 400 U/ml., Conclusions: Erythropoietin, at high concentrations, may significantly increase cellular damage in HK-2 cells subjected to oxidative stress, which may be due in part to decrease in activation of important signalling pathways involved in cell survival and/or cell proliferation.

Secondary hyperparathyroidism is a frequent complication of chronic renal failure that can induce severe bone disease and negatively influence the cardiovascular outcome. Therefore, nephrologists should attempt to reach the targets recommended by national and international guidelines using all the available therapeutic strategies. We describe the case of a 37-year-old woman affected by spina bifida and myelomeningocele who had been on hemodialysis since 1993. In July 2006 she developed secondary hyperparathyroidism complicated by peritrochanteric calcifications which did not respond to standard therapy. Because it was impossible to perform a parathyroidectomy, we started medical therapy with a combination of sevelamer hydrochloride, paracalcitol and cinacalcet, which resulted in progressive improvement of laboratory data and osteodystrophy. A diagnosis of mixed secondarytertiary hyperparathyroidism was made, but a progressive increase in iPTH to very high levels suggested a rapid evolution toward a pure tertiary form.

Backgrounds/aims: We recently demonstrated that pulsatile cardiopulmonary bypass (CPB) versus standard linear CPB is associated with better perioperative renal function. Since older subjects have a higher risk of acute renal failure, we have extended our study to evaluate the specific impact of pulsatile CPB on the perioperative renal function in elderly patients., Methods: We enrolled 50 patients with normal preoperative renal function: they were stratified by age (65-75 vs. 50-64 years) and randomized to nonpulsatile (group A) or pulsatile CPB (group B). Twenty-seven patients aged > or =50 years and <65 years were randomized to group A (n = 12) or to group B (n = 15) and 23, aged > or =65 years and < or =75 years, to group A (n = 13) or to group B (n = 10). Glomerular filtrate rate (GFR), daily diuresis, lactatemia and other parameters were measured during the pre- and perioperative period., Results: The percent perioperative decrease in GFR was lower in group A than in group B (p < 0.001), without differences between older and younger patients. By contrast, perioperative plasma lactate levels were higher in group A than in group B (p < 0.001), both in older and younger patients. No difference was observed for 24 h urine output and blood urea nitrogen., Conclusions: Pulsatile CPB preserves renal function better than standard CPB even in patients older than 65. CPB could be adopted as the procedure of choice in this subgroup of patients., (Copyright (c) 2009 S. Karger AG, Basel.)

Background: The monitoring program for patients on regular hemodialysis treatment (RDT) is not well defined yet by current international guidelines (CIG)., Methods: To evaluate the extent to which CIG are implemented, we sent a questionnaire to 100 Italian hemodialysis units (DU) with questions concerning: (a) the frequency with which routine tests were performed for the follow-up of patients on RDT; (b) which other non-routine tests were performed. We analyzed the response data and compared them with the CIG., Results: We received 37 replies. We found several differences between the monitoring program of our respondents and the CIG., Conclusion: Because of the small number of responses, this survey is only preliminary; however, it shows the difficulty nephrologists have in using the CIG to create a correct monitoring program in patients on RDT. Although our analysis is limited to 37 DUs, it suggests that specific guidelines are necessary to optimize the management of patients on RDT.

Background & Aims: Patients with constipation frequently complain of dyspeptic symptoms that may be explained by reflex inhibition of upper-gastrointestinal motor activity by colonic stimuli. We sought to evaluate the following: (1) the prevalence of functional constipation (FC) and gastric emptying characteristics in children with functional dyspepsia (FD), and (2) the efficacy of osmotic laxatives on constipation, dyspeptic symptoms, and gastric motility., Methods: We recruited 42 children (males/females, 22/20; mean age, 80.5 mo) affected by FD (Rome II criteria). All subjects underwent ultrasonographic measurement of the total gastric emptying time (TGEt) at baseline (T0) and after 3 months (T3). Children's bowel habits and the dyspeptic symptomatic score were evaluated at entry and after 1 (T1), 2 (T2), and 3(T3) months. Constipated patients were treated with osmotic laxatives for 3 months. Dyspeptic children without constipation represented the comparison group., Results: FC was present in 28 of 42 (66.6%) patients. Constipated dyspeptic children had significantly more prolonged TGEt than subjects without constipation (median value [interquartile range], 180 (50) vs 150 (28) min, respectively; P = .004). Patients on osmotic laxatives had a significant decrease in TGEt at 3 months (P < .001). The median dyspeptic symptomatic score as well as the number and consistency of evacuations per week significantly improved at T1 in comparison with T0 and even more at T2 and T3 (P < .001, for each)., Conclusions: In our study group, the majority of children with FD were affected by FC associated with delayed gastric emptying. Normalization of bowel habit may improve gastric emptying as well as dyspeptic symptoms.

Background: It is not known whether physical exercise increases daily proteinuria in patients with proteinuric nephropathies, thus accelerating progression of the renal lesion. This study evaluates the acute effects of physical exercise on proteinuria in young adults with immunoglobulin A (IgA) nephropathy., Methods: Changes induced by intense physical exercise on quantitative and qualitative proteinuria were evaluated in basal conditions and after 10 days of ramipril therapy in 10 patients with IgA nephropathy, normal glomerular filtration rate (GFR), proteinuria between 0.8 and 1.49 g/24 h, and "glomerular" microhematuria before and after the end of a maximal treadmill Bruce test (B-test). The basal study also was performed in 10 age- and sex-matched healthy volunteers., Results: At rest, GFR averaged 141 +/- 23 mL/min; it increased by 16.3% +/- 3.3% (P < 0.005) and 7.1% +/- 1.6% at 60 and 120 minutes after the B-test, respectively. At rest, GFR-corrected proteinuria averaged protein of 0.76 +/- 0.21 mg/min/100 mL GFR; it increased to 1.55 +/- 0.28 mg/min/100 mL GFR after 60 minutes (P < 0.001) and declined to 0.60 +/- 0.11 mg/min/100 mL GFR at 120 minutes after the end of the B-test. The pattern of urinary proteins remained unchanged, as did microhematuria. Daily proteinuria was not different from the basal value on the day of the B-test. After ramipril therapy, patients showed a reduction in GFR, but no change in daily GFR-corrected proteinuria, pattern of urinary proteins, or hematuria., Conclusion: The increase in proteinuria after exercise in our patients is significant and is not prevented by ramipril therapy, but lasts less than 120 minutes. Therefore, it cannot modify daily proteinuria. Thus, these data do not support the need to reduce acute physical activity in patients with nonnephrotic renal diseases.