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We have previously reported the one-year outcomes of 16 children with severe proliferative lupus nephritis (LN) who were treated using a multi-targeted induction protocol based on intravenous (IV) pulse methylprednisolone (MP), mycophenolate mofetil (MMF) and cyclosporine (CSA). This study examined the long-term renal outcomes of these 16 children, followed up for a median duration of 9.2 years (range 5.8–14.2 years). Primary treatment outcome was complete renal remission. Secondary outcomes included patient and renal survival as well as relapse-free and event-free survival. All patients achieved complete renal remission within 24 months (median 8.7 months, range 4.0–24.0 months). Comparing clinical and laboratory parameters at induction and last follow-up, respectively, Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score (25.4 ± 8.7 vs 0.4 ± 0.8), serum complement C3 (47 ± 21 vs 107 ± 27 mg/dL), estimated glomerular filtration rate (eGFR) (72 ± 57 vs 109.7 ± 43 ml/min/1.73m2) and urine protein (6.97 ± 7.09 vs 0.2 ± 0.02 g/day/1.73m2) improved significantly ( p 1 g/day/1.73m2. Cumulative probability that hospitalization would not be required was 93.8% at one year, and 71.4% at ten years. Our multi-targeted protocol for induction and maintenance therapy in Asian children with severe proliferative LN resulted in good long-term patient survival and renal preservation, with a good safety profile.

Animal models of chronic kidney disease (CKD) have suggested possible renoprotective roles for carbohydrate restriction, augmentation of polyphenol intake and restriction of dietary iron. Caloric restriction (principally achieved by carbohydrate restriction) has been shown in animal models to prevent renal failure progression independently of dietary protein intake1). Polyphenols inhibit the digestion and absorption of protein, energy and iron, and significantly prolong renal survival in experimental models of glomerulosclerosis2). Finally, iron has been identified as an important factor in the progression of experimental nephropathy after the inciting injury was removed3). The objective of this guideline was to assess the potential effectiveness of any of these dietary interventions on renal failure progression.

A retrospective case-control study of 71 patients with IgA nephropathy was performed to evaluate the long-term prognostic effect of tonsillectomy. Forty-one patients (19 males and 22 females) underwent tonsillectomy (Group A) and 30 patients (13 males and 17 females) did not (Group B). These patients were followed for more than 10 years after renal biopsy. The average age at the initial renal biopsy was 29.78 years in Group A and 33.0 years in Group B. The average follow-up period was 13 years and 3 months in Group A, and 12 years and 7 months in Group B. Glomerular damage demonstrated on renal biopsy was more extensive in Group A than in Group B. Renal prognosis after 10 years of follow-up was compared between these two groups. The clinical remission rate was 24.4% in Group A and 13.3% in Group B; the stable renal function rate was 82.9% in Group A and 70.0% in Group B; and the renal survival rate was 95.1% in Group A and 73.3% in Group B. The renal survival rate in Group A was significantly higher than that in Group B (p

Angiotensinogen gene variation and renoprotective efficacy of renin-angiotensin system blockade in IgA nephropathy.BackgroundBlockade of the renin-angiotensin system (RAS) is well documented to be renoprotective; however, not all patients with glomerulonephritis respond well to this therapy. The interindividual variation in response to the RAS blockade may be in part genetically determined, whereas the results have been controversial.MethodsWe investigated whether the therapeutic efficacy of angiotensin-converting enzyme (ACE) inhibitors and/or angiotensin receptor blocker on renal prognosis is modified by the angiotensinogen gene (AGT) polymorphism in immunoglobulin A nephropathy (IgAN). In total, 259 patients with histologically proven IgAN were analyzed for clinical manifestations, renal survival, and their associations with AGT A(-20)C and M235T.ResultsThe renal prognosis of 110 patients, who received ACE inhibitors/angiotensin receptor blocker during their clinical course, was significantly better than those without ACE inhibitors/angiotensin receptor blockers despite higher blood pressures and heavier proteinuria. The Cox proportional hazards regression model showed an increased hazard ratio (HR) for urinary protein (more than 1.0g/day) of 3.346 (P = 0.0001), hypertension of 1.949 (P = 0.01), deteriorated renal function of 3.040 (P < 0.0001), no ACE inhibitor/angiotensin receptor blocker administration of 2.725 (P = 0.0004), and the T235 and C(-20) haplotype of 1.608 (P = 0.0322). Only in patients carrying at least one M235 and A(-20) haplotype did the administration of ACE inhibitors/angiotensin receptor blockers have no significant effect on the prognosis of renal function (Kaplan-Meier, log rank test, χ2 = 0.700; P = 0.4028), whereas it was significant in patients who had other haplotypes of AGT (χ2 = 11.805; P = 0.0006).ConclusionThis study provides evidence that the M235T and A(-20)C genotype of AGT can influence the therapeutic efficacy of a RAS blockade on the renal survival in IgAN.

We report clinical and histopathological features, treatment and outcome of 37 Croatian children with biopsy-proven lupus nephritis seen over a 30-year period. The mean age at lupus nephritis presentation was 12.11 ± 2.59 years (range 4.66–17.0). The most frequent histopathological finding was class IV (37.8%), followed by class III (35.1%), class V (16.2 %) and class II (10.8 %) lupus nephritis. Compared with other classes there were more boys among patients with class IV lupus nephritis, and hypertension, nephrotic syndrome and decreased estimated glomerular filtration rate at presentation were more common. The median histopathological activity and total scores were highest in class IV lupus nephritis patients. The mean follow-up was 7.14 ± 4.71 years, ranging from 1.1 years to 21.0 years. Kaplan–Meier estimates of patient and kidney (without renal failure) survival rates were 90.5% and 87 % at five years. The renal survival rate of class IV lupus nephritis patients was found significantly lower compared with other histological classes combined. Decreased estimated glomerular filtration rate at the time of diagnosis, class IV lupus nephritis versus other lupus nephritis classes, and high total histological score were the parameters significantly associated with adverse outcome. The therapy with cyclophosphamide showed as superior to the therapy with azathioprine.

Serum bilirubin has been recognized as a novel endogenous antioxidant. The aim of our study was to evaluate the impact of serum bilirubin on kidney prognosis in IgA nephropathy (IgAN).We followed retrospectively 694 patients with IgAN diagnosed by renal biopsy between 1982 and 2010. The risk factors for developing end-stage renal disease (ESRD) were estimated using a Cox proportional hazard model. Predictive performance between models with or without serum bilirubin was evaluated by calculating the net reclassification improvement (NRI) and integrated discrimination improvement (IDI).Seventy-seven patients developed ESRD during the median 4.9 years of follow-up. Estimated glomerular filtration rate, proteinuria and histological severity were inversely related to bilirubin levels. In multivariate analysis, serum bilirubin was an independent risk factor for ESRD (hazard ratio for every 0.1 mg/dL decrease in serum bilirubin, 1.18; 95 % CI, 1.04-1.33). The incidence rate of ESRD decreased linearly with the increases in bilirubin levels (P for trend0.01). When bilirubin was incorporated into a model with conventional ESRD risk factors, the NRI and IDI were 0.281 (P = 0.02) and 0.019 (P = 0.01), respectively.We demonstrated that lower bilirubin levels were significantly associated with higher risk of ESRD in IgAN. In addition, bilirubin provided incremental predictive value in the risk assessment for progression of IgAN beyond that provided by standard risk factors.

Minimal change nephropathy is a common cause of primary nephrotic syndrome in adults. However, there are few studies of its clinical course, response to treatment, and long-term outcome.Retrospective cohort study.340 consecutive adult patients with nephrotic syndrome and biopsy-proven minimal change nephropathy treated in a university hospital from 1984 until 2004.Treatment response groups: primary steroid resistance, frequent relapse (≥4 relapses within 1 year), infrequent relapse (≥1 relapse but not frequent relapse), and no relapse (reference group); disease pattern.Medical problems after diagnosis; patient survival; renal survival.Median time to remission was 10 (IQR, 8-12) weeks; 179 (52.6%) had no relapse, 42 (12.4%) had infrequent relapses, 86 (25.3%) were frequent relapsers or steroid dependent, and 33 (9.7%) had primary steroid resistance. After a median follow-up of 174.7 (IQR, 119.7-235.0) months, 32 patients developed end-stage renal disease and 62 died (25 after progression to end-stage renal disease). Cox regression analysis showed that age and treatment response groups were the independent predictors of patient survival. Compared to the no-relapse group, the infrequent-relapse group had significantly better patient survival (adjusted HR, 0.19; 95% CI, 0.08-0.44; P0.001), whereas the primary-steroid-resistance group had significantly worse patient survival (adjusted HR, 5.87; 95% CI, 1.83-18.85; P0.001). Renal survival was excellent except in the primary-steroid-resistance group.Retrospective study.A substantial proportion of adult patients with minimal change nephropathy continue to have disease flares more than 10 years after the initial presentation, and medical problems after diagnosis are common.

Background: Diffuse mesangioproliferative glomerulonephritis (MesP) is the most commonly diagnosed type of glomerulonephritis (GN) in Denmark, with an incidence of 10.8 million per year. In the present study, the 30-year renal survival was estimated. Methods: A retrospective cohort investigation of 140 patients with biopsy-proven MesP was performed between the period 1967-2006. Factors influencing renal survival were investigated using Cox regression analysis. Results: Renal survival at 5, 10, 20 and 30 years was 87, 78, 59 and 50%, respectively. Female survival after 30 years was significantly better than male survival (70 vs. 40%, p = 0.049). Multivariate analysis, adjusted for age, estimated glomerular filtration rate (GFR) and nephrotic syndrome (NS) was performed for each sex individually. An increase in GFR was associated with a hazard risk (HR) of 0.98 (p = 0.02) in women and 0.99 (p = 0.006) in men. Older age was associated with a HR of 1.04 (p = 0.02) in women and 1.03 (p = 0.004) in men. NS had a poorer prognosis in men (HR 2.53, p = 0.01), but not in women (HR 0.54, p = 0.38). Conclusion: Increasing age and decreasing GFR were adversely associated with renal death. Renal prognosis was better for women after 30 years, and NS resulted in a poorer prognosis in men. This suggests that disease course and prognosis are different between men and women.

Focal and segmental glomerulosclerosis (FSGS) is a histological entity commonly found in children and adults with steroid-resistant nephrotic syndrome (SRNS), with a 10-year renal survival rate in the 40–60% range [1–3]. FSGS has diverse aetiologies, among which genetic, infectious, toxic and inflammatory factors have been identified. In the last few years, significant advances have been made in the identification of genes involved in the development of familial forms of FSGS, thereby revealing molecules which play a central role in podocyte function. Determination of the appropriate genetic diagnosis is crucial, as it allows accurate counselling to be provided for the patients and their families, particularly on the risk of disease transmission.

The importance of renal biopsy in systemic lupus erythematosus (SLE) patients with nephritis resides not only in the diagnosis, but mostly in its role in guiding and assessing therapy. The potential benefits of treatment in lupus nephritis include a dramatic clinical improvement and a direct impact on renal survival. However, these benefits can be achieved only if they are carefully weighed against the complex, prolonged, and potentially toxic treatment regimens. This review on lupus nephritis emphasizes on the histological appraisal of lupus nephritis, which may demonstrate some variations among institutions, but should be clearly defined if one expects to have more useful information for the routine management of patients, as well as in clinical trials.

Primary focal segmental glomerulosclerosis (FSGS) is the representative of refractory nephrotic syndrome in both adults and children. We review the clinical course and predictors of renal outcome in adult FSGS. Patients resistant to treatment frequently develop end-stage renal disease (ESRD), whereas patients achieving a remission show an excellent outcome. The renal survival rate in Japanese patients is 68.7% in 10 years and 31.4% in 20 years, indicating a better prognosis compared with the previous studies. When clinical and histological features at presentation have been evaluated by multivariate analysis, serum creatinine concentrations (>1.5 mg/dl) and the presence of tubulo-interstitial lesions (>20%) are significant positive predictors of progression to ESRD. We also discuss treatment for adult FSGS, with emphasis on intensive and prolonged therapy.

Primary chronic glomerulonephritis is the most common cause of end-stage renal failure in Japan. The incidence in dialysis patients in Japan is about four times higher than in the United States for reason which are unclear. We conducted a nationwide survey on the natural history and treatment of primary glomerulonephritis under a program project from the Ministry of Health and Welfare of Japan entitled ‘Progressive Chronic Renal Disease’. We analyzed patient characteristics, disease onset, clinical data, and histological findings in 1,850 patients with primary glomerulonephritis from 53 institutions in 1985 who underwent renal biopsy at least 5 years ago, and the follow-up study was carried out 8 years after registration. The incidence of diffuse-mesangial proliferative glomerulonephritis is 41.9%, that of minor glomerular abnormalities 17.5%, and that of focal-mesangial proliferative glomerulonephritis 13.0%. Of 1,045 biopsy specimens that were examined by immunofluorescence microscopy, 47.4% showed IgA nephropathy. Half of all cases with primary chronic glomerulonephritis were asymptomatic and were detected on routine health examination. The survival rates at 20 years from the apparent onset or earliest known renal abnormality are: focal glomerular sclerosis 49%, membranoproliferative glomerulonephritis 58%, diffuse-mesangial proliferative glomerulonephritis 66%, focal-proliferative glomerulonephritis 81%, membranous nephropathy 82%, minor glomerular abnormalities 94%, and IgA nephropathy 61%. In conclusion, a high incidence of IgA nephropathy and a better renal survival of membranous nephropathy are the features of primary chronic glomerulonephritis in Japan. This high incidence of IgA nephropathy together with its poor prognosis is probably the reason for the increased incidence of primary chronic glomerulonephritis in dialysis patients in Japan. In addition, the importance of routine health examination including urinalysis is demonstrated.

Despite aggressive therapy, lupus nephritis (LN) remains an important predictor of morbidity in patients with systemic lupus erythematosus. Clinical trials of novel drugs have not improved LN outcomes; however, re-analysis of well-characterized cohorts has identified surrogate end points of long-term renal survival, which will facilitate testing and qualification of novel treatments.

One of the characteristics of systemic lupus erythematosus (SLE) is the large inter-and intra-individual variability of the clinical course. Lupus nephritis is no exception. Some patients with kidney involvement may show rapid progression to renal failure, others may enter complete and stable remission after adequate therapy while a number of other patients, with similar clinical and histological pattern at presentation, may alternate periods of clinical quiescence with renal exacerbations of different severity. In this paper we focus on the incidence, the prognostic significance and the treatment of renal flares in patients with SLE nephritis.

Whether the long-term patient and renal survival of those diagnosed with lupus nephritis (LN) has improved over the decades is still debated. Eighty-nine patients diagnosed between 1968 and 1990 entered this study and their outcome was evaluated after 20 years. At presentation 54% of patients had class IV LN, 39.3% had renal insufficiency and 59.5% had nephrotic syndrome. Patients were divided into two groups: Group 1 consisted of 30 patients diagnosed between 1968 and 1980; Group 2 consisted of 59 patients diagnosed between 1981 and 1990. In Group 1 patient survival at 20 years was 84% versus 95% in Group 2 ( p = 0.05). Survivals without end-stage renal failure were respectively 75% and 84% at 20 years ( p = 0.05). Survivals without severe infection at 20 years were 44% in Group 1 and 66.5% in Group 2 ( p = 0.02). Survivals without cardiovascular events at 20 years were: 53% in Group 1 and 90% in Group 2 ( p = 0.005). At presentation, patients in Group 1 had higher serum creatinine (1.96 vs 1.15 mg/dl, p = 0.01), higher activity index (8 vs 5.5, p = 0.01), lower hematocrit (31% v s6%, p = 0.008) and lower serum C4 levels ( p = 0.04) than Group 2 patients. Patients in Group 1 also received less frequent methylprednisolone pulses (43% v s81%, p = 0.0006). In Italian patients with LN, long-term life expectancy and renal survival progressively improved over the decades, while morbidity progressively declined. An earlier referral and refinement of therapy achieved this goal.

The prevalence of hyperuricemia in patients with chronic kidney disease (CKD) is high, but the management is suboptimal under traditional treatment. This study was conducted to clarify whether febuxostat achieves better renal survival and patient outcome compared with traditional urate-lowering agents (ULAs). In total, 2,460 adults who had continuously received ULAs for at least three months before enrollment were investigated. Three groups were compared prospectively including non-conversion (n = 2,214), conversion (n = 206), and febuxostat first (n = 40). We evaluated laboratory changes, estimated glomerular filtration rate (eGFR) change, eGFR decline, renal survival, and all-cause mortality. The Cox proportional hazard risk analysis were also used for risk prediction. Multiple prescriptions for ULAs were found in both the non-conversion and conversion groups. However, improved median eGFR was noted in the febuxostat group (p

Human immunodeficiency virus (HIV)/AIDS is unique for its devastating impact on social, economic, and demographic developments. Recent studies have thrown more light on the reasons for the mortality and morbidity associated with HIV/AIDS in general and on its renal complications in particular. The classic involvement of the kidney by HIV infection in HIV-associated nephropathy (HIVAN) was reported in New York in 1984. HIVAN clinically presents with proteinuria and renal dysfunction and pathologically with focal segmental glomerulosclerosis. The imaging modality most commonly used in the assessment of renal dysfunction is ultrasonography, followed by computerized tomography. These renal lesions manifest in a protean number of ways in sonography. Renal survival has improved with the introduction of highly active antiretroviral therapy.

Objectives The objective of this study was to evaluate the clinical features, course, outcome, and prognostic indicators in lupus membranous nephritis (LMN) and to compare data of "pure" LMN vs "mixed" forms. Methods We retrospectively examined medical records and kidney biopsies of 103 patients with a diagnosis of LMN. Results Sixty-seven patients had "pure" LMN and 36 had "mixed" forms. Patients with mixed LMN had more frequent nephrotic syndrome (66.6 vs 44.7%, P = 0.05), low C3 (83.3 vs 62.6%, P = 0.05) and C4 (80.5 vs 52.2%, P = 0.005), anti-DNA positivity (86.0 vs 62.6%, P = 0.03), and a tendency toward a lower creatinine clearance (93 ± 29 vs 112 ± 50 mL/min, P = 0.07). Moreover, mixed membranous nephritis had a higher activity and chronicity index (6.5 ± 2.1 vs 1.4 ± 2.03, P = 0.005 and 2.4 ± 1.7 vs 1.4 ± 1.8, P = 0.0001, respectively). Methylprednisolone pulses and immunosuppressive therapy were more often used in patients with mixed forms (86.1 vs 60.6%, P = 0.016 and 83.3 vs 57.5%, P = 0.008, respectively). After a mean follow-up of 156.5 ± 104.5 months, there was no difference in the 2 subgroups concerning the number of patients achieving remission and patient/renal survival (94.5 vs 94.0% and 85.8 vs 86% at 10 years). At multivariate analysis, serum creatinine at presentation ( P = 0.0013), chronicity index ( P = 0.007), failure of achieving remission ( P = 0.000001), and occurrence of nephritic flares ( P = 0.00167) were independent predictors of chronic renal insufficiency. Conclusions Despite the differences in clinical and histological presentation, a therapy tailored on the grounds of clinical and histological features may reduce the differences in the outcome of white patients with mixed and pure membranous nephritis.

Even though IgA nephropathy (IgAN) is not the most common primary glomerulonephritis (GN) in India, the outcome of patients with IgAN in India is poor when compared with other parts of the world, which is a burden in itself.Basic and clinical research work in India on primary IgAN was systematically reviewed. Comparisons between data from India and those from other countries were made.IgAN constitutes between 7% and 16% of most biopsy samples from India, bearing in mind these figures may represent only the tip of the iceberg. Nephrotic syndrome and renal failure seem to be common presenting features. The renal survival rates appear to be dismally low. DD genotype of angiotensin-1 converting enzyme (ACE) gene may predispose the individual to IgAN in Indian population. As might be expected, IgAN can recur posttransplant though the posttransplant course is indolent. There are no data on the treatment aspects of IgAN.Low incidence but marked severity characterizes IgAN in India. It is apparent that IgAN seems to have a poor outcome in India. What we are unsure of is the reason behind it. In-depth basic studies and multicenter clinical trials are needed to address this bizarre pattern.

The discovery of the immunosuppressant Cyclosporin A (CsA) has improved the survival of transplanted kidneys and has made other solid organ transplants a practical reality. Transplant centres were initially reluctant to use CsA in pediatric patients because of a perceived poor-risk benefit ratio of the drug. However, improved transplant survival, largely because of CsA use in adult transplants, made all centres recognize that withholding this drug from children was not warranted. Hirsutism, coarsening of skin, and nephrotoxicity were the major problems associated with its use. However overall pediatric renal survival rates worldwide were below adult survival figures. 1 The need to improve kidney transplant survival has resulted in the widespread use of CsA along with changes in donor profiles and surgical and postoperative care. The change to CsA in pediatric transplantation has become the backbone of most posttransplant immunosuppressant regimes. The previously used myelotoxic drugs such as Imuran (Burroughs Wellcome Inc, Canada) remain in use for triple therapy regimes or for patients in whom CsA toxicity is particularly prominent. We have had a relatively long experience with CsA, and its use in children has been accepted. The question that was originally asked still looms: Do children have any unique toxicity to this drug, and will the dire predictions of centres that first refused to use it in children be borne out with time?

The role of hepatitis C virus infection (HCV) in the etiology and progression of chronic kidney disease (CKD) is controversial.To measure the prevalence of CKD and evaluate its course in patients with chronic HCV infection.A retrospective analysis was done after excluding patients with nephrolithiasis, structural kidney disease, and those with missing clinical information on 552 anti-HCV-positive patients and 313 patients without known HCV infection matched for age, race, and gender. CKD was defined as estimated glomerular filtration rate value of60 mL/min/1.73 m(2) and/or persistence of proteinuria (3 months) on urine analysis by dipstick. Viral load obtained during the initial evaluation was defined as "baseline viral load".The prevalence of CKD in the anti-HCV-positive group was significantly higher compared to control group [53 (9.6%) vs. 16 (5.1%), P = 0.02]. On multivariate regression analysis, higher age, hypertension, HCV PCR 7 × 10(5) cps/mL, and diabetes mellitus were significant independent positive predictors, whereas history of interferon treatment was significant independent negative predictor for CKD. Male gender, human immunodeficiency virus status, body weight, intravenous drug use, and HCV genotype were not predictors of CKD. Analysis of renal survival through Kaplan-Meyer curves revealed significantly shorter time to develop CKD (74 vs. 84 months, P 0.001; log rank) and end-stage renal disease (79.9 vs. 86.5 months, P = 0.005; log rank) in the HCV group compared to the control group.Chronic HCV infection was associated with a significantly higher prevalence of CKD compared with controls, as well as significantly shorter renal survival. A higher baseline viral load is an independent predictor of CKD.

There are three recognized types of Membranoproliferative glomerulonephritis (MPGN). Type II or Dense Deposit Disease (DDD)has a renal survival of 50% at 10 years. The goal of this study was to better identify patients at high risk of early renal failure,and to understand the factors that lead to fast progression of the disease. We identified six diagnostic features on the 98 DDD patients who responed to a Web-based survey, and examined the prognostic performance of these features in isolation and simple combinations. We then combined the features to build predictive models using both Cox proportional hazards regression (CHR), a standard statistical approach, and support vector machines (SVMs), a classification technique from the data mining literature. While the age and gender features showed some prognostic ability, the combined models -- particularly the SVM -- were superior in identifying cases with fast disease progression.This approach can be applied to disease survival analysis and prognosis, and might be useful to healthcare providers and patients in making healthcare decisions.

The production of cytokines by resident and non-resident renal cells during immunoglobulin A nephropathy (IgAN) plays a key role in the progression of renal damage. The aim of this study was to determine if measurements of urinary epidermal growth factor (EGF) and monocyte chemotactic peptide-1 (MCP-1), at the time of renal biopsy, were a predictor of end-stage renal disease ( ESRD) in a cohort of 132 patients with biopsy-proven IgAN. Outcome measures were a doubling of the baseline serum creatinine (sCr) and/or ESRD. Patients with ratios of EGF/MCP-1 in the lowest tertile had a significant decline in renal survival, while patients in the highest tertile maintained 100% renal survival at 48 and 84 months of follow-up. Multivariate Cox's regression analysis showed that the urine EGF/MCP-1 ratio was an independent prognostic factor and indirectly correlated with the combined outcome. The predictive value was also measured by the area under the receiver operating characteristic curve (ROC). The area of the EGF/MCP-1 ratio was significantly higher than that of EGF or MCP-1 alone, histologic grade, creatinine clearance, or proteinuria. Our study suggests that the urinary EGF/MCP-1 ratio may be used as a prognostic marker of ESRD for patients with IgAN.

Previous studies exploring the potential of glucocorticoid therapy on proteinuria and renal survival of patients with IgA nephropathy (IgAN) indicate that corticosteroid therapy is recommended if th

Background: Immunoglobulin A (IgA) nephropathy is the most common form of glomerulonephritis worldwide. Familial and sporadic cases are recognized, and a locus associated with the familial form of the disease was mapped to chromosome 6. Recent data suggest the familial IgA nephropathy form may have a poorer outcome than the sporadic form. Methods: We tested the hypothesis of unequal survival rates between the 2 forms of disease by analyzing time from biopsy to end-stage renal disease in patients of Italian ancestry; 589 patients with sporadic and 96 patients with familial IgA nephropathy. Results: Overall 10- and 20-year renal survival probabilities of the cohort as a whole were 71% and 50%, respectively. Macroscopic hematuria was the modality of clinical presentation in 51% of patients with familial IgA nephropathy and 39% of patients with sporadic IgA nephropathy. At univariable analysis, the sporadic form of IgA nephropathy was associated significantly with increased risk for renal death. However, patients with the sporadic form tended to be more hypertensive and diagnosed later, with signs of more advanced renal disease than those with familial disease at baseline. In the regression model, form of disease lost any independent effect. Only male sex, lower baseline glomerular filtration rate, greater proteinuria, and histopathologic score proved to be independent predictors of disease progression. Treatment with steroids or angiotensin-converting enzyme inhibitors was associated with improved outcomes. Conclusion: Our study does not confirm that familial IgA nephropathy has a worse prognosis than the sporadic form. The similar renal phenotype may support a common pathogenic mechanism underlying familial and sporadic IgA nephropathy.

Few data exist regarding the long-term sequelae of acute renal failure (ARF), and these studies are limited to a few renal conditions. We aim to assess the 3–5-year survival and incidence of renal injury in children who previously developed ARF of varying causes. We queried parents, physicians, and hospital/state vital statistics records to find patient survival in 174 children who previously had ARF and survived to hospital discharge. We assessed the following in 29 children for residual renal injury: (a) microalbuminuria, (b) glomerular filtration rate (GFR) by Schwartz formula, (c) hypertension, and (d) hematuria. The 3–5-year survival of children with ARF who survived to hospital discharge was 139/174 (79.9%). Most deaths (24/35 (68.5%)) occurred within 12 months after initial hospitalization. Combining those who died during initial hospitalization and in subsequent 3–5 years, the overall survival rate was 139/245 (56.8%). In all, 16 children progressed to end-stage renal disease; thus, renal survival was 127/173 (91%). Those with primary renal/urologic conditions had lower renal survival than others (24/35 (68.6%) vs 134/139 (96.4%); P

Monocyte chemoattractant protein (MCP)-1 is closely related to the pathogenesis of the progression of various chronic renal diseases, including IgA nephropathy (IgAN), through its chemoattractant effect on macrophages. However, the correlation of MCP-1 gene polymorphism with the long-term prognosis of Japanese patients with IgAN has not been clearly determined yet.We investigated 277 Japanese patients diagnosed with IgAN based on renal biopsy to clarify the association between the progression of IgAN and MCP-1 gene polymorphism at position A-2518G, which regulates the transcription of the MCP-1 gene.The incidence of endstage renal disease was significantly higher in patients with the AA genotype (47.1%) compared to those with the AG (24.1%) or GG (27.4%) genotype (P = 0.024). Moreover, Kaplan-Meier analysis revealed that the AA genotype significantly facilitated the progression of renal disease (log rank; P = 0.0029), and Cox proportional hazards regression model analysis showed that the AA genotype represented a 2.058-fold risk for the progression of renal disease (P = 0.026) compared to the AG/GG genotype. However, when the patients were treated with angiotensin-converting enzyme inhibitor and/or angiotensin receptor blocker, or corticosteroid, homozygosity for the -2518A allele was not associated with a higher rate of incidence of endstage renal disease. Serum MCP-1 levels were higher although not significantly so, in the patients with IgAN possessing the AA genotype.The AA genotype at MCP-1 -2518 was an independent risk factor for the progression of renal disease in Japanese patients with IgAN, and was closely associated with renal survival.