Presence Of Nephrotic Syndrome Research Articles

Clinicopathological characteristics and prognosis in patients with monoclonal gammopathy and renal damage in central China: a multicenter retrospective cohort study

Renal involvement is common in monoclonal gammopathy (MG); however, the same patient may have both MG and non-paraprotein-associated renal damage. Accordingly, distinguishing the cause of renal damage is necessary because of the different clinical characteristics and associated treatments. In this multicenter retrospective cohort study, we described the clinicopathological characteristics and prognosis of 703 patients with MG and renal damage in central China. Patients were classified as having MG of renal significance (MGRS), MG of undetermined significance (MGUS), or hematological malignancy. 260 (36.98%), 259 (36.84%), and 184 (26.17%) had MGRS, MGUS, and hematological malignancies, respectively. Amyloidosis was the leading pattern of MGRS (74.23%), followed by thrombotic microangiopathy (8.85%) and monoclonal immunoglobulin deposition disease (8.46%). Membranous nephropathy was the leading diagnosis of MGUS (39.38%). Renal pathological findings of patients with hematological malignancies included paraprotein-associated lesions (84.78%) and non-paraprotein-associated lesions (15.22%). The presence of nephrotic syndrome and an abnormal free light chain (FLC) ratio were independently associated with MGRS. The overall survival was better in patients with MGUS than in those with MGRS or hematological malignancies.

A case of seronegative microscopic polyangiitis following the diagnosis of renal pelvic carcinoma.

The incidence rate of malignancy in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is higher than that in the general population. Malignancy has been indicated to be a risk factor or inducer of AAV. Herein, we report the case of a healthy 84-year-old man with seronegative microscopic polyangiitis (MPA) after thediagnosis of renal pelvic carcinoma. Four weeks before admission, his estimated glomerular filtration rate (eGFR) was 85ml/min/1.73m2, and nohematuria or proteinuria was detected. Renal biopsy on admission revealed invasive urothelial carcinoma of the right renal pelvis. On day 15, his eGFR decreased to 30ml/min/1.73m2 without any incitement. The renal specimen extracted via right robot-assisted nephroureterectomy indicated the presence of ANCA-associated glomerulonephritis. On day 37, urinary protein/urinary creatinine level of 6.48g/gCre, serum albumin level of 2.1mg/dL, and eGFR of 20ml/min/1.73m2 indicated the presence of nephrotic syndrome. His blood sputum was analyzed via chest computed tomography, which revealed alveolar hemorrhage. Although his myeloperoxidase-ANCA was negative, he was diagnosed with MPA based on the 2022 American College of Rheumatology/European League Against Rheumatism classification criteria. This is the first case report of MPA orAAV complicated with renal pelvic carcinoma. The clinical indicators demonstrated that renal pelvic carcinoma preceded the onset of MPA. The spatial proximity of both diseases indicated that renal pelvic carcinoma had some influence on MPA development via the mechanism of inflammatory cytokines or neutrophil extracellular traps. Our report may be useful in elucidating the mechanism of MPA development.

#3269 EPIDEMIOLOGY OF PRIMARY FSGS INCLUDING CLUSTER ANALYSIS OVER A 20-YEAR PERIOD

Abstract Background and Aims Focal segmental glomerulosclerosis (FSGS) is one of the leading causes of nephrotic syndrome (NS) in adults. This epidemiological study describes a renal centre's 20 year ‘real world' experience of patients with primary FSGS including cluster analysis. Method This retrospective analysis was conducted on patients diagnosed with primary FSGS at a tertiary renal centre with a catchment population of 1.55 million, over 2 decades. Data collection included baseline demographics, laboratory results, immunosuppression (IS) and outcomes: Complete & partial remission, relapse, progression to renal replacement therapy (RRT) and mortality. A two-step cluster analysis was used to reveal groupings to aid prognostication and treatment decisions. Pre-specified cluster variables were: Serum albumin (sAlb), urine protein: creatinine ratio (uPCR) & estimated glomerular filtration rate (eGFR). Results 87 patients were identified with primary FSGS after exclusion of secondary causes. Mean age was 49.3 years, 60.9% male, 86.2% white with median eGFR 46 ml/min, uPCR 573 mg/mmol, sAlb 33g/l (Table 1). Rates of partial and complete remission were 23% & 49.4% respectively. Progression to RRT occurred in 27.4%, death in 27.4%. Comparing those receiving IS vs not: sAlb was lower 23 g/L vs 40 g/L (p<0.001), uPCR was higher 795 mg/mmol vs 318 mg/mmol (p <0.001). The IS group was more likely to achieve complete remission (62% vs 40%, p = 0.041), but relapsed more 48.6% vs 22% (p<0.027). There were no statistically significant differences between mortality or RRT. The 2-step cluster analysis separated the cohort into 3 clusters, see Fig. 1: Cluster 1 (n = 26) with ‘nephrotic range proteinuria’ mean sAlb 30 g/L, uPCR 778 mg/mmol and eGFR 72 ml/min; Cluster 2 (n = 43) with ‘non-nephrotic range proteinuria’, mean sAlb 39 g/L, uPCR 284 mg/mmol and eGFR 46 ml/min; Cluster 3 (n = 18) ‘NS’ sAlb 20 g/L, uPCR 1117 mg/mmol and eGFR 25 ml/min. IS use was comparable in the NS (cluster 3) and nephrotic range proteinuria (cluster 1) cohorts, but lower in cluster 2 (non-nephrotic range proteinuria) 77.8% & 69.2% vs 11.6% p <0.001. Rates of complete remission were greatest in clusters 1 & 3 vs cluster 2: 57.7% &66.7% vs 37.2% though this did not achieve statistical significance (p 0.067). Conclusion Although there have been several recent US & Asian reviews of primary FSGS, this study provides a review of the epidemiology of FSGS over a 20-year period in a predominantly white cohort in the UK. In general, the patients who received IS had lower sAlb and achieved remission more frequently. Although the aetiology of primary FSGS is unknown, it is hypothesised that it is due to an undiscovered circulating permeability factor. After the exclusion of secondary causes, KDIGO suggests only making the diagnosis in the presence of NS. Despite this the average sAlb in most epidemiological studies of primary FSGS have been above 30g/l. This is likely because of the inclusion of undiagnosed genetic or adaptive FSGS, but it may also be true that NS phenotype alone is too insensitive to diagnose primary ‘antibody driven’ FSGS. Our cluster analysis highlighted 3 potential FSGS phenotypes: A nephrotic cluster that clearly require IS; a cohort with preserved sAlb and non-nephrotic range proteinuria who will benefit from supportive care; lastly a cluster with heavy proteinuria but a sAlb > 30g/l. This group may still have antibody driven disease and thus benefit from immunosuppression and potentially genetic testing. The study received grant support from CSL Vifor

Prevalence of non-alcoholic fatty liver disease in patients with nephrotic syndrome: A population-based study.

Non-alcoholic fatty liver disease (NAFLD) is a global health concern with a prevalence of about 25% amongst United States adults. Its increased prevalence is attributed to increase in patients with obesity and metabolic syndrome, partly due to similar mechanisms of injury. Nephrotic syndrome (NS) is a clinical entity resulting from extensive proteinuria leading to hypoalbuminemia, hyperlipidemia, edema, and other complications. Given its association with hyperlipidemia, there is concern that patients with NS may be at increased risk of NAFLD. To perform a cross-sectional population-based study to investigate the prevalence and risk factors of NAFLD in patients with NS. A large multicenter database (Explorys Inc., Cleveland, OH, United States) was utilized for this retrospective cohort study. A cohort of 49700 patients with a diagnosis of "Non-Alcoholic fatty liver disease" using the Systematized Nomenclature of Medicine-Clinical Terms (SNOMED-CT) between 1999-2022 was identified. Inclusion criteria were age ≥ 18 years, presence of NAFLD, presence of NS. There were no specific exclusion criteria. Univariate and multivariate analysis were performed to adjust for multiple risk factors including age, gender, Caucasian race, NS, type II diabetes mellitus, hypothyroidism, dyslipidemia, obesity, metabolic syndrome and chronic kidney disease. Statistical analysis was conducted using R, and for all analyses, a 2-sided P value of < 0.05 was considered statistically significant. Among the 78734750 individuals screened in this database, there were a total of 49700 subjects with NAFLD. In univariate analysis, the odds of having NAFLD in patients with NS, type 2 diabetes mellitus, hypothyroidism, dyslipidemia, obesity, metabolic syndrome and chronic kidney disease were 14.84 [95% confidence interval (95%CI) 13.67-16.10], 17.05 (95%CI 16.78-17.32), 6.99 (95%CI 6.87-7.11), 13.61 (95%CI 13.38-13.84), 19.19 (95%CI 18.89-19.50), 29.09 (95%CI 28.26--29.95), and 9.05 (95%CI 8.88-9.22), respectively. In multivariate analysis, the odds of having NAFLD amongst patients with NS were increased to 1.85 (95%Cl 1.70-2.02), while the odds were also remained high in patients that have type 2 diabetes mellitus [odds ratio (OR) 3.84], hypothyroidism (OR 1.57), obesity (OR 5.10), hyperlipidemia (OR 3.09), metabolic syndrome (OR 3.42) and chronic kidney disease (OR 1.33). Patients with NS are frequently found to have NAFLD, even when adjusting for common risk factors. Hence, clinicians should maintain a high index of suspicion regarding presence of NAFLD in patients with NS.

Clinical and pathologic features of primary membranous nephropathy in Turkey: a multicenter study by the Turkish Society of Nephrology Glomerular Diseases Working Group

Background We aimed to evaluate the features of primary membranous nephropathy (MNP) in Turkish people. Methods This is a retrospective analysis of patients with biopsy-proven primary MNP. We obtained the data collected between 2009 and 2019 in the primary glomerulonephritis registry of the Turkish Society of Nephrology Glomerular Diseases Study Group (TSN-GOLD). Patients with a secondary cause for MNP were excluded. Clinical, demographic, laboratory, and histopathological findings were analyzed. Results A total of 995 patients with primary MNP were included in the analyses. Males constituted the majority (58.8%). The mean age was 48.4 ± 13.9 years. The most common presentation was the presence of nephrotic syndrome (81.7%) and sub nephrotic proteinuria (10.3%). Microscopic hematuria was detected in one-third of patients. The median estimated glomerular filtration rate (eGFR) was 100.6 mL/min/1.73 m2 (IQR, 75.4–116.3), and median proteinuria was 6000 mg/d (IQR, 3656–9457). Serum C3 and C4 complement levels were decreased in 3.7 and 1.7% of patients, respectively. Twenty-four (2.4%) patients had glomerular crescents in their kidney biopsy samples. Basal membrane thickening was detected in 93.8% of cases under light microscopy. Mesangial proliferation and interstitial inflammation were evident in 32.8 and 55.9% of the patients, respectively. The most commonly detected depositions were IgG (93%), C3 complement (68.8%), and kappa and lambda immunoglobulin light chains (70%). Although renal functions were normal at presentation, vascular, interstitial, and glomerular findings were more prominent on biopsy in hypertensive patients. No significant effect of BMI on biopsy findings was observed. Conclusions Despite some atypical findings, the main features of primary MNP in Turkey were similar to the published literature. This is the largest MNP study to date conducted in Turkish people.

Urinary thrombin as a marker of local disseminated intravascular coagulation in patients with chronic kidney disease

The aim of this research was to study the diagnostic markers of nonovert local disseminated intravascular coagulation (DIC) syndrome in the urine of patients with chronic kidney disease (CKD). We conducted a prospective study involving 140 patients with CKD, of these patients, 100 patients (71.4%; 95% CI 53.4-76.7) had glomerulonephritis (GN) and 40 patients (28.6%; 95% CI 21.3-36.8) had diabetic nephropathy (DN). We diagnosed overt DIC syndrome on the International Society of Thrombosis and Haemostasis (ISTH) scale (&gt;5 points) in 18.6 % of patients. We determined the level of thrombin in the urine of patients who had &lt;5 points on ISTH scale for the diagnosis of local nonovert DIC syndrome in the kidneys. In the urine of healthy individuals, the level of thrombin did not exceed 1 ng/ml, so we found no thrombinuria at a thrombin level &lt;1 ng/ml. In 56.1% of patients, we found urinary thrombin levels &gt;1 ng/ml. The average level of thrombin in the urine of these patients was 6.5 (4.8; 10.6) ng/ml. In our opinion, the presence of thrombinuria indicates the intensity of monocytic-macrophage inflammation in the glomeruli and may be a criterion for nonovert, local DIC syndrome in the kidneys. The association of overt DIC syndrome with decreased blood albumin, reduced glomerular filtration rate (GFR), increased daily protein excretion (DPE) indicates its occurrence in severe underlying disease, in the presence of nephrotic syndrome and in the severe stages of CKD. Early diagnosis of nonovert local DIC syndrome would be more useful, since the process is still reversible and controlled, and timely use of antiplatelet and anticoagulant therapy would affect the course and the progression of CKD.

Comparison of Patients with Hospital-Recorded Nephrotic Syndrome and Patients with Nephrotic Proteinuria and Hypoalbuminemia: A Nationwide Study in Denmark.

Registry-based studies of nephrotic syndrome (NS) may only include a subset of patients with biochemical features of NS. To address this, we compared patients with laboratory-recorded nephrotic proteinuria and hypoalbuminemia to patients with hospital-recorded NS. We identified adult patients with first-time hospital-recorded NS (inpatients, outpatients, or emergency-room visitors) in the Danish National Patient Registry and compared them with adults with first-time recorded nephrotic proteinuria and hypoalbuminemia in Danish laboratory databases during 2004-2018, defining the date of admission or laboratory findings as the index date. We characterized these cohorts by demographics, comorbidity, medication use, and laboratory and histopathologic findings. We identified 1139 patients with hospital-recorded NS and 5268 patients with nephrotic proteinuria and hypoalbuminemia; of these, 760 patients were identified in both cohorts. Within 1 year of the first record of nephrotic proteinuria and hypoalbuminemia, 18% had recorded hospital diagnoses indicating the presence of NS, and 87% had diagnoses reflecting any kind of nephropathy. Among patients identified with nephrotic proteinuria and hypoalbuminemia, their most recent eGFR was substantially lower (median of 35 versus 61 ml/min per 1.73 m2), fewer underwent kidney biopsies around the index date (34% versus 61%), and the prevalence of thromboembolic disease (25% versus 17%) and diabetes (39% versus 18%) was higher when compared with patients with hospital-recorded NS. Patients with nephrotic proteinuria and hypoalbuminemia are five-fold more common than patients with hospital-recorded NS, and they have a lower eGFR and more comorbidities. Selective and incomplete recording of NS may be an important issue when designing and interpreting studies of risks and prognosis of NS.

POS-143 SYPHILIS INDUCED MEMBRANOUS NEPHROPATHY. A CASE REPORT

The rate of reported cases of syphilis is rising. More than 85% of primary and secondary syphilis occurs in men. This is attributed to increased cases of men who have sex with men. It accounts for more than 50% of all cases of primary and secondary syphilis. Renal manifestations of syphilis may present with variable degrees of proteinuria, acute nephritis with hypertension (HTN) or acute kidney injury (AKI). Pathologically, membranous or endocapillary GN may be seen. We present a case of secondary syphilis in a young man with severe nephrotic syndrome, AKI and HTN A 28 years old male patient presents to the ER with severe facial edema for 2 days and skin rash for one week. He presents mouth and genital ulcers one month ago. He has no urinary symptoms, headache, fever or arthralgia. He had multiple unprotected sexual relations with the same sex for few months. His past and family history is irrelevant, and he doesn’t take any medication. Physical examination reveals generalized edema, maculopapular rash all over the body and increased blood pressure. Chest, heart, abdominal and neurological examination in normal. nPCR testing of CSF is negative. Lab work shows severe nephrotic syndrome with 24 h proteinuria of 32.89 g and serum Albumin of 9 g/L. Urine sediment reveals hyaline, granular, waxy casts and microscopic hematuria. Serum creatinine is increased with very low natriuresis denoting prerenal factor due to severe hypovolemia secondary to nephrotic syndrome. Liver enzymes are elevated. Blood picture is normal. Immunological profile including ANCA, Anti GBM, ANA, Anti ds DNA, ENA screen, RF, C3, C4, PPE is normal. VRDL and Syphilis RPR returns positive. Serology of other sexually transmitted and infectious diseases as HIV, HBV, HCV, CMV, Toxoplasma, gonorrhea, chlamydia is negative. Renal biopsy makes the diagnosis of Membranous Nephropathy (MN) stage 1. The absence of PLA2R antibodies and IgG4 with the predominance of IgG3 favors secondary form of MN. The patient received Benzathine penicillin, Albumin/Furosemide, anti-hypertensive treatment and thromboprophylaxis. Prognosis is excellent with dramatic response to penicillin, disappearance of proteinuria and normalization of renal function and liver enzymes after 4 weeks. The presence of nephrotic syndrome with maculopapular rash occurring in a male patient with history of unprotected sexual relations with the same sex may be related to renal complication of syphilis. The most common pathology is MN. Treatment of the cause is associated with complete cure and excellent prognosis.

Serum uric acid and renal survival prognosis in primary glomerulonephritis patients in a retrospective single-center cohort

Introduction: The role of serum uric acid (SUA) concentration in primary glomerulonephritis (PGN) aggravation is currently under active discussion. Objectives: This study primarily aimed to analyze the association between SUA concentration and renal survival prognosis in PGN patients and secondarily to determine whether hyperuricemia is an independent risk factor for reduced glomerular filtration rate (GFR) in the presence of nephrotic syndrome. Patients and Methods: We performed a retrospective observational single-center study involving 344 patients with biopsy-proved or clinically diagnosed PGN with the mean follow-up period of 5.3 [3.8-6.2] years. The rate of annual decline in estimated GFR (eGFR) was used to assess chronic kidney disease progression. Primary outcome measures were eGFR decline or transfer to renal replacement therapy (RRT) during the 5-year follow-up period. Results: There were 78/344 (22.7%) patients who eventually progressed to eGFR &lt;15 mL/min/1.73 m2 or started RRT. In multivariate logistic regression analysis eGFR at diagnosis, proteinuria and hyperuricemia were associated with increased renal risk in PGN patients during the 5-year follow-up period. However, a less significant effect of SUA on rapid eGFR decline was found in the patients with nephrotic-range proteinuria compared with the patients with mild proteinuria. Conclusion: Our study revealed that a higher level of SUA was significantly associated with a greater annual decline in GFR and, consequently, a worse 5-year renal survival prognosis in PGN patients. The effect of hyperuricemia on the risk of rapid CKD progression was greater in PGN patients with mild proteinuria compared with the patients with nephrotic-range proteinuria.

P0377THE RELATIONSHIP BETWEEN SEVERITY OF INTERSTITIAL FIBROSIS AND ANEMIA IN PATIENT WITH PRIMARY GLOMERULONEPHRITIS: THE DATA FROM TSN-GOLD WORKING GROUP

Abstract Background and Aims Anemia is one of the most important complications in chronic renal disease and inadequate erythropoietin production is the most important reason for anemia. Fibrosis of renal interstitium may be associated with inadequate synthesis of erythropoietin and anemia. In our study, we investigated the relationship between the severity of interstitial fibrosis (IF) in patients with primary glomerulonephritis (PGN) and anemia parameters. Method A total of 2794 patients who were recorded to the database of the Turkish Society of Nephrology, Glomerular Diseases (TSN-GOLD) Working Group between May 2009 and June 2019 were included in our national multicenter (44 centers) study. Patients aged 16 years or more with documented biopsy were included in the study. Patients were divided into four groups according to IF severity. IF was not detected in the interstitial area, was defined as none, if fibrosis was present in less than 25% of the area, defined as mild, if fibrosis was present in 25-50%, defined as moderate and if more than 50%, defined as severe. Anemia was defined as hemoglobin &amp;lt;12 g/dl in adult female patients and &amp;lt;13 g/dl in adult male patients, according to Clinical Practice Guidelines for anemia of CKD published by The Kidney Disease: Improving Global Outcomes Foundation. The relationship between IF severity and anemia parameters at the time of biopsy was investigated. Results In the study, 57% of the patients were male and their mean age was 41.0 ± 14.3 years. The mean eGFR was 82.7 ± 36.9 ml / min / 1.73 m2 and the hemoglobin level was 13.1 ± 1.9 g/dl. General characteristics of patients with and without anemia are given in Table 1. While 41.1% of patients had no IF, 41.2% had mild, 14.1% had moderate and 3.5% had severe IF. When the patients were divided into four groups according to the severity of IF, the mean hemoglobin and hematocrit levels were significantly different between the groups (Figure 1). Anemia was present in 960 patients (34.4%) (Figure 2). Anemia rate was significantly higher in the patients with IF than patients without IF (36.8% vs 30.9%, p = 0.001). Anemia rate was %25.1 in patients with eGFH≥60 ml/min/1.73m2 and 56% in patients with eGFR&amp;lt;60 ml/min/1.73m2. Gender, serum albumin, eGFR, proteinuria, presence of diabetes, presence of nephrotic syndrome, and presence of IF were the independent variables in the regression analysis of hemoglobin-related factors (Table 2). Conclusion Approximately one-third of PGN patients had anemia at the time of biopsy and the frequency of anemia increases in patients with IF. The presence of IF is an independent risk factor associated with the development of anemia in PGN. The elucidation of the etiopathogenesis of anemia in PGN patients may play an important role in the follow-up and treatment of patients. Studies on IF can play a critical role.

Acute Kidney Injury Following Methimazole Initiation: A Case Report

Objective: Nephritis has been rarely associated with methimazole, primarily in the development of nephrotic syndrome. We describe a case of acute kidney injury without evidence of nephrotic syndrome following methimazole initiation. Methods: We present the relevant history, laboratory data, and nuclear medicine data and review relevant documentation from the literature. Results: A 72-year-old male recently diagnosed with new-onset atrial fibrillation was found to have suppressed thyroid-stimulating hormone (TSH) levels; elevated free T3, T4, and thyroid-stimulating immunoglobulin (TSI) levels; and a nonnodular thyroid gland with normal iodine uptake. He was diagnosed with Graves’ disease and treated with propylthiouracil (PTU) for 5 years. When his poor compliance with PTU was impeding his antithyroid treatment, he was converted to methimazole. Within 1 month following methimazole initiation, his serum creatinine (SCr) had risen to 1.6× baseline in the absence of other contributing nephrotoxins. SCr returned to baseline within 2 weeks of methimazole discontinuation, and the patient was subsequently managed on PTU. Conclusion: Acute kidney injury with or without the presence of nephrotic syndrome may occur during treatment with methimazole. Renal function should be closely monitored after the initiation of methimazole to prevent progressive renal dysfunction.

Differentiating Primary, Genetic, and Secondary FSGS in Adults: A Clinicopathologic Approach.

FSGS describes a renal histologic lesion with diverse causes and pathogenicities that are linked by podocyte injury and depletion. Subclasses of FSGS include primary, genetic, and secondary forms, the latter comprising maladaptive, viral, and drug-induced FSGS. Despite sharing certain clinical and histologic features, these subclasses differ noticeably in management and prognosis. Without an accepted nongenetic biomarker that discriminates among these FSGS types, classification of patients is often challenging. This review summarizes the clinical and histologic features, including the onset and severity of proteinuria as well as the presence of nephrotic syndrome, that may aid in identifying the specific FSGS subtype. The FSGS lesion is characterized by segmental sclerosis and must be differentiated from nonspecific focal global glomerulosclerosis. No light microscopic features are pathognomonic for a particular FSGS subcategory. The characteristics of podocyte foot process effacement on electron microscopy, while helpful in discriminating between primary and maladaptive FSGS, may be of little utility in detecting genetic forms of FSGS. When FSGS cannot be classified by clinicopathologic assessment, genetic analysis should be offered. Next generation DNA sequencing enables cost-effective screening of multiple genes simultaneously, but determining the pathogenicity of a detected genetic variant may be challenging. A more systematic evaluation of patients, as suggested herein, will likely improve therapeutic outcomes and the design of future trials in FSGS.

Kidney disease and venous thromboembolism: Does being woman make the difference?

The risk of venous thromboembolism (VTE) is increased across the spectrum of chronic kidney disease (CKD), from mild to more advanced CKD, and typically characterizes nephrotic syndrome (NS). VTE risk in patients with kidney disease may be due to underlying hemostatic abnormalities, including activation of pro-thrombotic factors, inhibition of endogenous anticoagulation systems, enhanced platelet activation and aggregation, and decreased fibrinolytic activity. The mechanisms involved differ depending on the cause of the kidney impairment (i.e. presence of NS or CKD stage). Sex and gender differences, as well as, environmental factors or comorbidities may play a modulating role; however, specific sex and gender data on this topic are still rare. The aim of the present review is to discuss the VTE risk associated with impairment of kidney function, the potential mechanism accounting for it and the impact of sex differences in this clinical setting.

Correlations of serum magnesium with dyslipidemia in patients on maintenance hemodialysis

Correlations of serum magnesium with dyslipidemia in patients on maintenance hemodialysis

Serum levels of 12 renal function and injury markers in patients with glomerulonephritis.

INTRODUCTION Glomerulonephritis (GN) is a complex disease that affects the function of the whole nephron. There are few data on the serum levels of the most common biomarkers of kidney function and injury in GN, or the studies provide ambiguous results. OBJECTIVES The aim of the study was to evaluate the levels of known kidney-specific and nonspecific markers of renal function or injury in the serum of patients with diagnosed primary or secondary GN, with or without the presence of nephrotic syndrome (NS) and arterial hypertension (AH). PATIENTS AND METHODS The study included 58 patients with diagnosed GN and 6 patients with congenital defects (CD) of the kidney and AH (CD+AH). The serum levels of β2-microglobulin (β2M), neutrophil‑gelatinase associated lipocalin (NGAL), osteopontin, trefoil factor 3 (TFF-3), calbindin, glutathione-S‑transferase- π (GST-π), interleukin 18 (IL-18), kidney injury molecule 1 (KIM-1), and monocyte chemoattractant protein 1 (MCP-1) were measured with Kidney Toxicity Panels 1 and 2 using the Bio-Plex method. Renalase levels were measured using an enzyme-linked immunosorbent assay. RESULTS In the whole group and in the subgroups (GN, GN+AH, GN+NS, CD+AH), NGAL, KIM-1, TFF-3, IL-18, β2M, and calbindin levels correlated with estimated glomerular filtration rate (eGFR). In patients with NS, this correlation for calbindin was reversed. Renalase, MCP-1, GST-π, and osteopontin levels were independent of eGFR. Increase in IL-18 levels in the group with GN was assiociated with lower odds of the kidney disease. When this group was divided according to eGFR into subgroups G1-G5, TFF-3, NGAL, and β2M levels increased with the stage of the disease. CONCLUSIONS In patients with NS, renalase and MCP-1 might regulate each other's levels. Further studies are needed to investigate associations between renalase, MCP-1, and osteopontin as factors unrelated to eGFR in GN. NS may contribute to the loss of calbindin from serum. NGAL, KIM-1, TFF-3, IL-18, β2M, and calbindin are good indicators of kidney function loss in patients with GN.

Estimation of Daily Proteinuria in Patients with Amyloidosis by Using the Protein-To-Creatinine ratio in Random Urine Samples.

Measurement of daily proteinuria in patients with amyloidosis is recommended at the time of diagnosis for assessing renal involvement, and for monitoring disease activity. Renal involvement is usually defined by proteinuria >500 mg/day. We evaluated the accuracy of the random urine protein-to-creatinine ratio (Pr/Cr) in predicting 24 hour proteinuria in patient with amyloidosis. We compared results of random urine Pr/Cr ratio and concomitant 24-hour urine collections in 44 patients with amyloidosis. We found a strong correlation (Spearman’s ρ=0.874) between the Pr/Cr ratio and the 24 hour urine protein excretion. For predicting renal involvement, the optimal cut-off point of the Pr/Cr ratio was 715 mg/g. The sensitivity and specificity for this point were 91.8% and 95.5%, respectively, and the area under the curve value was 97.4%. We conclude that the random urine Pr/Cr ratio could be useful in the screening of renal involvement in patients with amyloidosis. If validated in a prospective study, the random urine Pr/Cr ratio could replace the 24 hour urine collection for the assessment of daily proteinuria and presence of nephrotic syndrome in patients with amyloidosis.

C1q Nephropathy: The Unique Underrecognized Pathological Entity.

C1q nephropathy is a rare glomerular disease with characteristic mesangial C1q deposition noted on immunofluorescence microscopy. It is histologically defined and poorly understood. Light microscopic features are heterogeneous and comprise minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), and proliferative glomerulonephritis. Clinical presentation is also diverse, and ranges from asymptomatic hematuria or proteinuria to frank nephritic or nephrotic syndrome in both children and adults. Hypertension and renal insufficiency at the time of diagnosis are common findings. Optimal treatment is not clear and is usually guided by the underlying light microscopic lesion. Corticosteroids are the mainstay of treatment, with immunosuppressive agents reserved for steroid resistant cases. The presence of nephrotic syndrome and FSGS appear to predict adverse outcomes as opposed to favorable outcomes in those with MCD. Further research is needed to establish C1q nephropathy as a universally recognized distinct clinical entity. In this paper, we discuss the current understanding of pathogenesis, histopathology, clinical features, therapeutic options, and outcomes of C1q nephropathy.

Focal segmental glomerulosclerosis: towards a better understanding for the practicing nephrologist

Focal and segmental glomerulosclerosis (FSGS) is a common histopathological lesion that can represent a primary podocytopathy, or occur as an adaptive phenomenon consequent to nephron mass reduction, a scar from a healing vasculitic lesion, direct drug toxicity or viral infection among other secondary causes. Thus, the presence of an FSGS lesion in a renal biopsy does not confer a disease diagnosis, but rather represents the beginning of an exploratory process, hopefully leading ultimately to identification of a specific etiology and its appropriate treatment. We define primary FSGS as a 'primary' podocytopathy characterized clinically by the presence of nephrotic syndrome in a patient with an FSGS lesion on light microscopy and widespread foot process effacement on electron microscopy (EM). Secondary FSGS is commonly characterized by the absence of nephrotic syndrome and the presence of segmental foot process effacement on EM. Failure to accurately differentiate between the primary and secondary forms of FSGS has resulted in many patients undergoing unnecessary immunosuppressive treatment. Here, we review some key points that may assist the practicing nephrologist to distinguish between primary and secondary FSGS.

Histological pattern of primary glomerular diseases among adult Sudanese patients: A single center experience

In Sudan, like most developing countries, the incidence and histologic patterns of primary glomerulonephritis (GN) remains undetermined. A cross-sectional hospital-based prospective study was performed to determine the pattern of primary GN among adult Sudanese patients. The study was conducted at Khartoum Teaching Hospital from September 2010 to August 2011. It included all adult patients with suspected primary glomerular disease and who underwent native kidney biopsy. A total of 83 adult patients were biopsied with 71 patients (85.5%) being diagnosed as having primary GN. Among those with primary GN the mean age was 34.6 ± 18 years and males represent 54.9%. In 46.5% kidney biopsy was indicated by the presence of nephrotic syndrome, whereas in 33.8% biopsies were performed due to unexplained elevations in renal parameters. Primary focal segmental glomerulosclerosis (FSGS) was diagnosed in 29.6% of patients, followed by membrano-proliferative glomerulonephritis (MPGN) in 26.8% and minimal change disease in 16.9%. IgA nephropathy was observed in 5.6%. Complications following kidney biopsies were reported in 9.6% of biopsied patients. Nephrotic syndrome in an adult was the most common indication for kidney biopsy in our unit. A large number of patients were biopsied due to elevated renal parameters, which reflected the increasing awareness toward thoroughly diagnosing patients with suspected reversible kidney damage. In conclusion, FSGS and MPGN make the most common causes of primary GN among Sudanese adults. Creation of a national renal registry is essential for obtaining more specified epidemiological data.

Acute Tubular Necrosis and Renal Failure in Patients with Glomerular Disease

Renal failure is common in patients with glomerular disease. Although renal failure may result from the glomerular lesion itself, it is also observed in patients with minimal glomerular alterations. Degenerative changes and necrosis of the tubular epithelium are common findings in kidney biopsies from these patients. The aim of this work is to examine the association between acute tubular necrosis (ATN) and renal failure in patients with glomerulopathy and to estimate the relationship between the degree of ATN and renal failure in these patients. Data on age, sex, presence of nephrotic syndrome, and renal failure were recorded for 149 patients, who underwent a renal biopsy for the diagnosis of glomerulopathy. The biopsies were reviewed, and ATN, when present, was classified as one of four grades depending on its intensity. The mean age of the patients was 21 ± 16 years. Eighty patients (54%) were male, 43 (42%) had renal failure, 104 (72%) had nephrotic syndrome, and 66 (45%) had minimal change disease or focal segmental glomerulosclerosis. ATN was present in 115 (77%) patients. The frequency of renal failure was directly correlated with the intensity of ATN [odds ratio (OR) of 26.0 for patients with grade 2 lesions and OR of 45.5 for patients with grade 3 lesions]. ATN is a common finding in the biopsies of patients with glomerulopathy. The severity of ATN is directly associated with the frequency of renal failure in these patients.