This study was carried out to look for diagnostic and prognostic role of neutrophil gelatinase-associated lipocalin (NGAL) in early diabetic nephropathy (DN) in type 2 diabetes individuals. NGAL was measured in both urinary and serum sample of 144 type 2 diabetes individuals stratified into three categories based on urinary albumin-creatinine ratio and 54 control populations with estimated glomerular filtration rate >60 mL/min/1.73 m² and serum creatinine <1.2 mg/dl. The serum NGAL (sNGAL), urine NGAL (uNGAL), and uNGAL/urine creatinine were significantly higher in diabetic individuals than in the control populations with significant difference in between the groups (P < 0.05). Difference of above values between control value and normoalbuminuria was also statistically significant (P < 0.05). Again, sNGAL and uNGAL correlate positively with albuminuria (P < 0.05). Tubular injury may precede glomerular injury in diabetic individuals, and NGAL can be used as a biomarker to diagnose DN even earlier to incipient nephropathy. Both sNGAL and uNGAL can predict albuminuria and be used as a noninvasive tool for diagnosis, staging, and progression of DN.

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Systemic lupus erythematosus is common in our country, and renal involvement is an important cause of chronic kidney disease. This study was aimed at comparing the three regimens, i.e., cyclophosphamide-based regimes (low dose and high dose) and mycophenolate mofetil (MMF)-based regime and determining if cyclophosphamide (CPM)-based regime can be an effective, safe, and cheap alternative to MMF-based regime in a resource-limited setting. Out of 144 patients, females constituted 89%. Nephrotic nephritic presentation was the most common. Rapidly progressive renal failure was seen in in 42 (29.1%) patients. Class IV was the most common 66 (45.8%) histological class. Crescentic glomerulonephritis was seen in 18 (12.5%). Overall remission (complete + partial) at 6 months was seen in 71.4% in National Institute of Health regime, 65% in European lupus nephritis trial protocol and 72.9% in MMF regime. End-stage renal disease and switching to other therapies were comparable among the three groups. Although infections were more with CPM, the difference was not statistically significant. CPM-based therapies were associated with a significantly lower cost.

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Calcineurin inhibitors (CNIs) are the preferred drugs for treatment of childhood steroid-resistant nephrotic syndrome (SRNS) who are also resistant to cyclophosphamide (CYC). Although few studies have shown a benefit of one over the other, efficacy and safety of either CNIs (tacrolimus [TAC] or cyclosporine [CSA]) in this special population remained to be assessed in long-term studies. Forty-five children with SRNS who were also resistant to CYC (CYC-SRNS) from January 2006 to June 2011, were included in the study. Patients were treated with CNI either TAC or CSA based on 1:1 allocations and were prospectively observed. Patients who were nonresponsive to CNIs had been treated with mycophenolate mofetil. Outcomes were measured in terms of remission of NS, adverse effects of drugs, and progression of disease. After 6 months of treatment, 16/23 (69.5%) patients on CSA achieved remission and 18/22 (81.8%) on TAC achieved remission (P = 0.3). The side effects hypertrichosis, and gum hyperplasia were significantly less in TAC group as compared to CSA group (P < 0.001). The 1-, 2-, 3-, 4-, and 5-year estimated renal survival (doubling of serum creatinine as event) in CSA group was 96%, 91%, 85%, 54%, and 33% and in TAC group was 96%, 95%, 90%, 89%, and 79%, respectively (P = 0.02). Although TAC and CSA are equally efficacious, TAC has significantly less side effects. The long-term outcome of renal function was significantly better in patients who were treated with TAC as compared to CSA.

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Suspicion and subsequent detection of renal disease is by an assessment of the urinalysis and renal function in the clinical context. Our attempt in this study is to correlate initial presenting features of urinalysis and renal function to the final histopathological diagnosis. A retrospective analysis of 1059 native kidney biopsies performed from January 2002 to June 2015 at Amrita Institute of Medical Sciences was conducted. Correlative patterns between urinalysis, renal function, and final histopathological diagnosis were studied. Five hundred and eleven (48%) patients had nephrotic syndrome. Out of these, 193 (38%) had pure: nephrotic syndrome, 181 (35.8%) had associated microhematuria, 110 (21.7%) had microhematuria and renal failure, and 27 (5.3%) had only associated renal failure. Minimal change disease (MCD) (30%), membranous nephropathy (30%), and IgA nephropathy (29%) were the major diseases in the respective groups. Five hundred and five (47.6%) patients had subnephrotic proteinuria. Out of these, 29 (5.6%) had only subnephrotic proteinuria, 134 (27%) had additional microhematuria, 300 (59%) had subnephrotic proteinuria, microhematuria, and renal failure, and 42 (8%) had subnephrotic proteinuria with renal failure. Lupus Nephritis (45% and 40%) and IgA Nephropathy (32% and 21%) were the major disorders in the subgroups respectively. Forty-two patients (3.7%) were biopsied for isolated renal failure with bland urinary sediment. Cast nephropathy and acute interstitial nephritis were the major diseases. Out of 89 patients with diabetes who were biopsied, 15 (16.8%) had diabetic nephropathy, 45 (50.5%) had no diabetic nephropathy, and 29 (32.5%) had diabetic nephropathy along with a non-diabetic renal disease. Postinfectious glomerulonephritis was the major glomerular disease. IgA nephropathy (22.2%) and membranous nephropathy (15.5%) were the major diseases in patients with diabetes with no diabetic nephropathy. In our population, MCD and membranous nephropathy formed the majority of diseases in biopsied nephrotic syndrome. Added microhematuria did not seem to decrease the incidence of either disease on the whole. We found a significant number of patients with membranous nephropathy with nephrotic syndrome, microhematuria, and additional renal failure. IgA nephropathy formed a majority of cases with nephrotic syndrome, microhematuria, and renal failure. The presence of renal failure regardless of other abnormalities in urinalysis showed a trend toward IgA nephropathy. Membranous nephropathy may have a more varied presentation than was originally thought and IgA nephropathy presenting as nephrotic syndrome may not be uncommon. MCD is the major subgroup of diseases in the pediatric population and presents both as nephrotic syndrome as well as nephrotic syndrome with microhematuria. Thus, urinalysis and renal failure may be a valuable tool in assessing renal disease.

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Radiocephalic arteriovenous fistula (RCAVF) is the first choice for native arteriovenous fistula (AVF). Preoperative vessel assessment with ultrasonography (USG) has been reported to enhance the outcome of native AVF, but data regarding its predictive value for functional maturation of RCAVF are scanty. We aimed to determine the role of preoperative duplex USG (DUS) for prediction of functional maturity of radiocephalic fistula in the wrist. The data from 173 patients were analyzed prospectively. The estimated duplex variable included size, patency, and continuity of cephalic vein and size, peak systolic velocity, and wall calcifications in radial artery at the wrist. The subjects underwent RCAVF creation and were reviewed 6–8 weeks post procedure for adequacy of maturation. Doppler variables between successful and failed maturation groups were compared. Successful functional fistula maturation was noted in 138 (80.9%) patients. Values of radial artery diameter, cephalic vein diameter, and peak systolic velocity were >2 mm, 2.2 mm, and 32.8 cm/s, respectively, for successful maturation of RCAVF in more than 90% of cases. Vascular calcifications were detected preoperatively in 15 diabetic patients and 9 (60%) of them had fistula failure. Preoperative DUS can provide a good prediction on functional maturation of RCAVF. Vascular calcifications were associated with high risk of maturation failure in diabetics.

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Monoclonal gammopathy of renal significance is a recently described entity in which a small B-cell clone not meeting the criteria for the diagnosis of multiple myeloma produces renal disease usually through deposition of a secreted monoclonal immunoglobulin. Here, we describe a case of Type I cryoglobulinemic glomerulonephritis diagnosed on a kidney biopsy and caused by a monoclonal IgM produced by a small bone marrow clone. The patient made a complete renal recovery after chemotherapy to suppress the clone.

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Eculizumab is the therapy of choice for patients with atypical hemolytic uremic syndrome (aHUS). Dosing recommendations stem from two trials: one retrospective trial (19 children and 5 infants) and one prospective trial (22 patients and 5 infants). This case report highlights the need for more precise dosing recommendations in children, particularly in infants, and for smaller vials of the medication to facilitate more precise dosing. Such changes would ensure that adverse events are minimized and that the children with aHUS who are treated with eculizumab experience an optimal clinical response.

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The spectrum of biopsy-proven glomerular disease was studied from a single center in Northwestern India, among adolescents aged 13–19 years. From January 2009 to December 2012, a total of 177 patients with biopsy-proven glomerular disease were studied. The same pathologist reported all the biopsy specimens after subjecting to light, immunofluorescence, and electron microscopy. The clinical profile and laboratory findings of the patients were correlated with the histopathological spectrum of glomerular diseases. Males formed 71.19% (n = 126) and the remaining 28.81% (n = 51) were females. Lupus nephritis had a strong female predominance, whereas minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) had a male predominance. Nephrotic syndrome was the indication for biopsy in 65% of the cases. Rapidly progressive renal failure and acute nephritis were the next common indications accounting for 14% and 7%, respectively. FSGS was the most common glomerular disease in adolescents (n = 45, 25.4%). The next common were MCD and lupus nephritis each contributing 21.6% and 10.7%, respectively. Primary glomerular diseases accounted for 84.75% (n = 150) of the total. The remaining 15.25% (n = 27) were attributed to secondary glomerular diseases, of which lupus nephritis was the most common, i.e., in 70.4% patients (n = 19). FSGS was the most common histology in adolescent nephrotic participants (37%). MCD was the next common, found in 31% of nephrotic patients. Electron microscopy changed the diagnosis made by light microscopy and immunofluorescence in 5.6% cases only, and it confirmed the diagnosis in another 21.6%. Kidney biopsy in adolescents is a safe procedure. The spectrum of glomerular diseases in adolescents is different from that seen in adults and smaller children.

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Screening of individuals at increased risk of developing chronic kidney disease (CKD) has been advocated by several guidelines. Among individuals at increased risk are first-degree relatives (FDRs) of patients with CKD. There is a paucity of data on the prevalence and risk of CKD in FDRs of patients with CKD in sub-Saharan African population. This study aimed to screen FDRs of patients with CKD for albuminuria and reduced estimated glomerular filtration rate (eGFR). A cross-sectional survey of 230 FDRs of patients with CKD and 230 individuals without family history of CKD was conducted. Urinary albumin: creatinine ratio (ACR) was determined from an early morning spot urine. Glomerular filtration rate was estimated from serum creatinine. Reduced eGFR was defined as eGFR <60 ml/min/1.73 m² and albuminuria defined as ACR ≥30 mg/g. A higher prevalence of albuminuria was found in the FDRs compared to the controls (37.0% vs. 22.2%; P < 0.01). Reduced eGFR was more prevalent among the FDRs compared with the controls (5.7% vs. 1.7%, P < 0.03). Hypertension (odds ratio [OR], 2.9) and reduced eGFR (OR, 9.1) were independent predictors of albuminuria while increasing age (OR, 6.7) and proteinuria (OR, 10.7) predicted reduced eGFR in FDRs. The odds of developing renal dysfunction were increased 2-fold in the FDRs of patients with CKD, OR 2.3, 95% confidence interval, 1.29–3.17. We concluded that albuminuria and reduced eGFR are more prevalent among the FDRs of patient with CKD and they are twice as likely to develop kidney dysfunction as healthy controls.

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Nail–Patella syndrome (NPS) is an inherited disease with characteristic nail, limb, and renal anomalies. While almost all patients manifest classical skeletal and nail abnormalities, renal involvement is seen in only 40% of patients. Asymptomatic proteinuria is the most common renal presentation. Although a substantial number of patients can progress to nephrotic range proteinuria, development of end-stage kidney disease is rare. Pathological abnormalities are appreciable only by electron microscopy. Our patient who had not been diagnosed with NPS previously presented with new-onset proteinuria during the second trimester of her first pregnancy. The characteristic physical and radiological features confirmed the diagnosis of NPS. Her clinical course was complicated by nephrotic syndrome with fetal loss at 22 weeks and postpartum renal vein thrombosis. Nephrotic state improved after termination of pregnancy. She was managed with angiotensin receptor blocker and anticoagulation. At the end of 2 years of follow-up, she has normal glomerular filtration rate with minimal proteinuria.

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Pseudohypoaldosteronism (PHA) Type 1 is characterized by mineralocorticoid resistance, manifesting as neonatal salt wasting, hypotension, hyperkalemia, hyponatremia, and metabolic acidosis in spite of elevated aldosterone levels and plasma renin activity. It is important to differentiate children with systemic PHA from renal PHA, as these children are likely to decompensate even with mild symptoms. Here, we report two neonates with PHA that presented to us with multiorgan involvement.

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Dense deposit disease (DDD), earlier called Type II membranoproliferative glomerulonephritis is distinct disease having frequent relapses reaching end-stage kidney disease by 10-year in up to 50%–60% of cases and high recurrence rate in the allograft. The term DDD is derived from its distinctive ribbon-like osmiophilic deposits in the lamina densa of glomerular basement membrane by electron microscopy. Pathogenetically, alternate pathway dysfunction leads to this disease, which is diagnosed by ultrastructure. Herein, we describe our observation of C4d positivity in an adolescent boy with DDD.

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The aim of this study was to measure insulin resistance (IR) in dialysis patients and its relation to Vitamin D status and nutritional parameters. We included patients on maintenance dialysis, both hemodialysis and peritoneal dialysis (HD and PD). IR was measured by homeostatic model assessment (HOMA)-IR index defined as fasting serum insulin (μU/L) × fasting blood sugar (mmol/L)/22.5. Baseline Vitamin D levels were measured by chemiluminescence immunoassay (CLIA) method. HOMA-IR index correlated with nutritional parameters such as 7-point subjective global assessment (SGA) and anthropometric measures, for example, body fat percentage, lean body weight (LBW), mid-arm circumference (MAC), and mid-arm muscle circumference (MAMC). A total of 55 patients were studied, of them 74.55% were male with mean age of the study population being 37.44 ± 14.96 years. The prevalence values of Vitamin D deficiency <30 ngm/ml, <20 ngm/ml, and <10 ngm/ml levels were 96.36%, 70.91%, and 23.64%, respectively. Mean HOMA-IR index was 3.14 ± 3.86. The correlation of HOMA-IR with Vitamin D was negative (r = −0.140, 95% confidence interval [CI] = −0.397–0.138 and P = 0.309); however, in subgroup analysis, patients with Vitamin D level <20 ng/ml had significantly high IR compared to those with Vitamin D >20 ng/ml, i.e., 3.74 ± 4.37 and 1.67 ± 1.47, respectively (P = 0.018). The other measured parameter which had a significant positive correlation with IR was serum uric acid (r = 0.303, 95% CI = 0.021–0.534, and P = 0.025). In nutritional assessment, body mass index, MAC, and MAMC had statistically significant positive correlation with HOMA-IR index (P ≤ 0.001, 0.004, and 0.004, respectively) unlike SGA (P = 0.480). The mode of dialysis did not have a significant effect on IR (HD vs. PD, P = 0.227). The majority of the patients on maintenance dialysis are Vitamin D deficient. Low Vitamin D level, especially <20 ng/ml, muscle mass, and high serum uric acid level are likely to have more IR in dialysis-dependent patients.

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