Carbamylated hemoglobin (CarHb) was found to have a potential role in the differentiation of patients with acute kidney injury (AKI) from chronic kidney disease (CKD). This study was aimed at the evaluation of the diagnostic performance and usefulness of CarHb in the differentiation of AKI from CKD. Forty patients with renal disease and twenty age- and sex-matched healthy controls were included in the study. Urea, creatinine, Hb, and CarHb were measured in all the subjects. Patients with AKI and CKD were found to have significantly increased levels of CarHb when compared to controls (P < 0.05 for both groups). Patients with CKD had significantly increased levels of CarHb when compared to patients with AKI (P < 0.05). CarHb showed significant positive correlation with urea in patients with renal disease (r = 0.776, P < 0.0001). Significant area under curve (AUC = 0.840, P < 0.0001) was obtained for CarHb and a cut-off value of 98.33 μg VH/g Hb resulted with the best combination of 85% sensitivity and 75% specificity. CarHb may provide clinical utility since patients with AKI and CKD have similar clinical presentation usually. A cut-off value of 98.33 μg VH/g Hb has been found to be useful to differentiate AKI from CKD.

Escherichia coli is the major causative pathogen of urinary tract infection (UTI) in humans. Virulence and drug resistance play important roles in the pathogenesis of E. coli infections. The aims were to investigate the presence of uropathogenic virulence genes and to evaluate a relationship between antibiotic resistance and virulence in E. coli from UTI. A total of 132 E. coli were collected between April and June 2015 in two hospitals of Sanandaj, Iran. Isolates were examined for susceptibility to 16 antibiotic disks using the disk diffusion method and for possession of virulence genes by polymerase chain reaction. Associations between antimicrobial resistance and virulence genes were investigated. A P < 0.05 was considered significant. Of the 132 isolates, the most prevalent virulence gene was pap (31.1%), followed by cnf (28.8%), hly (16.7%), and afa (10.6%). Different patterns of virulence genes were identified. A significant association was detected between the simultaneous presence of hly and pap. The most effective antibiotics were nitrofurantoin, cefoxitin, and imipenem and the least effective were ampicillin, trimethoprim-sulfamethoxazole, and cefotaxime. An association was seen between the presence of cnf and susceptibility to the certain antibiotics, whereas strains with a reduced susceptibility to the certain antibiotics were associated with a significantly increased prevalence of afa and hly (P < 0.05). These findings suggest a correlation between the presence of virulence gene and resistance in E. coli strains from UTI. The results indicate that there is a need for surveillance programs to monitor drug resistance in pathogenic E. coli.

The adult population above the age of 60 years has significantly increased in India, with a life expectancy of 68.4 years in 2016. Data regarding the renal histopathology in these patients are scarce though the number of native kidney biopsies done in this subset of population is increasing. The present study is a retrospective analysis of 231 biopsies from a total of 700 biopsies, from patients above 60 years of age (M = 65.8%; F = 34.2%) with a mean age of 64 ± 6.03 years. The indications for kidney biopsy included nephrotic syndrome (NS) (30.4%), nephritic syndrome (19.1%), rapidly progressive renal failure (11.7%), acute kidney injury (AKI) (15.7%), and acute worsening of preexisting chronic kidney disease (CKD) (23%). The median percentage of glomerulosclerosis was 22% (5%–45%), and interstitial fibrosis and tubular atrophy was 30% (10%–50%). The most common cause for nephrotic syndrome was membranous nephropathy (31.4%) and for nephritic syndrome was benign arterionephrosclerosis (22.7%). Postinfectious glomerulonephritis (29.6%) was the leading cause for rapidly progressive renal failure. Acute injury on CKD was notable in patients with diabetic nephropathy (30.2%). The predominant causes for AKI were acute tubulointerstitial nephritis (33.3%), acute tubular necrosis (22.2%), and acute pyelonephritis (19.4%). The biopsy proven histopathological features enabled us in tailoring the therapy. None of the patients developed life-threatening complications following ultrasonography-guided biopsy.

Corticosteroids form the mainstay of therapy for all forms of nephrotic syndrome. The long-term use of this medication is associated with serious side effects including adrenocortical suppression. The primary objective of this study was to identify adrenocortical suppression (assessed by single morning serum cortisol levels) in children with nephrotic syndrome on treatment with low-dose alternate day steroids. This cross-sectional study was conducted in the Department of Pediatrics in a tertiary care hospital from January 2014 to January 2015. Seventy children (1–18 years) with nephrotic syndrome (steroid sensitive and resistant) who were in remission and on low-dose alternate day steroids for at least 8 weeks or had received steroids of 2 mg/kg/d for at least 2 weeks in the last 1 year (infrequent relapsers) were enrolled. Relevant history was taken, clinical examination was done and blood samples were drawn for serum cortisol, lipid profile, kidney function tests, fasting blood sugar, glycated hemoglobin (HbA_1c), and serum albumin. Forty percent (28/70) children had adrenocortical suppression as assessed by low morning serum cortisol levels. The mean serum cortisol levels were 188 nmol/L and were significantly lower in frequently relapsing individuals (85.9 nmol/L) as compared to other types of nephrotic syndrome (P = 0.05). The prevalence of adrenocortical suppression was higher in steroid-resistant patients (57%) as compared to 28% in frequently relapsing and 11% in steroid-dependent patients. Fifty-seven percent of patients with adrenocortical suppression had short stature while 50% had obesity. All individuals had normal serum HbA_1clevels. The cumulative steroid doses and total duration of corticosteroid therapy were significantly higher in patients with adrenocortical suppression. Children with nephrotic syndrome treated with low-dose alternate day steroids have a high prevalence of adrenocortical suppression on screening with single morning cortisol sample. Those with frequently relapsing or steroid-resistant diseases are at a higher risk of suppression.

IgG4-related kidney disease (IgG4 RKD) is increasingly reported with varied manifestations. The present study was carried out to study the spectrum of IgG4 RKD. All patients with renal manifestation associated with conditions known to be associated with IgG4-related diseases (IgG4 RDs), or renal imaging or histology suggestive of IgG4 RKD were included and evaluated further. Patients with known extrarenal IgG4RD were also screened for renal involvement. Out of 40 patients screened over a period of 15 months, IgG4 RKD was diagnosed in 8. Majority were male (87.5%) with mean age being 56 years. Disease spectrum ranged from normal renal function in one to renal failure requiring dialysis in another two patients. Significant proteinuria was uncommon (12.5%) while hematuria was not seen in any patient. Tubulointerstitial nephritis was seen in all four patients who underwent kidney biopsy. Two patients had associated glomerular lesions in the form of immune complex crescentic glomerulonephritis. The most common imaging abnormality was hypodense renal lesions seen in 2 patients. Elevated IgG4 levels had 87.5% sensitivity and 78.3% specificity for IgG4 RKD and levels did not correlate with disease severity. Of 4 patients treated with steroids, 3 showed improvement in renal function. IgG4 RKD is an uncommon disease even at a referral tertiary care center. Elevated IgG4 levels alone are neither sensitive nor specific for the diagnosis of IgG4 RKD, and a combination of clinical, imaging, serological, and histological features are required for diagnosis.

Focal segmental glomerulosclerosis (FSGS) is a disease that is defined entirely by its histopathological appearance. The recent Columbian classification has grouped this disease into various types based on the light microscopic description. There is a paucity of data describing the distribution of its various subtypes from the Indian subcontinent. This study was undertaken with the aim to throw light on the epidemiology and clinical features of primary FSGS in Eastern India. This retrospective study includes our cohort of biopsy-proven FSGS who presented to us from June 2009 to July 2011 and the analysis of their presenting clinical and histopathological features from our center in East India. Out of 347 patients diagnosed with FSGS in this period, 224 patients were included in the study. A total of 167 cases were of not otherwise specified (NOS) variant (74.5%), 30 tip variant (13.39%), 14 perihilar (6.25%), 8 cellular (3.57%), and 5 to the collapsing variant (2.23%). The maximum proteinuria at presentation was seen with the tip variant (7.98 ± 6.6 g/24 h), and the renal functions were most deranged at presentation with the collapsing variant. These findings were different from those described in other populations including higher prevalence of the tip and the perihilar variant, significant difference in the degree of hypertension, proteinuria, and renal dysfunction among the different variants. The Columbian classification has helped to stratify the outcomes of this glomerular disease with respect to its clinical presentation as well as histopathological features. However, the characteristics of the various variants do show a distinctive pattern in various populations based on ethnicities.

Hepatitis C virus (HCV) infection in renal allograft recipient is associated with increased morbidity and mortality. At present, only few studies related to treatment and outcomes of HCV-infected renal allograft recipients with DAAs have been published. We aimed the study to assess the efficacy and safety of sofosbuvir-based regimens in HCV-infected renal allograft recipients. We analyzed data of 22 eligible HCV-infected renal allograft recipients (14 genotype-3, 6 genotype-1, one each genotype-2 and 4) who were treated with DAAs at our institute. DAA regimen included sofosbuvir and ribavirin with or without ledipasvir or daclatasvir for 12–24 weeks. Patients were followed up for 24 weeks after completion of treatment. A rapid viral response of 91%, end of therapy response of 100%, and sustained viral response at 12 and 24 weeks of 100% with rapid normalization of liver enzymes were observed. Therapy was well tolerated except for ribavirin-related anemia. A significant decrease in tacrolimus trough levels was observed and most patients required increase in tacrolimus dose during the study. Treatment with newer DAAs is effective and safe for the treatment of HCV-infected renal allograft recipients.

Waardenburg syndrome (WS) is a rare genetic disorder characterized by varying degrees of hearing loss, pigmentary anomalies, and defects of other neural crest cell-derived structures. The association of WS with renal anomalies has been described in the literature. However, nephrotic syndrome is a very rare association with WS, and only one case has been reported in the literature. We report a case of WS2 associated with biopsy-proven nephrotic syndrome (minimal change disease).

Monoclonal gammopathy of renal significance (MGRS) can present with myriad of morphological features. We report a case of MGRS in a 46-year-old man who presented with nephrotic-range proteinuria and renal insufficiency. Renal biopsy showed amorphous eosinophilic periodic acid–Schiff positive deposits in capillary loops and lamda light chain positivity on immunofluorescence, suggestive of cyoglobulinemic glomerulonephritis. Serum cryoglobulins were positive. Serum immunoelectrophoresis and immunofixation showed a M band of 0.5 g/dl of IgG lambda type. Bone marrow showed 8% of plasma cells which confirmed the diagnosis of MGRS.

Invasive fungal infection is a complication seen in immunocompromised patients. A disseminated fungal infection has a high rate of mortality. Although disseminated infection is known to be seen in most organs, thyroid involvement is rarely reported. Hence, we report a fatal case of thyroid mucormycosis which resulted into laryngeal nerve paralysis and death of a renal allograft recipient.

Deep vein thrombosis, renal vein thrombosis, and cerebral venous sinus thrombosis in children are frequently described complications of nephrotic syndrome (NS). Early diagnosis and treatment with anticoagulants is the key for a good outcome. There are a few reported cases of portal vein and superior mesenteric thrombosis in adults in association with NS. Here, we describe two cases of portal vein thrombosis with variable extent of involvement of superior mesenteric vein in association with relapse of NS. A high degree of suspicion, ultrasonography of the abdomen along with Doppler study of abdominal vessels, and computed tomography angiography can only pick up such unusual sites of thrombosis and facilitate early management.

Hyperoxaluria and resultant oxalate nephropathy are infrequently reported causes of irreversible renal failure. A rapid decline in renal function in an otherwise insidiously progressive oxalate nephropathy may be triggered by various superimposed insults like the use of nephrotoxic drugs. We present the case of a patient with rapidly progressive renal failure due to oxalate nephropathy that lead to a retrospective diagnosis of chronic pancreatitis. This case highlights the importance of timely assessment for enteric hyperoxaluria in patients with unexplained renal failure of tubulointerstitial nature.

Antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis in a patient of scleroderma is very rare. Very few cases have been reported in English literature. We report a case of a 58-year-old male with long-standing limited cutaneous scleroderma (Scl-70 positive) presenting with normotensive scleroderma renal crisis. Perinuclear ANCA with antimyeloperoxidase antibody was found to be strongly positive. Renal biopsy showed pauci immune-necrotizing crescentic glomerulonephritis. We believe that this case report will be helpful in understanding clinical features of normotensive ANCA-associated glomerulonephritis in scleroderma patients.