Rare Association of Waardenburg Syndrome with Minimal Change Disease.

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).

Immunohistochemical Analysis of Anti-phospholipase A2 Receptor Antibody on Renal Biopsies: A Single Tertiary Care Center Study.

Membranous nephropathy (MN) is one of the common cause of nephrotic syndrome. The discrimination between primary MN (iMN) and secondary MN is essential because of treatment implications. Immunohistochemical (IHC) evaluation with the help of anti-phospholipase A2 receptor (PLA2R) antibody helps in tissue evaluation of iMN, which is an easy, cost-effective, and pathologist-friendly technique. The study included 82 cases of MN over a period of 3 years. IHC using PLA2R antibody was performed on iMN and secondary cases with adequate tissue. Cases of minimal change disease (MCD) were included as control. Granular staining along the basement membrane in the absence of staining of podocytes was considered positive. Medical records were verified for clinical information, baseline biochemical parameters, details of viral markers, connective tissue disease profile, and basic imaging workup. Of the 82 cases of MN, 51 were iMN and 31 secondary MN (sMN). Thirteen MCD cases were included as control. IHC with PLA2R antibody showed a sensitivity of 91.8% and specificity of 95.1%, positive predictive value of 95.7%, and negative predictive value of 90.7% in the diagnosis of iMN. The other parameters, either clinical or laboratory, did not show significant differences between iMN and sMN groups. The results of PLA2R staining by IHC were comparable with other studies and showed a higher sensitivity (91.8%) and specificity (95.1%). IHC with anti-PLA2R antibody can be considered as the standard diagnostic approach to identify iMN and offer scope for individualized treatment.

Risk Factors for Urinary Tract Infections in Renal Allograft Recipients: Experience of a Tertiary Care Center in Hyderabad, South India.

Renal transplantation is an effective and commonly performed procedure for end-stage renal disease. Urinary tract infections are a major cause of morbidity and mortality in renal transplant patients. As data on postrenal transplant urinary tract infections from the Indian subcontinent are limited, the present study was conducted to estimate the burden of urinary tract infections in this vulnerable group of patients. This was a prospective study on patients undergoing renal transplantation in 2014 at our tertiary hospital in South India with a follow-up of 2 years to evaluate the risk factors for urinary tract infections. The prevalence of urinary tract infections was 41.9% with a male preponderance of 76.9%. Mean age of the 31 patients was 32.4 ± 10.2 years (range: 16-55 years). Gram-negative bacilli were the most common isolates with Escherichia coli being the predominant pathogen (53.3%). All the infections occurred within 1 year of transplantation with delayed graft function (P < 0.001; confidence interval [CI]: 29.0-96.3) and prolonged hospital stay (P = 0.0281; CI: 42.1-99.6) being the significant risk factors for acquiring urinary tract infections. Carbapenemase production was noted in 33.3% of isolates and all the Gram-negative organisms isolated in the 1st month of transplantation were carbapenem-resistant (CR) E. coli. The high rate of carbapenem-resistant organisms in the early posttransplant period is a point of concern, especially with cadaver transplants. Infection control practices and catheter care need to be strictly monitored to minimize the risk for UTI in the immediate posttransplant period.

Acute Ciprofloxacin-Induced Crystal Nephropathy with Granulomatous Interstitial Nephritis.

Crystal-induced acute kidney injury (AKI) is caused by the intratubular precipitation of crystals, which results in obstruction and kidney injury. Ciprofloxacin, a commonly used antibiotic, causes AKI secondary to immune-mediated interstitial injury. Rare mechanisms of ciprofloxacin-induced renal injury include crystalluria, rhabdomyolysis, and granulomatous interstitial nephritis. Clinical and experimental studies have suggested that crystalluria and crystal nephropathy due to ciprofloxacin occur in alkaline urine. Preexisting kidney function impairment, high dose of the medication, and advanced age predispose to this complication. We report a case of ciprofloxacin-induced crystal nephropathy and granulomatous interstitial nephritis in a young patient with no other predisposing factors. The patient responded to conservative treatment without the need for glucocorticoids.

Saprochaete capitata fungal infection in renal transplant recipient.

Saprochaete capitata is a fungus that rarely causes human infections; majority of infections were reported in patients with hematological malignancies. Here, we report a case of Saprochaete capitata infection in a renal transplant recipient. To the best of our knowledge, this is the first case report of infection with this unusual organism in renal transplant recipients. In our patient, this organism was isolated from broncho alveolar lavage, and it responded dramatically to the combination of amphotericin and voriconazole.

Rituximab in the treatment of refractory late acute antibody-mediated rejection: Our initial experience.

Antibody-mediated rejection (AMR) is not uncommon after renal transplantation and is harder to handle compared to cell-mediated rejection. When refractory to conventional therapies, rituximab is an attractive option. This study aims to examine the effectiveness of rituximab in refractory late acute AMR. This is a retrospective study involving nine renal transplant recipients. Four doses of rituximab were administered at weekly interval for 4 weeks, at a dose of 375 mg/m2. The mean age of patients was 35.3 ± 7.38 years. The median period between transplantation and graft dysfunction was 30 ± 20 months. Mean serum creatinine at the time of discharge after transplantation and at the time of acute AMR diagnosis was 1.14 ± 0.19 mg/dl and 2.26 ± 0.57 mg/dl, respectively. After standard therapy, it was 2.68 ± 0.62 mg/dl. One patient died of Pseudomonas sepsis and three patients progressed to end-stage renal disease (ESRD). Four biopsies showed significant plasma cell infiltrations. Mean serum creatinine among non-ESRD patients at the end of 1 year progressed from 2.3 ± 0.4 to 3.8 ± 1.2 mg/dl (P value 0.04). eGFR prior to therapy and at the end of 1 year were 34.4 ± 6.18 and 20.8 ± 7.69 ml/min (P value 0.04), respectively. Only one patient showed improvement in graft function in whom donor-specific antibody (DSA) titers showed significant improvement. Rituximab may not be effective in late acute AMR unlike in early acute AMR. Monitoring of DSA has a prognostic role in these patients and plasma cell rich rejection is associated with poor prognosis.

Expanding the antibody-mediated component of plasma cell-rich acute rejection: A case series.

Renal allograft rejection is mediated by T-cells (T-cell mediated rejection) or by donor-specific antibodies (DSAs) (antibody mediated rejection, ABMR). Plasma cell-rich acute rejection (PCAR) is a unique entity due to its peculiar morphology and poor prognostic behavior. All allograft biopsies done at our center from January 2013 to October 2014 were reviewed, and seven were identified with a diagnosis of PCAR with antibody mediated rejection (ABMR). The allograft biopsies were classified as per the Banff 2007 schema. Immunohistochemistry with C4d, SV 40, CD3, CD20, CD138, kappa and lambda light chain was performed. Total 210 allograft biopsies were performed in the study period of which seven biopsies (3.3%) were diagnosed as PCAR with ABMR. All these were late ABMRs (more than 6 months) with median posttransplant duration of 17 months. The allograft biopsy showed features of PCAR along with glomerulitis, peritubular capillaritis, and positive C4d. DSA was positive in six patients. All the patients were treated with standard therapeutic measures of acute cellular rejection (ACR) and ABMR including steroids, plasma exchange, rituximab and intravenous immunoglobulins. All the patients had persistent graft dysfunction or graft loss on follow-up.

Successful catheter reinsertion in a case of Paecilomyces varioti peritonitis in a patient on continuous ambulatory peritoneal dialysis.

Peritonitis is one of the most common and important complications in patients on continuous ambulatory peritoneal dialysis (CAPD). Fungal peritonitis isreported in 4-8% of peritonitis episodes. Fungal peritonitis due to Paecilomyces species is not common. We report a case of CAPD peritonitis due to P. varioti. We immediately removed the CAPD catheter and IV amphotericin was administered for 4 weeks along with temporary hemodialytic support followed by successful catheter reinsertion.