Fibrilo-Tactoid Glomerulonephritis: A Possible Novel Morphological Variant.

Fibrillary and immunotactoid glomerulonephritis are infrequent causes of primary nephrotic range proteinuria and are poorly understood. Recent significant developments include the discovery of DNA JB9 antigen in fibrillary glomerulonephritis. Here, we present a case of a middle-aged woman who presented with nephrotic range proteinuria, hematuria, and normal renal function. Renal biopsy revealed fibrils that were randomly arranged on electron microscopy. They were of small size and congo red negative similar to the ones found in fibrillary glomerulonephritis, but were also DNA JB 9 negative, and had a hollow core like in immunotactoid glomerulopathy. Though we try to classify these conditions into either immunotactoid glomerulonephropathy (ITGN) or fibrillary glomerulonephritis (FGN), there are scenarios such as this case where it does not fit into either and is probably an overlap or intermediate variant of these two conditions. Pathological features of these glomerulonephrites are discussed together with their clinical implications, treatment choices, and diagnostic importance.

Previously Unidentified Gene Variation Associated with Fabry Disease: The Impact on One Family.

Fabry disease is an X-linked lysosomal storage genetic disorder associated with over 1000 mutations in the alpha-galactosidase-A gene region. We report here a 69-year-old male who underwent a kidney biopsy to evaluate progressive renal failure. He was found to have zebra bodies in visceral epithelial cells on biopsy, with electron microscopy showing inclusions within the cytoplasm of multiple podocytes consistent with Fabry disease. An alpha-galactosidase level was found to be 21 nm/hr/mg (normal range 50-150 nm/hr/mg). Genetic studies revealed a missense variant in the GLA gene with alanine replaced by cysteine at position 682 (c.682 A > C, p.N228H) that had not been previously associated with Fabry disease. The same variant was detected in two additional family members. The pathologic findings, clinical features, and low alpha-galactosidase level suggest that the c.682 A > C variant is associated with Fabry disease.

Controversies in Treating Asymptomatic Bacteriuria and Urinary Tract Infection: A Case Based Review of Antibiotic Use in Renal Transplant Patients and its Impact on the Development of Resistance.

There is excessive use of both broad spectrum and niched antibiotics for urinary tract infections (UTIs) in hospital and ambulatory setting in spite of clear guidelines on appropriate use. Majority of antibiotics prescribed in United States for UTIs are for nonspecific indications such as positive urine cultures in the absence of symptoms, etc. For these conditions especially asymptomatic bacteriuria, a large proportion of the antibiotics prescribed are unlikely to provide clinical benefit to patients. Asymptomatic bacteriuria (ASB) is a common finding in healthy women and persons with underlying urological abnormalities. Guidelines from Infectious Diseases Society of America (IDSA) clearly define the use of antibiotics in ASB only in pregnant patients and in individuals prior to undergoing invasive urological procedures. IDSA updated the guidelines in 2019 on the use of antibiotics for UTI in special groups such as patients with neutropenia, solid organ transplants, and non-urologic surgery. Considering the implications of antibiotic resistance in the setting of indiscriminate use, there is definitely a need to improve their use in asymptomatic bacteriuria as well as in UTIs. In this review, we follow case-based approach to identify the barriers to appropriate antibiotic prescribing practices in renal transplant recipients.

Corrigendum to "Pericardial Tamponade: An Uncommon Clinical Presentation in cANCA Related Vasculitis and Glomerulonephritis in Association with Very High Titres of ANA".

[This corrects the article DOI: 10.1155/2019/4983139.].

Pericardial Tamponade: An Uncommon Clinical Presentation in cANCA Related Vasculitis and Glomerulonephritis in Association with Very High Titres of ANA.

ANCA (anti-neutrophil cytoplasmic antibody) vasculitides are systemic autoimmune diseases in which anti-neutrophilic cytoplasmic antibodies activate primed neutrophils, thereby generating an inflammatory cascade resulting in the damage of small sized blood vessels in various organs of the body, including the heart. Pleuropericardial involvement is underrecognized as a complication of ANCA vasculitis and is highlighted in this case report of a 51-year-old male who presented with an initial symptomatic presentation of pleuropericardial effusion progressing to pericardial tamponade in the setting of a later renal biopsy proven pauci-immune crescentic glomerulonephritis with high ANA titres along with positive cANCA (cytoplasmic ANCA) and PR3 (proteinase 3) antibodies. He was found to have acute renal failure which progressively got better with cyclophosphamide.