Fibrillary Glomerulonephritis, DNAJB9, and the Unfolded Protein Response.

Background: Fibrillary glomerulonephritis (FGN) is found in approximately 1% of native kidney biopsies and was traditionally defined by glomerular deposition of fibrils larger than amyloid (12-24 nm diameter) composed of polyclonal IgG. Recent identification of DNAJB9 as a sensitive and specific marker of FGN has revolutionized FGN diagnosis and opened new avenues to studying FGN pathogenesis. In this review, we synthesize recent literature to provide an updated appraisal of the clinical and pathologic features of FGN, discuss diagnostic challenges and pitfalls, and propose molecular models of disease in light of DNAJB9. Summary: DNAJB9 tissue assays, paraffin immunofluorescence studies, and IgG subclass testing demonstrate that FGN is distinct from other glomerular diseases with organized deposits and highlight FGN morphologic variants. Additionally, these newer techniques show that FGN is only rarely monoclonal, and patients with monoclonal FGN usually do not have a monoclonal gammopathy. DNAJB9 mutation does not appear to affect the genetic architecture of FGN; however, the accumulation of DNAJB9 in FGN deposits suggests that disease is driven, at least in part, by proteins involved in the unfolded protein response. Treatments for FGN remain empiric, with some encouraging data suggesting that rituximab-based therapy is effective and that transplantation is a good option for patients progressing to ESKD. Key Messages: DNAJB9 aids in distinguishing FGN from other glomerular diseases with organized deposits. Further investigations into the role of DNAJB9 in FGN pathogenesis are necessary to better understand disease initiation and progression and to ultimately develop targeted therapies.

Case Report: Life Saving Application of Commercial Tourniquet in Pediatric Extremity Hemorrhage.

Hemorrhage is the leading preventable cause of death in civilian and military trauma. Recent data from the ongoing conflicts in Iraq and Afghanistan suggest that early and aggressive tourniquet utilization is a safe and effective way to dramatically reduce mortality from extremity hemorrhage. As a result, prehospital tourniquet use is now endorsed by a majority of professional emergency medicine, emergency medical service and trauma professional societies. However, there currently exists scant evidence supporting the efficacy of commercially available tourniquets in controlling extremity hemorrhage in pediatric trauma patients.

The influence of race length on arterial compliance following an ultra-endurance marathon.

There is inconclusive evidence concerning the effects of routine participation in ultra-endurance events on cardiovascular disease (CVD) risk. Arterial compliance is a reliable, non-invasive, and effective tool for evaluating CVD risk. The purpose of this research was to examine if race length influences acute changes in arterial compliance following an ultra-marathon event. A total of 46 ultra-marathon runners were recruited including 21 participants (39.8 ± 8.3 years, 6 females) in the 80-km event and 25 participants (43.7 ± 9.8 years, 3 female) in the 195-km event. Arterial compliance was measured via radial applanation tonometry (CR-2000, HDI) for diastolic pulse contour analysis before and following the race. Significant between-group differences were found for changes in large arterial compliance with a decrease (increase in stiffness) following the 195-km event and an increase following the 80-kilometre event (p < .05). Longer race lengths are associated with greater reductions in large arterial compliance following recreational ultra-marathon running. Assessment of arterial compliance might be a useful prognostic tool to assess the long-term risk of CVD among ultra-marathon runners.

The Ranger First Responder Program and Tactical Emergency Casualty Care Implementation: A Whole-Community Approach to Reducing Mortality From Active Violent Incidents.

Active violent incidents are dynamic and challenging situations that can produce a significant amount of preventable deaths. Lessons learned from the military?s experience in Afghanistan and Iraq through the Committee on Tactical Combat Casualty Care and the 75th Ranger Regiment?s Ranger First Responder Program have helped create the Committee for Tactical Emergency Casualty Care (C-TECC) to address the uniqueness of similar wounding patterns and to end preventable deaths. We propose a whole-community approach to active violent incidents, using the C-TECC Trauma Chain of Survival and a tiered approach for training and responsibilities: the first care provider, nonmedical professional first responders, medical first responders, and physicians and trauma surgeons. The different tiers are critical early links in the Chain of Survival and this approach will have a significant impact on active violent incidents.

Another civilian life saved by law enforcement-applied tourniquets.

Increasing data and anecdotal operational reports are supporting the early, aggressive, prehospital application of tourniquets in potentially life-threatening extremity trauma. Especially in the civilian urban setting where transport times are short, the benefit in terms of lives saved far outweighs the potential risk to the extremity. The popular press has reported frequently on law enforcement-applied tourniquets, but to date, no group has published a scientific review of any of these cases. This case report suggests that law enforcement personnel can be trained to safely identify indications for tourniquet application, properly apply them with limited training, and function as effective first care providers.