Clinical and Genotype Characteristics and Symptom Migration in Patients With Mixed Phenotype Transthyretin Amyloidosis from the Transthyretin Amyloidosis Outcomes Survey

Introduction: Transthyretin amyloidosis (ATTR amyloidosis) is primarily associated with a cardiac or neurologic phenotype, but a mixed phenotype is increasingly described. Methods: This study describes the mixed phenotype cohort in the Transthyretin Amyloidosis Outcomes Survey (THAOS). THAOS is an ongoing, longitudinal, observational survey of patients with ATTR amyloidosis, including both hereditary (ATTRv) and wild-type disease, and asymptomatic carriers of pathogenic transthyretin variants. Baseline characteristics of patients with a mixed phenotype (at enrollment or reclassified during follow-up) are described (data cutoff: January 4, 2022). Results: Approximately one-third of symptomatic patients (n = 1185/3542; 33.5%) were classified at enrollment or follow-up as mixed phenotype (median age, 66.5 years). Of those, 344 (29.0%) were reclassified to mixed phenotype within a median 1–2 years of follow-up. Most patients with mixed phenotype had ATTRv amyloidosis (75.7%). The most frequent genotypes were V30M (38.9%) and wild type (24.3%). Conclusions: These THAOS data represent the largest analysis of a real-world mixed phenotype ATTR amyloidosis population to date and suggest that a mixed phenotype may be more prevalent than previously thought. Patients may also migrate from a primarily neurologic or cardiologic presentation to a mixed phenotype over time. These data reinforce the need for multidisciplinary evaluation at initial assessment and follow-up of all patients with ATTR amyloidosis. Trial Registration: ClinicalTrials.gov: NCT00628745

Brief report on the effect of providing single versus assorted brand name condoms to hospital patients: a descriptive study

OBJECTIVES: This study examined condom acquisition by persons in a hospital setting when single versus assorted brand name condoms were provided. METHODS: Condom receptacles were placed in exam rooms of two clinics. During Phase 1, a single brand name was provided; for Phase 2, assorted brand names were added. Number of condoms taken was recorded for each phase. RESULTS: For one clinic there was nearly a two-fold increase in number of condoms taken (Phase 1 to Phase 2); for the second clinic there was negligible difference in number of condoms taken. CONCLUSIONS: The provision of assorted brand name condoms, over a single brand name, can serve to increase condom acquisition. Locations of condoms and target population characteristics are related factors

Review and Evaluation of the J100â 10 Risk and Resilience Management Standard for Water and Wastewater Systems

Risk analysis standards are often employed to protect critical infrastructures, which are vital to a nation’s security, economy, and safety of its citizens. We present an analysis framework for evaluating such standards and apply it to the J100â 10 risk analysis standard for water and wastewater systems. In doing so, we identify gaps between practices recommended in the standard and the state of the art. While individual processes found within infrastructure risk analysis standards have been evaluated in the past, we present a foundational review and focus specifically on water systems. By highlighting both the conceptual shortcomings and practical limitations, we aim to prioritize the shortcomings needed to be addressed. Key findings from this study include (1) risk definitions fail to address notions of uncertainty, (2) the sole use of â worst reasonable caseâ assumptions can lead to mischaracterizations of risk, (3) analysis of risk and resilience at the threatâ asset resolution ignores dependencies within the system, and (4) stakeholder values need to be assessed when balancing the tradeoffs between risk reduction and resilience enhancement.Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/154262/1/risa13421_am.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/154262/2/risa13421.pd

Immunomodulatory Effects of Streptococcus suis Capsule Type on Human Dendritic Cell Responses, Phagocytosis and Intracellular Survival

Streptococcus suis is a major porcine pathogen of significant commercial importance worldwide and an emerging zoonotic pathogen of humans. Given the important sentinel role of mucosal dendritic cells and their importance in induction of T cell responses we investigated the effect of different S. suis serotype strains and an isogenic capsule mutant of serotype 2 on the maturation, activation and expression of IL-10, IL-12p70 and TNF-α in human monocyte-derived dendritic cells. Additionally, we compared phagocytosis levels and bacterial survival after internalization. The capsule of serotype 2, the most common serotype associated with infection in humans and pigs, was highly anti-phagocytic and modulated the IL-10/IL-12 and IL-10/TNF-α cytokine production in favor of a more anti-inflammatory profile compared to other serotypes. This may have consequences for the induction of effective immunity to S. suis serotype 2 in humans. A shielding effect of the capsule on innate Toll-like receptor signaling was also demonstrated. Furthermore, we showed that 24 h after phagocytosis, significant numbers of viable intracellular S. suis were still present intracellularly. This may contribute to the dissemination of S. suis in the body

Implementing a Low-Threshold Analysis with the Askaryan Radio Array (ARA)

The Askaryan Radio Array (ARA) is a ground-based radio detector at the South Pole designed to capture Askaryan emission from ultra-high energy neutrinos interacting within the Antarctic ice. The newest ARA station has been equipped with a phased array trigger, in which radio signals in multiple antennas are summed in predetermined directions prior to the trigger. In this way, impulsive signals add coherently, while noise likely does not, allowing the trigger threshold to be lower than a traditional ARA station. Early results on just a fraction of available data from this new system prove the feasibility of a low-threshold analysis