Generation of two hiPSCs lines of two patients carrying truncating mutations in the dimerization domain of filamin C.

Here we introduce the human induced pluripotent stem cell lines (hiPSCs), HIMRi004-A and HIMRi005-A from dermal fibroblasts of a 48-year-old female (HIMRi004-A) carrying missense mutation that translate to the first described filamin C isoform p.W2710X and from a 56-year-old female (HIMRi005-A) carrying a recently described mutation in the same domain p.Y2704X. Both lines are generated via lentiviral expression of OCT4, SOX2, KLF4 and c-MYC. The lines display a typical embryonic stem cell-like morphology, express pluripotency markers, retain a normal karyotype (46, XX) and have the differentiation capacity in all three germ layers. The two lines can be used to elucidate the pathomechanisms of FLNC myofibrillar myopathies and to develop novel therapeutic options.

Disaster response using the circular economy: A timeline review of the literature.

Construction materials are, by the very nature of building something, an investment in the future. Every project highlights the possibilities of the building or structure, but when a catastrophic event renders it useless, the landfills grow, and the resources' potential is lost. The end of a building does not need to mean the end of those materials in it. As part of the growing momentum behind sustainability metrics sought out by investment and construction firms alike, the potential for returns on investment already exists. The United States has the National Response Framework and international commitments like the Sendai Framework for Disaster Risk Reduction that signify the appetite to embrace the latest innovations in response techniques and technologies. This literature review will focus on how the construction and demolition industry can lead as the primary ameliorators and enablers of remedying this shortfall in innovative adoption uptake by engaging and applying the growing practice of the circular economy to their own debris management regimens.

[Periprosthetic infection after total hip replacement : Risk factors for an early infection after primary implantation]. / Periprothetischer Infekt nach Hüftprothesenimplantation : Risikofaktoren für die Entwicklung einer Frühinfektion nach Primärimplantation.

BACKGROUND: Periprosthetic infection is one of the most serious complications in primary arthroplasty. The infection rates reported in the current literature range from 0.36 to 2.23%. OBJECTIVES: The aim of this retrospective case-control study was to determine preoperative risk factors for the occurrence of early periprosthetic infection after primary hip arthroplasty. MATERIALS AND METHODS: The influence of patient age, gender, body mass index (BMI), C­reactive protein, preoperative leukocyte count and morbidity level (American Society of Anaesthesiologists score) on the occurrence of periprosthetic early infection of the hip joint was examined, and their correlation was investigated. RESULTS: Of the 1383 patients followed up, 25 were diagnosed with early infection. With an increase in BMI of 1 kg/m2, the risk of periprosthetic early infection increased by 12.1% (p < 0.001). In addition, using the receiver operating characteristic (ROC) curve, a BMI ≥ 29 kg/m2 is identified as a significant cut-off for the increased likelihood of periprosthetic hip infection. Using the ROC curve, a preoperative CrP value > 5 mg/l can be validated as a cut-off value for an increased risk of early infection. Using binary logistic regression, no influence of CrP > 5 mg/l on the development of early infection was statistically proven (p = 0.065). CONCLUSIONS: Patients with a BMI ≥ 29 kg/m2 should be informed about the increased risk of periprosthetic early infection after hip prosthesis implantation and a risk assessment should be performed. Furthermore, the determination of the preoperative CrP value should be considered standard.

Active shooter mitigation techniques.

This paper looks at current active shooter mitigation techniques employed by schools throughout the country in an attempt to mitigate casualties during an active shooter event. The researchers modified an existing Columbine High School agent-based model created by Jae Lee, MS, to examine if the introduction of a school resource officer (SRO), concealed carry weapon (CCW) holder, or both would change the outcome of the previous research. RUN.HIDE.FIGHT® scenarios were modeled with the same parameters of the previous work, but now included armed first responders during the incident to assess whether their presence decreased casualties through a reduction in response time. The researchers determined that the addition of either an SRO, CCW holder, or both significantly reduced casualty rates during an active shooter scenario.

Active shooter mitigation strategies in small rural churches.

Gun violence in places of worship (POW) has long been an issue and has been addressed repeatedly in the literature. Contextually, most of the research has been pertinent to relatively large POW, situated in an urban setting. However, rural churches have not been addressed, and they appear to have a far less defensive posture, mainly because of their remote location and the extended time required for first responders to arrive, which in turn requires a higher level of independent operation in terms of defense and medical response. Having retained an off-duty officer is a strong deterrent and provides the ability to handle any issues that may arise, including lower violence level events. If retaining an officer is not an option, having a well-trained volunteer armed team and a clear plan of action is vital to surviving such an event. Furthermore, due to the extended response and transport time, it is critical to have proper medical training, such as Stop the Bleed® and cardio-pulmonary resuscitation. This article's focus is not only on response but preparedness, which reinforces the response, as well as prevention and deterrence. An exhaustive best practices review has informed the solutions offered, supplemented by experience and recommendations of a highly experienced physical security expert and a police officer member of a Special Weapons and Tactics (SWAT) team.

Software library for agent-based modeling and simulation of active shooter events.

Software libraries have been used for decades to produce code in a quick and cost-effective manner. The use of well-designed libraries permits software developers and other professionals to create applications due in part to code reusability. Also, good libraries grant lesser skilled developers the opportunity to make high-quality applications they otherwise could not produce. In the field of active shooting incident (ASI) research, various tools have been used for years that give researchers the ability to conduct exploratory research. However, as good as these tools might be, there has been little thought about reusability of these models and associated code. This has hindered the proper advancement of the research field given that researchers must often start from nothing when building a new model. Constant repetition of the same basic tasks has not enabled researchers to expand model fidelity and has limited time to dedicate toward the problem set. This paper proposes the creation of a new agent-based ASI library, made for the AnyLogic® system. The library assists researchers in quickly creating models using a well-defined application programming interface. It also abstracts away implementation details so that the library user does not get waylaid in development. The authors also recreated parts of a large and powerful AnyLogic model to observe the resultant library employment. It was determined that a significant amount of time can be saved building new models, even with the initial version of the library implementation.

Magazine capacity limits and home security.

In response to several mass-casualty shootings in the United States, multiple legislative bodies have promoted or adopted laws that restrict the magazine capacity of firearms. The stated intent of these laws is to reduce the number of rounds a bad actor can fire in a mass-casualty situation. However, objectors argue that the laws not only are ineffective but could also cause an undue burden on the ability of law-abiding gun owners to protect themselves. Using agent-based simulation modeling, a home-invasion model including two-armed bad actors and a one-armed homeowner was created to test this hypothesis. The homeowner engaged the bad actors with different capacity magazines, and the survival rate was recorded and compared across the spectrum of capacities. This model showed that reducing the homeowner's magazine capacity resulted in an increased casualty rate for the homeowner.

Agent-based modeling for theme park evacuation.

Each year theme parks can see up to 20 million patrons, but often little effort is put into planning for an emergency evacuation. In this study, we built a multiagent simulation model using AnyLogic® 8.5.1. The model was based on a preliminary design of a theme park provided by AOA Builds, Orlando. This research had two goals: the first was to compare evacuation time when the park is full (1) using only the main guest gate and (2) using all seven available exits. The second goal was to model first responder response time between various start and end locations within the park. Using only the main gate, evacuation took an average of 14 minutes and 51 seconds. Using all seven gates results in an average evacuation time of 11 minutes and 58 seconds. This was due to a gate being overwhelmed causing a delay in overall evacuation time. If that gate is not included in the calculation, the average evacuation time drops to 6 minutes and 44 seconds. For the purpose of measuring response times, four starting locations were chosen with the guidance of a subject matter expert. These locations included response teams positioned at the front gate, at a police station, at the service area behind a main attraction, and mobile patrol walking around the park. Based on our testing, walking around the park was the best option in terms of response time, using the main gate was 53.7 percent faster than other options and, using all seven gates, was 60.7 percent faster during an evacuation using all seven exits.

A case for standardized communications for point of distribution operations.

OBJECTIVE: Propose standardized communication formats and procedures at the point of distribution (POD) sites to facilitate the effective management and alleviate friction points that may occur while responding to a widespread medical incident within the National Incident Management System (NIMS). DESIGN: Through observation of POD operations and interviews with key personnel in Indiana and county emergency management agencies (EMA), identify current communication practices and propose a structured command and control (C2) framework that would streamline management requirements during a widespread activation. SETTING: The State of Indiana, various counties throughout Indiana. CONCLUSIONS: Current C2 practices are adequate for small-scale events. However, a standardized approach to the C2 and reporting framework may provide more responsive situational awareness and incident management of larger-scale medical incidents within Indiana.

POD variant comparisons.

Points of distribution, also known as points of dispensing (POD), are a means for public and private organizations to assist their communities in times of crisis. There are two principal categories of PODs, open and closed, but all PODs differ in design, properties, and application. This study investigates two POD variations: drive-through and supervisor, which have their own unique requirements for being stood up, run, and shut down, as well as differing requirements for planning, staffing, and logistics. There are also similarities in the requirements that each POD category share which lead to certain efficiencies in planning for POD standup, execution, and shutdown. The primary findings of this paper are that planners cannot rely on one POD design and its properties to accommodate every situation, and each POD design has its own strengths and weaknesses. These are related to staffing, security, space requirements, and material logistics needs. Flexibility should be exercised when choosing the correct design, and implementing the proper strategy is key to standing up and executing a POD that will best serve a community. Every situation is different and factors such as population, available infrastructure, resource requirements, and individual skill of the POD staff all influence the design of a POD. Planners should consider resources such as available volunteers, trained personnel (medical and security), and buildings or outdoor space available to run a POD. With proper planning, a POD is an excellent tool to effectively and efficiently serve the public.

Impact of HBV genotype and mutations on HBV DNA and qHBsAg levels in patients with HBeAg-negative chronic HBV infection.

BACKGROUND: HBV DNA and quantitative (q)HBsAg levels as prognostic markers for HBV-related disease are mostly validated in Asia and their significance in Western populations is uncertain. AIM: To analyse the impact of the HBV genotype and frequent mutations in precore (PC), basal core promoter (BCP) and preS on HBV DNA and qHBsAg levels. METHODS: HBV DNA and qHBsAg serum levels of 465 patients with HBeAg-negative chronic HBV infection were correlated with the HBV genotype and mutations in PC, BCP and preS. For a detailed analysis of the molecular virology, genotype A2 genomes harbouring these mutations were analysed for replication efficacy and HBsAg release in cell culture. RESULTS: While no impact of the HBV genotype on HBV DNA levels was observed, qHBsAg levels differed up to 1.4 log among the genotypes (P < 0.001), reflected by large differences regarding the 1000 IU/mL HBsAg cut-off. While PC mutations were associated with higher (P < 0.001), BCP mutations were associated with lower HBV DNA levels (P < 0.001). Higher qHBsAg levels were associated with preS and lower levels with PC mutations (P < 0.001 and P = 0.001, respectively). The cell culture experiments revealed a higher HBsAg release and shorter filaments in case of a HBV genome harbouring a preS deletion. In contrast, a perinuclear HBsAg accumulation was detected for the PC and BCP-variants, reflecting an impaired HBsAg release. CONCLUSIONS: qHBsAg serum levels depend on the HBV genotype and together with HBV DNA levels on frequent mutations in PC, BCP and preS in HBeAg-negative patients. qHBsAg cut-offs when used as prognostic markers require genotype-dependent validation.

Cutaneous and Subcutaneous Soft Tissue Tumours in Snakes: A Retrospective Study of 33 Cases.

Cutaneous and subcutaneous soft tissue tumours have been rarely described in detail in snakes. Several malignant entities show strikingly similar histological patterns and therefore the term soft tissue sarcoma (STS) has become a standard histopathological diagnosis. The present study characterizes soft tissue tumours in 33 snakes. Samples included 29 surgically excised masses and four carcasses. Additionally, six animals were humanely destroyed and submitted for necropsy examination following tumour recurrence. Benign neoplasms (n = 8) were described as lipomas of varying differentiation. Recurrence was observed in two of five snakes in which the clinical course was recorded. Malignant neoplasms (n = 25) were diagnosed as STS and graded according to a three-point system previously applied to canine STS. Five (20%) of the primary tumours were classified as grade 1, eleven (44%) as grade 2 and nine (36%) as grade 3 sarcomas. Clinically, recurrence of STS was observed in 11 of 17 cases with available follow-up information. Pathologically, multiple cutaneous metastases were found in one grass snake (Natrix natrix), while visceral metastases were observed in one carpet python (Morelia spilota) and two corn snakes (Pantherophis guttatus). Metastatic risk appears to increase with histological grade. Surgical excision generally represents the current therapy of choice for STS. This study includes the first reports of conventional lipomas in a ribbon snake (Thamnophis radix), angiolipomas in a black-headed python (Aspidites melanocephalus) and a corn snake as well as of STS in a Jamaican boa (Epicrates subflavus), emerald tree boa (Corallus caninus), grass snake (N. natrix), African house snake (Lamprophis fuliginosus), California kingsnake (Lampropeltis getula californiae) and common garter snake (Thamnophis sirtalis).

Mitigating active shooter impact: Analysis for policy options based on agent/computer-based modeling.

UNLABELLED: Active shooting violence at confined settings, such as educational institutions, poses serious security concerns to public safety. In studying the effects of active shooter scenarios, the common denominator associated with all events, regardless of reason/intent for shooter motives, or type of weapons used, was the location chosen and time expended between the beginning of the event and its culmination. This in turn directly correlates to number of casualties incurred in any given event. The longer the event protracts, the more casualties are incurred until law enforcement or another barrier can react and culminate the situation. OBJECTIVE: Using AnyLogic technology, devise modeling scenarios to test multiple hypotheses against free-agent modeling simulation to determine the best method to reduce casualties associated with active shooter scenarios. DESIGN, SETTING, SUBJECTS: Test four possible scenarios of responding to active shooter in a public school setting using agent-based computer modeling techniques-scenario 1: basic scenario where no access control or any type of security is used within the school; scenario 2, scenario assumes that concealed carry individual(s) (5-10 percent of the work force) are present in the school; scenario 3, scenario assumes that the school has assigned resource officer; scenario 4, scenario assumes that the school has assigned resource officer and concealed carry individual(s) (5-10 percent) present in the school. MAIN OUTCOMES MEASURED: Statistical data from modeling scenarios indicating which tested hypothesis resulted in fewer casualties and quicker culmination of event. RESULTS: The use of AnyLogic proved the initial hypothesis that a decrease on response time to an active shooter scenario directly reduced victim casualties. CONCLUSIONS: Modeling tests show statistically significant fewer casualties in scenarios where on scene armed responders such as resource officers and concealed carry personnel were present.

Do social media have a place in public health emergency response?

OBJECTIVES: To ascertain whether analyses of social media trends for various Twitter responses following a major disaster produce implications for improving the focus on public health resources and messaging to disaster victims. METHODS: Radian6 and trend analyses were used to analyze 12-hour counts of Twitter data before, during, and after the March 2011 Japanese earthquake and tsunami. Radian6 was used to organize tweets into categories of preparedness, emergency response, and public health. RESULTS: Radian6 revealed that 49 percent of tweets were either positive or somewhat positive in sentiment about preparedness and only 7 percent were negative or somewhat negative. Trend analyses revealed a rapid onset of tweet activity associated with all keywords followed by mostly fast exponential decline. Analyses indicate that opportunities for improving public health awareness by leveraging social media communications exist for as much as 5 days after a disaster. CONCLUSIONS: Analyses suggest key times for public health social media communication to promote emergency response.

[Bone tumours in lizards and snakes. A rare clinical finding]. / Knochentumoren bei Echsen und Schlangen. Ein seltener klinischer Befund in der Reptilienpraxis.

OBJECTIVE: This article presents the pathological findings of 13 bone and cartilage tumours in lizards (n=8) and snakes (n=5) within the clinical context. MATERIAL AND METHODS: Within a 12-year period (2001-2013), 13 cases of bone tumours in reptiles were diagnosed from 358 submitted tumour specimens. Pathological examination was performed on eight excisions, two biopsies, two amputates and four carcasses. Macroscopically, the samples displayed a light-coloured surface when cut and had a generally solid consistency. For the histological examination, representative specimens were decalcified when necessary, embedded in paraffin and stained using haematoxylin and eosin. Diagnosis was made based on the World Health Organisation classification for veterinary and human medicine. RESULTS: Benign proliferations of the bone (ossifying fibroma [n=2], fibrous dysplasia [n=1]) as well as malignant cartilage (chondrosarcoma [n=2]) and bone tumours (fibroblastic osteosarcoma [n=2], small cell osteosarcoma [n=1]) were found on the head (n=5) and limbs (n=3) of various lizard species. In snakes only malignant cartilage neoplasms (chondrosarcoma [n=2], dedifferentiated chondrosarcoma [n=3]) of the spine were diagnosed. The histological appearance of the malignant tumours ranged from low to highly malignant differentiated aggregations of tumour cells, that produced varying amounts of osteoid or a hyaline matrix. Curative therapy was achieved in one ossifying fibroma by complete surgical removal and in two chondrosarcomas through amputation. No metastasis was observed in any of the four necropsies. CONCLUSION AND CLINICAL RELEVANCE: Primary neoplasias of the bone are rare tumours in reptiles. Considering the information on therapeutic procedures and clinical course, the therapy of choice in lizards is complete surgical removal whereas in snakes reductive surgery may prolong the survival time.

Implementation and modeling of a Regional Hub Reception Center during mass evacuation operations.

When developing response plans in the aftermath of a catastrophic incident, jurisdictions often fail to conduct the necessary interdisciplinary planning needed to fully address the needs across jurisdictional borders. The Purdue Homeland Security Institute (PHSI) was selected by the City of Chicago Office of Emergency Management and Communications (OEMC) in 2010 to lead an effort to address planning across jurisdictional borders during mass evacuations following a catastrophic incident. Specifically, PHSI was chosen to lead the effort in developing a planning and implementation guide for standing up a conceptual Regional Hub Reception Center (RHRC). A major component within the mass evacuation and sheltering continuum, the RHRC is designed to provide evacuees with quickresponse mass care and emergency assistance while their other needs are assessed and appropriate shelter locations are identified. The RHRC also provides a central location to leverage governmental, nongovernmental, and private sector resources and is the first point in the evacuation, mass care, and sheltering concept of operations where more comprehensive support (food, shelter, medical, psychological, household pet sheltering, reunification, etc) can be expected. PHSI undertook this lead role working within the Illinois-Indiana-Wisconsin (IL-IN-WI) Combined Statistical Area (CSA) as part of the US Department of Homeland Security Regional Catastrophic Planning Grant Program. Coordinating closely with the City of Chicago OEMC and IL-IN-WI CSA Regional Catastrophic Planning Team, PHSI lead the research effort using resource and capability data compiled from all 17 jurisdictions within the IL-IN-WI CSA and validated the RHRC concept using three tabletop exercises. Upon completion, the PHSI team published the RHRC planning guide complete with procedures and processes that define the roles and responsibilities of government, nongovernment organizations, and private sector for providing RHRC mass care functions and RHRC capability and capacity assessments. This article further examines the potential for using simulation modeling as a cost-effective means to rapidly evaluate any facility for potential use as a RHRC and to measure and maximize RHRC operational efficiency. Using AnyLogic simulation software, PHSI developed a first-ever model of a theoretical RHRC capable of simulating, measuring, and manipulating RHRC operations under specified conditions/scenarios determined by the emergency management planner. Future simulation modeling research promises to promote the Whole Community Approach to response and recovery by reinforcing interdisciplinary planning, enhancing regional situational awareness, and improving overall jurisdictional coordination and synchronization.

The late positive potential, emotion and apathy in Parkinson's disease.

Parkinson's disease is associated with emotional changes including depression, apathy, and anxiety. The current study investigated emotional processing in non-demented individuals with Parkinson disease (PD) using an electrophysiological measure, the centro-parietal late positive potential (LPP). Non-demented patients with Parkinson's disease (n=17) and healthy control participants (n=16) viewed pleasant, neutral, and unpleasant pictures while EEG was recorded from a 64-channel geodesic net. The Parkinson patients did not differ from controls in terms of early electrophysiological components that index perceptual processing (occipital P100, N150, P250). Parkinson patients, however, showed reduced LPP amplitude specifically when viewing unpleasant, compared to pleasant, pictures as well as when compared to controls, consistent with previous studies suggesting a specific difference in aversive processing between PD patients and healthy controls. Importantly, LPP amplitude during unpleasant picture viewing was most attenuated for patients reporting high apathy. The data suggest that apathy in PD may be related to a deficit in defensive activation, and may be indexed cortically using event-related potentials.

Emotion and ocular responses in Parkinson's disease.

Parkinson's disease (PD) is a neurodegenerative disease that affects motor, cognitive, and emotional functioning. Previous studies reported reduced skin conductance responses in PD patients, compared to healthy older adults when viewing emotionally arousing pictures. Attenuated skin conductance changes in PD may reflect peripheral autonomic dysfunction (e.g., reduced nerve endings at the sweat gland) or, alternatively, a more central emotional deficit. The aim of the current study was to investigate a second measure of sympathetic arousal-change in pupil dilation. Eye movements, a motor-based correlate of emotional processing, were also assessed. Results indicated that pupil dilation was significantly greater when viewing emotional, compared to neutral pictures for both PD patients and controls. On the other hand, PD patients made fewer fixations with shorter scan paths, particularly when viewing pleasant pictures. These results suggest that PD patients show normal sympathetic arousal to affective stimuli (indexed by pupil diameter), but differences in motor correlates of emotion (eye movements).