Diagnosis and treatment of hereditary angioedema: An expert opinion

Hereditary angioedema (HAE) is a rare disease, but it severely interrupts daily life activities and can sometimes be life-threatening. Therefore, early diagnosis and prompt treatment of HAE attacks are critical. Physicians should be aware of how to diagnose and manage HAE to prepare not to miss a diagnosis when treating HAE patients. Physicians must also carry out tests to confirm the diagnosis of HAEs caused by C1 inhibitor deficiency (type 1) or C1 inhibitor dysfunction (type 2) in patients with recurrent angioedema. In addition, recent studies revealed another type of HAE which is not related to C1 inhibitor (normal C1 inhibitor HAE). Once HAE is confirmed, patients and their caregivers should be given with short-term and long-term treatment plans to relieve or prevent HAE attacks. HAE requires life-long measures, including psychological support for patients and self-management education.ope

Management of hereditary angioedema in pediatric, pregnant, and breast-feeding patients: An expert opinion

Hereditary angioedema (HAE) is a rare inherited condition marked by recurrent skin and submucosal edema. HAE is caused by a C1 inhibitor deficiency or decreased C1 inhibitor function. The initial attack may occur during childhood or pregnancy, with symptoms ranging from classic angioedema to nonspecific stomach cramps. In this review, we discuss strategies for children and pregnant women to manage HAE attacks effectively and safely in light of the recent increase in HAE diagnosis. To begin, aggressive work-up is necessary to confirm HAE–1/2 and to determine the most effective countermeasures. Secondly, in the event of an acute attack, plasma-derived C1-inhibitor is the first line of defense for children and pregnant women. Icatibant is also appropriate for use, except in pregnant women. Fresh frozen plasma (FFP) may be suggested as an alternative. Thirdly, proactive measures to prevent HAE attacks should be considered whenever a procedure is performed that may result in an exacerbation. Finally, FFP, attenuated androgen and antifibrinolytic agents are recommended for long-term prophylaxis in South Korea where the C1-inhibitor is scarce. However, when making a decision, it is necessary to consider both the efficacy and the risk of adverse effects. For proper management, written action plans and first-aid kits are required. The action plans should be customized to the patients’ unique circumstances.ope

Genetic Markers for Differentiating Aspirin-Hypersensitivity

Aspirin-induced asthma (AIA) and aspirin-induced urticaria/angioedema (AIU) are two major aspirin-related allergies. We summarize recent findings related to their molecular genetic mechanisms in order to identify genetic susceptibility markers for differentiating AIU and AIA. The overproduction of cysteinyl leukotriene has been suggested as a mechanism in both AIU and AIA. Increased expression of CYSLTR1 with CYLSTR1 and CYSLTR2 polymorphisms are new findings in AIA, while the ALOX5 promoter polymorphism has been noted in AIU. An HLA study suggested that DPB1*0301 is a strong genetic marker for AIA, and that HLA DRB1*1302 and DQB1*0609 are markers for AIU susceptibility. Several single nucleotide polymorphisms (SNPs) in the promoters of EP2, TBX21, COX-2, FcεRIβ, and TBXA2R were associated with AIA, while an FcεRIα promoter polymorphism was associated with AIU. The functional studies of the key genes involved in AIA and AIU are summarized. The identification and functional study of genetic markers for AIA and AIU susceptibility would further elucidate the pathogenic mechanisms and facilitate the development of early diagnostic markers to establish therapeutic targets

An Initial Assessment of Head Acceleration Events in Rugby League using Instrumented Mouthguards and Qualitative Video Analysis

There is a growing concern that the long-term effects potentially associated with contact sport are not only caused by concussions, but by the accumulation of subconcussive impacts. It is therefore important to understand the head acceleration event (HAE) exposure sustained by contact sports individuals. In this thesis, a combination of qualitative video analysis and biomechanical instrumented mouthguard data is used to quantify and characterise HAE sustained by professional male rugby league players during competitive matches. Peak linear acceleration (PLA), peak angular acceleration (PAA) and peak change in angular velocity (ΔPAV) were collected using custom-fit instrumented mouthguards with an 8 g or 800 rad/s2 resultant data acquisition threshold. A total of 725 HAE were collected from 31 player matches throughout 10 male players. Multiple HAE often occurred in the same tackle phase and 47.6% of HAE occurred after the initial contact between a tackler and the ball-carrier. Indirect HAE accounted for around half of HAE from carries (50.4%) and a quarter of HAE from tackles (23.8%), they also led to significantly greater ΔPAV than direct HAE (p < 0.001, ES = 0.69, interpretation = moderate). Each kinematic was also skewed to lower values, therefore applying a data acquisition threshold of 10 g led to an exposure rate of around half (13.8 ± 6.9 HAE per player, per match) than the exposure rate reported using an 8 g or 800 rad/s2 threshold (28.0 ± 10.6 HAE per player, per match). The results and methodology from this study demonstrate the efficacy of a combination of biomechanical data with qualitative video analysis in quantifying and characterising HAE exposure in rugby league and will form the basis for a future league wide instrumented mouthguard study

NAD+-dependent DNA ligases of Mycobacterium tuberculosis and Streptomyces coelicolor

Sequencing of the genomes of Mycobacterium tuberculosis H37Rv and Streptomyces coelicolor A3(2) identified putative genes for an NAD+-dependent DNA ligase. We have cloned both open reading frames and overexpressed the protein products in Escherichia coli. In vitro biochemical assays confirm that each of these proteins encodes a functional DNA ligase that uses NAD+ as its cofactor. Expression of either protein is able to complement E. coli GR501, which carries a temperature-sensitive mutation in ligA. Thus, in vitro and in vivo analyses confirm predictions that ligA genes from M. tuberculosis and S. coelicolor are NAD+-dependent DNA ligases

Evaluación del impacto de los eventos adversos cutáneos en la calidad de vida de los pacientes oncológicos que reciben tratamiento antineoplásico

Los avances en el conocimiento de la biología tumoral y el desarrollo de fármacos nuevos han permitido mejorar el control de la enfermedad en los pacientes oncológicos. Conocer el impacto en la calidad de vida que pueden tener los eventos adversos cutáneos derivados de los tratamientos antineoplásicos puede ser decisivo en el manejo de estos pacientes. En nuestro estudio, gracias al uso de diversos cuestionarios de calidad de vida observamos que los eventos adversos cutáneos pueden afectar negativamente a la calidad de vida, siendo este efecto mayor en los casos moderados o graves y en aquellos con más de un evento concomitante. Esto refuerza la importancia de tener en cuenta las valoraciones realizadas por los propios pacientes con respecto a su calidad de vida

Toward the recovery of effective youth ministry for Korean ethnic churches in the United States

https://place.asburyseminary.edu/ecommonsatsdissertations/1706/thumbnail.jp

Dynamic Context Awareness of Universal Middleware based for IoT SNMP Service Platform

This study focused on the Universal Middleware design for the IoT (Internet of Things) service gateway for the implementation module of the convergence platform. Recently, IoT service gateway including convergence platform could be supported on dynamic module system that is required mounting and recognized intelligent status with the remote network protocol. These awareness concepts support the dynamic environment of the cross-platform distributed computing technology is supported by these idea as a Universal Middleware for network substitution. Distribution system commonly used in recent embedded systems include CORBA (Common Object Request Broker Architecture), RMI (Remote Method Invocation), DCE (Distributed Computing Environment) for dynamic service interface, and suggested implementations of a device object context. However, the aforementioned technologies do not support each standardization of application services, communication protocols, and data, but are also limited in supporting inter-system scalability. In particular, in order to configure an IoT service module, the system can be simplified, and an independent service module can be configured as long as it can support the standardization of modules based on hardware and software components. This paper proposed a design method for Universal Middleware that, by providing IoT modules and service gateways with scalability for configuring operating system configuration, may be utilized as an alternative. This design could be a standardized interface provisioning way for hardware and software components as convergence services, and providing a framework for system construction. Universal Middleware Framework could be presented and dynamic environment standardization module of network protocols, various application service modules such as JINI (Apache River), UPnP (Universal Plug & Play), SLP (Service Location Protocol) bundles that provide communication facilities, and persistence data module. In this IoT gateway, management for based Universal Middleware framework support and available for each management operation, application service component could be cross-executed over SNMP (Simple Network Management Protocol) version 1, version 2, and version 3. The way of SNMP extension service modules are conducted cross-support each module and independent system meta-information that could be built life cycle management component through the MIB (Management Information Base) information unit analysis. Therefore, the MIB role of relation with the Dispatcher applied to support multiple concurrent SNMP messages by receiving incoming messages and managing the transfer of PDU (Protocol Data Unit) between the RFC 1906 network in this study. Results of the study revealed utilizing Universal Middleware that dynamic situations of context objects with mechanisms and tools to publish information could be consisted of IoT to standardize module interfaces to external service clients as a convergence between hardware and software platforms