Subcutaneous Immunoglobulin Replacement Therapy with Hizentra® is Safe and Effective in Children Less Than 5 Years of Age.

BACKGROUND:Hizentra® (IGSC 20%) is a 20% liquid IgG product approved for subcutaneous administration in adults and children 2 years of age and older who have primary immunodeficiency disease (PIDD). There is limited information about the use of IGSC 20 % in very young children including those less than 5 years of age. METHODS:A retrospective chart review involved 88 PIDD infants and children less than 5 years of age who received Hizentra®. RESULTS:The mean age at the start of Hizentra® was 34 months (range 2 to 59 months). IGSC 20 % was administered weekly to 86 infants (two additional infants received twice weekly and three times weekly infusions, respectively) and included an average of 63 infusions (range 6-182) for an observation period up to 45.5 months. Infusion by manual delivery occurred in 15 patients. The mean dose was 674 mg/kg/4 weeks. The mean IgG level was 942 mg/dL while on IGSC 20 %, compared to a mean trough IgG level of 794 mg/dL (p < 0.0001) during intravenous or subcutaneous IgG administration prior to IGSC 20 %. Average infusion time was 47 (range 5-120) minutes, and the median number of infusion sites was 2 (range 1-4). Local reactions were mostly mild and observed in 36/88 (41%) children. No serious adverse events were reported. A significant increase in weight percentile (7 % ± 19.2, p = 0.0012) among subjects was observed during IGSC 20% administration. The rate of serious bacterial infections was 0.067 per patient-year while receiving IGSC 20%, similar to previously reported efficacy studies. CONCLUSIONS:Hizentra® is effective in preventing infections, and is well tolerated in children less than age 5 years

Using GENI for experimental evaluation of Software Defined Networking in smart grids

The North American Electric Reliability Corporation (NERC) envisions a smart grid that aggressively explores advance communication network solutions to facilitate real-time monitoring and dynamic control of the bulk electric power system. At the distribution level, the smart grid integrates renewable generation and energy storage mechanisms to improve the reliability of the grid. Furthermore, dynamic pricing and demand management provide customers an avenue to interact with the power system to determine the electricity usage that best satisfies their lifestyle. At the transmission level, efficient communication and a highly automated architecture provide visibility in the power system and as a result, faults are mitigated faster than they can propagate. However, such higher levels of reliability and efficiency rest on the supporting communication infrastructure. To date, utility companies are moving towards Multiprotocol Label Switching (MPLS) because it supports traffic engineering and virtual private networks (VPNs). Furthermore, it provides Quality of Service (QoS) guarantees and fail-over mechanisms in addition to meeting the requirement of non-routability as stipulated by NERC. However, these benefits come at a cost for the infrastructure that supports the fullMPLS specification. With this realization and given a two week implementation and deployment window in GENI, we explore the modularity and flexibility provided by the low cost OpenFlow Software Defined Networking (SDN) solution. In particular, we use OpenFlow to provide 1.) automatic fail-over mechanisms, 2.) a load balancing, and 3.) Quality of Service guarantees: all essential mechanisms for smart grid networks

Caenorhabditis elegans ETR-1/CELF has broad effects on the muscle cell transcriptome, including genes that regulate translation and neuroblast migration

Migration of neuroblasts and neurons from their birthplace is central to the formation of neural circuits and networks. ETR-1 is the Caenorhabditis elegans homolog of the CELF1 (CUGBP, ELAV-like family 1) RNA-processing factor involved in neuromuscular disorders. etr-1 regulates body wall muscle differentiation. Our previous work showed that etr-1 in muscle has a non-autonomous role in neuronal migration, suggesting that ETR-1 is involved in the production of a signal emanating from body wall muscle that controls neuroblast migration and that interacts with Wnt signaling. etr-1 is extensively alternatively-spliced, and we identified the viable etr-1(lq61) mutant, caused by a stop codon in alternatively-spliced exon 8 and only affecting etr-1 isoforms containing exon 8. We took advantage of viable etr-1(lq61) to identify potential RNA targets of ETR-1 in body wall muscle using a combination of fluorescence activated cell sorting (FACS) of body wall muscles from wild-type and etr-1(lq61) and subsequent RNA-seq. This analysis revealed genes whose splicing and transcript levels were controlled by ETR-1 exon 8 isoforms, and represented a broad spectrum of genes involved in muscle differentiation, myofilament lattice structure, and physiology. Genes with transcripts underrepresented in etr-1(lq61) included those involved in ribosome function and translation, similar to potential CELF1 targets identified in chick cardiomyocytes. This suggests that at least some targets of ETR-1 might be conserved in vertebrates, and that ETR-1 might generally stimulate translation in muscles. As proof-of-principle, a functional analysis of a subset of ETR-1 targets revealed genes involved in AQR and PQR neuronal migration. One such gene, lev-11/tropomyosin, requires ETR-1 for alternative splicing, and another, unc-52/perlecan, requires ETR-1 for the production of long isoforms containing 3′ exons. In sum, these studies identified gene targets of ETR-1/CELF1 in muscles, which included genes involved in muscle development and physiology, and genes with novel roles in neuronal migration

Ceramic matrix composite turbine engine vane

A vane has an airfoil shell and a spar within the shell. The vane has an outboard shroud at an outboard end of the shell and an inboard platform at an inboard end of the shell. The spar has a first chamber essentially along the suction side and a second chamber along the pressure side opposite the first chamber

The effect of SO2 on mineral carbonation in batch tests

CO2 sequestration is a key element of future emission-free fossil-fueled power plants. Other constituents of flue gas must also be captured and rendered innocuous. Contemporary power plants remove SOx from exit gases, but next-generation plants may simultaneously treat CO2, SOx, and other pollutants. Pioneering tests at the U.S. Department of Energy's Albany Research Center investigated the combined treatment of CO2 and SO2 in a mineral-carbonation process. SO2 was removed from the gas stream, and as a small fraction of the total volume of mineralizing gas, it did not inhibit the carbonation reaction. The results indicate that this approach to CO2 sequestration could be used to treat multiple pollutants

A framework for human-like behavior in an immersive virtual world

Just as readers feel immersed when the story-line adheres to their experiences, users will more easily feel immersed in a virtual environment if the behavior of the characters in that environment adheres to their expectations, based on their life-long observations in the real world. This paper introduces a framework that allows authors to establish natural, human-like behavior, physical interaction and emotional engagement of characters living in a virtual environment. Represented by realistic virtual characters, this framework allows people to feel immersed in an Internet based virtual world in which they can meet and share experiences in a natural way as they can meet and share experiences in real life. Rather than just being visualized in a 3D space, the virtual characters (autonomous agents as well as avatars representing users) in the immersive environment facilitate social interaction and multi-party collaboration, mixing virtual with real

Characterizing the Decision Process in Setting Corn and Soybean Seeding Rates

Selecting optimal corn and soybean seeding rates are difficult decisions to make. A survey of Ohio and Michigan farm operators finds that, although generally keen to learn from others, they tend to emphasize their own experience over outside information sources. Soybean growers declare university and extension recommendations as more important than do corn growers. In response to direct queries and in free comments, growers place more emphasis on understanding the agronomic and technological problems at hand than on adjusting to the market environment. Given the decision environment, we argue that these responses are reasonable

Common variants in FOXP1 are associated with generalized vitiligo

In a recent genome-wide association study of generalized vitiligo, we identified ten confirmed susceptibility loci. By testing additional loci that showed suggestive association in the genome-wide study, using two replication cohorts of European descent, we observed replicated association of generalized vitiligo with variants at 3p13 encompassing FOXP1 (rs17008723, combined P = 1.04 × 10−8) and with variants at 6q27 encompassing CCR6 (rs6902119, combined P = 3.94 × 10−7)