Andersen-Tawil Syndrome

Andersen-Tawil syndrome (ATS) is a rare condition consisting of ventricular arrhythmias, periodic paralysis, and dysmorphic features. In 2001, mutations in KCNJ2, which encodes the α subunit of the potassium channel Kir2.1, were identified in patients with ATS. To date, KCNJ2 is the only gene implicated in ATS, accounting for approximately 60% of cases. ATS is a unique channelopathy, and represents the first link between cardiac and skeletal muscle excitability. The arrhythmias observed in ATS are distinctive; patients may be asymptomatic, or minimally symptomatic despite a high arrhythmia burden with frequent ventricular ectopy and bidirectional ventricular tachycardia. However, patients remain at risk for life-threatening arrhythmias, including torsades de pointes and ventricular fibrillation, albeit less commonly than observed in other genetic arrhythmia syndromes. The characteristic heterogeneity at both the genotypic and phenotypic levels contribute to the continued difficulties with appropriate diagnosis, risk stratification, and effective therapy. The initial recognition of a syndromic association of clinically diverse symptoms, and the subsequent identification of the underlying molecular genetic basis of ATS has enhanced both clinical care, and our understanding of the critical function of Kir2.1 on skeletal muscle excitability and cardiac action potentia

Multi-wavelength Observations of LS I +61 303 with VERITAS, Swift and RXT E: 2006-2008

A long term, multi-wavelength monitoring campaign on the TeV binary LS I +61 303 has been performed utilizing >300 GeV observations with VERITAS along with monitoring in the 0.2-10 keV band by RXTE and Swift between September 2006 and February 2008. The source was detected by VERITAS as a variable TeV source with flux values ranging from 5-20% of the Crab Nebula flux with the strongest flux levels appearing around apastron. X-ray observations by RXTE and Swift show the source as a highly variable hard X-ray source with flux values varying in the range of 0.5-3*10^-11 ergs cm^-2 s^-1 over a single orbital cycle. The 2007-2008 RXTE data set also shows the presence of several extremely large flaring episodes presenting a flux of up to 7.2*10^-11 ergs cm^-2 s^-1, the largest such flare recorded from this source. Comparison of the contemporaneous TeV and X-ray data does not show a correlation at this time, however, the sparsity of data sets do not preclude the existence of such a correlation.Comment: Submitted to Proceedings of "4th Heidelberg International Symposium on High Energy Gamma-Ray Astronomy 2008

Data Note: Shifts in VR Outcome Trends for VR Customers With and Without Intellectual Disabilities

People with intellectual disabilities (ID) aspire to gainful employment1. To assist them with this goal, state vocational rehabilitation (VR) agencies offer employment services based upon Individualized Plans for Employment (IPEs). A commonly used measure of outcomes is the rehabilitation rate, defined as the percentage of individuals exiting the program who have achieved an employment outcome after receiving services with an IPE. This indicator does not consider that not all eligible consumers progress to receive services after an IPE is developed. This occurs for a variety of individual, service, and systems reasons. Reviewing data for eligible consumers who do not receive services provides a more complete picture of the experiences of individuals who engage with and exit the VR system

Data Note: WIA Employment Outcomes

The Workforce Investment Act (WIA) requires One-Stop system partners who provide employment services funded by the U.S. Department of Labor to report data on performance measures. These data include the rate of customers entering employment, their employment retention rate, and their rate of earning a work credential. WIA tracks a number of funding streams for different audiences. This Data Note focuses on adults in the general population and dislocated workers