Computer-Assisted Prototyping of Advanced Microsystems

Contains reports on five research projects.Defense Advanced Research Projects Agency Contract DABT 63-95-C-0088Stanford Universit

Custom Integrated Circuits

Contains table of contents for Part III, table of contents for Section 1 and reports on eleven research projects.IBM CorporationMIT School of EngineeringNational Science Foundation Grant MIP 94-23221Defense Advanced Research Projects Agency/U.S. Army Intelligence Center Contract DABT63-94-C-0053Mitsubishi CorporationNational Science Foundation Young Investigator Award Fellowship MIP 92-58376Joint Industry Program on Offshore Structure AnalysisAnalog DevicesDefense Advanced Research Projects AgencyCadence Design SystemsMAFET ConsortiumConsortium for Superconducting ElectronicsNational Defense Science and Engineering Graduate FellowshipDigital Equipment CorporationMIT Lincoln LaboratorySemiconductor Research CorporationMultiuniversity Research IntiativeNational Science Foundatio

Nanostructures, Technology, Research, and Applications

Contains reports on twenty research projects and a list of publications.Joint Services Electronics Program Grant DAAH04-95-1-0038National Science Foundation Grant ECS-94-07078Semiconductor Research CorporationU.S. Army Research Office Grant DAAH04-95-1-0564Defense Advanced Research Projects Agency/Naval Air Systems Command Contract N00019-95-K-0131National Aeronautics and Space Administration Contract NAS8-38249National Aeronautics and Space Administration Grant NAGW-2003IBM Corporation Contract 1622National Science Foundation Graduate FellowshipU.S. Navy - Office of Naval Research Grant N00014-95-1-1297U.S. Army Research Office Contract DAAH04-94-G-0377U.S. Air Force - Office of Scientific Research Grant F49620-92-J-0064U.S. Air Force - Office of Scientific Research Grant F49620-95-1-0311National Science Foundation Contract DMR 94-0034U.S. Air Force - Office of Scientific Research Contract F49620-96-0126Harvard-Smithsonian Astrophysical Observatory Contract SV630304National Aeronautics and Space Administration Grant NAG5-5105Los Alamos National Laboratory Contract E57800017-9

Outcomes and treatment strategies for autoimmunity and hyperinflammation in patients with RAG deficiency

BACKGROUND: While autoimmunity and hyperinflammation secondary to recombinase activating gene (RAG) deficiency have been associated with delayed diagnosis and even death, our current understanding is limited primarily to small case series. OBJECTIVE: Understand the frequency, severity, and treatment responsiveness of autoimmunity and hyperinflammation in RAG deficiency. METHODS: In reviewing the literature and our own database, we identified 85 patients with RAG deficiency, reported between 2001 and 2016, and compiled the largest case series to date of 63 patients with prominent autoimmune and/or hyperinflammatory pathology. RESULTS: Diagnosis of RAG deficiency was delayed a median of 5 years from the first clinical signs of immune dysregulation. The majority of patients (55.6%) presented with more than one autoimmune or hyperinflammatory complication, with the most common etiologies being cytopenias (84.1%), granulomas (23.8%), and inflammatory skin disorders (19.0%). Infections, including live viral vaccinations, closely preceded the onset of autoimmunity in 28.6% of cases. Autoimmune cytopenias had early onset (median 1.9, 2.1, and 2.6 years for autoimmune hemolytic anemia (AIHA), immune thrombocytopenia (ITP) and autoimmune neutropenia (AN), respectively) and were refractory to intravenous immunoglobulin, steroids, and rituximab in the majority of cases (64.7%, 73.7%, and 71.4% for AIHA, ITP, and AN, respectively). Evans syndrome specifically was associated with lack of response to first-line therapy. Treatment-refractory autoimmunity/hyperinflammation prompted hematopoietic stem cell transplantation in 20 patients. CONCLUSIONS: Autoimmunity/hyperinflammation can be a presenting sign of RAG deficiency and should prompt further evaluation. Multi-lineage cytopenias are often refractory to immunosuppressive treatment and may require hematopoietic cell transplantation for definitive management

System-Level Design Methods for Low-Energy Architectures Containing Variable Voltage Processors

This paper focuses on system-level design methods for low energy consumption in architectures employing variable-voltage processors. Two low-energy design flows are introduced