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FFTF implementation of training requirments for government owned facilities
The Fst Flux Facility (FFTF) is a liquid metal cooled, fast flux reactor plant. It is owned by the Department of Energy (DOE) and is operated by the Hanford Engineering Development Laboratory (HEDL) under a DOE contract with Westinghouse Hanford Company. The FFTF is presently undergoing acceptance testing of sodium systems in preparation for initial criticality in August 1979. It will be used to test fuels and materials, to develop associated components and to gain operating experience for future LMFBR's. The FFTF Training Program is a specific example of how training guidelines provided by NRC can be implemented in principle in a DOE owned reactor. The DOE requirements for government owned facilities and the NRC requirements for commercially owned facilities are compared to components of the FFTF Training program. These components will be described, actual status provided and evaluated as to meeting requirements. Cold Plant Qualification (equivalent to NRC Col Plant Licensing) is the next major milestone for FFTF Operator training. Additional requirements or constraints such as operator time utilization, recruiting and turnover, plant availbility and startup testing greatly affect the ability to meet this milestone. These constraints and requirements and the resulting compromises will be evaluated relative to meeting DOE requirements
Early Career Aquatic Scientists Forge New Connections at Eco-DAS XV
A sense of kuleana (personal responsibility) in caring for the land and sea. An appreciation for laulima (many hands cooperating). An understanding of aloha ’āina (love of the land). The University of Hawai’i at Manoa hosted the 2023 Ecological Dissertations in Aquatic Sciences (Eco-DAS) program, which fostered each of these intentions by bringing together a team of early career aquatic ecologists for a week of networking and collaborative, interdisciplinary project development (Fig. 1)
Growth and health of pre-weaned Holstein dairy heifers fed PROTERNATIVE SF in combination with LEVU-CELLS.
The African Project Failure Syndrome: The Conundrum of Project Management Knowledge Base—The Case of SADC
Better Together: Early Career Aquatic Scientists Forge New Connections at Eco‐DAS XV
A sense of kuleana (personal responsibility) in caring for the land and sea. An appreciation for laulima (many hands cooperating). An understanding of aloha 'āina (love of the land). The University of Hawai'i at Manoa hosted the 2023 Ecological Dissertations in Aquatic Sciences (Eco-DAS) program, which fostered each of these intentions by bringing together a team of early career aquatic ecologists for a week of networking and collaborative, interdisciplinary project developmen
Nusinersen Versus Sham Control In Infantile-Onset Spinal Muscular Atrophy
BACKGROUND & para;& para;Spinal muscular atrophy is an autosomal recessive neuromuscular disorder that is caused by an insufficient level of survival motor neuron (SMN) protein. Nusinersen is an antisense oligonucleotide drug that modifies pre-messenger RNA splicing of the SMN2 gene and thus promotes increased production of full-length SMN protein.& para;& para;METHODS & para;& para;We conducted a randomized, double-blind, sham-controlled, phase 3 efficacy and safety trial of nusinersen in infants with spinal muscular atrophy. The primary end points were a motor-milestone response (defined according to results on the Hammersmith Infant Neurological Examination) and event-free survival (time to death or the use of permanent assisted ventilation). Secondary end points included over all survival and subgroup analyses of event-free survival according to disease duration at screening. Only the first primary end point was tested in a prespecified interim analysis. To control the overall type I error rate at 0.05, a hierarchical testing strategy was used for the second primary end point and the secondary end points in the final analysis.& para;& para;RESULTS & para;& para;In the interim analysis, a significantly higher percentage of infants in the nusinersen group than in the control group had a motor-milestone response (21 of 51 infants [41 %] vs. 0 of 27 [0%], P<0.001), and this result prompted early termination of the trial. In the final analysis, a significantly higher percentage of infants in the nusinersen group than in the control group had a motor-milestone response (37 of 73 infants [51%] vs. 0 of 37 [0%]), and the likelihood of event-free survival was higher in the nusinersen group than in the control group (hazard ratio for death or the use of permanent assisted ventilation, 0.53; P=0.005). The likelihood of overall survival was higher in the nusinersen group than in the control group (hazard ratio for death, 0.37; P=0.004), and infants with a shorter disease duration at screening were more likely than those with a longer disease duration to benefit from nusinersen. The incidence and severity of adverse events were similar in the two groups.& para;& para;CONCLUSIONS & para;& para;Among infants with spinal muscular atrophy, those who received nusinersen were more likely to be alive and have improvements in motor function than those in the control group. Early treatment may be necessary to maximize the benefit of the drug.Wo
Nusinersen versus Sham Control in Later-Onset Spinal Muscular Atrophy
International audienceBACKGROUND Nusinersen is an antisense oligonucleotide drug that modulates pre-messenger RNA splicing of the survival motor neuron 2 (SMN2) gene. It has been developed for the treatment of spinal muscular atrophy (SMA). METHODS We conducted a multicenter, double-blind, sham-controlled, phase 3 trial of nusinersen in 126 children with SMA who had symptom onset after 6 months of age. The children were randomly assigned, in a 2: 1 ratio, to undergo intrathecal administration of nusinersen at a dose of 12 mg (nusinersen group) or a sham procedure (control group) on days 1, 29, 85, and 274. The primary end point was the least-squares mean change from baseline in the Hammersmith Functional Motor Scale-Expanded (HFMSE) score at 15 months of treatment; HFMSE scores range from 0 to 66, with higher scores indicating better motor function. Secondary end points included the percentage of children with a clinically meaningful increase from baseline in the HFMSE score (>= 3 points), an outcome that indicates improvement in at least two motor skills. RESULTS In the prespecified interim analysis, there was a least-squares mean increase from baseline to month 15 in the HFMSE score in the nusinersen group (by 4.0 points) and a least-squares mean decrease in the control group (by -1.9 points), with a significant between-group difference favoring nusinersen (least-squares mean difference in change, 5.9 points; 95% confidence interval, 3.7 to 8.1; P< 0.001). This result prompted early termination of the trial. Results of the final analysis were consistent with results of the interim analysis. In the final analysis, 57% of the children in the nusinersen group as compared with 26% in the control group had an increase from baseline to month 15 in the HFMSE score of at least 3 points (P< 0.001), and the overall incidence of adverse events was similar in the nusinersen group and the control group (93% and 100%, respectively). CONCLUSIONS Among children with later-onset SMA, those who received nusinersen had significant and clinically meaningful improvement in motor function as compared with those in the control group. (Funded by Biogen and Ionis Pharmaceuticals; CHERISH ClinicalTrials. gov number, NCT02292537.