Senior Leaders Should Use Leading Companies’ Key Practices to Improve Performance

Symposium PresentationApproved for public release; distribution is unlimited

Federal Contracting: Senior Leaders Should Use Leading Companies’ Key Practices to Improve Performance

Excerpt from the Proceedings of the Nineteenth Annual Acquisition Research SymposiumEach year, federal agencies spend over $500 billion to buy a wide variety of products and services, ranging from cutting-edge military aircraft to common office supplies. Given the federal funds spent and the missions these contracts support, it is critical that agencies’ procurement leaders manage their organizations effectively. GAO found procurement leaders at six of the federal government’s largest agencies did not consistently use key practices that leading companies use to improve the performance of their procurement organizations. For example, only the procurement leaders at NASA collaborated with end users when developing performance metrics. Corporate procurement leaders told GAO that collaboration with end users during the development and implementation of performance metrics increases coordination and improves performance at the strategic level. Additionally, GAO found procurement leaders at most of the agencies reviewed had ongoing or planned efforts to use performance metrics to measure at least one of the four procurement outcomes identified as important by corporate procurement leaders: (1) cost savings/avoidance, (2) timeliness of deliveries, (3) quality of deliverables, and (4) end-user satisfaction. However, all of the leaders had work to do to fully implement metrics measuring these outcomes. The original GAO report is accessible at www.gao.gov/products/gao-21-491.Approved for public release; distribution is unlimited

Federal Contracting: Senior Leaders Should Use Leading Companies’ Key Practices to Improve Performance

Excerpt from the Proceedings of the Nineteenth Annual Acquisition Research SymposiumEach year, federal agencies spend over $500 billion to buy a wide variety of products and services, ranging from cutting-edge military aircraft to common office supplies. Given the federal funds spent and the missions these contracts support, it is critical that agencies’ procurement leaders manage their organizations effectively. GAO found procurement leaders at six of the federal government’s largest agencies did not consistently use key practices that leading companies use to improve the performance of their procurement organizations. For example, only the procurement leaders at NASA collaborated with end users when developing performance metrics. Corporate procurement leaders told GAO that collaboration with end users during the development and implementation of performance metrics increases coordination and improves performance at the strategic level. Additionally, GAO found procurement leaders at most of the agencies reviewed had ongoing or planned efforts to use performance metrics to measure at least one of the four procurement outcomes identified as important by corporate procurement leaders: (1) cost savings/avoidance, (2) timeliness of deliveries, (3) quality of deliverables, and (4) end-user satisfaction. However, all of the leaders had work to do to fully implement metrics measuring these outcomes. The original GAO report is accessible at www.gao.gov/products/gao-21-491.Approved for public release; distribution is unlimited

Association of Variants in the SPTLC1 Gene With Juvenile Amyotrophic Lateral Sclerosis

Importance: Juvenile amyotrophic lateral sclerosis (ALS) is a rare form of ALS characterized by age of symptom onset less than 25 years and a variable presentation.Objective: To identify the genetic variants associated with juvenile ALS.Design, Setting, and Participants: In this multicenter family-based genetic study, trio whole-exome sequencing was performed to identify the disease-associated gene in a case series of unrelated patients diagnosed with juvenile ALS and severe growth retardation. The patients and their family members were enrolled at academic hospitals and a government research facility between March 1, 2016, and March 13, 2020, and were observed until October 1, 2020. Whole-exome sequencing was also performed in a series of patients with juvenile ALS. A total of 66 patients with juvenile ALS and 6258 adult patients with ALS participated in the study. Patients were selected for the study based on their diagnosis, and all eligible participants were enrolled in the study. None of the participants had a family history of neurological disorders, suggesting de novo variants as the underlying genetic mechanism.Main Outcomes and Measures: De novo variants present only in the index case and not in unaffected family members.Results: Trio whole-exome sequencing was performed in 3 patients diagnosed with juvenile ALS and their parents. An additional 63 patients with juvenile ALS and 6258 adult patients with ALS were subsequently screened for variants in the SPTLC1 gene. De novo variants in SPTLC1 (p.Ala20Ser in 2 patients and p.Ser331Tyr in 1 patient) were identified in 3 unrelated patients diagnosed with juvenile ALS and failure to thrive. A fourth variant (p.Leu39del) was identified in a patient with juvenile ALS where parental DNA was unavailable. Variants in this gene have been previously shown to be associated with autosomal-dominant hereditary sensory autonomic neuropathy, type 1A, by disrupting an essential enzyme complex in the sphingolipid synthesis pathway.Conclusions and Relevance: These data broaden the phenotype associated with SPTLC1 and suggest that patients presenting with juvenile ALS should be screened for variants in this gene.</p

Defense Acquisitions: Senior Leaders Should Emphasize Key Practices to Improve Weapon System Reliability

Panel #13: Lessons Learned in MDAP Reliability, Maintainability and SustainmentAcquisition Research: Creating Synergy for Informed Change. 17th Annual Acquisition Research Symposiu