Stormont Vail Health

About: Stormont Vail Health is a(n) healthcare organization based out in Topeka, Kansas, United States. It is known for research contribution in the topic(s): Ferritin & Population. The organization has 22 authors who have published 27 publication(s) receiving 154 citation(s).

ALG1-CDG: clinical and molecular characterization of 39 unreported patients

Abstract: Congenital disorders of glycosylation (CDG) arise from pathogenic mutations in over one hundred genes leading to impaired protein or lipid glycosylation. ALG1 encodes a β1,4 mannosyltransferase that catalyzes the addition of the first of nine mannose moieties to form a dolichol-lipid linked oligosaccharide intermediate (DLO) required for proper N-linked glycosylation. ALG1 mutations cause a rare autosomal recessive disorder termed ALG1-CDG. To date thirteen mutations in eighteen patients from fourteen families have been described with varying degrees of clinical severity. We identified and characterized thirty-nine previously unreported cases of ALG1-CDG from thirty-two families and add twenty-six new mutations. Pathogenicity of each mutation was confirmed based on its inability to rescue impaired growth or hypoglycosylation of a standard biomarker in an alg1-deficient yeast strain. Using this approach we could not establish a rank order comparison of biomarker glycosylation and patient phenotype, but we identified mutations with a lethal outcome in the first two years of life. The recently identified protein-linked xeno-tetrasaccharide biomarker, NeuAc-Gal-GlcNAc2, was seen in all twenty-seven patients tested. Our study triples the number of known patients and expands the molecular and clinical correlates of this disorder.

Time to Change Dosing of Inactivated Quadrivalent Influenza Vaccine in Young Children: Evidence From a Phase III, Randomized, Controlled Trial

Abstract: Background. Children under 3 years of age may benefit from a double-dose of inactivated quadrivalent influenza vaccine (IIV4) instead of the standard-dose. Methods. We compared the only United States-licensed standard-dose IIV4 (0.25 mL, 7.5 µg hemagglutinin per influenza strain) versus double-dose IIV4 manufactured by a different process (0.5 mL, 15 µg per strain) in a phase III, randomized, observer-blind trial in children 6-35 months of age (NCT02242643). The primary objective was to demonstrate immunogenic noninferiority of the double-dose for all vaccine strains 28 days after last vaccination. Immunogenic superiority of the double-dose was evaluated post hoc. Immunogenicity was assessed in the per-protocol cohort (N = 2041), and safety was assessed in the intent-to-treat cohort (N = 2424). Results. Immunogenic noninferiority of double-dose versus standard-dose IIV4 was demonstrated in terms of geometric mean titer (GMT) ratio and seroconversion rate difference. Superior immunogenicity against both vaccine B strains was observed with double-dose IIV4 in children 6-17 months of age (GMT ratio = 1.89, 95% confidence interval [CI] = 1.64-2.17, B/Yamagata; GMT ratio = 2.13, 95% CI = 1.82-2.50, B/Victoria) and in unprimed children of any age (GMT ratio = 1.85, 95% CI = 1.59-2.13, B/Yamagata; GMT ratio = 2.04, 95% CI = 1.79-2.33, B/Victoria). Safety and reactogenicity, including fever, were similar despite the higher antigen content and volume of the double-dose IIV4. There were no attributable serious adverse events. Conclusions. Double-dose IIV4 may improve protection against influenza B in some young children and simplifies annual influenza vaccination by allowing the same vaccine dose to be used for all eligible children and adults.

Baccalaureate outcomes met by associate degree nursing programs.

Abstract: In a statewide study of nurse educators from nationally accredited ADN programs, 42 of 109 baccalaureate outcomes were reported met in their programs Those outcomes clustered in 3 areas: information management and application of technology, professionalism and professional values, and generalist nursing practice The 67 outcomes that were not met were in the categories of liberal education, organizational and systems leadership, evidence-based practice, healthcare policy, finance and regulatory environments, interprofessional collaboration, and population health This research provides important evidence from which to construct an outcomes-based associate degree to baccalaureate curriculum

Effect of Maternal Cigarette Smoking on Newborn Iron Stores.

Abstract: Background Maternal smoking has been known to have a negative impact on the well being of the developing fetus. Prenatal smoking has been associated with premature births, low birth weight and with certain birth defects. Small research studies have also found a negative correlation between maternal smoking and neonatal body iron. Objectives To study and compare the relationship between maternal and infants' body iron in smokers and non-smokers in a large matched-pair cohort. Methods This was a prospective cohort study involving 144 mothers - 72 smokers and 72 non-smokers and their respective infants. Samples were obtained from maternal and infants' cord blood at delivery for Serum transferrin receptor (sTfR) and ferritin levels. Serum TfR and ferritin were measured by RAMCO ELISA and RIA assays. Total Body Iron (TBI) was calculated using the sTfR/ferritin ratio in a previously described formula by Cook et al. Results Women who smoked had lower sTfR, higher ferritin and higher body iron compared to nonsmoking women. In contrast to their respective mothers, we found a small, but statistically significant negative correlation between smoking and infants' total body iron. The number of packs per day smoked was also negatively correlated with infants' ferritin and total body iron. Lower birth weight was noted in babies of smokers compared to nonsmokers (mean /- SD =3270 +/-475 vs. 3393 g +/- 475 g, p=0.03). Conclusion Women who smoked during pregnancy had higher iron stores but their newborn infants had lower iron stores than those of non-smoking mothers. The more packs per day (PPD) and more days smoked during pregnancy led to lower total body iron of the babies. There may be a negative dose-dependent response between fetal smoke exposure and infant iron stores.

Does Systems Thinking Improve the Perception of Safety Culture and Patient Safety

Abstract: Introduction Adverse patient events are frequently associated with medication administration errors. Despite implementation of barcode technology, medication administration errors continue, often because of system issues. Integrating systems thinking into nursing practice facilitates identification and correction of factors that interfere with patient safety. Safety culture is positively associated with patient outcomes. The purpose of this study was to improve patient safety with respect to medication administration through an intervention designed to enhance systems thinking (Systems Thinking Education Program, STEP). Aims Specific aims were to identify nurse workarounds during medication administration, to assess changes in the rates of medication events and workarounds after STEP, to assess changes in systems thinking and safety culture after STEP, and to correlate safety culture and systems thinking. Methods This study was a pre-post comparison with a STEP intervention (including medication huddles) and organization-wide monthly education for 1 year. Outcome measures included perception of safety culture, as measured by the Safety Attitudes Questionnaire, and systems thinking, as measured by the Systems Thinking Scale. All organization nurses were invited to complete preintervention and postintervention electronic surveys via an e-mail link. Additionally, medication event rates and workaround rates were determined by direct medication administration observations on eight units (six inpatient and two ambulatory) that were conducted before and after intervention with trained data collectors. Results A total of 1,652 medication observations before intervention and 1,998 observations after intervention were reported. The workaround rate was significantly lower after STEP (175 workarounds out of 1,998 observations; 8.8%) compared with before (305 workarounds out of 1,652 observations; 18.5%), p Conclusion The STEP intervention strengthened understanding of systems thinking and revealed the importance of addressing the nurse as a second victim of medication errors, which is likely to be central to safety culture for nurses. Medication huddles may be a useful intervention to improve systems thinking.