Tyrosinemia type I is a recessive inborn error of metabolism caused by mutations in the fumarylacetoacetate hydrolase (FAH) gene, coding for the final enzyme in the metabolism of tyrosine. This renders FAH nonfunctional and without treatment, toxic metabolites accumulate causing liver and kidney damage. Introduction of the drug NTBC in 2002 offered a treatment which inhibits an upstream enzyme, preventing the production of the toxic metabolites. There is now a long-term survival rate of greater than 90 % in children, but there are reports of lower cognitive function and IQ as well as schooling and behavioral problems in these children. We studied a mouse model of tyrosinemia type I to gain insight into the effects of tyrosinemia type I and treatment with NTBC on mouse learning, memory, and behavior. In the Barnes maze, visual and spatial cues can be used by mice to remember the location of a dark escape box. The primary time, distance, and strategy taken by the mice to locate the escape box is a measure of learning and memory. Our findings show that mice with tyrosinemia type I were slower to learn than wild-type mice treated with NTBC and made more mistakes, but were capable of learning and storing long-term memory. After learning the location of the target hole, mice with tyrosinemia type I respond differently to a change in location and were less flexible in learning the new target hole location. Our findings suggest that this slower learning and cognitive difference is caused by tyrosinemia type I and not by the treatment with NTBC.
PURPOSE: No risk assessment scale exists in the United States specifically designed for use among patients with critical illness. The aim of this project was to modify the Norton Scale for Pressure Sore Risk to improve its predictive power when used in the critical care setting. PARTICIPANTS AND SETTING: The setting for this quality improvement project was a 1157-bed academic medical center in the Southeast United States. Data were collected from 114 clinicians; 111 were critical care nurses and 3 were certified wound care nurses. METHODS: Participants assessed the pressure injury risks of a video-simulated critical care patient using the optimized Norton Scale (oNS); this instrument was modified from the Norton Scale. Data were collected on reliability, validity, usability, and preference. OUTCOMES: All 114 participants accurately predicted a patient's severe high risk for pressure injury using the oNS. Predictive validity and reliability of the oNS were excellent based on a correlation coefficient of more than 0.6 and a Cronbach α = 0.944, respectively. The intraclass correlation coefficient (ICC) was 0.933 (95% confidence interval, 0.911-0.950). From 71.2% to 84.9% of the participants agreed that the oNS represented the desired characteristics for optimal usability in the critical-care setting. Preference for the oNS was associated with perceptions that it was easier, quicker, and more critical-care-specific than the Braden Scale for Pressure Sore Risk currently used in critical care units in the project facility. IMPLICATIONS FOR PRACTICE: The oNS offered critical care nurses in our facility a quick, easy-to-use, critical care- specific risk assessment tool that focused on the unique vulnerabilities of patients with critical illness.
Nursing faculty who desire to expand their research portfolios will benefit from collaboration with researchers with complimentary interests from different universities across the world. International collaboration can enhance the productivity of researchers who seek to conduct studies with similar populations in different environments, and who desire a larger impact based on the findings of their studies. International collaborative teams have the potential to make important discoveries that affect the health of populations across the world. Communication is a critical step in defining the roles and professional relationships of researchers involved in international collaboration. Researchers need to be cognizant of rules affecting data security, intellectual property, data ownership, and funding sources in each country. International collaborative research can be exciting and rewarding, especially when participants are culturally aware, respect universities' policies, and are mindful of the ethical and legal principles for the countries in which the research is conducted. This article describes ways to enhance the success of nursing faculty who desire a rich experience with international research collaborators.
Hereditary tyrosinemia type I (HT1) is caused by mutations in the fumarylacetoacetate hydrolase (FAH) gene, the template for the final enzyme in the tyrosine catabolism pathway. If left untreated this deficiency of functional FAH leads to a buildup of toxic metabolites that can cause liver disease, kidney dysfunction and high mortality. The current treatment with the drug NTBC prevents the production of these metabolites and has consequently increased the survival rate in HT1 children. As a result of this increased survival, long term complications of HT1 are now being observed, including slower learning, impaired cognition and altered social behavior. We studied a mouse model of HT1 to gain insight into the effects of HT1 and treatment with NTBC on social behavior in mice. We showed that mice with HT1 display abnormal social behavior in that they spend more time in the absence of another mouse and do not discriminate between a novel mouse and an already familiar mouse. This altered behavior was due to HT1 and not treatment with NTBC. Quantification of cerebral cortex myelin in mice with HT1 showed a two to threefold increase in myelin expression. Our findings suggest that absence of FAH expression in the brain produces an altered brain biochemistry resulting in increased expression of myelin. This increase in myelination could lead to abnormal action potential velocity and altered neuronal connections that provide a mechanism for the altered learning, social behavior and cognitive issues recently seen in HT1.
School nurses are advocates, caregivers, and teachers. It is the responsibility of school nurses to understand current prevention and treatment options. In understanding how and why coronavirus disease 2019 (COVID-19) mRNA vaccines work, school nurses are in a trusted position to explain and advocate vaccination to students and their caregivers. The messenger ribonucleic acid (mRNA) vaccine is a product of the latest scientific and medical technology. A better understanding of how and why this vaccination is effective may prevent vaccination hesitancy and provide reassurance to those choosing to accept vaccination. In December 2020, the National Association of School Nurses publicized its support for vaccination against COVID-19. As the COVID-19 pandemic lingers school nurses will step toward the front line to aid in the abatement of poor public health outcomes that may be severely affecting their schools, students, and caregivers.
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