Loss of appetite in amyotrophic lateral sclerosis is associated with weight loss and decreased calorie consumption independent of dysphagia.

BACKGROUND: Loss of appetite has been reported to affect up to half of people with amyotrophic lateral sclerosis (ALS) and to be associated with weight loss. We wished to test whether loss of appetite correlates with reduced dietary intake independent of dysphagia. METHODS: Appetite was measured repeatedly using the Council on Nutrition Appetite Questionnaire (CNAQ) in participants in the Electronic health Application To Measure Outcomes REmotely study. Dietary intake and weight were compared to appetite, ALS Functional Rating Scale-Revised total and bulbar scores (dysphagia). RESULTS: The average baseline CNAQ score was 30.4 (n = 61; SD = 3.9) with 18.0% scoring <28 points (severe loss of appetite). Lower CNAQ scores correlated with greater weight loss since diagnosis (Pearson correlation coefficient, r = -0.34; P = 0.009) and lower baseline energy intake (P = 0.007), independent of dysphagia. CONCLUSIONS: Our results support an association between loss of appetite and decreased calorie intake and weight in ALS which is independent of dysphagia.

Nutritional counseling with or without mobile health technology: a randomized open-label standard-of-care-controlled trial in ALS.

BACKGROUND: Nutritional status is an important prognostic factor in Amyotrophic Lateral Sclerosis (ALS). We wished to study the safety, tolerability and efficacy of nutritional counseling with or without an mHealth application to maintain or increase body weight in ALS, compared to standard care. METHODS: In this randomized open-label, standard-of-care-controlled, single-center clinical trial, we randomly assigned adults with ALS to one of three nutritional interventions: counseling by their physician or nurse ("standard care"), counseling by a registered dietitian (RD) ("in-person"), or counseling supported by a mHealth app ("mHealth"). Both intervention arms received tailored nutrition recommendations and recorded dietary intake and weight biweekly (mHealth) or monthly (in-person). The primary outcome of weight and secondary and tertiary outcomes of calorie intake, ALSFRS-R, and quality of life (QOL) were recorded at each clinic visit and analyzed in an ITT mixed model analysis. RESULTS: A total of 88 participants were enrolled of whom 78 were included in this analysis. The three arms were well-balanced except for excess males in the mHealth arm and greater weight lost at baseline in the in-person arm. Participants in the mHealth arm increased their calorie intake at month 3 to an average of 94% (95% CI: 85, 103) of recommended calories, compared to 81% (95% CI: 72, 91, p = 0.06 vs. mHealth) in the standard care arm. After 6 months, calorie intake was not different among the three arms. Overall weight was stable across all three groups. QOL scores in the mHealth arm were stable over 3 months (0.3 points, 95% CI: - 1.7, 2.2) compared to worsening in standard care (- 2.1 points, 95% CI: - 4.0, - 0.2, p = 0.09 vs. mHealth), but all scores declined by 6 months. ALSFRS-R total scores declined by an average of - 2.6 points (95% CI: - 5.1, - 0.1) over 6 months in the mHealth arm (p = 0.13 vs. standard care) compared to - 5.8 points (95% CI: - 8.2, - 3.4, p = 0.74 vs. standard care) in the in-person and - 5.2 points (95% CI: - 7.6, - 2.9) in the standard care arm. CONCLUSIONS: Nutritional counseling by a registered dietitian (with or without support by an mHealth app) is safe but did not maintain weight significantly better than standard care in ALS patients. TRIAL REGISTRATION: https://clinicaltrials.gov/ identifier NCT02418546. Registered April 16, 2015.

Girl Talk: A Smartphone Application to Teach Sexual Health Education to Adolescent Girls.

STUDY OBJECTIVE: Produce Girl Talk, a free smartphone application containing comprehensive sexual health information, and determine the application's desirability and appeal among teenage girls. DESIGN, SETTING, PARTICIPANTS, AND INTERVENTIONS: Thirty-nine girls ages 12 to 17 years from Rhode Island participated in a 2-phase prospective study. In phase I, 22 girls assessed a sexual health questionnaire in focus groups. In phase II, 17 girls with iPhones used Girl Talk for 2 weeks and answered the revised sexual health questionnaire and interview questions before and after use. MAIN OUTCOME MEASURES: Participants' responses to the sexual health questionnaire, interviews, and time viewing the application were used to determine feasibility and desirability of Girl Talk. RESULTS: Girl Talk was used on average for 48 minutes during participants' free time on weekends for 10- to 15-minute intervals. Reported usefulness of Girl Talk as a sexual health application from baseline (6 participants) to follow-up (16 participants) increased significantly (35.3% vs 94.1%; P < .001). Knowledge improved most in topics related to anatomy and physiology (70.5% to 74.7% out of 7 questions), sexuality and relationships (76.5% to 80.0% out of 10 questions), and STI prevention (75.6% to 79.0% out of 7 questions). Most phase II participants (13 out of 17, or 76.5%) were exposed to sexual health education before using Girl Talk, but 16 out of 17 participants (94.1%) stated that the application provided new and/or more detailed information than health classes. CONCLUSION: Girl Talk can potentially connect teenage girls to more information about sexual health vs traditional methods, and participants recommended the application as a valuable resource to learn about comprehensive sexual health.

Interdisciplinary Development of an Improved Emergency Department Procedural Work Surface Through Iterative Design and Use Testing in Simulated and Clinical Environments.

STUDY OBJECTIVE: A stable and readily accessible work surface for bedside medical procedures represents a valuable tool for acute care providers. In emergency department (ED) settings, the design and implementation of traditional Mayo stands and related surface devices often limit their availability, portability, and usability, which can lead to suboptimal clinical practice conditions that may affect the safe and effective performance of medical procedures and delivery of patient care. We designed and built a novel, open-source, portable, bedside procedural surface through an iterative development process with use testing in simulated and live clinical environments. METHODS: The procedural surface development project was conducted between October 2014 and June 2016 at an academic referral hospital and its affiliated simulation facility. An interdisciplinary team of emergency physicians, mechanical engineers, medical students, and design students sought to construct a prototype bedside procedural surface out of off-the-shelf hardware during a collaborative university course on health care design. After determination of end-user needs and core design requirements, multiple prototypes were fabricated and iteratively modified, with early variants featuring undermattress stabilizing supports or ratcheting clamp mechanisms. Versions 1 through 4 underwent 2 hands-on usability-testing simulation sessions; version 5 was presented at a design critique held jointly by a panel of clinical and industrial design faculty for expert feedback. Responding to select feedback elements over several surface versions, investigators arrived at a near-final prototype design for fabrication and use testing in a live clinical setting. This experimental procedural surface (version 8) was constructed and then deployed for controlled usability testing against the standard Mayo stands in use at the study site ED. Clinical providers working in the ED who opted to participate in the study were provided with the prototype surface and just-in-time training on its use when performing bedside procedures. Subjects completed the validated 10-point System Usability Scale postshift for the surface that they had used. The study protocol was approved by the institutional review board. RESULTS: Multiple prototypes and recursive design revisions resulted in a fully functional, portable, and durable bedside procedural surface that featured a stainless steel tray and intuitive hook-and-lock mechanisms for attachment to ED stretcher bed rails. Forty-two control and 40 experimental group subjects participated and completed questionnaires. The median System Usability Scale score (out of 100; higher scores associated with better usability) was 72.5 (interquartile range [IQR] 51.3 to 86.3) for the Mayo stand; the experimental surface was scored at 93.8 (IQR 84.4 to 97.5 for a difference in medians of 17.5 (95% confidence interval 10 to 27.5). Subjects reported several usability challenges with the Mayo stand; the experimental surface was reviewed as easy to use, simple, and functional. In accordance with experimental live environment deployment, questionnaire responses, and end-user suggestions, the project team finalized the design specification for the experimental procedural surface for open dissemination. CONCLUSION: An iterative, interdisciplinary approach was used to generate, evaluate, revise, and finalize the design specification for a new procedural surface that met all core end-user requirements. The final surface design was evaluated favorably on a validated usability tool against Mayo stands when use tested in simulated and live clinical settings.

Reduced heme levels underlie the exponential growth defect of the Shewanella oneidensis hfq mutant.

The RNA chaperone Hfq fulfills important roles in small regulatory RNA (sRNA) function in many bacteria. Loss of Hfq in the dissimilatory metal reducing bacterium Shewanella oneidensis strain MR-1 results in slow exponential phase growth and a reduced terminal cell density at stationary phase. We have found that the exponential phase growth defect of the hfq mutant in LB is the result of reduced heme levels. Both heme levels and exponential phase growth of the hfq mutant can be completely restored by supplementing LB medium with 5-aminolevulinic acid (5-ALA), the first committed intermediate synthesized during heme synthesis. Increasing expression of gtrA, which encodes the enzyme that catalyzes the first step in heme biosynthesis, also restores heme levels and exponential phase growth of the hfq mutant. Taken together, our data indicate that reduced heme levels are responsible for the exponential growth defect of the S. oneidensis hfq mutant in LB medium and suggest that the S. oneidensis hfq mutant is deficient in heme production at the 5-ALA synthesis step.

Shewanella oneidensis Hfq promotes exponential phase growth, stationary phase culture density, and cell survival.

BACKGROUND: Hfq is an RNA chaperone protein that has been broadly implicated in sRNA function in bacteria. Here we describe the construction and characterization of a null allele of the gene that encodes the RNA chaperone Hfq in Shewanella oneidensis strain MR-1, a dissimilatory metal reducing bacterium. RESULTS: Loss of hfq in S. oneidensis results in a variety of mutant phenotypes, all of which are fully complemented by addition of a plasmid-borne copy of the wild type hfq gene. Aerobic cultures of the hfq∆ mutant grow more slowly through exponential phase than wild type cultures, and hfq∆ cultures reach a terminal cell density in stationary phase that is ~2/3 of that observed in wild type cultures. We have observed a similar growth phenotype when the hfq∆ mutant is cultured under anaerobic conditions with fumarate as the terminal electron acceptor, and we have found that the hfq∆ mutant is defective in Cr(VI) reduction. Finally, the hfq∆ mutant exhibits a striking loss of colony forming units in extended stationary phase and is highly sensitive to oxidative stress induced by H2O2 or methyl viologen (paraquat). CONCLUSIONS: The hfq mutant in S. oneidensis exhibits pleiotropic phenotypes, including a defect in metal reduction. Our results also suggest that hfq mutant phenotypes in S. oneidensis may be at least partially due to increased sensitivity to oxidative stress.