Spastic Ataxias (SA) are a group of neurodegenerative disorders with combined pyramidal and cerebellar system affection, leading to an overlap phenotype between Hereditary Spastic Paraplegias (HSP) ...and Cerebellar Ataxias (CA). Here we describe the generation of iPSCs from three unrelated patients with an ultra-rare subtype of SA caused by compound heterozygous mutations in POLR3A, that encodes the largest subunit of RNA polymerase III. iPSCs were reprogrammed from normal human dermal fibroblasts (NHDFs) using episomal reprogramming with integration-free plasmid vectors: HIHRSi004-A, derived from a 44 year-old male carrying the mutations c.1909 + 22G > A/c.3944_3945delTG, HIHRSi005-A obtained from a 66 year-old male carrying the mutations c.1909 + 22G > A/c.1531C > T, and HIHRSi006-A from a 27 year-old male carrying the mutations c.1909 + 22G > A/c.2472_2472delC (ENST00000372371.8).
Early transfer to specialized centers improves trauma and burn outcomes; however, overtriage can result in unnecessary burdens to patients, providers, and health systems. Our institution developed ...novel burn triage pathways in 2016 to improve resource allocation. We evaluated the implementation of these pathways, analyzing trends in adoption, resource optimization, and pathway reliability after implementation.
Triage pathways consist of transfer nurses (RNs) triaging calls based on review of burn images and clinical history: green pathway for direct outpatient referral, blue pathway for discussion with the on-call provider, red pathway for confirmation of transfer as requested by referring provider, and black pathway for the rapid transfer of severe burns. We used the RE-AIM (Reach, Effectiveness, Adoption, Implementation, Maintenance) framework to evaluate implementation. These pathways affected all acute burn referrals to our center from January 2017 to December 2019 (reach). Outcomes of interest were pathway assignment over time (adoption), changes to burn provider call volume (effectiveness), and the concordance of pathway assignment with final disposition (implementation reliability).
Transfer RNs triaged 5,272 burn referrals between 2017 and 2019. By January 2018, >98% of referrals were assigned a pathway. In 2018-2019, green pathway calls triaged by RNs reduced calls to burn providers by a mean of 40 (SD, 11) per month. Patients in green/blue pathways were less likely to be transferred, with >85% receiving only outpatient follow-up ( p < 0.001). Use of the lower acuity pathways increased over time, with a concordant decrease in use of the higher acuity pathways. Younger adults, patients referred from Level III to Level V trauma centers and nontrauma hospitals, and patients referred by APPs were less likely to be triaged to higher acuity pathways.
Implementation of highly adopted, reliable triage pathways can optimize existing clinical resources by task-shifting triage of lower acuity burns to nursing teams.
Prognostic and Epidemiological; Level III.
P-V-T equation of state (P-V-T EOS) of gold to high pressure and temperature are studied by the statistical moment method. We obtain P-V-T equation of state for gold of simple analytic form, without ...adjustment parameters. The numerical results of the pressure P at compression x on the isotherms T of gold by this equation of state in good agreement with the data calculated by the semi-empirical equations of state, which have adjustable parameters, up to 600kbar and 2000K. The thermodynamic quantities of gold are also obtained from this equation of state. The numerical results of the thermal expansion coefficient, heat capacity of gold are in excellent agreement with the experimental data. Due to the simplicity and efficiency of gold equation of state obtained, this equation of state can be used as pressure scales to 600kbar and 2000K.
•P-V-T EOS of gold to high pressure and temperature derived by statistical moment method (SMM).•Isotherms at temperatures 300K to 2000K and pressures up to 600kbar have been evaluated.•Thermodynamic quantities at high pressure and temperature have been calculated and evaluated.•Very good agreement with the experimental data in the literature has been achieved.
This study deals with a fault-tolerant control (FTC) strategy for a marine current energy conversion system based on a five-phase permanent magnet synchronous generator. First, a finite control ...set-model predictive control is adopted to highlight the advantages of this kind of generator in normal mode. The speed tracking performance is evaluated when the system operates under swell effect. Second, its fault tolerance is evaluated under various open-circuit fault conditions. In this case, the reference currents are reconfigured online to achieve the reference torque while minimising the copper losses. Extensive simulations, based on real-tidal speed data measured at the Raz-de-Sein site in Bretagne, France, are carried out for the validation of the proposed FTC strategy.
Regionalized care for burn-injured patients requires accurate triage. In 2016, we implemented a tele-triage system for acute burn consultations. We evaluated resource utilization following ...implementation, hypothesizing that this system would reduce short-stay admissions and prioritize inpatient care for those with higher burn severity.
We conducted a retrospective study of all transferred patients with acute burn injuries from January 1, 2010 to December 31, 2015, and January 1, 2017 to December 31, 2019. We evaluated the proportions of short-stay admissions (discharges less than 24 hours without operative intervention, ICU admission, or concern for nonaccidental trauma) among patients transferred before (2010 to 2015) and after (2017 to 2019) triage system implementation. Multivariable Poisson regression was used to evaluate factors associated with short-stay admissions. Interrupted time series analysis was used to evaluate the effect of the triage system.
There were 4,688 burn transfers (3,244 preimplementation and 1,444 postimplementation) in the study periods. Mean age was higher postimplementation (32 vs 29 years, p < 0.001). Median hospital length of stay (LOS) and ICU LOS were both 1 day higher, more patients underwent operative intervention (19% vs 16%), and median time to first operation was 1 day lower postimplementation. Short-stay admissions decreased from 50% (n = 1,624) to 39% (n = 561), and patients were 17% less likely to have a short-stay admission after implementation (adjusted relative risk aRR, 0.83; 95% CI, 0.8 to 0.9). Pediatric patients younger than 15 years old composed 43% of all short-stay admissions and were much more likely than adult patients to have a short-stay admission independent of transfer timing (aRR, 2.36; 95% CI, 1.84 to 3.03).
Tele-triage burn transfer center protocols reduced short-stay admissions and prioritized inpatient care for patients with more severe injuries. Pediatric patients remain more likely to have short-stay admission after transfer.
This Clinical Practice Guideline (CPG) addresses the topic of acute fluid resuscitation during the first 48 hours following a burn injury for adults with burns ≥20% of the total body surface area ...(%TBSA). The listed authors formed an investigation panel and developed clinically relevant PICO (Population, Intervention, Comparator, Outcome) questions. A systematic literature search returned 5978 titles related to this topic and after 3 levels of screening, 24 studies met criteria to address the PICO questions and were critically reviewed. We recommend that clinicians consider the use of human albumin solution, especially in patients with larger burns, to lower resuscitation volumes and improve urine output. We recommend initiating resuscitation based on providing 2 mL/kg/% TBSA burn in order to reduce resuscitation fluid volumes. We recommend selective monitoring of intra-abdominal and intraocular pressure during burn shock resuscitation. We make a weak recommendation for clinicians to consider the use of computer decision support software to guide fluid titration and lower resuscitation fluid volumes. We do not recommend the use of transpulmonary thermodilution-derived variables to guide burn shock resuscitation. We are unable to make any recommendations on the use of high-dose vitamin C (ascorbic acid), fresh frozen plasma (FFP), early continuous renal replacement therapy, or vasopressors as adjuncts during acute burn shock resuscitation. Mortality is an important outcome in burn shock resuscitation, but it was not formally included as a PICO outcome because the available scientific literature is missing studies of sufficient population size and quality to allow us to confidently make recommendations related to the outcome of survival at this time.
Health-related quality of life is decreased in burn survivors, with scars implicated as a cause. The authors aim to characterize the use of reconstructive surgery following hospitalization and ...determine whether patient-reported outcomes change over time. The authors hypothesized improvement in health-related quality of life following reconstructive surgery.
Adult burn survivors undergoing reconstructive surgery within 24 months after injury were extracted from a prospective, longitudinal database from 5 U.S. burn centers (Burn Model System). Surgery was classified by problem as follows: scar, contracture, and open wound. The authors evaluated predictors of surgery using logistic regression. Short Form-12/Veterans RAND 12 health survey outcomes at 6, 12, and 24 months were compared at follow-up intervals and matched with nonoperated participants using propensity score matching.
Three hundred seventy-two of 1359 participants (27.4 percent) underwent one or more reconstructive operation within 24 months of injury. Factors that increased the likelihood of surgery included number of operations during index hospitalization (p < 0.001), hand (p = 0.001) and perineal involvement (p = 0.042), and range-of-motion limitation at discharge (p < 0.001). Compared to the physical component scores of peers who were not operated on, physical component scores increased for participants undergoing scar operations; however, these gains were only significant for those undergoing surgery more than 6 months after injury (p < 0.05). Matched physical component scores showed nonsignificant differences following contracture operations. Mental component scores were unchanged or lower following scar and contracture surgery.
Participants requiring more operations during index admission were more likely to undergo reconstructive surgery. There were improvements in Short Form-12/Veterans RAND 12 scores for those undergoing scar operations more than 6 months after injury, although contracture operations were not associated with significant differences in Short Form-12/Veterans RAND 12 scores.
Skin tissue is recognized to exhibit rate-dependent mechanical behavior under various loading conditions. Here, we report that the full-thickness burn human skin exhibits rate-independent behavior ...under uniaxial tensile loading conditions. Mechanical properties, namely, ultimate tensile stress, ultimate tensile strain, and toughness, and parameters of Veronda-Westmann hyperelastic material law were assessed via uniaxial tensile tests. Univariate hypothesis testing yielded no significant difference (p > 0.01) in the distributions of these properties for skin samples loaded at three different rates of 0.3 mm/s, 2 mm/s, and 8 mm/s. Multivariate multiclass classification, employing a logistic regression model, failed to effectively discriminate samples loaded at the aforementioned rates, with a classification accuracy of only 40%. The median values for ultimate tensile stress, ultimate tensile strain, and toughness are computed as 1.73 MPa, 1.69, and 1.38 MPa, respectively. The findings of this study hold considerable significance for the refinement of burn care training protocols and treatment planning, shedding new light on the unique, rate-independent behavior of burn skin.
Copper (Cu) is an essential dietary supplement in animal feeds, which plays an important role in maintaining the balance of all living organisms. Copper nanoparticles (nCu) participate in catalysing ...activities of multiple antioxidant/defensive enzymes and exerts pro-inflammatory and pro-apoptotic effects on systemic organs and tissues. The present study explored whether nCu affects maize growth and yield and grain mineral nutrients as well as physiological functions in mice.
Maize seeds were treated with nCu (20 mg/kg and 1000 mg/kg dry weight (DW)) and their grain productions were used for mouse feed. For testing of autoimmune response, mice were treated with nCu at concentration of 2 mg/l and 1000 mg/l and ultimately serum biochemical indicators, numbers and activation of immune cells infiltrated in mouse spleens were examined.
Treatment of maize seeds with nCu at dose of 20 mg/kg DW, but not 1000 mg/kg DW enhanced germination rate, plant growth and grain yield as well as grain mineral nutrients as compared to control group. Importantly, administration of mice with 1000 mg/l nCu resulted in their morphological change due to excessive accumulation of nCu in liver and blood, leading to inflammatory responses involved in upregulated expression of serum biochemical indicators of liver and kidney as well as increased infiltration and activation of splenic immune cells.
nCu concentration at 20 mg/kg DW facilitated the morphological and functional development of maize plants, whose production was safe to feed mice.
