Simulation is a valuable, immersive educational tool for both health professional trainees and experienced clinicians. By promoting a realistic, collaborative, safe, hands‐on, learning environment, simulation allows interprofessional teams to come together and practise both routine and high stakes, low‐frequency events. The COVID‐19 pandemic and the need for social distancing have shifted traditional simulation‐based medical education towards a virtual platform: telesimulation. Telesimulation is an evolving field and the speed at which clinical educators need to adapt to use this platform is unprecedented. Educators must quickly navigate and leverage the differences between traditional simulation and telesimulation to create robust remote educational experiences. Telesimulation has unique goals and objectives, technology needs, and participant roles that need to be understood and properly operationalized to maximize opportunities for learning. This article reviews the authors’ recommendations for developing and delivering successful telesimulations.
Severe bile salt export pump (BSEP) deficiency is a hereditary cholestatic condition that starts in infancy and leads to end-stage liver disease. Three children who underwent orthotopic liver transplantation for severe BSEP deficiency had post-transplantation episodes of cholestatic dysfunction that mimicked the original disease. Remission of all episodes was achieved by intensifying the immunosuppressive regimen. The phenotypic recurrence of the disease correlated with the presence of circulating high-titer antibodies against BSEP that inhibit transport by BSEP in vitro. When administered to rats, these antibodies targeted the bile canaliculi and impaired bile acid secretion.
Farnesoid X receptor (FXR) is a transcription factor that controls bile acid homeostasis. The phenotype of Fxr null mice is characterized by hypercholanaemia, impaired secretion of bile acids and failure to thrive. Human disorders with these characteristics include FIC1 disease (caused by mutations in ATP8B1, which encodes a putative aminophospholipid translocase, FIC1, whose function in bile handling is unknown) and bile salt export pump (BSEP) disease (caused by mutation in ABCB11, which encodes BSEP, the primary canalicular bile salt export pump). We investigated the possibility of hepatic down-regulation of FXR in FIC1 disease and BSEP disease. Three siblings with this phenotype, born to consanguine parents, were initially studied. The children were demonstrated to be compound heterozygotes for missense and nonsense mutations in ATP8B1. Expression of specific genes in liver was analysed, comparing one of these siblings with a child homozygous for missense mutation in ABCB11, as well as with a child having idiopathic cholestatic liver disease, a child with extrahepatic biliary atresia and a normal organ donor. The expression of two main FXR isoforms was specifically decreased in the liver of the FIC1 disease patient. A consistent and concomitant reduction in messenger RNA levels of FXR targets, such as BSEP and small heterodimer partner, was also found. Gene-profiling experiments identified 163 transcripts whose expression changed significantly in FIC1-disease liver. Of note was that several genes involved in synthesis, conjugation and transport of bile acids were down-regulated. A cluster of genes involved in lipid metabolism was also differentially expressed. Our findings suggest that hepatic down-regulation of FXR contributes to the severe cholestasis of FIC1 disease.
When infection of a prosthesis is suspected the diagnostic procedure should start with a WBC scan followed, if positive, by a BM scan. This procedure reduces the cost, the time required for a diagnosis, and the dose of radiation received by the patient.
Background: Multi-institutional, international practice variation of pediatric anaphylaxis management by healthcare providers has not been reported.Objective: Characterize variability in epinephrine administration for pediatric anaphylaxis across institutions, including frequency and types of medication errors. Methods:A prospective, observational, study using a standardized in situ simulated anaphylaxis scenario was performed across 28 healthcare institutions in six countries. The on-duty healthcare team was called for a child (patient simulator) in anaphylaxis. Real medications and supplies were obtained from their actual locations. Demographic data about team members, institutional protocols for anaphylaxis, timing of epinephrine delivery, medication errors, and systems safety issues discovered during the simulation were collected.Results: Thirty-seven in situ simulations were performed. Anaphylaxis guidelines existed in 41% (15/37) of institutions. Teams used a cognitive aid for medication dosing 41% (15/37) of the time and 32% (12/37) for preparation. Epinephrine auto injectors (EAIs) were not available in 54% (20/37) of institutions and were used in only 14% (5/37) simulations. Median time to epinephrine administration was 95 seconds (IQR 77, 252) for EAI and 263 seconds (IQR 146, 407.5) for manually prepared epinephrine (p=.12). At least one medication error occurred in 68% (25/37) of simulations. Prior nursing experience with epinephrine administration for anaphylaxis was associated with fewer preparation (p=.04) and administration (p=.01) errors.Latent safety threats (LSTs) were reported by 30% (11/37) of institutions, more than half of these (6/11) involved a cognitive aid. Conclusion and Relevance:A multicenter, international study of simulated pediatric anaphylaxis reveals: 1) variation in management between institutions in usage of protocols,
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