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1.
MedEdPORTAL ; 13: 10550, 2017 Mar 07.
Article in English | MEDLINE | ID: mdl-30800752

ABSTRACT

INTRODUCTION: The patient is a 40-kg, 12-year-old Caucasian male with history of asthma who is undergoing an elective inguinal hernia repair. There is no family history of anesthesia-related complications. The surgery proceeds under general anesthesia with an IV induction with propofol, fentanyl, and succinylcholine; intubation under direct laryngoscopy; and maintenance with isoflurane. During the surgery, he develops malignant hyperthermia (MH). METHODS: Learners are to identify the signs of MH, including tachycardia, hypercapnia, muscle rigidity, and renal failure, and provide the appropriate treatment, resuscitation, and follow-up care. Anesthesiology faculty in the room assist and offer guided instruction to aid the learners in achieving these goals. RESULTS: The simulation was completed by 24 medical students with 2 weeks of anesthesia training and daily lectures on various anesthesia topics. Verbal feedback from the learners was positive, and many appreciated the preparation in how to prioritize the management of such a rare but life-threatening anesthesia emergency. Based on reviewers' recommendations, a learner evaluation of the session and pre- and posttest exams have been developed but have not yet been used with learners. DISCUSSION: The simulation not only was received well by the students but was also crucial to understanding the benefits of simulation training in the field of anesthesiology, especially when rare diseases are difficult to encounter in real life. Future simulations will incorporate other rare but important disease processes in the simulation training environment to allow anesthesia providers to learn in a safe setting without detriment to any patient.

3.
Contemp Nurse ; 46(1): 18-27, 2013 Dec.
Article in English | MEDLINE | ID: mdl-24716758

ABSTRACT

BACKGROUND: This paper identifies the latent opportunities and challenges inherent in the formative stages of a project that was resumed after partial completion by other on-the-ground research teams. METHODS: Grounded theory methods were used to analyse project documentation from previous research teams and to generate new process-oriented data. RESULTS: The intention of all research teams was moving towards Engaging in Community-Based Participatory Research; this was conceptually identified as the core category. The social process involved in achieving community engagement practice was named Coming to an Ethics of Practice. Four different facets comprised the core category: Developing meaningful relationships; being reflective; recognising difference; and making research relevant. CONCLUSIONS: To achieve mutually beneficial outcomes, researchers conducting community-based research with Aboriginal people must implement strengths-based approaches to realise ethically sound research; prioritise the relevance of the research to the daily lives, needs and aspirations of those with whom they work; and in doing so, remain cognisant of their own philosophical position and context in which the research is located.


Subject(s)
Ethics, Research , Health Services Research/ethics , Native Hawaiian or Other Pacific Islander , Rural Population , Humans , Queensland
4.
J Biol Chem ; 287(34): 28943-55, 2012 Aug 17.
Article in English | MEDLINE | ID: mdl-22740690

ABSTRACT

Mutations in the erythroid-specific aminolevulinic acid synthase gene (ALAS2) cause X-linked sideroblastic anemia (XLSA) by reducing mitochondrial enzymatic activity. Surprisingly, a patient with the classic XLSA phenotype had a novel exon 11 mutation encoding a recombinant enzyme (p.Met567Val) with normal activity, kinetics, and stability. Similarly, both an expressed adjacent XLSA mutation, p.Ser568Gly, and a mutation (p.Phe557Ter) lacking the 31 carboxyl-terminal residues also had normal or enhanced activity, kinetics, and stability. Because ALAS2 binds to the ß subunit of succinyl-CoA synthetase (SUCLA2), the mutant proteins were tested for their ability to bind to this protein. Wild type ALAS2 bound strongly to a SUCLA2 affinity column, but the adjacent XLSA mutant enzymes and the truncated mutant did not bind. In contrast, vitamin B6-responsive XLSA mutations p.Arg452Cys and p.Arg452His, with normal in vitro enzyme activity and stability, did not interfere with binding to SUCLA2 but instead had loss of positive cooperativity for succinyl-CoA binding, an increased K(m) for succinyl-CoA, and reduced vitamin B6 affinity. Consistent with the association of SUCLA2 binding with in vivo ALAS2 activity, the p.Met567GlufsX2 mutant protein that causes X-linked protoporphyria bound strongly to SUCLA2, highlighting the probable role of an ALAS2-succinyl-CoA synthetase complex in the regulation of erythroid heme biosynthesis.


Subject(s)
5-Aminolevulinate Synthetase/metabolism , Anemia, Sideroblastic , Genetic Diseases, X-Linked , Mutation, Missense , Succinate-CoA Ligases/metabolism , 5-Aminolevulinate Synthetase/genetics , Adult , Amino Acid Substitution , Anemia, Sideroblastic/enzymology , Anemia, Sideroblastic/genetics , Enzyme Stability/genetics , Genetic Diseases, X-Linked/enzymology , Genetic Diseases, X-Linked/genetics , Heme/biosynthesis , Heme/genetics , Humans , Male , Protein Binding , Protoporphyria, Erythropoietic/enzymology , Protoporphyria, Erythropoietic/genetics , Succinate-CoA Ligases/genetics , Vitamin B 6/genetics , Vitamin B 6/metabolism
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