Integrated Care for People Living With Rare Disease: A Scoping Review on Primary Care Models in Organization for Economic Cooperation and Development Countries.

Introduction/objectives: Individually rare, rare diseases are collectively common resulting in frequent health system use. Navigating the health system persists as a challenge. Primary care provides longitudinal contact with the health system and is placed to provide integrated rare-disease-care.

Evolution of out-of-hospital emergency cardiac care: Heart attack therapy for a retired president helped modernize American emergency medical services.

In the late 1960s, American emergency medical services (EMS) began to upgrade from mere Red Cross first aid to systems that now provide sophisticated advanced life support. This revolution in EMS stemmed from two pioneering Belfast reports in that described how early out-of-hospital coronary care saved lives. Inspired, a handful of American physicians implemented avant-garde programs in the USA.

Attitudes of health professionals to using routinely collected clinical data for performance feedback and personalised professional development.

Objectives: To learn the attitudes of health professionals, health informaticians and information communication technology professionals to using data in electronic health records (eHRs) for performance feedback and professional development.

Design: Qualitative research in a co-design framework. Health professionals' perceptions of the accessibility of data in eHRs, and barriers to and enablers of using these data in performance feedback and professional development were explored in co-design workshops.

Bridging the gap: a five stage approach for developing specialty-specific entrustable professional activities.

Background: Entrustable Professional Activities (EPAs) are increasingly used as a focus for assessment in graduate medical education (GME). However, a consistent approach to guide EPA design is currently lacking, in particular concerning the actual content (knowledge, skills and attitude required for specific tasks) for EPAs. This paper describes a comprehensive five stage approach, which was used to develop two specialty-specific EPAs in emergency medicine focused on the first year of GME.

Correlations between short-term mobile monitoring and long-term passive sampler measurements of traffic-related air pollution.

Mobile monitoring has provided a means for broad spatial measurements of air pollutants that are otherwise impractical to measure with multiple fixed site sampling strategies. However, the larger the mobile monitoring route the less temporally dense measurements become, which may limit the usefulness of short-term mobile monitoring for applications that require long-term averages. To investigate the stationarity of short-term mobile monitoring measurements, we calculated long term medians derived from a mobile monitoring campaign that also employed 2-week integrated passive sampler detectors (PSD) for NO, Ozone, and nine volatile organic compounds at 43 intersections distributed across the entire city of Baltimore, MD.

Measurement of spatial and temporal variation in volatile hazardous air pollutants in Tacoma, Washington, using a mobile membrane introduction mass spectrometry (MIMS) system.

The objective of this study was to use membrane introduction mass spectrometry (MIMS), implemented on a mobile platform, in order to provide real-time, fine-scale, temporally and spatially resolved measurements of several hazardous air pollutants. This work is important because there is now substantial evidence that fine-scale spatial and temporal variations of air pollutant concentrations are important determinants of exposure to air pollution and adverse health outcomes. The study took place in Tacoma, WA during periods of impaired air quality in the winter and summer of 2008 and 2009.

Caring for prisoners-patients: a quandary for registered nurses.

Purpose: The purpose of this study was to unveil the complexity of registered nurse (RN) care for prisoner-patients in an acute care perioperative setting.

Design: The study design was based on phenomenology and the philosophy of Hans George Gadamer.

Method: This study used researcher journaling and two audio-taped in-depth interviews with each of the 12 nurse participants.

Testing of glove efficacy against sprayed isocyanate coatings utilizing a reciprocating permeation panel.

Objectives: Modify a permeation panel to evaluate dermal protective clothing for resistance to sprayed coatings with minimal variability in spray paint loading across the test panel. Determine isocyanate protection effectiveness of natural rubber latex (5 mil or 0.13mm), nitrile rubber (5 mil or 0.

A review of prevocational medical trainee assessment in New South Wales.

Objective: To evaluate the effectiveness of formal assessments of prevocational medical trainees (ie, interns, first-year residents and international medical graduates undergoing supervised training).

Design And Setting: Retrospective review of structured assessment forms that are completed by all prevocational trainees and their supervisors in New South Wales public hospitals. We reviewed the first 3390 assessment forms, representing assessments of 1072 trainees at 43 training sites (83% of all prevocational trainees in NSW) since January 2009.

Do medical courses adequately prepare interns for safe and effective prescribing in New South Wales public hospitals?

Aims: To assess ability of interns immediately before starting clinical practice in New South Wales (NSW) teaching hospitals to prescribe medications safely and appropriately and to describe their impressions of the adequacy of their clinical pharmacology training in medical school.

Methods: A cross-sectional study was performed on all interns (n= 191) who attended intern orientation programmes at four NSW hospitals in January 2008. A clinical case scenario that tested prescribing ability and a survey investigating impressions of clinical pharmacology training in medical school were administered to the interns in exam format.

The relationship between admission blood glucose levels and hospital mortality.

Aims/hypothesis: The purpose of this study was to examine the relationship between blood glucose level (BGL) on admission with mortality rates among patients admitted to hospital through the Emergency Department.

Methods: In a prospective observational study, BGLs were routinely measured on 6,187 consecutive patients requiring blood testing and admitted through the Emergency Department of a tertiary referral hospital. These measurements were matched against demographic data and hospital mortality rates.

Rapid loading of sotalol or amiodarone for management of recent onset symptomatic atrial fibrillation: a randomized, digoxin-controlled trial.

Background: Amiodarone and sotalol are commonly used for the maintenance of sinus rhythm, but the efficacy of these agents administered as high-dose infusions for rapid conversion of atrial fibrillation is unknown. Use in this context would facilitate drug initiation in patients in whom ongoing prophylactic therapy is indicated.

Methods: We assessed the efficacy and safety of rapid high-dose intravenous infusions of amiodarone and sotalol for heart rate control and rapid reversion to sinus rhythm in patients who came to the emergency department with recent-onset symptomatic atrial fibrillation.

Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome.

Background: beta-blockers are routinely prescribed in congenital long-QT syndrome (LQTS), but the effectiveness and limitations of beta-blockers in this disorder have not been evaluated.

Methods And Results: The study population comprised 869 LQTS patients treated with beta-blockers. Effectiveness of beta-blockers was analyzed during matched periods before and after starting beta-blocker therapy, and by survivorship methods to determine factors associated with cardiac events while on prescribed beta-blockers.

The long QT syndrome. Prospective longitudinal study of 328 families.

Background: The Long QT Syndrome (LQTS) is an infrequently occurring familial disorder in which affected individuals have electrocardiographic QT interval prolongation and a propensity to ventricular tachyarrhythmic syncope and sudden death. We prospectively investigated the clinical characteristics and the long-term course of 3,343 individuals from 328 families in which one or more members were identified as affected with LQTS (QTc greater than 0.44 sec1/2).

Left cardiac sympathetic denervation in the therapy of congenital long QT syndrome. A worldwide report.

Background: Long QT syndrome (LQTS) is a congenital disorder accompanied by a high incidence of sudden cardiac death. beta-Adrenergic blockade is the therapy of choice, and it is successful in 75-80% of patients. For those in whom cardiac events (syncope or cardiac arrest) are not prevented by beta-blockade, experimental studies suggest that left cardiac sympathetic denervation (LCSD) may be useful.

Acute myocardial infarction associated with single vessel coronary artery disease: an analysis of clinical outcome and the prognostic importance of vessel patency and residual ischemic myocardium.

The long-term outcome and the significance of residual ischemic myocardium, as assessed by predischarge exercise thallium scintigraphy and vessel patency, were studied in 97 patients with single vessel coronary artery disease by angiography 12 +/- 4 days after uncomplicated myocardial infarction. During a mean follow-up period of 39 +/- 17 months, no patients died, 6 (6%) had a recurrent nonfatal infarction and 25 (26%) experienced rapidly progressive angina requiring hospitalization. Although neither exercise-induced angina nor ST segment depression was predictive of a recurrent cardiac event, the mean number of infarct zone scan segments showing thallium redistribution (1.

Acute non-Q wave myocardial infarction associated with early ST segment elevation: evidence for spontaneous coronary reperfusion and implications for thrombolytic trials.

The clinical significance of early ST segment elevation in patients with non-Q wave infarction is unknown. Therefore, 150 consecutive patients with creatine kinase isoenzyme-confirmed acute uncomplicated myocardial infarction who had ST segment elevation of 1 mm or more in at least two contiguous leads on the admission electrocardiogram were analyzed. None received thrombolytic therapy or acute coronary angioplasty.

Lidocaine enhances intraoperative ventricular defibrillation.

The efficacy of lidocaine during myocardial reperfusion in coronary artery bypass surgery was evaluated in 20 patients randomly assigned to a control group (n = 10) or to receive lidocaine, 1 mg/kg intravenously 5 min before aortic unclamping and cardiac reperfusion, followed by infusion at 40 micrograms X kg-1 X min-1 (n = 10). We recorded ECG leads II and V5 continuously, and number, energy, and current of direct current (DC) shocks starting at 1 joule. The number of low energy DC shocks to sustained defibrillation (5.

Effects of coronary grafting technique upon reperfusion cardiac rhythm, ventricular function, and other variables.

The effects of different techniques of aortocoronary bypass grafting on reperfusion cardiac rhythm and ventricular function have not been systematically evaluated for possible advantages or disadvantages. The placement of proximal anastomoses before cardiopulmonary bypass and sequential coronary grafting with reperfusion via both the grafts and the native circulation were prospectively compared to traditional grafting and reperfusion via native arteries. More than 40 biochemical, thermal, temporal, hemodynamic, and other variables, including arrhythmias and myocardial failure, were measured intraoperatively and postoperatively.

Low-energy defibrillation: safe and effective.

During cardiopulmonary bypass, 150 cardiac surgical patients were prospectively evaluated for the number, energy, current, and success rates of direct current (DC) shocks required to terminate reperfusion ventricular fibrillation (1 degree) or ventricular fibrillation occurring subsequent to a nonfibrillatory reperfusion rhythm (2 degrees). Thirty-one percent of 1-J shocks and 58% of 2.5-J shocks defibrillated.