One-year outcomes of out-of-hospital administration of intravenous glucose, insulin, and potassium (GIK) in patients with suspected acute coronary syndromes (from the IMMEDIATE [Immediate Myocardial Metabolic Enhancement During Initial Assessment and Treatment in Emergency Care] Trial).

The Immediate Myocardial Metabolic Enhancement During Initial Assessment and Treatment in Emergency care Trial of very early intravenous glucose-insulin-potassium (GIK) for acute coronary syndromes (ACS) in out-of-hospital emergency medical service (EMS) settings showed 80% reduction in infarct size at 30 days, suggesting potential longer-term benefits. Here we report 1-year outcomes. Prespecified 1-year end points of this randomized, placebo-controlled, double-blind, effectiveness trial included all-cause mortality and composites including cardiac arrest, mortality, or hospitalization for heart failure (HF).

Enzyme therapy and immune response in relation to CRIM status: the Dutch experience in classic infantile Pompe disease.

Background: Enzyme-replacement therapy (ERT) in Pompe disease--an inherited metabolic disorder caused by acid α-glucosidase deficiency and characterized in infants by generalized muscle weakness and cardiomyopathy--can be complicated by immune responses. Infants that do not produce any endogenous acid α-glucosidase, so-called CRIM-negative patients, reportedly develop a strong response. We report the clinical outcome of our Dutch infants in relation to their CRIM status and immune response.

Phenotypical variation within 22 families with Pompe disease.

Background: Pompe disease has a broad clinical spectrum, in which the phenotype is partially explained by the genotype. The aim of this study was to describe phenotypical variation among siblings with non-classic Pompe disease. We hypothesized that siblings and families with the same genotype share more similar phenotypes than the total population of non-classic Pompe patients, and that this might reveal genotype-phenotype correlations.

Severely impaired health status at diagnosis of Pompe disease: a cross-sectional analysis to explore the potential utility of neonatal screening.

Since the introduction of enzyme replacement therapy for Pompe disease, awareness and early diagnosis have gained importance. Because the therapy is most effective when started early and methods for dried bloodspot screening for Pompe disease are currently being explored, neonatal screening is getting increased attention. The objective of this study was to investigate the gains that might be achieved with earlier diagnosis by neonatal screening.

Treatment options for lysosomal storage disorders: developing insights.

Introduction: Lysosomal storage disorders (LSDs) are clinically heterogeneous disorders that result primarily from lysosomal accumulation of macromolecules in various tissues. LSDs are always progressive, and often lead to severe symptoms and premature death. The identification of the underlying genetic and enzymatic defects has prompted the development of various treatment options.

Rapid ultraperformance liquid chromatography-tandem mass spectrometry assay for a characteristic glycogen-derived tetrasaccharide in Pompe disease and other glycogen storage diseases.

Background: Urinary excretion of the tetrasaccharide 6-α-D-glucopyranosyl-maltotriose (Glc₄) is increased in various clinical conditions associated with increased turnover or storage of glycogen, making Glc₄ a potential biomarker for glycogen storage diseases (GSD). We developed an ultraperformance liquid chromatography-tandem mass spectrometry (UPLC-MS/MS) assay to detect Glc₄ in urine without interference of the Glc₄ isomer maltotetraose (M₄).

Methods: Urine samples, diluted in 0.

Out-of-hospital administration of intravenous glucose-insulin-potassium in patients with suspected acute coronary syndromes: the IMMEDIATE randomized controlled trial.

Context: Laboratory studies suggest that in the setting of cardiac ischemia, immediate intravenous glucose-insulin-potassium (GIK) reduces ischemia-related arrhythmias and myocardial injury. Clinical trials have not consistently shown these benefits, possibly due to delayed administration.

Objective: To test out-of hospital emergency medical service (EMS) administration of GIK in the first hours of suspected acute coronary syndromes (ACS).

Prehospital electronic patient care report systems: early experiences from emergency medical services agency leaders.

Background: As the United States embraces electronic health records (EHRs), improved emergency medical services (EMS) information systems are also a priority; however, little is known about the experiences of EMS agencies as they adopt and implement electronic patient care report (e-PCR) systems. We sought to characterize motivations for adoption of e-PCR systems, challenges associated with adoption and implementation, and emerging implementation strategies.

Methods: We conducted a qualitative study using semi-structured in-depth interviews with EMS agency leaders.

Helmet use in Connecticut motorcycle crashes: a state without a universal helmet law.

Objectives: Assess the association of helmet use with motorcycle crash mortality and identify characteristics of riders who do not wear helmets in Connecticut crashes.

Methods: Police crash data for Connecticut motorcycle crashes 2001-2007 were analyzed. Bivariate analysis and multivariable logistic regressions were performed including age, gender, seating position, road type, season, time of day, and recklessness.

An open, interoperable, and scalable prehospital information technology network architecture.

Some of the most intractable challenges in prehospital medicine include response time optimization, inefficiencies at the emergency medical services (EMS)-emergency department (ED) interface, and the ability to correlate field interventions with patient outcomes. Information technology (IT) can address these and other concerns by ensuring that system and patient information is received when and where it is needed, is fully integrated with prior and subsequent patient information, and is securely archived. Some EMS agencies have begun adopting information technologies, such as wireless transmission of 12-lead electrocardiograms, but few agencies have developed a comprehensive plan for management of their prehospital information and integration with other electronic medical records.

Fireground use of an emergency escape respirator.

Introduction: Firefighters who become lost, disoriented, or trapped in a burning building may die after running out of air in their self-contained breathing apparatus (SCBA). An emergency escape device has been developed that attaches to the firefighter's mask in place of the SCBA regulator. The device filters out particulate matter and a number of hazardous components of smoke (but does not provide oxygen), providing additional time to escape after the firefighter runs out of SCBA air.

Early cardiac catheterization laboratory activation by paramedics for patients with ST-segment elevation myocardial infarction on prehospital 12-lead electrocardiograms.

Background: Prompt reperfusion in ST-segment elevation myocardial infarction (STEMI) saves lives. Although studies have shown that paramedics can reliably interpret STEMI on prehospital 12-lead electrocardiograms (p12ECGs), prehospital activation of the cardiac catheterization laboratory by emergency medical services (EMS) has not yet gained widespread acceptance.

Objective: To quantify the potential reduction in time to percutaneous coronary intervention (PCI) by early prehospital activation of the cardiac catheterization laboratory in STEMI.

A mystery: one wound, multiple bullets.

This case report provides an unusual presentation of a gunshot wound (GSW) and stresses the importance of gathering complete clinical, scene and historical information, if possible. Sufficient details regarding an injured patient's mechanism of injury (MOI) should be elicited by the treating physician when hemodynamic status of the patient allows. A careful physical exam is essential as are appropriate laboratory investigations and diagnostic imaging.

An experimental model of heat storage in working firefighters.

Objective: Develop experimental models to study uncompensable heat stress (UCHS) in working firefighters (FFs).

Methods: FFs ingested core temperature (Tc) capsules prior to performing sequential tasks in 40 degrees C and personal protective ensemble (PPE), or 18 degrees C and no PPE. Both trials were conducted in an environmental chamber with FFs using self-contained breathing apparatus (SCBA).

Emergency medical services in Connecticut.

This article describes emergency medical services (EMS) systems in Connecticut, beginning with a historical perspective. The discussion of statewide oversight of the EMS system includes legislative and regulatory mandates as well as recent external reviews of the system. Medical oversight of EMS care and services is provided by sponsor hospitals rather than individual medical directors.