Arterial aneurysm and dissection: toward the evolving phenotype of Tatton-Brown-Rahman syndrome.

BACKGROUND: Tatton-Brown-Rahman syndrome (TBRS) is a rare disorder, caused by DNMT3A heterozygous pathogenic variants, and first described in 2014. TBRS is characterised by overgrowth, intellectual disability, facial dysmorphism, hypotonia and musculoskeletal features, as well as neurological and psychiatric features. Cardiac manifestations have also been reported, mainly congenital malformations such as atrial septal defect, ventricular septal defect and cardiac valvular disease. Aortic dilatation has rarely been described. METHODS: Here we have undertaken a detailed clinical and molecular description of eight previously unreported individuals, who had TBRS and arterial dilatation and/or dissection, mainly thoracic aortic aneurysm (TAA). We have also reviewed the seven previously published cases of TAA in individuals with TBRS to try to better delineate the vascular phenotype and to determine specific follow-up for this condition. RESULTS: We include eight new patients with TBRS who presented with arterial aneurysms mainly involving aorta. Three of these patients presented with dissection that required critical surgery. CONCLUSIONS: Arterial aneurysms and dissections are a potentially lethal, age-dependent manifestation. The prevalence of aortic disease in individuals with TBRS is far in excess of that expected in the general population. This cohort, together with individuals previously published, illustrates the importance to consider dilatation/dissection, mainly in aorta but also in other arteries. Arterial vascular weakness may therefore also be a cardinal feature of TBRS and vascular surveillance is recommended.

Block by block: Building on our knowledge to better care for LGBTQIA+ patients.

Background: Emergency physicians need to recognize the diversity of identities held by sexual and gender minorities, as well as the health implications and inequities experienced by these communities. Identities such as lesbian, gay, bisexual, transgender, queer, questioning, intersex, asexual, aromantic, and many others fall under the LGBTQIA+ acronym. This wide spectrum is seldom discussed in emergency medicine but nonetheless impacts both patient care and patient experience in acute and critical care settings. Aims: This commentary aims to provide a brief but nonexhaustive review of LGBTQIA+ identities and supply a critical framework for applying this understanding to patient encounters in the emergency department, as well as describe the challenges and educational aims at the level of medical school, residency, and postresidency. Materials and Methods: The commonly used and widely accepted definitions of LGBTQIA+ terms are described, as well as implications for patient care and emergency physician education. The authors of this writing group represent the Society for Academic Emergency Medicine, LGBTQ Task Force of the Academy of Diversity Inclusion in Medicine. Results: LGB terms are addressed, with LGBTQIA+ adding "intersex," "asexual," and "+," to include other gender identities and sexual orientations which are not already included. This paper also addresses the terms "transition," "nonbinary," "polyamorous." "two-spirit," "queer," and others. These acronyms and terms continually expand and evolve in the pursuit of inclusivity. Additionally, with some health issues potentially related to medications, hormones, surgery, or to internal or external genitalia, important EM physician tools include gathering an "organ inventory," asking about sexual history, and conducting a physical exam. Discussion: Most persons have congruent biological sex, gender identity, and attraction to the "opposite" gender. However, humans can have every imaginable variation and configuration of chromosomes, genitalia, gender identities, sexual attractions, and sexual behaviors. Terms and definitions are constantly changing and adapting; they may also vary by local culture. Obtaining relevant medical history, conducting an "organ inventory," asking about sexual history in a nonjudgmental way, and conducting a physical exam when warranted can all be important in delivering best possible medical care. Although there has been increased focus on education at the medical school, residency, and faculty level on LGBTQIA+ patient care in the ED, much work remains to be done. Conclusion: Emergency physicians should feel confident in providing a model of care that affirms the sexual and gender identities of all the patient populations we serve. Optimal patient-centric care requires a deeper understanding of the patient's biology, gender identity, and sexual behavior encapsulated into the ever-growing acronym LGBTQIA+.

Emergency care of LGBTQIA+ patients requires more than understanding the acronym.

Emergency physicians (EPs) frequently deliver care to members of the LGBTQIA+ community in the emergency department. This community suffers from many health disparities important to understand as part of comprehensive care, and these disparities are infrequently discussed in emergency medicine education. Previous data also suggest a need for broader education to increase the comfort of EPs caring for LGBTQIA+ patients. A group of content experts identified key disparities, opportunities for expanded education, and strategies for more inclusive care of LGBTQIA+ patients.

Review of 20 Years of Continuous Quality Improvement of a Rapid Response System, at Four Institutions, to Identify Key Process Responsible for Its Success.

Rapid response systems are still in development, and their practices vary significantly from hospital to hospital. Although the literature supports their value and a four-arm structure, it is unclear within these arms (efferent, afferent, quality assurance, administrative arms) which processes and procedures are responsible for their efficacy. This article reports the evolution of a rapid response system over many years at four institutions and considers the key elements that likely contribute to its efficacy. METHODS: Retrospective evaluation of the processes, procedures, and outcomes of an adult general-ward rapid response system as it evolved, at four nonaffiliated community medical centers, spanning 2 decades of development and refinement. System and patient outcomes examined included the number of rapid response system activations/1,000 admissions, time to rapid response system activation and/or interventions, cardiac arrest rate, and/or hospital mortality over time. RESULTS: In the three hospitals that collected control and intervention data, there was significant increase in earlier and total number of rapid response system activations, more rapid administration of protocolized interventions, and associated decreases in cardiac arrest rate and hospital mortality of the respective population. In all four institutions three important common rapid response system processes were identified: early identification of at-risk patient using a novel focused bedside-assessment tool, leading to classification of the pathophysiologic process, linked to goal-directed intervention protocols. CONCLUSIONS: Our review of a rapid response system that evolved over 20 years across four unrelated institutions revealed a common care pathway that coupled a focused bedside at-risk patient assessment leading to pathophysiologic classification of the patients decline linked to goal-directed intervention protocols. We speculate that the improved outcomes observed are a consequence of effective implementation and coupling of these three processes, as they are important in identifying and treating early the signs of tissue hypoxia and hypoperfusion, which remain the basic pathophysiologic threats of acute deterioration.

Age-Related Child Sexual Abuse Experiences.

The study aimed to describe differences in the types of sexual abuse injuries between two age groups of children presenting to an emergency department. This descriptive correlational study explored the relationships between sexual abuse type and child's age using data from the hospital health records of 95 children ages 6-14 years. Descriptive analysis indicated that sexually abused child victims' ages were significantly associated with different types of penetrations and mucosal tears or injuries (p < 0.05). These findings highlighted the need for increased awareness about the different types of physical trauma associated with the ages of the child victims.

Sleep and immune function among people living with human immunodeficiency virus (HIV).

People living with HIV are at increased risk for sleep disturbances. Up to 75% of the HIV-infected individuals in the United States experience sleep disturbances of some kind. Previous studies have suggested an association between patient-reported sleep disturbances and impaired immune function. This study evaluates data obtained via sleep actigraphy to evaluate the relationship between objectively measured sleep, HIV viral load, and immune function. While this study found no relationship between objective sleep and CD4+ T- lymphocyte count, higher sleep efficiency was weakly correlated with lower HIV viral loads, τb(93) = -.165, p = .043. More research is warranted to clarify the nature of these relationships.

Acquiring data in medical research: A research primer for low- and middle-income countries.

Without data, there is no new knowledge generated. There may be interesting speculation, new paradigms or theories, but without data gathered from the universe, as representative of the truth in the universe as possible, there will be no new knowledge. Therefore, it is important to become excellent at collecting, collating and correctly interpreting data. Pre-existing and new data sources are discussed; variables are discussed, and sampling methods are covered. The importance of a detailed protocol and research manual are emphasized. Data collectors and data collection forms, both electronic and paper-based are discussed. Ensuring subject privacy while also ensuring appropriate data retention must be balanced.

What every emergency physician should know about research: Introduction to a research primer for low- and middle-income countries.

Research is the search for new, generalisable knowledge (Truth in the Universe) to improve our collective ability to correctly diagnose and treat human suffering. In the formal sense, medical research implies both creating new knowledge, and also disseminating that new knowledge as well as putting it into practice. This is the first paper in this Research Primer. It briefly covers why each emergency physician should know and care about research. The paper reminds us that it does not take a physician to do research, but that it is the practicing physician who best knows what new knowledge is needed at the bedside. It introduces the scope of the other papers included in this special issue. The paper reviews the definitions of research and the scope of research practice in emergency medicine; overviews the hows and whys of research, as well as discusses the research question, study justification, literature search and touching on research design.

The curious case of a 75-year-long dislocated hip causing abdominal pain.

We present a case of abdominal pain due to chronic hip dislocation of 75 years duration. Hip dislocations are not uncommon, but long-term, unreduced dislocations are vanishingly rare in the developed world. This 80-year-old female, who emigrated to the United States as an adult, presented to the emergency department for acute abdominal pain. Workup showed no intra-abdominal cause for her pain. History revealed she had suffered a traumatic hip dislocation at 5 years of age that was unable to receive adequate treatment because of limited health care access. After several years, she regained functional ability because of anatomic and compensatory musculoskeletal changes in the pelvis. The adaptations likely caused excessive muscular strain resulting in muscle spasm at the location of her abdominal pain. To our knowledge, this is the only reported case of a hip that remained dislocated for 75 years.

Nuevo Amanecer-II: Results of a randomized controlled trial of a community-based participatory, peer-delivered stress management intervention for rural Latina breast cancer survivors.

OBJECTIVE: We report results of a community-based multisite, randomized controlled trial of Nuevo Amanecer (NA-II), a 10-week stress management program for rural, low literacy Latina breast cancer survivors. METHODS: Trained peers delivered NA-II to Spanish-speaking Latinas with non-metastatic breast cancer in three rural communities. Women were randomized to receive the program immediately or wait 6 months. Assessments were conducted at baseline, 3 months, and 6 months. Primary outcomes were breast cancer-specific quality of life domains; secondary outcomes included general distress symptoms and stress management skills. Intention-to-treat analyses using repeated-measures linear regression models estimated changes in slope between groups. RESULTS: Of 153 participants (76 randomized to intervention, 77 to control group), 92% were retained at 6 months. Mean age was 54.8 years (SD = 10.5); 80% had less than high school education. There were no statistically significant treatment × time effects on quality of life. Compared to women in the control group, intervention group women reported greater improvements in anxiety at 6 months (-0.20 vs -0.02, P = .049; range 0-4) as well as three stress management skills: relaxation at 3 months (+0.98 vs -0.07, P < .0001; range 0-4) and 6 months (+0.82 vs +0.04, P < .001), awareness of tension at 3 months (+0.31 vs -0.19, P < .01; range 0-4) and 6 months (+0.29 vs -0.11, P < .05), and coping confidence at 3 months (+0.12 vs -0.23, P < .01; range 0-4). CONCLUSIONS: Stress management programs delivered by trained peers in rural community settings can reduce anxiety and improve stress management skills among Latina breast cancer survivors.

Translating a stress management intervention for rural Latina breast cancer survivors: The Nuevo Amanecer-II.

OBJECTIVES: Adapt a cognitive-behavioral stress management program (Nuevo Amanecer or NA) to be generalizable to rural, low literacy Spanish-speaking Latinas with breast cancer survivors at all phases of survivorship. METHODS: Apply the Transcreation Framework, a community-engaged translational model, to develop the adapted program (Nuevo Amanecer or NA-II), design a randomized controlled trial for community settings, identify recruiters and interventionists, and recruit participants into the trial. RESULTS: Adaptations included expanding the program from eight to ten weeks, simplifying materials, and increasing skills practice. We added stress management videos, healthy lifestyles information, and survivorship information. Interventionists were trained Latina breast cancer survivors. All core components of NA were retained in NA-II including managing the impact of cancer, information on breast cancer and its treatment, finding cancer information, getting support, managing thoughts, stress management techniques, and setting goals. Participants receive a program manual. Each session includes a review of that week's content using the manual, practicing a stress-management skill, setting a specific goal, and reviewing videos. Spanish-speaking Latinas with non-metastatic breast cancer were recruited by community recruiters. Of 231 women approached, 24% refused, 10% were ineligible, and 153 (66%) were randomized to the intervention or a wait-list control group. The sample was vulnerable: 69% had < high school education, more than half had only Medicaid or no insurance, 91% was foreign born, and 48% reported financial hardship in the past year. CONCLUSIONS: Applying the Transcreation Framework to engage stakeholders in designing community-based RCTs enhanced congruence with community contexts and recruitment of this vulnerable population.

Creating Consensus: Revisiting the Emergency Medicine Resident Scholarly Activity Requirement.

INTRODUCTION: In the context of the upcoming single accreditation system for graduate medical education resulting from an agreement between the Accreditation Council for Graduate Medical Education (ACGME), American Osteopathic Association and American Association of Colleges of Osteopathic Medicine, we saw the opportunity for charting a new course for emergency medicine (EM) scholarly activity (SA). Our goal was to engage relevant stakeholders to produce a consensus document. METHODS: Consensus building focused on the goals, definition, and endpoints of SA. Representatives from stakeholder organizations were asked to help develop a survey regarding the SA requirement. The survey was then distributed to those with vested interests. We used the preliminary data to find areas of concordance and discordance and presented them at a consensus-building session. Outcomes were then re-ranked. RESULTS: By consensus, the primary role(s) of SA should be the following: 1) instruct residents in the process of scientific inquiry; 2) expose them to the mechanics of research; 3) teach them lifelong skills, including search strategies and critical appraisal; and 4) teach them how to formulate a question, search for the answer, and evaluate its strength. To meet these goals, the activity should have the general elements of hypothesis generation, data collection and analytical thinking, and interpretation of results. We also determined consensus on the endpoints, and acceptable documentation of the outcome. CONCLUSION: This consensus document may serve as a best-practices guideline for EM residency programs by delineating the goals, definitions, and endpoints for EM residents' SA. However, each residency program must evaluate its available scholarly activity resources and individually implement requirements by balancing the ACGME Review Committee for Emergency Medicine requirements with their own circumstances.

Intra-abdominal Rupture of a Live Cervical Pregnancy with Placenta Accreta but Without Vaginal Bleeding.

We describe an unusual ruptured ectopic pregnancy. The unique features of the case include abdominal pain without vaginal bleeding; cervical implantation and a placenta accreta; and the late presentation at 16 weeks of gestation without prior symptoms. Both the initial point-of-care ultrasound and the formal ultrasound were interpreted as showing an intrauterine pregnancy. The clinical presentation was misleading; the correct diagnosis was made by magnetic resonance imaging. We show the ultrasonic images. We discuss cervical ectopic pregnancies, their diagnosis and management. The woman survived but required emergency hysterectomy and many units of blood.

Communicating Value in Simulation: Cost-Benefit Analysis and Return on Investment.

Value-based health care requires a balancing of medical outcomes with economic value. Administrators need to understand both the clinical and the economic effects of potentially expensive simulation programs to rationalize the costs. Given the often-disparate priorities of clinical educators relative to health care administrators, justifying the value of simulation requires the use of economic analyses few physicians have been trained to conduct. Clinical educators need to be able to present thorough economic analyses demonstrating returns on investment and cost-effectiveness to effectively communicate with administrators. At the 2017 Academic Emergency Medicine Consensus Conference "Catalyzing System Change through Health Care Simulation: Systems, Competency, and Outcomes," our breakout session critically evaluated the cost-benefit and return on investment of simulation. In this paper we provide an overview of some of the economic tools that a clinician may use to present the value of simulation training to financial officers and other administrators in the economic terms they understand. We also define three themes as a call to action for research related to cost-benefit analysis in simulation as well as four specific research questions that will help guide educators and hospital leadership to make decisions on the value of simulation for their system or program.

Communicating Value in Simulation: Cost Benefit Analysis and Return on Investment.

Value-based health care requires a balancing of medical outcomes with economic value. Administrators need to understand both the clinical and economic effects of potentially expensive simulation programs to rationalize the costs. Given the often-disparate priorities of clinical educators relative to health care administrators, justifying the value of simulation requires the use of economic analyses few physicians have been trained to conduct. Clinical educators need to be able to present thorough economic analyses demonstrating returns on investment and cost effectiveness to effectively communicate with administrators. At the 2017 Academic Emergency Medicine Consensus Conference "Catalyzing System Change through Health Care Simulation: Systems, Competency, and Outcomes", our breakout session critically evaluated the cost benefit and return on investment of simulation. In this paper we provide an overview of some of the economic tools that a clinician may use to present the value of simulation training to financial officers and other administrators in the economic terms they understand. We also define three themes as a call to action for research related to cost benefit analysis in simulation as well as four specific research questions that will help guide educators and hospital leadership to make decisions on the value of simulation for their system or program. This article is protected by copyright. All rights reserved.

A Case of Osteomyelitis of the toe caused by Coccidioidomycosis in a 17 year-old with Diabetes Insipidus.

We report a case of a 17-year-old male who presented with pain in his right first toe. His pain and swelling had worsened and x-rays of his foot revealed erosive changes of the great toe distal phalanx suggesting possible osteomyelitis. His co-morbidities were morbid obesity and diabetes insipidus. He was admitted to the hospital, blood cultures were drawn, and he was started on vancomycin for presumed bacterial osteomyelitis. He underwent incision and drainage of the fluctuant abscess of the toe, where a culture of the wound was taken. Preliminary results grew fungi. Being located in an endemic area, he was started on anti-fungal treatment for presumed disseminated coccidioidomycosis; culture was positive for Coccidiodes immitis. He also had serology positive for coccidioidomycosis titers. He had uneventful hospital stay and was discharged on long-term oral antifungal therapy.

Is Metabolic Syndrome On the Radar? Improving Real-Time Detection of Metabolic Syndrome and Physician Response by Computerized Scan of the Electronic Medical Record.

OBJECTIVE: Metabolic syndrome is a common underdiagnosed condition among psychiatric patients exacerbated by second-generation antipsychotics, with the exception of aripiprazole and ziprasidone. This study evaluated the prescribing and treating behavior with regard to antipsychotics and metabolic syndrome of psychiatrists before and after implementation of a mandatory admission order set and electronic notification of results. METHOD: Baseline data from 9,100 consecutive psychiatric admissions to a mental health hospital (July 2013-July 2014) were compared to postintervention data (July 2014-January 2015), which included 1,499 consecutive patient records. The intervention initiated standardized admission testing with electronic notification to psychiatrists when patients met metabolic syndrome criteria (according to Axis III of the DSM-IV). Charts were examined for inclusion of this diagnosis at discharge and for treatment changes. RESULTS: At baseline, only 2.4% of patients (n = 214) were evaluated for metabolic syndrome. Of these, 34.5% (0.8% of the total sample) met metabolic syndrome criteria. Only 15 patients (0.16%) were comprehensively treated. No chart listed metabolic syndrome under Axis III of the DSM-IV. After the intervention, the diagnosis of patients meeting the criteria for metabolic syndrome increased from 0% to 29.3%. Less than 3% of patients were switched to drugs with a more benign metabolic profile. All patients who continued on second-generation antipsychotics had metabolic retesting. Thirty-eight experienced a significant and rapid increase in triglyceride levels after only 3 to 17 days. CONCLUSIONS: Mandatory intake testing increases the number of patients evaluated for metabolic syndrome. Electronic alerts increase the inclusion of metabolic syndrome among discharge diagnoses but rarely affect prescribing practices.

A model for predicting angiographically normal coronary arteries in survivors of out-of-hospital cardiac arrest.

BACKGROUND: It has been recommended that all survivors of out-of-hospital cardiac arrest (OHCA) have immediate coronary angiography (CAG), even though it has been reported that half of the survivors have normal coronary arteries. Our aim was to develop a model which might identify those who have angiographically normal coronary arteries. Reliable prediction would reduce unnecessary CAG. METHODS: A retrospective, observational, cohort study was conducted on 47 consecutive adult survivors who received immediate CAG after resuscitation from OHCA, between June 1, 2006 and March 31, 2011. We analyzed the clinical and electrocardiographic characteristics of the survivors with and without normal coronary arteries. RESULTS: All subjects had CAG. Normal coronary arteries were found in 25/47. These persons did not have diabetes mellitus (p = 0.0069) or a history of acute coronary syndrome (ACS) (p = 0.0069). Any abnormality of the ST segment or ST segment elevation on electrocardiogram (ECG) was strongly related to abnormal coronary arteries (p = 0.0045 and p = 0.0200, respectively). The partitioning model for predicting angiographically normal coronary arteries showed that all patients (8/8) with no ST segment change on their ECG had normal coronary arteries. Eight out of ten patients with ST segment abnormalities also had normal coronary arteries with a history of arrhythmia without a history of ACS. CONCLUSIONS: Survivors of OHCA who have no history of diabetes mellitus, who have no past history of ACS, and who present with no ST segment abnormalities may not require urgent/emergent CAG. Further studies are needed to guide clinicians in the determination of emergent cardiac catheterization following resuscitation of OHCA.