Natural History, Phenotype Spectrum and Clinical Outcomes of Desmin (DES)-Associated Cardiomyopathy.

Background: Pathogenic/likely pathogenic (P/LP) desmin (DES) variants cause heterogeneous cardiomyopathy and/or skeletal myopathy phenotypes. Limited data suggest a high incidence of major adverse cardiac events (MACE), including cardiac conduction disease (CCD), sustained ventricular arrhythmias (VA), and heart failure (HF) events (HF hospitalization, LVAD/cardiac transplant, HF-related death), in patients with P/LP DES variants. However, pleiotropic presentation and small cohort sizes have limited clinical phenotype and outcome characterization. Objectives: We aimed to describe the natural history, phenotype spectrum, familial penetrance and outcomes in patients with P/LP DES variants through a systematic review and individual patient data meta-analysis using published reports. Methods: We searched Medline (PubMed) and Embase for studies that evaluated cardiac phenotypes in patients with P/LP DES variants. Cardiomyopathy diagnosis or occurrence of MACE were considered evidence of cardiac involvement/penetrance. Lifetime event-free survival from CCD, sustained VA, HF events, and composite MACE was assessed. Results: Out of 4,212 screened publications, 71 met the inclusion criteria. A total of 230 patients were included (52.6% male, 52.2% probands, median age: 31 years [22.0; 42.8] at first evaluation, median follow-up: 3 years [0; 11.0]). Overall, 124 (53.9%) patients were diagnosed with cardiomyopathy, predominantly dilated cardiomyopathy (14.8%), followed by restrictive cardiomyopathy (13.5%), whereas other forms were less common: arrhythmogenic cardiomyopathy (7.0%), hypertrophic cardiomyopathy (6.1%), arrhythmogenic right ventricular cardiomyopathy (5.2%), and other forms (7.4%). Overall, 132 (57.4%) patients developed MACE, with 96 [41.7%] having CCD, 36 [15.7%] sustained VA, and 43 [18.7%] HF events. Familial penetrance of cardiac disease was 63.6% among relatives with P/LP DES variants. Male sex was associated with increased risk of sustained VA (HR 2.28, p=0.02) and HF events (HR 2.45, p=0.008). Conclusions: DES cardiomyopathy exhibits heterogeneous phenotypes and distinct natural history, characterized by high familial penetrance and substantial MACE burden. Male patients face higher risk of sustained VA events.

Transcatheter Aortic Valve Replacement in Low-Risk Patients at Four or More Years.

BACKGROUND: Transcatheter aortic valve replacement (TAVR) is accepted as an alternative to surgical aortic valve replacement (SAVR) in patients with severe symptomatic aortic valve stenosis. Prior studies have shown that TAVR has comparable or superior outcomes to SAVR in intermediate and high-risk patients. However, there is paucity of data about outcome of TAVR vs SAVR in low-surgical-risk patients evaluated at 4 or more years post-procedure. METHODS: A systematic review of all published randomized controlled trials comparing TAVR and SAVR in low-risk patients was completed. A random-effects model meta-analysis was performed to study major outcomes, including all-cause mortality, stroke, myocardial infarction, and aortic valve reintervention. RESULTS: Three randomized trials comprising 2644 patients (1371 TAVR and 1273 SAVR) with a mean age of 74.3 ± 5.8 years were included in this analysis. There was no significant difference in all-cause and cardiovascular mortality, stroke, myocardial infarction, or aortic valve reintervention between the TAVR and SAVR groups at long-term follow-up. Transcatheter aortic valve replacement was associated with higher rate of pacemaker implantation, whereas SAVR was associated with more atrial fibrillation. CONCLUSIONS: At 4 or more years of follow-up, TAVR is safe and has comparable outcomes to SAVR in low-surgical-risk patients. Possibility of TAVR and its risks and benefits should be discussed with low-surgical-risk patients.

A systematic review of association between use of hair products and benign and malignant gynecological conditions.

Hair products often contain chemicals like para-phenylenediamine (PPD) and endocrine-disrupting chemicals (EDCs); giving rise to concerns about the possible adverse effects such as hormonal disturbances and carcinogenicity. The objective of this systematic review was to evaluate the association between the use of different hair products and benign and malignant gynecological conditions. Studies were identified from three databases including PubMed, Embase, and Scopus, and evaluated in accordance with PRISMA guidelines. The risk of bias was assessed using the Newcastle-Ottawa Scale. A total of 17 English-language studies met the inclusion criteria. Associations of hair relaxer or hair dye use with breast and ovarian cancer were observed in at least one well-designed study, but these findings were not consistent across studies. Further sub-analysis showed 1.08 times (95 % CI: 1.01-1.15) increased risk of breast cancer in females with permanent hair dye use. Chang et al. reported strong association between uterine cancer risk and hair relaxer use (HR 1.8, 95 % CI: 1.12-2.88), with no observed association with hair dye use. Studies conducted by Wise et al. and James-Todd et al. for benign gynecological conditions; including uterine leiomyoma (IRR 1.17, 95 % CI: 1.06-1.30), early onset of menarche (RR 1.4, 95 % CI: 1.1-1.9), and decreased fecundability (FR 0.89, 95 % CI: 0.81-0.98) revealed positive associations with hair relaxer use, but these findings were based on small sample sizes. In summary, the available evidence regarding personal use of hair products and gynecological conditions is insufficient to determine whether a positive association exists.

Elucidating symptoms of COVID-19 illness in the Arizona CoVHORT: a longitudinal cohort study.

OBJECTIVE: To elucidate the symptoms of laboratory-confirmed COVID-19 cases as compared with laboratory-confirmed negative individuals and to the untested general population among all participants who reported symptoms within a large prospective cohort study. SETTING AND DESIGN: This work was conducted within the framework of the Arizona CoVHORT, a longitudinal prospective cohort study conducted among Arizona residents. PARTICIPANTS: Eligible participants were any individual living in Arizona and were recruited from across Arizona via COVID-19 case investigations, participation in testing studies and a postcard mailing effort. PRIMARY AND SECONDARY OUTCOME MEASURES: The primary outcome measure was a comparison of the type and frequency of symptoms between COVID-19-positive cases, tested but negative individuals and the general untested population who reported experiencing symptoms consistent with COVID-19. RESULTS: Of the 1335 laboratory-confirmed COVID-19 cases, 180 (13.5%) reported having no symptoms. Of those that did report symptoms, the most commonly reported were fatigue (82.2%), headache (74.6%), aches, pains or sore muscles (66.3%), loss of taste or smell (62.8) and cough (61.9%). In adjusted logistic regression models, COVID-19-positive participants were more likely than negative participants to experience loss of taste and smell (OR 12.1; 95% CI 9.6 to 15.2), bone or nerve pain (OR 3.0; 95% CI 2.2 to 4.1), headache (OR 2.6; 95% CI 2.2 to 3.2), nausea (OR 2.4; 95% CI 1.9 to 3.1) or diarrhoea (OR 2.1; 95% CI 1.7 to 2.6). Fatigue (82.9) and headache (74.9) had the highest sensitivities among symptoms, while loss of taste or smell (87.2) and bone or nerve pain (92.9) had the high specificities among significant symptoms associated with COVID-19. CONCLUSION: When comparing confirmed COVID-19 cases with either confirmed negative or untested participants, the pattern of symptoms that discriminates SARS-CoV-2 infection from those arising from other potential circulating pathogens may differ from general reports of symptoms among cases alone.

Inpatient Addiction Medicine Consultation Service Impact on Post-discharge Patient Mortality: a Propensity-Matched Analysis.

BACKGROUND: Inpatient addiction medicine consultation services (AMCS) have grown rapidly, but there is limited research of their impact on patient outcomes. OBJECTIVE: To examine whether AMCS is associated with all-cause mortality and hospital utilization post-discharge. DESIGN: This was a propensity-score-matchedcase-control study from 2018 to 2020. PARTICIPANTS: The intervention group included patients referred to the AMCS from October 2018 to March 2020. Matched control participants included patients hospitalized from October 2017 to September 2018 at an urban academic hospital with a large suburban and rural catchment area. MAIN MEASURES: The effect of treatment was estimated as the difference between the proportion of subjects experiencing the event (7-day and 30-day readmission, emergency department visits, and mortality within 90 days) for each group in the matched sample. KEY RESULTS: There were 711 patients in the intervention group and 2172 patients in the control group. The most common substance use disorders among the intervention group were primary alcohol use disorder (n=181; 25.5%) and primary opioid use disorder (n=175, 24.6%) with over a third with polysubstance use (n=257, 36.1%). Intervention patients showed a reduction in 90-day mortality post-hospital discharge (average treatment effect [ATE]: -2.35%, 95% CI: -3.57, -1.13; p-value <0.001) compared to propensity-matched controls. We found a statistically significant reduction in 7-day hospital readmission by 2.15% (95% CI: -3.65, -0.65; p=0.005) and a nonsignificant reduction in 30-day readmission (ATE: -2.38%, 95% CI: -5.20, 0.45; p=0.099). There was a statistically significant increase in 30-day emergency department visits (ATE: 5.32%, 95% CI: 2.19, 8.46; 0.001) compared to matched controls. CONCLUSIONS: There was a reduction in 90-day all-cause mortality for the AMCS intervention group compared to matched controls, although the impact on hospital utilization was mixed. AMCS are systems interventions that are effective tools to improve patient health and reduce all-cause mortality.

On the importance of a procedurally fair organizational climate for openness to change in law enforcement.

OBJECTIVE: Drawing on recent work in policing and organizational psychology, we examined factors related to openness to organizational change and to adopting evidence-based interview techniques among law enforcement investigators. HYPOTHESES: We hypothesized that a procedurally fair organizational climate would predict outcomes tied to organizational change, mediated by organizational identification and perceived legitimacy. We also predicted that procedural justice factors would be stronger predictors than outcome-oriented factors (i.e., rewards and sanctions). METHOD: Study 1 surveyed law enforcement investigators (N = 711) about their attitudes toward and behaviors within their organization (i.e., perceived procedural fairness of one's organization, identification, legitimacy, compliance, empowerment, and extra-role behavior). Study 2 conceptually extended this survey to interviewers (N = 71) trained in a new, evidence-based interviewing approach adding likelihood of future use of the novel interviewing approach as an outcome. RESULTS: In Study 1, the more investigators thought their organization had a procedurally fair climate, the more they identified with the organization and perceived it as legitimate. Framing compliance, empowerment and extra-role behavior as associated with openness to change, we found that legitimacy predicted compliance and tendency toward extra-role behavior (i.e., going "above and beyond"), while level of identification predicted feelings of empowerment and extra-role behavior. Study 2 partially replicated findings from Study 1 and found that motivation to attend the training also predicted likelihood of future use. CONCLUSIONS: These studies highlight the value of a procedurally just organizational climate framework in understanding law enforcement interrogators' propensity toward implementing new evidence-based interrogation techniques. (PsycInfo Database Record (c) 2020 APA, all rights reserved).

Implementation of STFM's "Smiles for Life" oral health curriculum in a medical school interclerkship.

BACKGROUND AND OBJECTIVES: While oral health is an important topic for medical education, it is often not covered in medical school. The Association of American Medical Colleges (AAMC) has recently set guidelines for oral health training in medical education. Our objective was to demonstrate how a mandatory interclerkship (half-day workshops taught between third-year clerkships) that covers pediatric, urgent care, examination skills, and prevention topics in oral health can lead to an increase in knowledge for medical students. METHODS: Teaching methods included the use of interactive lectures, an audience response system, and small-group workshops taught by medical and dental educators. The curriculum was based on the Society of Teachers of Family Medicine (STFM) Smiles for Life National Oral Health Curriculum. Students were given pretests and posttests, including a 6-month follow-up test. RESULTS: Students showed a significant improvement in knowledge between pretesting and immediate posttesting across a range of topics. Long-term knowledge retention was more limited. The majority of students reported enthusiasm for this topic and found the materials essential for their training. CONCLUSIONS: A brief interclerkship can improve medical students' oral health knowledge and be engaging. More research is needed to evaluate means to sustaining the knowledge.