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Sinus tachycardia (ST) is ubiquitous, but its presence outside of normal physiological triggers in otherwise healthy individuals remains a commonly encountered phenomenon in medical practice. In many cases, ST can be readily explained by a current medical condition that precipitates an increase in the sinus rate, but ST at rest without physiological triggers may also represent a spectrum of normal. In other cases, ST may not have an easily explainable cause but may represent serious underlying pathology and can be associated with intolerable symptoms. The classification of ST, consideration of possible etiologies, as well as the decisions of when and how to intervene can be difficult. ST can be classified as secondary to a specific, usually treatable, medical condition (eg, pulmonary embolism, anemia, infection, or hyperthyroidism) or be related to several incompletely defined conditions (eg, inappropriate ST, postural tachycardia syndrome, mast cell disorder, or post-COVID syndrome). While cardiologists and cardiac electrophysiologists often evaluate patients with symptoms associated with persistent or paroxysmal ST, an optimal approach remains uncertain. Due to the many possible conditions associated with ST, and an overlap in medical specialists who see these patients, the inclusion of experts in different fields is essential for a more comprehensive understanding. This article is unique in that it was composed by international experts in Neurology, Psychology, Autonomic Medicine, Allergy and Immunology, Exercise Physiology, Pulmonology and Critical Care Medicine, Endocrinology, Cardiology, and Cardiac Electrophysiology in the hope that it will facilitate a more complete understanding and thereby result in the better care of patients with ST.

BackgroundPediatric anesthesiology has been greatly impacted by COVID-19 in the delivery of care to patients and to the individual providers. With this study, we sought to survey pediatric centers and highlight the variations in care related to perioperative medicine during the COVID-19 pandemic, including the availability of protective equipment, the practice of pediatric anesthesia, and economic impact.AimThe aim of the survey was to determine how COVID-19 directly impacted pediatric anesthesia practices during the study period.MethodsA survey concerning four major domains (testing, safety, clinical management/policy, economics) was developed. It was pilot tested for clarity and content by members of the Pediatric Anesthesia COVID-19 Collaborative. The survey was administered by email to all Pediatric Anesthesia COVID-19 Collaborative members on September 1, 2020. Respondents had six weeks to complete the survey and were instructed to answer the questions based on their institution's practice during September 1 - October 13, 2020.ResultsSixty-three institutions (100% response rate) participated in the COVID-19 Pediatric Anesthesia Survey. Forty-one hospitals (65%) were from the United States, and 35% included other countries. N95 masks were available to anesthesia teams at 91% of institutions (n = 57) (95% CI: 80%-96%). COVID-19 testing criteria of anesthesia staff and guidelines to return to work varied by institution. Structured simulation training aimed at improving COVID-19 safety and patient care occurred at 62% of institutions (n = 39). Pediatric anesthesiologists were economically affected by a reduction in their employer benefits and restriction of travel due to employer imposed quarantine regulations.ConclusionOur data indicate that the COVID-19 pandemic has impacted the testing, safety, clinical management, and economics of pediatric anesthesia practice. Further investigation into the long-term consequences for the specialty is indicated.

BACKGROUND: A subset of patients with postural tachycardia syndrome (POTS) are thought to have a primary hyperadrenergic cause. We assessed clinical biomarkers to identify those that would benefit from sympatholytic therapy. METHODS: We measured sympathetic function (supine muscle sympathetic nerve activity, upright plasma norepinephrine, and blood pressure responses to the Valsalva maneuver) in 28 patients with POTS (phenotyping cohort) to identify clinical biomarkers that are associated with responsiveness to the central sympatholytic guanfacine in a separate uncontrolled treatment cohort of 38 patients that had received guanfacine clinically for suspected hyperadrenergic POTS (HyperPOTS). RESULTS: In the phenotyping cohort, an increase in diastolic blood pressure (DBP) >17 mm Hg during late phase 2 of the Valsalva maneuver identified patients with the highest quartile of resting muscle sympathetic nerve activity (HyperPOTS) with 71% sensitivity and 85% specificity. In the treatment cohort, patients with HyperPOTS, identified by this clinical biomarker, more often reported clinical improvement (85% versus 44% in nonhyperadrenergic; P=0.016), had better orthostatic tolerance (∆Orthostatic Hypotension Daily Activities Scale: −1.9±0.9 versus 0.1±0.5; P=0.032), and reported less chronic fatigue (∆PROMIS Fatigue Short Form 7a: −12.9±2.7 versus −2.2±2.2; P=0.005) in response to guanfacine. CONCLUSIONS: These results are consistent with the concept that POTS is caused by a central sympathetic activation in a subset of patients, which can be identified clinically by an exaggerated DBP increase during phase 2 of the Valsalva maneuver and improved by central sympatholytic therapy. These results support further clinical trials to determine the safety and efficacy of guanfacine in patients with POTS enriched for the presence of this clinical biomarker. [ABSTRACT FROM AUTHOR]

The US Food and Drug Administration (FDA) and National Organization for Rare Disease (NORD) convened a public workshop titled "Immune Responses to Enzyme Replacement Therapies: Role of Immune Tolerance Induction" to discuss the impact of anti-drug antibodies (ADAs) on efficacy and safety of enzyme replacement therapies (ERTs) intended to treat patients with lysosomal storage diseases (LSDs). Participants in the workshop included FDA staff, clinicians, scientists, patients, industry, and advocacy group representatives. The risks and benefits of implementing prophylactic immune tolerance induction (ITI) to reduce the potential clinical impact of antibody development were considered. Complications due to immune responses to ERT are being recognized with increasing experience and lengths of exposure to ERTs to treat several LSDs. Strategies to mitigate immune responses and to optimize therapies are needed. Discussions during the workshop resulted in the identification of knowledge gaps and future areas of research, as well as the following proposals from the participants: (1) systematic collection of longitudinal data on immunogenicity to better understand the impact of ADAs on long-term clinical outcomes; (2) development of disease-specific biomarkers and outcome measures to assess the effect of ADAs and ITI on efficacy and safety; (3) development of consistent approaches to ADA assays to allow comparisons of immunogenicity data across different products and disease groups, and to expedite reporting of results; (4) establishment of a system to widely share data on antibody titers following treatment with ERTs; (5) identification of components of the protein that are immunogenic so that triggers and components of the immune responses can be targeted in ITI; and (6) consideration of early ITI in patients who are at risk of developing clinically relevant ADA that have been demonstrated to worsen treatment outcomes.

Known disparities exist in pain treatment between African American, Latino, and White children. A recent study described 'adultification' of Black children, with Black children being less likely to have a parent present at induction of anesthesia and less likely to receive an anxiolytic premedication before proceeding to the operating room. The aim of this study is to identify differences based on race and socioeconomic status when treating children and their families for anesthetic induction. We hypothesize that differences exist such that certain populations are less likely to receive sedative premedication and less likely to have parents present at induction of anesthesia. This was a retrospective cohort study. Demographic data were obtained along with type of surgical procedure, type of anesthesia induction, use of premedication, and involvement of child life services (including the plan for parental presence at induction) for all pediatric patients presenting for anesthetics from February 2019 to March 2020. Statistical analysis consisted of fitting logistic mixed effects models for caregiver presence or for midazolam use during induction, with fixed effects for sex, race, ethnicity, language, public/private insurance, and anesthetic risk, and with the provider as a random effect. A total of 7,753 patients were included in our statistical analyses, and parental presence focused on 4,102 patients with documentation from child life specialists. Females were less likely than males to have parents present at induction (odds ratio [OR] 0.77, confidence interval [CI] [0.67, 0.89]). When looking at race, American Indian/Alaskan Native patients (OR 0.23 [CI 0.093, 0.47]) and Black/African American patients OR 0.64 [CI 0.47, 0.89]) were less likely to have a parent present induction than White patients. Patients with private insurance were more likely to have parents present than patients with public insurance (OR 0.63 CI [0.5, 0.78]). These findings held true in age-separated sensitivity analysis. Asian patients were less likely to receive midazolam premedication (OR 0.65 CI [0.49, 0.86]). This study supports previous work showing differential use of parental presence at induction based on race. Additionally, it also shows different treatment based on sex and public insurance status, a surrogate for socioeconomic status. [ABSTRACT FROM AUTHOR]

Although diagnostic criteria have been developed characterizing postural orthostatic tachycardia syndrome (POTS), no single set of criteria is universally accepted. Furthermore, there are gaps in the present criteria used to identify individuals who have this condition. The reproducibility of the physiological findings, the relationship of symptoms to physiological findings, the presence of symptoms alone without any physiological findings and the response to various interventions confuse rather than clarify this condition. As many disease entities can be confused with POTS, it becomes critical to identify what this syndrome is. What appears to be POTS may be an underlying condition that requires specific therapy. POTS is not simply orthostatic intolerance and symptoms or intermittent orthostatic tachycardia but the syndrome needs to be characterized over time and with reproducibility. Here we address critical issues regarding the pathophysiology and diagnosis of POTS in an attempt to arrive at a rational approach to categorize the syndrome with the hope that it may help both better identify individuals and better understand approaches to therapy. [ABSTRACT FROM AUTHOR]

Objective: To evaluate and better understand variations in practice patterns, we analyzed ambulatory surgery claims data from 3 demographically diverse states to assess the relationship between age at umbilical hernia repair and patient, hospital, and geographic characteristics.Study Design: We performed a cross-sectional descriptive study of uncomplicated hernia repairs performed as a single procedure in 2012-2014, using the State Ambulatory Surgery and Services Database for Wisconsin, New York, and Florida. Age and demographic characteristics of umbilical hernia repair patients are described.Results: The State Ambulatory Surgery and Services Database analysis included 6551 patients. Across 3 states, 8.2% of hernia repairs were performed in children <2 years, 18.7% in children age 2-3 years, and 73.0% in children age ≥4 years, but there was significant variability (P < .001) in practice patterns by state. In regression analysis, race, Medicaid insurance and rural residence were predictive of early repair, with African American patients less likely to have a repair before age 2 (OR 0.62, P = .046) and rural children (OR 1.53, P = .009) and Medicaid patients (OR 2.01, P < .001) more likely to do so. State of residence predicted early repair even when holding these variables constant.Conclusions: The age of pediatric umbilical hernia repair varies widely. As hernias may resolve over time and can be safely monitored with watchful waiting, formal guidelines are needed to support delayed repair and prevent unnecessary operations. [ABSTRACT FROM AUTHOR]

Orthostatic hypotension (OH), a common, often overlooked, disorder with many causes, is associated with debilitating symptoms, falls, syncope, cognitive impairment, and risk of death. Chronic OH, a cardinal sign of autonomic dysfunction, increases with advancing age and is commonly associated with neurodegenerative and autoimmune diseases, diabetes, hypertension, heart failure, and kidney failure. Management typically involves a multidisciplinary, patient-centered, approach to arrive at an appropriate underlying diagnosis that is causing OH, treating accompanying conditions, and providing individually tailored pharmacological and nonpharmacological treatment. We propose a novel streamlined pathophysiological classification of OH; review the relationship between the cardiovascular disease continuum and OH; discuss OH-mediated end-organ damage; provide diagnostic and therapeutic algorithms to guide clinical decision making and patient care; identify current gaps in knowledge and try to define future research directions. Using a case-based learning approach, specific clinical scenarios are presented highlighting various presentations of OH to provide a practical guide to evaluate and manage patients who have OH. [ABSTRACT FROM AUTHOR]