Results of genetic analysis of 11 341 participants enrolled in the My Life, Our Future hemophilia genotyping initiative in the United States.

Background: Hemophilia A (HA) and hemophilia B (HB) are rare inherited bleeding disorders. Although causative genetic variants are clinically relevant, in 2012 only 20% of US patients had been genotyped.

Objectives: My Life, Our Future (MLOF) was a multisector cross-sectional US initiative to improve our understanding of hemophilia through widespread genotyping.

Impact of obesity on factor VIII and von Willebrand factor levels in patients with Type 1 von Willebrand disease and low von Willebrand factor: An analysis of the ATHNdataset.

Introduction: Obesity is associated with endothelial dysfunction, haemostatic and fibrinolytic disturbances, however the impact of obesity on von Willebrand factor (VWF) is unclear.

Aim: The aim of this study was to test our hypothesis that the prevalence of obesity is higher among participants with low VWF (LVWF) compared to type 1 von Willebrand disease (T1VWD).

Methods: A retrospective review of the ATHNdataset as of March 2018 was performed.

Descriptive analysis of bleeding symptoms in haemophilia carriers enrolled in the ATHNdataset.

Background: Several studies have reported that haemophilia carriers have a bleeding tendency independent of factor activity. However, investigations have been fraught with methodological concerns. The ATHNdataset houses the largest data set of haemophilia carriers in the world.

Inhibitors and mortality in persons with nonsevere hemophilia A in the United States.

Although persons with nonsevere hemophilia A (NSHA) account for about one-half of the hemophilia A population, epidemiological data in this subset of individuals are scarce. We set out to describe the clinical characteristics of persons with NSHA with inhibitors, and to determine mortality rates, predictors of mortality, and primary causes of death in persons with NSHA in the United States over a 9-year period (2010-2018). We queried the American Thrombosis and Hemostasis Network dataset (ATHNdataset) for information on demographics, inhibitor status, and date and cause of death.

The impact of extended half-life factor concentrates on prophylaxis for severe hemophilia in the United States.

With licensure of extended half-life (EHL) factor products and the changing landscape of available hemophilia products, patients and providers have options for less treatment-intense prophylaxis. The impact of these products in clinical practice to date remains understudied. We aimed to quantify the use of EHL products in prophylaxis in the US using the ATHN-dataset, a database of 145 ATHN-affiliated hemophilia treatment centers (HTCs).

A Cross-Sectional Study of Women and Girls with Congenital Bleeding Disorders: The American Thrombosis and Hemostasis Network Cohort.

The number of women and girls (WG) with bleeding disorders cared for at hemophilia treatment centers has increased dramatically over the last 30 years, owing to improved recognition of bleeding symptoms specific to WG. However, basic epidemiologic data of this population remain elusive. The ATHNdataset (American Thrombosis and Hemostasis Network) is a surveillance tool for people with bleeding disorders in the United States, providing demographic as well as bleeding symptom and treatment information.

Regional variation and cost implications of prescribed extended half-life factor concentrates among U.S. Haemophilia Treatment Centres for patients with moderate and severe haemophilia.

Background: Extended half-life (EHL) factor VIII (FVIII) and IX (FIX) products are intended to decrease the burden of prophylaxis for patients with haemophilia A or B. Whether these newer concentrates have led to meaningful clinical practice change remains vague.

Aim: To characterize the longitudinal use of standard (SHL) and EHL factor concentrates at haemophilia treatment centres (HTCs), using the ATHNdataset, a US database of 138 ATHN-affiliated HTCs.

Impact of the 340B Pharmacy Program on Services and Supports for Persons Served by Hemophilia Treatment Centers in the United States.

Purpose Hemophilia Treatment Centers (HTCs) provide integrated and comprehensive services to individuals affected with rare bleeding disorders, such as hemophilia and Von Willebrand disease. Through the 340 Drug Pricing Program, HTCs may use pharmacy income to support clinical staff and patient services. The objective of this study was to describe the impact of the 340B program funding on services and support provided by HTCs to persons affected by rare bleeding disorders.

A cross-sectional analysis of cardiovascular disease in the hemophilia population.

Men with hemophilia were initially thought to be protected from cardiovascular disease (CVD), but it is now clear that atherothrombotic events occur. The primary objective of the CVD in Hemophilia study was to determine the prevalence of CVD and CVD risk factors in US older men with moderate and severe hemophilia and to compare findings with those reported in age-comparable men in the Atherosclerosis Risk in Communities (ARIC) cohort. We hypothesized if lower factor levels are protective from CVD, we would see a difference in CVD rates between more severely affected and unaffected men.

Transition of care for patients with venous thromboembolism: Rationale, design and implementation of a quality intervention project conducted at American Thrombosis and Hemostasis Network (ATHN) affiliated sites.

Introduction: Medication errors frequently occur during transition from the inpatient to outpatient setting. Anticoagulants are associated with serious medical errors, including major bleeding. Standardized transition of care (TOC) techniques in patients with venous thromboembolism (VTE) have not been developed.

Clinical relevance of the Humpty Dumpty Falls Scale in a pediatric specialty hospital.

The Joint Commission requires pediatric hospitals to implement fall prevention programs and evaluate the efficacy of such programs. The Humpty Dumpty Falls Scale (HDFS), a seven-item assessment scale used to document age, gender, diagnosis, cognitive impairments, environmental factors, response to surgery/sedation, and medication usage, is one of several instruments developed to assess fall risk in pediatric patients. To determine the specificity and sensitivity of the HDFS in predicting falls in children hospitalized for pediatric specialty care, a team at a pediatric specialty hospital conducted a nonexperimental retrospective study that used a matched case-control design and chart review analysis.

Use of the bootstrap method to develop a physical fitness test for public safety officers who serve as both police officers and firefighters.

Physical fitness testing is a common tool for motivating employees with strenuous occupations to reach and maintain a minimum level of fitness. Nevertheless, the use of such tests can be hampered by several factors, including required compliance with US antidiscrimination laws. The Highland Park (Texas) Department of Public Safety implemented testing in 1991, but no single test adequately evaluated its sworn employees, who are cross-trained and serve as police officers and firefighters.

Comparison of force exerted on the sternum during a sneeze versus during low-, moderate-, and high-intensity bench press resistance exercise with and without the valsalva maneuver in healthy volunteers.

Sternal precautions are intended to prevent complications after median sternotomy, but little data exist to support the consensus recommendations. To better characterize the forces on the sternum that can occur during everyday events, we conducted a prospective nonrandomized study of 41 healthy volunteers that evaluated the force exerted during bench press resistance exercise and while sneezing. A balloon-tipped esophageal catheter, inserted through the subject's nose and advanced into the thoracic cavity, was used to measure the intrathoracic pressure differential during the study activities.

The impact of electronic health records on workflow and financial measures in primary care practices.

Objective: To estimate a commercially available ambulatory electronic health record's (EHR's) impact on workflow and financial measures.

Data Sources/study Setting: Administrative, payroll, and billing data were collected for 26 primary care practices in a fee-for-service network that rolled out an EHR on a staggered schedule from June 2006 through December 2008.

Study Design: An interrupted time series design was used.

Sedentary behaviour and physical inactivity assessment in primary care: the Rapid Assessment Disuse Index (RADI) study.

Background: The emerging evidence of the effects of sedentary time on health outcomes suggests a need to better measure this exposure. Healthcare settings, however, are not equipped with a tool that can quickly assess the sedentary habits of their patient population. The purpose of this study was to validate a tool for rapidly quantifying and tracking the sedentary time and low levels of daily lifestyle physical activity among primary care patients.

Development and evaluation of a treadmill-based exercise tolerance test in cardiac rehabilitation.

Cardiac rehabilitation exercise prescriptions should be based on exercise stress tests; however, limitations in performing stress tests in this setting typically force reliance on subjective measures like the Duke Activity Status Index (DASI). We developed and evaluated a treadmill-based exercise tolerance test (ETT) to provide objective physiologic measures without requiring additional equipment or insurance charges. The ETT is stopped when the patient's Borg scale rating of perceived exertion (RPE) reaches 15 or when any sign/symptom indicates risk of an adverse event.

Evaluation of quality improvement performance in the Community Diabetes Education (CoDE) program for uninsured Mexican Americans: results of a randomized controlled trial.

The objective of this article is to quantify quality improvement using data from a randomized controlled trial that tested the effectiveness of a community health worker in the primary role of diabetes educator in a clinic serving uninsured Mexican Americans. The intervention group received 7 hours of diabetes education/case management in excess of usual medical care. Of 16 process and outcome measures evaluated, the intervention group was significantly more likely to have received a dilated retinal examination, and 53% achieved a hemoglobin A1c below 7% compared with 38% of the control group participants.

High-intensity, occupation-specific training in a series of firefighters during phase II cardiac rehabilitation.

Six male firefighters who were referred to phase II cardiac rehabilitation after coronary revascularization participated in a specialized regimen of high-intensity, occupation-specific training (HIOST) that simulated firefighting tasks. During each session, the electrocardiogram, heart rate, and blood pressure were monitored, and the patients were observed for adverse symptoms. No patient had to discontinue HIOST because of adverse arrhythmias or symptoms.

Phthalate concentrations and dietary exposure from food purchased in New York State.

Background: Phthalates have been found in many personal care and industrial products, but have not previously been reported in food purchased in the United States. Phthalates are ubiquitous synthetic compounds and therefore difficult to measure in foods containing trace levels. Phthalates have been associated with endocrine disruption and developmental alteration.