Suchergebnisse

Background--Little is known about the burden of sudden cardiac death (SCD) among active, presumably healthy persons. Weinvestigated the incidence of SCD among US male career firefighters.Methods and Results--All on-duty SCDs among US male career firefighters between 1998 and 2012 were identified from theUS Fire Administration and the US National Institute for Occupational Safety and Health databases. Age-specific incidence rates(IRs) of SCD with 95% CIs were computed. A joinpoint model was fitted to analyze the trend in IR and to help estimate the annualpercentage change of SCD rates over the years. The effects of seasonality were assessed through a Poisson regression model.We identified 182 SCDs; based on 99 available autopsy reports, the leading underlying cause of death was coronary heartdisease (79%). The overall IR was 18.1 SCDs per 100 000 person-years. The age-specific IRs of SCD ranged between 3.8 (forthose aged 18 to 24 years) and 45.2 (for those aged 55 to 64 years) per 100 000 person-years. The annual rate of SCD steadilydeclined over time (annual percentage change 3.9%, 95% CI 5.8 to 2.0). SCD events were more frequent during January(peak-to-low ratio 1.70; 95% CI 1.09 to 2.65). In addition, the IR was 3 times higher during high-risk duties compared with lowriskduties. IRs among firefighters were lower than those observed among the US general population and US military personnel.Conclusions--SCD risk in this active working population is overestimated using statistics from the general population. To addresspublic health questions among these subpopulations, more specific studies of active adults should be conducted.

Background Transthyretin amyloidosis (also known as ATTR amyloidosis) is a systemic, life-threatening disease characterized by transthyretin (TTR) fibril deposition in organs and tissue. A definitive diagnosis of ATTR amyloidosis is often a challenge, in large part because of its heterogeneous presentation. Although ATTR amyloidosis was previously considered untreatable, disease-modifying therapies for the treatment of this disease have recently become available. This article aims to raise awareness of the initial symptoms of ATTR amyloidosis among general practitioners to facilitate identification of a patient with suspicious signs and symptoms. Methods These consensus recommendations for the suspicion and diagnosis of ATTR amyloidosis were developed through a series of development and review cycles by an international working group comprising key amyloidosis specialists. This working group met to discuss the barriers to early and accurate diagnosis of ATTR amyloidosis and develop a consensus recommendation through a thorough search of the literature performed using PubMed Central. Results The cardiac and peripheral nervous systems are most frequently involved in ATTR amyloidosis; however, many patients often also experience gastrointestinal and other systemic manifestations. Given the multisystemic nature of symptoms, ATTR amyloidosis is often misdiagnosed as a more common disorder, leading to significant delays in the initiation of treatment. Although histologic evaluation has been the gold standard to confirm ATTR amyloidosis, a range of tools are available that can facilitate early and accurate diagnosis. Of importance, genetic testing should be considered early in the evaluation of a patient with unexplained peripheral neuropathy. Conclusions A diagnostic algorithm based on initial red flag symptoms and manifestations of cardiac or neurologic involvement will facilitate identification by the general practitioner of a patient with clinically suspicious symptoms, enabling subsequent referral of the patient to a multidisciplinary specialized medical center.