58 research outputs found
"Pi of the Sky" - all-sky, real-time search for fast optical transients
An apparatus to search for optical flashes in the sky is described. It has
been optimized for gamma ray bursts (GRB) optical counterparts. It consists of
2x16 cameras covering all the sky. The sky is monitored continuously and the
data are analysed on-line. It has self-triggering capability and can react to
external triggers with negative delay. The prototype with two cameras has been
installed at Las Campanas (Chile) and is operational from July 2004. The paper
presents general idea and describes the apparatus in detail. Performance of the
prototype is briefly reviewed and perspectives for the future are outlined
Cor triatriatum and lipomatous hypertrophy of the interatrial septum in the elderly: a case report
Cor triatriatum is a rare congenital heart defect characterized by the presence of a fibromuscular membrane dividing the left atrium into two distinct chambers. Lipomatous hypertrophy of the atrial septum is an infrequently observed benign abnormality caused by large fatty tissue deposits in the interatrial septum. An increased incidence of atrial arrhythmias is described in both pathologies, while a significant obstruction of blood flow mimicking mitral stenosis is typically manifested in cor triatriatum. We report the case of a 75-year-old woman with a previously undescribed association of the above stated abnormalities detected by both transthoracic and transeosophageal echocardiography. Diagnosis was confirmed by means of computed tomography. The singular physiologic and anatomic factors underlying survival until such a late age are described. The diagnostic, therapeutic and surgical management is discussed and a short review of the literature performed
Mutations in HYAL2, Encoding Hyaluronidase 2, Cause a Syndrome of Orofacial Clefting and Cor Triatriatum Sinister in Humans and Mice.
Orofacial clefting is amongst the most common of birth defects, with both genetic and environmental components. Although numerous studies have been undertaken to investigate the complexities of the genetic etiology of this heterogeneous condition, this factor remains incompletely understood. Here, we describe mutations in the HYAL2 gene as a cause of syndromic orofacial clefting. HYAL2, encoding hyaluronidase 2, degrades extracellular hyaluronan, a critical component of the developing heart and palatal shelf matrix. Transfection assays demonstrated that the gene mutations destabilize the molecule, dramatically reducing HYAL2 protein levels. Consistent with the clinical presentation in affected individuals, investigations of Hyal2-/- mice revealed craniofacial abnormalities, including submucosal cleft palate. In addition, cor triatriatum sinister and hearing loss, identified in a proportion of Hyal2-/- mice, were also found as incompletely penetrant features in affected humans. Taken together our findings identify a new genetic cause of orofacial clefting in humans and mice, and define the first molecular cause of human cor triatriatum sinister, illustrating the fundamental importance of HYAL2 and hyaluronan turnover for normal human and mouse development
EQ-5D in Central and Eastern Europe : 2000-2015
Objective: Cost per quality-adjusted life year data are required for reimbursement decisions in many Central and Eastern European (CEE) countries. EQ-5D is by far the most commonly used instrument to generate utility values in CEE. This study aims to systematically review the literature on EQ-5D from eight CEE countries. Methods: An electronic database search was performed up to July 1, 2015 to identify original EQ-5D studies from the countries of interest. We analysed the use of EQ-5D with respect to clinical areas, methodological rigor, population norms and value sets. Results: We identified 143 studies providing 152 country-specific results with a total sample size of 81,619: Austria (n=11), Bulgaria (n=6), Czech Republic (n=18), Hungary (n=47), Poland (n=51), Romania (n=2), Slovakia (n=3) and Slovenia (n=14). Cardiovascular (20%), neurologic (16%), musculoskeletal (15%) and endocrine/nutritional/metabolic diseases (14%) were the most frequently studied clinical areas. Overall 112 (78%) of the studies reported EQ VAS results and 86 (60%) EQ-5D index scores, of which 27 (31%) did not specify the applied tariff. Hungary, Poland and Slovenia have population norms. Poland and Slovenia also have a national value set. Conclusions: Increasing use of EQ-5D is observed throughout CEE. The spread of health technology assessment activities in countries seems to be reflected in the number of EQ-5D studies. However, improvement in informed use and methodological quality of reporting is needed. In jurisdictions where no national value set is available, in order to ensure comparability we recommend to apply the most frequently used UK tariff. Regional collaboration between CEE countries should be strengthened
Impact of Optimized Breastfeeding on the Costs of Necrotizing Enterocolitis in Extremely Low Birthweight Infants
To estimate risk of NEC for ELBW infants as a function of preterm formula and maternal milk (MM) intake and calculate the impact of suboptimal feeding on NEC incidence and costs
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