bluuebunny/crossref_metadata_2025_split huggingface dataset:
Deferasirox limits cartilage damage following haemarthrosis in haemophilic mice
Authors: Roosendaal Goris , Mastbergen Simon , Coeleveld Katja , Biesma Douwe , Lafeber Floris , Schutgens Roger , Nieuwenhuizen Laurens 
Published: September 2014

SummaryJoint bleeds in haemophilia result in iron-mediated synovitis and cartilage damage. It was evaluated whether deferasirox, an iron chelator, was able to limit the development of haemophilic synovitis and cartilage damage. Haemophilic mice were randomly assigned to oral treatment with deferasirox (30 mg/kg) or its vehicle (control) (30 mg/kg). Eight weeks after start of treatment, haemarthrosis was induced. After another five weeks of treatment, blood-induced synovitis and cartilage damage were determined. Treatment with deferasirox resulted in a statistically significant (p< 0.01) decrease in plasma ferritin levels as compared to the control group (823 ng/ml ± 56 and 1220 ng/ml ±114, respectively). Signs of haemophilic synovitis, as assessed by the Valentino score [range 0 (normal) – 10 (most affected)], were not different (p=0.52) when comparing the control group with the deferasirox group. However, deferasirox treatment resulted in a statistically significant (p< 0.01) reduction in cartilage damage, as assessed by the loss in Safranin O staining [range 0 (normal) – 6 (most affected)], when comparing the deferasirox group with the control group: score 2 (65.4 % vs 4.2 %), score 3 (26.9 % vs 4.2 %), score 4 (7.7 % vs 20.8 %), score 5 (0 % vs 54.2 %), and score 6 (0 % vs 16.7 %). Treatment with deferasirox limits cartilage damage following the induction of a haemarthrosis in haemophilic mice. This study demonstrates the role of iron in blood-induced cartilage damage. Moreover, these data indicate that iron chelation may be a potential prevention option to limit the development of haemophilic arthropathy.
Novel α-Actinin 2 Variant Associated With Familial Hypertrophic Cardiomyopathy and Juvenile Atrial Arrhythmias
Authors: Girolami Francesca , Iascone Maria , Tomberli Benedetta , Bardi Sara , Benelli Matteo , Marseglia Giuseppina , Pescucci Chiara , Pezzoli Laura , Sana Maria Elena , Basso Cristina , Marziliano Nicola , Merlini Piera Angelica , Fornaro Alessandra , Cecchi Franco , Torricelli Francesca , Olivotto Iacopo 
Published: August 2014

Background—
Next-generation sequencing might be particularly advantageous in genetically heterogeneous conditions, such as hypertrophic cardiomyopathy (HCM), in which a considerable proportion of patients remain undiagnosed after Sanger. In this study, we present an Italian family with atypical HCM in which a novel disease-causing variant in α-actinin 2 (ACTN2) was identified by next-generation sequencing.

Methods and Results—
A large family spanning 4 generations was examined, exhibiting an autosomal dominant cardiomyopathic trait comprising a variable spectrum of (1) midapical HCM with restrictive evolution with marked biatrial dilatation, (2) early-onset atrial fibrillation and atrioventricular block, and (3) left ventricular noncompaction. In the proband, 48 disease genes for HCM, selected on the basis of published reports, were analyzed by targeted resequencing with a customized enrichment system. After bioinformatics analysis, 4 likely pathogenic variants were identified: TTN c.21977G>A (p.Arg7326Gln); TTN c.8749A>C (p.Thr2917Pro); ACTN2 c.683T>C (p.Met228Thr); and OBSCN c.13475T>G (p.Leu4492Arg). The novel variant ACTN2 c.683T>C (p.Met228Thr), located in the actin-binding domain, proved to be the only mutation fully cosegregating with the cardiomyopathic trait in 18 additional family members (of whom 11 clinically affected). ACTN2 c.683T>C (p.Met228Thr) was absent in 570 alleles of healthy controls and in 1000 Genomes Project and was labeled as Damaging by in silico analysis using polymorphism phenotyping v2, as Deleterious by sorts intolerant from tolerant, and as Disease-Causing by Mutation Taster.

Conclusions—
A targeted next-generation sequencing approach allowed the identification of a novel ACTN2 variant associated with midapical HCM and juvenile onset of atrial fibrillation, emphasizing the potential of such approach in HCM diagnostic screening.
Dose–Response Relationship of Total and Leisure Time Physical Activity to Risk of Heart Failure
Authors: Andersen Kasper , Mariosa Daniela , Adami Hans-Olov , Held Claes , Ingelsson Erik , Lagerros Ylva Trolle , Nyrén Olof , Ye Weimin , Bellocco Rino , Sundström Johan 
Published: September 2014

Background—
The nature of the association between levels of physical activity and risk of heart failure is little known. We investigated nonlinear associations of total and leisure time physical activity with risk of heart failure.

Methods and Results—
In 1997, 39 805 persons without heart failure completed a questionnaire of lifestyle factors and medical history. We used Cox regression models to investigate total (adjusting for education and previous myocardial infarction) and direct (multivariable-adjusted) effects of self-reported total and leisure time physical activity on risk of heart failure of any cause and heart failure of nonischemic origin. Heart failure diagnoses were obtained until December 31, 2010. Higher leisure time physical activity was associated with lower risk of heart failure of any cause; hazard ratio of the total effect of leisure time physical activity was for fifth versus first quintile 0.54; 95% confidence interval was 0.44 to 0.66. The direct effect was similar. High total daily physical activity level was associated with lower risk of heart failure, although the effect was less pronounced than for leisure time physical activity (total effect hazard ratio, 0.81; 95% confidence interval, 0.69–0.95; fifth versus first quintile). A similar direct effect observed.

Conclusions—
Leisure time physical activity was inversely related to risk of developing heart failure in a dose–response fashion. This was reflected in a similar but less pronounced association of total physical activity with risk of heart failure. Only part of the effects appeared to be mediated by traditional risk factors.
Development and Validation of a Short Version of the Seattle Angina Questionnaire
Authors: Chan Paul S. , Jones Philip G. , Arnold Suzanne A. , Spertus John A. 
Published: September 2014

Background—
Clinical trials and national performance measures increasingly mandate reporting patients’ perspectives of their health status: their symptoms, function, and quality of life. Although the Seattle Angina Questionnaire (SAQ) is a validated disease-specific health status instrument for coronary artery disease (CAD) with high test–retest reliability, predictive power, and responsiveness, its use in routine clinical practice has been limited, in part, by its length (19 items).

Methods and Results—
Using data from 10 408 patients with CAD from 5 multicenter registries, we derived and validated a shortened version of the SAQ (SAQ-7) among patients presenting with stable CAD, undergoing percutaneous coronary intervention, and after acute myocardial infarction. We examined the psychometric properties of the SAQ-7 as compared with the full SAQ. Seven items from the Physical Limitation, Angina Frequency, and Quality of Life domains were identified for the SAQ-7, with high levels of concordance (0.88–1.00) with each original SAQ domain. The SAQ-7 demonstrated good construct validity (compared with Canadian Cardiovascular Society class for angina), with a correlation of 0.62 and 0.38 for patients with stable CAD and undergoing percutaneous coronary intervention, respectively. It was highly reproducible in patients with stable CAD (intraclass correlation, ≥0.78) and exhibited excellent responsiveness in patients after percutaneous coronary intervention (≥18 points in each SAQ domain). Finally, the SAQ-7 was predictive of 1-year mortality and readmission.

Conclusions—
To increase the feasibility of measuring patient-reported outcomes in patients with CAD, we developed and validated a shortened 7-item SAQ instrument for use in clinical trials and routine care.
Long-Term Outcomes After Combined Revascularization Surgery in Adult Moyamoya Disease
Authors: Cho Won-Sang , Kim Jeong Eun , Kim Chang Hyeun , Ban Seung Pil , Kang Hyun-Seung , Son Young Je , Bang Jae Seung , Sohn Chul-Ho , Paeng Jin Chul , Oh Chang Wan 
Published: September 2014

Background and Purpose—
The surgical outcomes of adult moyamoya disease are rarely reported. We aimed to evaluate the long-term outcomes of combined revascularization surgery in patients with adult moyamoya disease.

Methods—
Combined revascularization surgery consisting of superficial temporal artery–middle cerebral artery anastomosis with encephalodurogaleosynangiosis was performed on 77 hemispheres in 60 patients. Clinical, angiographic, and hemodynamic states were evaluated retrospectively using quantitative methods preoperatively and postoperatively in the short-term (≈6 months) and long-term (≈5 years) periods. The mean clinical follow-up duration was 71.0±10.1 months (range, 60–104 months).

Results—

Clinical status improved until 6 months after surgery and remained stable thereafter, as assessed by the Karnofsky Performance Scale and modified Rankin Scale. The revascularization area relative to supratentorial area significantly increased in the long-term period compared with that in the short-term period (54.8% versus 44.2%;
P
<0.001). Cerebral blood flow in the territory of the middle cerebral artery improved in the short-term period compared with that in the preoperative period (68.7 versus 59.1; considering blood flow of the pons as 50;
P
<0.001) and thereafter became stable (65.5 in the long term;
P
=0.219). The annual risks of symptomatic hemorrhage and infarction were 0.4% and 0.2%, respectively, in the operated hemispheres.

Conclusions—
Combined revascularization surgery resulted in satisfactory long-term improvement in clinical, angiographic, and hemodynamic states and prevention of recurrent stroke.
Moisture Asymmetry and MJO Eastward Propagation in an Aquaplanet General Circulation Model*
Authors: Hsu Pang-Chi , Li Tim , Murakami Hiroyuki 
Published: September 2014

Abstract
The role of zonal moisture asymmetry in the eastward propagation of the Madden–Julian oscillation (MJO) is investigated through a set of aquaplanet atmospheric general circulation model (AGCM) experiments with a zonally symmetric sea surface temperature distribution. In the control experiment, the model produces eastward-propagating MJO-like perturbations with a dominant period of 30–90 days. The model MJO exhibits a clear zonal asymmetry in the lower-tropospheric specific humidity field, with a positive (negative) anomaly appearing to the east (west) of the MJO convection. A diagnosis of the lower-tropospheric moisture budget indicates that the asymmetry primarily arises from vertical moisture advection associated with boundary layer convergence, while horizontal moisture advection has the opposite effect.
In a sensitivity experiment, the lower-tropospheric specific humidity field is relaxed toward a zonal-mean basic state derived from the control simulation. In this case, the model’s mean state remains the same, but its intraseasonal mode becomes quasi-stationary. The numerical model experiments clearly demonstrate the importance of the zonal moisture asymmetry in MJO eastward propagation.
Analysis of Vortex Dynamics of Lateral Circulation in a Straight Tidal Estuary*
Authors: Li Ming , Cheng Peng , Chant Robert , Valle-Levinson Arnoldo , Arnott Kim 
Published: September 2014

Abstract
The dynamics associated with lateral circulation in a tidally driven estuarine channel is analyzed on the basis of streamwise vorticity. Without rotational effects, differential advection and diffusive boundary mixing produce two counterrotating vortices (in the cross-channel section) whose strength and sense of circulation may change during a tidal cycle. The streamwise vorticity equation is determined by a balance between baroclinic forcing and turbulent diffusion, which explains the flood–ebb asymmetry of the lateral circulation. Analysis of the lateral salinity gradient shows that differential advection is the main driver of lateral flows, but boundary mixing can also be an important contributor in stratified estuaries. The strength of lateral circulation decreases with increasing stratification. With rotational effects, the lateral Ekman forcing in the bottom boundary layer drives a one-cell lateral circulation that switches its sense of rotation over the tidal cycle. The vorticity budget analysis reveals a three-way balance among the tilting of planetary vorticity by the vertical shear in the along-channel current, baroclinic forcing, and turbulent diffusion. The structure and magnitude of the lateral circulation change with the width of the estuary, expressed nondimensionally as the Kelvin number Ke. This lateral circulation features two counterrotating vortices in narrow estuaries, one vortex filling up the entire cross section in estuaries of intermediate widths and one vortex confined to the left side (looking into the estuary) in wide estuaries. The magnitude of the streamwise vorticity increases rapidly with Ke, reaches a maximum at , and decreases slightly in wide estuaries subject to strong rotational control.
Why Scanning Instruments Are a Necessity for Constraining Temperature and Humidity Fields in the Lower Atmosphere
Authors: Themens David , Fabry Frédéric 
Published: September 2014

AbstractThe ability of different ground-based measurement strategies for constraining thermodynamic variables in the troposphere, particularly at the mesoscale, is investigated. First, a preliminary assessment of the capability of pure-vertical sounders for constraining temperature and water vapor fields in clear-sky conditions to current accuracy requirements is presented. Using analyses over one month from the Rapid Refresh model as input to an optimal estimation technique, it is shown that the horizontal density of a network of nonexisting, ideal vertical profiling instruments must be greater than 30 km in order to achieve accuracies of 0.5 g kg−1 for water vapor and 0.5 K for temperature. Then, an assessment of a scanning microwave radiometer’s capability for retrieving water vapor and temperature fields in a cloud-free environment over two- and three-dimensional mesoscale domains is also presented. The information content of an elevation and azimuthal scanning microwave radiometer is assessed using the same optimal estimation framework. Even though, in any specific pointing direction, the scanning radiometer does not provide much information, it is capable of providing considerably more constraints on thermodynamic fields, particularly water vapor, than a near-perfect vertical sounder. These constraints on water vapor are largely located within 80 km of the radiometer and between 1000- and 7000-m altitude, while temperature constraints are limited to within 35 km of the instrument at altitudes between the ground and 1500 m. The findings suggest that measurements from scanning radiometers will be needed to properly constrain the temperature and especially moisture fields to accuracies needed for mesoscale forecasting.
The Convective Evolution and Rapid Intensification of Hurricane Earl (2010)
Authors: Stevenson Stephanie N. , Corbosiero Kristen L. , Molinari John 
Published: September 2014

Abstract
The relationship between an inner-core (r &lt; 100 km) lightning outbreak and the subsequent rapid intensification (RI) of Hurricane Earl (2010) is examined using lightning strikes recorded by the World Wide Lightning Location Network (WWLLN) and in situ observations from various aircraft missions. Moderate (8.4 m s−1) northeasterly deep-layer (850–200 hPa) vertical wind shear, caused by outflow from Hurricane Danielle, existed over Earl at the beginning of a prolonged period of RI. Over 70% of the lightning strikes within a 500-km radius occurred downshear, with a preference toward downshear right in the outer rainbands, in agreement with previous studies.
The location of inner-core strikes in Earl differed markedly from previous studies. The inner-core lightning activity precessed from left of shear to upshear, an extremely rare event, beginning just prior to the onset of RI. Diagnosis of the vortex tilt midway through the lightning precession showed this convection was occurring downtilt in the upshear-left quadrant; however, limited observations could not confirm if the vortex tilt was precessing with the lightning. Elevated values of low-level relative humidity and CAPE were also found upshear and supported the inner-core convection, which was found to occur within the radius of maximum wind (RMW). Previous studies have shown that convection located inside the RMW promotes intensification. It is hypothesized that intensification may have occurred in part because the vertical wind shear acted to reduce the upshear tilt, and the occurrence of convection inside the RMW helped to enhance the warm core.
Forecasting a Large Number of Tropical Cyclone Intensities around Japan Using a High-Resolution Atmosphere–Ocean Coupled Model
Authors: Ito Kosuke , Kuroda Tohru , Saito Kazuo , Wada Akiyoshi 
Published: September 2014

AbstractThis work quantifies the benefits of using a high-resolution atmosphere–ocean coupled model in tropical cyclone (TC) intensity forecasts in the vicinity of Japan. To do so, a large number of high-resolution calculations were performed by running the Japan Meteorological Agency (JMA) nonhydrostatic atmospheric mesoscale model (AMSM) and atmosphere–ocean coupled mesoscale model (CMSM). A total of 281 3-day forecasts were compiled for 34 TCs from April 2009 to September 2012 for each model. The performance of these models is compared with the JMA global atmospheric spectral model (GSM) that is used for the operational TC intensity guidance. The TC intensities are better predicted by CMSM than the other models. The improvement rates in CMSM relative to GSM and AMSM generally increase with increasing forecast time (FT). CMSM is better than GSM and AMSM by 27.4% and 21.3% at FT = 48 h in terms of minimum sea level pressure, respectively. Regarding the maximum wind speed, CMSM is better than GSM and AMSM by 12.8% and 19.5% at FT = 48 h, respectively. This is due to smaller initial intensity errors and sea surface cooling consistent with in situ observations that suppress erroneous TC intensification. Thus, a high-resolution coupled model is promising for TC intensity prediction in the area surrounding Japan, where most of the TCs are in a decay stage. In contrast, coupling to the upper-ocean model yields only a negligible difference in the TC track forecast skill on average.
Board of Governors’ Hot Topic 2014: Will Fee for Service Survive?
Authors: Parikh Sanjay R. , Kennedy David W. , Waguespack Richard W. , Denneny James C. , Setzen Michael 
Published: September 2014

Program Description:On the hinge of health care reform, the Board of Governors’ (BOG's) 2014 hot topic is: will fee for service survive? Expert panelists, consisting of American Academy of Otolaryngology—Head and Neck Surgery presidents, BOG chairs, and physician payment and policy workgroup members, will discuss if fee for service will be superseded by payments based on work relative value units, quality measures, hospital employment, and bundled payments. Presentations will be made on current and past payment structures, bundled payments, quality metrics, and the sustainability of private practice. Time for interaction between the audience and panelists will be ensured through a final round table discussion.Educational Objectives:(1) Describe the history of fee for service and how it has changed in the past 10 years. (2) Relate the impact of health care reform on current and future payment systems. (3) Recognize the challenges private practice will have with new reimbursement models.
Facial Plastic Surgery Maintenance of Certification Review Course
Authors: Jordan James R. , Fedok Fred G. , Jarchow Andrea , McCarn Kate E. , Chi John J. 
Published: September 2014

Program Description:This 3‐hour course sponsored by the American Academy of Otolaryngology—Head and Neck Surgery Foundation Facial Plastic and Reconstructive Surgery Education Committee will provide a concise review of facial plastic surgery topics that will provide attendees a starting point in preparing for the recertification examination (maintenance of certification [MOC] part III). Faculty will use a combination of didactic and case‐based formats to cover essential content areas including facial trauma, aging face, facial defect repair, and surgical management of chronic facial paralysis. The course will also fulfill the MOC part II requirements by providing continuing medical education credit.Educational Objectives:(1) Demonstrate increased understanding of key topics in facial plastic surgery. (2) Guide further study on these topics in facial plastic surgery. (3) Access available resources in facial plastic surgery that will assist in test preparation.
Facial Trauma, Concussions, and Return to Play: What Otolaryngologists Need to Know
Authors: Kellman Robert M. , Tatum Sherard A. , Jeyakumar Anita S. , Tollefson Travis T. , Doerr Timothy D. , Brickman Todd M. 
Published: September 2014

Program Description:Concussions and return to play are public health concerns that are gaining coverage as incidence and public awareness increases. Otolaryngologists play an increasing role in the diagnosis of facial trauma and concussions. Not only does the public and health community look to head and neck surgeons for facial trauma treatment, but there is also increasing need for otolaryngologists to play a role in helping concussed athletes return to play. This miniseminar will present the relationship of facial trauma to concussions, review guidelines for diagnosing concussions, review guidelines for returning athletes to play, and review how otolaryngologists fit into the treatment algorithm.Educational Objectives:(1) Consider the relationship between facial trauma and concussions. (2) Cite the criteria for diagnosing concussions and returning athletes back to play. (3) Determine the role the otolaryngologist plays in helping the athlete return to play.
Academic Bowl
Authors: Wax Mark K. , Malekzadeh Sonya 
Published: September 2014

Program Description:The academic bowl has been an annual interactive resident competition at the American Academy of Otolaryngology—Head and Neck Surgery Foundation annual meeting for the past 8 years. Four teams from across the country are selected based on strict academic criteria. These programs send 3 residents to represent them in an interactive question‐and‐answer format. The educational committees of the foundation develop questions. They are presented to the teams and the audience. Each team responds, and the audience is given a chance to pit their knowledge against the residents’. The resident team with the best score at the end of the competition wins a prize.Educational Objectives:(1) Attendees will be able to see what the current level of knowledge is in various subspecialties of otolaryngology. (2) Attendees will be able to compare their knowledge base to residents across a wide range of topics. (3) Attendees will be able to determine areas of weakness in their knowledge base as compared with residents.