bluuebunny/crossref_metadata_2025_split huggingface dataset:
Joint assessment in von Willebrand disease
Authors: van Galen Karin , Timmer Merel , de Kleijn Piet , Fischer Kathelijn , Foppen Wouter , Schutgens Roger , Eikenboom Jeroen , Meijer Karina , Cnossen Marjon , Fijnvandraat Karin , van der Bom Johanna , Larosvan Gorkom Britta , Leebeek Frank , Mauser-Bunschoten Eveline ,   on behalf of the Win study group
Published: May 2017

SummaryAssessment of clinical outcome after joint bleeding is essential to identify joint damage and optimise treatment, to prevent disability. However, disease-specific tools to assess the musculoskeletal status in patients with von Willebrand disease (VWD) are lacking. We aimed to determine validity and reliability of the Haemophilia Joint Health Score (HJHS) and Haemophilia Activities List (HAL) in patients with Von Willebrand disease (VWD). Ninety-six patients with VWD were included (mean age 46 years) of whom 27 had more than five documented joint bleeds. The HJHS was performed in all patients and all patients completed the HAL and Impact on Participation and Autonomy (IPA) questionnaires. Health-related quality of life (SF36) results were obtained from the prior ‘Willebrand in the Netherlands’ study. Joint X-rays of knees, elbows and ankles were scored according to Pettersson (PS). Internal consistency of the HJHS (Cronbach’s α (α)=0.75) and HAL (α=0.89) were good. Inter-observer agreement of the HJHS was good (ICC 0.84; Limits of Agreement ± 10.3). The HJHS showed acceptable correlation with the X-ray PS (Spearman’s r (rs)>0.60 all joints) and HAL (rs=0.71). The HAL also showed acceptable correlation with the SF36 physical functioning (rs=0.65) and IPA (rs=0.69). Hypothesis testing showed adequate discriminative power of both instruments: in patients with a history of >5 versus ≤ 5 joint bleeds (median HJHS 10 vs 2 (p<0.01); median HAL 77 vs 98 (p<0.01)), independent from age. In conclusion, both the HJHS and HAL are feasible to assess clinical outcome after joint bleeds in VWD.
Survival in Women Versus Men Following Implantation of Pacemakers, Defibrillators, and Cardiac Resynchronization Therapy Devices in a Large, Nationwide Cohort
Authors: Varma Niraj , Mittal Suneet , Prillinger Julie B. , Snell Jeff , Dalal Nirav , Piccini Jonathan P. 
Published: May 2017

Background

Whether outcomes differ between sexes following treatment with pacemakers (
PM
), implantable cardioverter defibrillators, and cardiac resynchronization therapy (
CRT
) devices is unclear.

Methods and Results

Consecutive US patients with newly implanted
PM
, implantable cardioverter defibrillators, and
CRT
devices from a large remote monitoring database between 2008 and 2011 were included in this observational cohort study. Sex‐specific all‐cause survival postimplant was compared within each device type using a multivariable Cox proportional hazards model, stratified on age and adjusted for remote monitoring utilization and
ZIP
‐based socioeconomic variables. A total of 269 471 patients were assessed over a median 2.9 [interquartile range, 2.2, 3.6] years. Unadjusted mortality rates (
MR
; deaths/100 000 patient‐years) were similar between women versus men receiving
PM
s (n=115 076, 55% male;
MR
4193 versus
MR
4256, respectively; adjusted hazard ratio, 0.87; 95%
CI
, 0.84–0.90;
P
<0.001) and implantable cardioverter defibrillators (n=85 014, 74% male;
MR
4417 versus
MR
4479, respectively; adjusted hazard ratio, 0.98; 95%
CI
, 0.93–1.02;
P
=0.244). In contrast, survival was superior in women receiving
CRT
defibrillators (n=61 475, 72% male;
MR
5270 versus male MR 7175; adjusted hazard ratio, 0.73; 95%
CI
, 0.70–0.76;
P
<0.001) and also
CRT
pacemakers (n=7906, 57% male;
MR
5383 versus male MR 7625, adjusted hazard ratio, 0.69; 95%
CI
, 0.61–0.78;
P
<0.001). This relative difference increased with time. These results were unaffected by age or remote monitoring utilization.

Conclusions

Women accounted for less than 30% of high‐voltage implants and fewer than half of low‐voltage implants in a large, nation‐wide cohort. Survival for women and men receiving implantable cardioverter defibrillators and
PM
s was similar, but dramatically greater for women receiving both defibrillator‐ and PM‐based
CRT
.
Prestimulus Alpha Oscillations and the Temporal Sequencing of Audiovisual Events
Authors: Grabot Laetitia , Kösem Anne , Azizi Leila , van Wassenhove Virginie 
Published: May 2017

Abstract
Perceiving the temporal order of sensory events typically depends on participants' attentional state, thus likely on the endogenous fluctuations of brain activity. Using magnetoencephalography, we sought to determine whether spontaneous brain oscillations could disambiguate the perceived order of auditory and visual events presented in close temporal proximity, that is, at the individual's perceptual order threshold (Point of Subjective Simultaneity [PSS]). Two neural responses were found to index an individual's temporal order perception when contrasting brain activity as a function of perceived order (i.e., perceiving the sound first vs. perceiving the visual event first) given the same physical audiovisual sequence. First, average differences in prestimulus auditory alpha power indicated perceiving the correct ordering of audiovisual events irrespective of which sensory modality came first: a relatively low alpha power indicated perceiving auditory or visual first as a function of the actual sequence order. Additionally, the relative changes in the amplitude of the auditory (but not visual) evoked responses were correlated with participant's correct performance. Crucially, the sign of the magnitude difference in prestimulus alpha power and evoked responses between perceived audiovisual orders correlated with an individual's PSS. Taken together, our results suggest that spontaneous oscillatory activity cannot disambiguate subjective temporal order without prior knowledge of the individual's bias toward perceiving one or the other sensory modality first. Altogether, our results suggest that, under high perceptual uncertainty, the magnitude of prestimulus alpha (de)synchronization indicates the amount of compensation needed to overcome an individual's prior in the serial ordering and temporal sequencing of information.
Bias Correction of Climate Modeled Temperature and Precipitation Using Artificial Neural Networks
Authors: Moghim Sanaz , Bras Rafael L. 
Published: May 2017

Abstract
Climate studies and effective environmental management require unbiased climate datasets. This study develops a new bias correction approach using a three-layer feedforward neural network to reduce the biases of climate variables (temperature and precipitation) over northern South America. Air and skin temperature, specific humidity, and net longwave and shortwave radiation are used as inputs to the network for bias correction of 6-hourly temperature. Inputs to the network for bias correction of monthly precipitation are precipitation at lag 0, 1, 2, and 3 months, and also the standard deviation of precipitation from 3 × 3 neighbors around the pixel of interest. The climate model data are provided by the Community Climate System Model, version 3 (CCSM3). Results show that the trained artificial neural network (ANN) can improve the estimation error and correlation of the variables for both calibration and validation periods even when there is a low temporal consistency between the time series of the model data and targets. The developed model is also able to modify the probabilistic structure of the variables although the quantile-based information is not directly considered in the network. The ANN model outperforms linear regression, which is used for comparison purposes. The new method can be used to produce bias-corrected climate variables that can be used as forcing to hydrological and ecological models.
Langerhans cell histiocytosis (eosinophilic granuloma) of the skull mimicking nummular headache. Report of two cases
Authors: Silva Rosas Carlos , Angus-Leppan Heather , Lemp Melchor Bruno , Rozas Juan Pablo , Quijada Alonso Humberto 
Published: May 2017

Background Nummular headache is a rare, recently described topographic headache defined by the circumscribed coin-shaped area of pain. It is classified as a primary headache. There is debate about whether it is due to a peripheral or central disturbance, and its relationship to migraine. Case reports We report two patients with presumed nummular headache secondary to Langerhans cell histiocytosis, both with resolution of their headaches after surgical resection. Conclusion Imaging in patients with clinical features of nummular headache is recommended, as this and other cases highlight that it may be symptomatic. There are no distinguishing clinical features to separate nummular headache from secondary mimics, and treatment of the underlying cause may be curative.
The Effects of Repeated Insulin Hypoglycaemia on Capillary and Tissue Metabolism in Man
Authors: Lovett Doust John W. , Salna Marta E. 
Published: May 2017

(1) The response of 2 male schizophrenic patients in terms of quantified extent of mental state disturbance, 12 measures of capillary vascular physiology and oxidative metabolism and 5 estimates of tissue cell metabolism were employed kinetically to determine the influence of insulin hypoglycaemia in man. Longitudinal observations were continued for 5 months, serial measurements being obtained daily prior to commencement of the insulin course and in the afternoons after interruption of the hypoglycaemic state by glucose. (2) Clinically, both patients improved initially but only one of them maintained this improvement and returned to his job; the other was finally committed to an Ontario Hospital. This difference was reflected in a mean fall of 10 points in the quantified disturbance rating scale in the former and of only 3 points in the latter patient. (3) The post-insulin state differed significantly from the pre-insulin baseline state in the patient who improved in that the body weight increased while tissue oedema diminished; the C.F.F. rose; pCO2 was increased; pulse pressure widened; capillary blood pressure fell; arteriolar and capillary blood flow both increased; and capillary oxygen saturation also rose. In the patient who finally failed to improve clinically, similar changes were also found save that although his pO2 increased, arteriolar blood flow fell somewhat and, in general, the physiological gains were less than in the former patient. Improvement in capillary resistance occurred in both patients, but the results could not be analysed statistically. (4) Microscopic observations showed a gain in capillary tone in both patients following the hypoglycaemic episodes, and an increase in translucency in the inter-vascular and peri-vascular tissue field. Together with the decrease in waterlogging of the tissues, it was felt that these findings supported an improved local cellular metabolism. Again, the changes in the clinically indifferent patient were differentially less well marked than in the patient who did well from his treatment.
Personality Patterns and Duodenal Ulcer Relapse under Antisecretory Treatment*
Authors: Magni Guido , Di Mario Francesco , Conlon Patrick , Naccarato Remo 
Published: May 2017

The personality pattern of duodenal ulcer (DU) patients on maintenance treatment who relapsed (N = 22) was compared to a similar group who did not relapse (N = 57) using the Sixteen Personality Factors Questionnaire Multivariate analysis of data indicated that a subgroup of DU patients with personality marked by dependent and anxiety traits was more prone to relapse of illness.
Recruitment of macrophages from the spleen contributes to myocardial fibrosis and hypertension induced by angiotensin II
Authors: Wang Ning-Ping , Erskine James , Zhang Wei-Wei , Zheng Rong-Hua , Zhang Li-Hui , Duron Garret , Gendreau Julian , Zhao Zhi-Qing 
Published: May 2017

Introduction: The purpose of this study was to determine whether macrophages migrated from the spleen are associated with angiotensin II-induced cardiac fibrosis and hypertension. Methods: Sprague-Dawley rats were subjected to angiotensin II infusion in vehicle (500 ng/kg/min) for up to four weeks. In splenectomy, the spleen was removed before angiotensin II infusion. In the angiotensin II AT1 receptor blockade, telmisartan was administered by gastric gavage (10 mg/kg/day) during angiotensin II infusion. The heart and aorta were isolated for Western blot analysis and immunohistochemistry. Results: Angiotensin II infusion caused a significant reduction in the number of monocytes in the spleen through the AT1 receptor-activated monocyte chemoattractant protein-1. Comparison of angiotensin II infusion, splenectomy and telmisartan comparatively reduced the recruitment of macrophages into the heart. Associated with this change, transforming growth factor β1 expression and myofibroblast proliferation were inhibited, and Smad2/3 and collagen I/III were downregulated. Furthermore, interstitial/perivascular fibrosis was attenuated. These modifications occurred in coincidence with reduced blood pressure. At week 4, invasion of macrophages and myofibroblasts in the thoracic aorta was attenuated and expression of endothelial nitric oxide synthase was upregulated, along with a reduction in aortic fibrosis. Conclusions: These results suggest that macrophages when recruited into the heart and aorta from the spleen potentially contribute to angiotensin II-induced cardiac fibrosis and hypertension.
Unmet Primary Care Needs in Diabetic Patients with Multimorbidity in a Medically Underserved Area
Authors: Jackson Bianca M. , Gutierrez Mary Lou , Relyea George E. , Carlton Erik L. , Ahn SangNam , Binkley Bonnie L. , Bailey James E. 
Published: May 2017

Background: Diabetic patients with multimorbidity in medically underserved minority communities are less engaged in primary care and experience high emergency department (ED) utilization. This study assesses unmet primary care needs among diabetic patients in a medically underserved area (MUA). Community Context: A suburb of Memphis—Whitehaven, Tennessee (Shelby County, ZIP codes 38109 and 38116)—majority African American (96.6%) with 30.5% below the poverty level. Methods: Community case study using multiple data sources including diabetes registry, individual interviews, focus groups, and a survey of 30 ED patients with diabetes and multimorbidity. Results: Diabetes registry data indicated that 95.5% of 5723 diabetic patients had multimorbidity. Over 91.5% were uncontrolled at some point in 2014 to 2015. Only 83% of patients with diabetes and multimorbidity reported having a primary care provider (PCP) and those without a PCP were more likely to report delays in needed care. Patients expressed strong interest in health coaching (88%) and receiving text messages from the doctor’s office (73%). Individual patient interviews (n = 9) and focus groups (n = 11) revealed common primary care and self-care experiences and needs including diabetes education, improved patient–provider communication, health-care access and coverage, and strengthened primary care and community. Conclusion: This study demonstrates that almost 1 in 5 ED complex diabetic patients in an MUA do not have a PCP, and that difficulty accessing primary care often results in patients forgoing needed care. Qualitative findings support these conclusions. These results suggest that primary care capacity and infrastructure to support diabetes self-care need strengthening in MUAs.
Hydroxycalciomicrolite, Ca1.5Ta2O6(OH), a new member of the microlite group from Volta Grande pegmatite, Nazareno, Minas Gerais, Brazil
Authors: Andrade M. B. , Yang H. , Atencio D. , Downs R. T. , Chukanov N. V. , Lemée-Cailleau M. H. , Persiano A. I. C. , Goeta A. E. , Ellena J. 
Published: May 2017

AbstractHydroxycalciomicrolite, Ca1.5Ta2O6(OH) is a new microlite-group mineral found in the Volta Grande pegmatite, Nazareno, Minas Gerais, Brazil. It occurs as isolated octahedral and as a combination of octahedral and rhombic dodecahedral crystals, up to1.5 mm in size. The crystals are yellow and translucent, with a white streak and vitreous to resinous lustre. The mineral is brittle, with a Mohs hardness of 5–6. Cleavage is not observed and fracture is conchoidal. The calculated density is 6.176 g cm–3. Hydroxycalciomicroliteis isotropic, ncalc. = 2.010. The infrared and Raman spectra exhibit bands due to O–H stretching vibrations. The chemical composition determined from electron microprobe analysis (n = 13) is (wt.%): Na2O 0.36(8), CaO 15.64(13), SnO2 0.26(3),Nb2O5 2.82(30), Ta2O5 78.39(22), MnO 0.12(2), F 0.72(12) and H2O 1.30 (from the crystal structure data), O = F –0.30, total 99.31(32), yielding an empirical formula, (Ca1.48Na0.06Mn0.01)∑1.55(Ta1.88Nb0.11Sn0.01)∑2.00O6.00[(OH)0.76F0.20O0.04].Hydroxycalciomicrolite is cubic, with unit-cell parameters a = 10.4205(1) Å, V = 1131.53(2) Å3 and Z = 8. It represents a pyrochlore supergroup, microlite-group mineral exhibiting P4332 symmetry, instead of Fd3m. Thereduction in symmetry is due to long-range ordering of Ca and vacancies on the A sites. This is the first example of such ordering in a natural pyrochlore, although it is known from synthetic compounds. This result is promising because it suggests that other species with P4332or lower-symmetry space group can be discovered and characterized.
Noninvasive detection of F8 int22h-related inversions and sequence variants in maternal plasma of hemophilia carriers
Authors: Hudecova Irena , Jiang Peiyong , Davies Joanna , Lo Y. M. Dennis , Kadir Rezan A. , Chiu Rossa W. K. 
Published: May 2017

Key PointsDroplet digital PCR is an affordable and user-friendly method for the detection of F8 and F9 sequence variants in maternal plasma. Targeted MPS accurately determines fetal inheritance of F8 int22h-related inversions, using maternal plasma of pregnant hemophilia carriers.
USP7 inhibition alters homologous recombination repair and targets CLL cells independently of ATM/p53 functional status
Authors: Agathanggelou Angelo , Smith Edward , Davies Nicholas J. , Kwok Marwan , Zlatanou Anastasia , Oldreive Ceri E. , Mao Jingwen , Da Costa David , Yadollahi Sina , Perry Tracey , Kearns Pamela , Skowronska Anna , Yates Elliot , Parry Helen , Hillmen Peter , Reverdy Celine , Delansorne Remi , Paneesha Shankara , Pratt Guy , Moss Paul , Taylor A. Malcolm R. , Stewart Grant S. , Stankovic Tatjana 
Published: May 2017

Key Points
USP7 is overexpressed and regulates HRR in CLL cells. USP7 inhibition is selectively cytotoxic to CLL cells independently of ATM and p53 and synergizes with chemotherapy.
A randomized trial (MA.17R) of extending adjuvant letrozole for 5 years after completing an initial 5 years of aromatase inhibitor therapy alone or preceded by tamoxifen in postmenopausal women with early-stage breast cancer.
Authors: Goss Paul E. , Ingle James N. , Pritchard Kathleen I. , Robert Nicholas J. , Muss Hyman , Gralow Julie , Gelmon Karen A. , Whelan Timothy Joseph , Strasser-Weippl Kathrin , Rubin Sheldon , Sturtz Keren , Wolff Antonio C. , Winer Eric P. , Hudis Clifford A. , Stopeck Alison , Beck J. Thaddeus , Kaur Judith Salmon , Whelan Kate , Tu Dongsheng , Parulekar Wendy R. 
Published: May 2017

LBA1 Background: Five years of aromatase inhibitor (AI) therapy either as up-front treatment or after 2-5 years of tamoxifen has become the standard of care for postmenopausal women with hormone receptor positive early breast cancer. Extending treatment with an AI to 10 years may further reduce the risk of breast cancer recurrence. Methods: We conducted a double-blind, placebo-controlled trial (Canadian Cancer Trials Group MA.17R) to test the efficacy of extending AI treatment for an additional five years using letrozole. The primary endpoint was disease-free survival. Results: A total of 1,918 women with early stage breast cancer were enrolled (median follow-up 75 months, 6.3 years). A total of 165 disease-free survival (DFS) events (67 on letrozole and 98 on placebo) occurred, of which 42 versus 53 were distant recurrences on letrozole and placebo, respectively. There were 200 deaths (100 in each treatment group). The 5 year DFS was respectively 95% for patients receiving letrozole versus 91% for those on placebo (HR 0.66; P = 0.01) from a two-sided log-rank test stratified by nodal status, prior adjuvant chemotherapy, interval between last dose of AI therapy and randomization, and duration of prior tamoxifen at randomization. The 5 year overall survival was respectively 93% for subjects on letrozole and 94% on placebo with a HR of 0.97 (P = 0.83). The annual incidence rate of contralateral breast cancer was 0.21% for subjects on letrozole versus 0.49% on placebo (P = 0.007). Conclusions: Compared to 5 years of AI treatment as initial therapy or preceded by 2-5 years of tamoxifen, extending AI treatment to 10 years significantly improves disease-free survival. Further analyses will provide a comprehensive picture of toxicities and QOL. Clinical trial information: NCT00754845.
A phase III randomized controlled trial of short-course radiotherapy with or without concomitant and adjuvant temozolomide in elderly patients with glioblastoma (CCTG CE.6, EORTC 26062-22061, TROG 08.02, NCT00482677).
Authors: Perry James R. , Laperriere Normand , O'Callaghan Christopher J. , Brandes Alba Ariela , Menten Johan , Phillips Claire , Fay Michael F. , Nishikawa Ryo , Cairncross J. Gregory , Roa Wilson , Osoba David , Sahgal Arjun , Hirte Hal W. , Wick Wolfgang , Laigle-Donadey Florence , Franceschi Enrico , Chinot Olivier L. , Winch Chad , Ding Keyue , Mason Warren P. 
Published: May 2017

LBA2 Background: The EORTC (26981-22981)/NCIC CTG (CE.3) RCT in newly diagnosed glioblastoma (GB) showed increased overall survival (OS) with concomitant and adjuvant temozolomide (TMZ) added to radiotherapy (RT). Pts were 18-71 (median 56) years; however, a trend of decreasing benefit from the addition of TMZ with increasing age was noted. Recent RCTs in elderly GB detected non-inferiority of 40 Gy/15 v 60 Gy/30 RT and superior survival was noted for MGMT-methylated pts treated with TMZ alone. However, whether the addition of TMZ to RT improves survival in elderly pts remained unanswered. Methods: We conducted a global randomized phase III clinical trial for patients ≥ 65 yrs with histologically confirmed newly diagnosed GB, ECOG 0-2, randomized 1:1 to receive 40Gy/15 RT v 40Gy/15 RT with 3 weeks of concomitant TMZ plus monthly adjuvant TMZ until progression or 12 cycles. Stratification was by centre, age (65-70, 71-75, or 76+), ECOG 0,1 vs 2, and biopsy vs resection. Results: 562 pts were randomized, 281 on each arm; median age 73 yrs (range 65-90), male 61%, PS 0/1 77%, resection 68%. RT+TMZ significantly improved OS over RT alone (median 9.3m v 7.6m, HR 0.67, 95%CI 0.56-0.80, p < 0.0001) and significantly improved PFS (median 5.3m v 3.9m, HR 0.50, 95%CI 0.41 – 0.60, p < 0.0001). Tissue from 462 pts was provided and adequate for MGMT analysis in 354 to date. In MGMT methylated patients (n = 165) OS for RT+TMZ v RT was 13.5 m and 7.7m respectively (HR: 0.53 (95% C.I. 0.38, 0.73, p = 0.0001). In MGMT unmethylated patients (n = 189) OS for RT + TMZ v RT was 10.0m vs 7.9m respectively (HR 0.75 (95% C.I. 0.56 – 1.01, p = 0.055). QoL analyses showed no differences in functional domains of QLQC30 and BN20 but were worse in the RT/TMZ arm for nausea, vomiting, and constipation. Systemic therapy after PD was reported in 39% on RT+TMZ v 41% on RT. Conclusions: The addition of concomitant and adjuvant TMZ to hypofractionated RT for elderly pts with GB significantly improves OS and PFS in all patients and is well tolerated. Patients with MGMT methylated tumors benefit the most from the addition of TMZ to RT where median OS is nearly doubled. Clinical trial information: NCT00482677.