bluuebunny/crossref_metadata_2025_split huggingface dataset:
Biological and clinical response to desmopressin (DDAVP) in a retrospective cohort study of children with low von Willebrand factor levels and bleeding history
Authors: Meschengieser Susana , Woods Adriana , Chuit Roberto , Turdó Karina , Blanco Alicia , Lazzari María , Sánchez-Luceros Analía 
Published: October 2010

SummaryThe diagnosis and management of von Willebrand disease (VWD) in paediatrics is challenging. Our aim was to review patient’s characteristics related to biological and clinical response to DDAVP in children with low von Willebrand factor (VWF) levels and bleeding history from a single institution. We included a retrospective cohort of 221 children (median age 11 years; 137 females): 27 type 1 (VWF levels within 15–30 IU dL-1) and 194 possible type 1 (VWF levels within 31–49 IU dL-1). The DDAVP infusion-test was performed in 214/221 children, 93.4% of whom showed good response. Patients with type 1 were at higher risk of DDAVP-test failure: 9/26 (34.6%) vs. 18/188 (9.6%) with possible type 1 (RR 3.44, 1.75–6.79; p= 0.002, Fisher’s exact test). In 68 children, the clinical response to DDAVP was evaluated 87 times: i) to stop bleeding: menorrhagia (13), mucocutaneous (12), haemarthrosis (1); and ii) to prevent surgical bleeding: adenotonsillectomy (17), major (15) and minor surgery (10); and dental procedures (19). No major adverse events or bleeding were observed. The treatment was effective with one single dose of DDAVP in almost all patients, without antifibrinolytic or local therapy, except in a girl with severe haemorrhage during menarche who required replacement therapy. In conclusion, patients with VWD type 1 were at higher risk of no response to DDAVP infusion-test. In this series, one dose of DDAVP proved effective and safe for children with VWD. Since this is a safe, effective and affordable therapy, we consider that a wider use should be promoted, especially in developing countries.
The effect of initiating combined antiretroviral therapy on endothelial cell activation and coagulation markers in South African HIV-infected individuals
Authors: van Gorp Eric C. M. , Meijers Joost C. M. , Cate Hugo ten , Jacobson Barry F. , Jong Eefje , Louw Susan 
Published: October 2010

SummaryAn increased incidence of venous thromboembolism (VTE) is observed in human immunodeficiency virus (HIV)-infected patients. Only a limited number of studies described the effect of combined antiretroviral therapy (cART) on coagulation markers. In a prospective cohort study in cART-naive South African HIV-infected individuals the effect of initiating cART on markers of endothelial cell activation, coagulation and natural anticoagulation was studied. These markers were compared to the reference ranges for an HIV-uninfected control population recruited from hospital staff. A venous ultrasound of both legs was performed to detect asymptomatic deep venous thrombosis (DVT). A total number of 123 HIV-infected participants were included. The patients were predominantly black and severely immuno-compromised. The CD4 cell count increased and the HIV viral load decreased significantly after the initiation of cART (p<0.001). The median follow-up period was 7.2 (± 1.6) months. Laboratory testing before and after initiation of cART was completed by 86 patients. Before initiating cART significantly elevated von Willebrand factor and D-dimer levels, increased activated protein C sensitivity ratio (APCsr) and decreased total and free protein S and protein C levels were observed compared to HIV-negative controls. At follow-up all markers, except APCsr, improved towards the normal range for controls without showing complete normalisation. In a subgroup of 57 patients no asymptomatic DVT was found. Compared to the controls, abnormal levels of coagulation markers were observed in HIV-infected individuals before and after the initiation of cART. Most markers improved after starting cART, but remained significantly different from the controls, indicating a persistent disturbed haemostatic balance.
Safety of thrombolysis in cerebral venous thrombosis
Authors: Squizzato Alessandro , Gianni Monica , De Lodovici Maria , Venco Achille , Paciaroni Maurizio , Crowther Mark , Ageno Walter , Dentali Francesco 
Published: October 2010

SummarySeveral small series have suggested the efficacy of thrombolysis in patients with cerebral vein thrombosis (CVT). However, since no randomised controlled trials have compared the use of thrombolysis with anticoagulant treatment in these patients, the risk to benefit ratio of this approach remains uncertain. The aim of this study is therefore to assess the safety of thrombolysis in CVT estimating mortality and major bleeding complications. MEDLINE and EMBASE databases were searched up to June 2010. Two reviewers performed study selection independently. Studies providing data on mortality and/or on the incidence of major bleeding complications were potentially eligible for the study. Two reviewers independently extracted data on study and population characteristics, type, dose and administration route of thrombolytic treatment; use and dose of concomitant heparin. Weighted mean proportion of the mortality rate and of the rate of major and non-major bleeding complications were calculated. Fifteen studies for a total of 156 patients were included. Twelve patients died after thrombolysis (weighted mean 9.2%; 95% CI 4.3, 15.7%) and 15 patients had a major bleeding complication (weighted mean 9.8%; 95% CI 5.3, 15.6%). Twelve haemorrhages were intracranial (weighted mean 7.6%; 95% CI 3.5, 13.1%), and seven of these patients died (58.3%; 95% CI 32.0, 80.7%). Our results suggest that thrombolysis is associated with a nonnegligible incidence of major bleeding complications, including intracranial bleeding potentially affecting patients outcome. Future studies are necessary to evaluate the safety of thrombolysis in comparison to more conservative strategies.
Inconsistencies in the planning of the duration of anticoagulation among outpatients with acute deep-vein thrombosis
Authors: Willenberg Torsten , Banyai Martin , Frank Ulrich , Baldi Thomas , Amann-Vesti Beatrice , Baumgartner Iris , Spirk David , Husmann Marc , Kucher Nils 
Published: October 2010

SummaryThree-month anticoagulation is recommended to treat provoked or first distal deep-vein thrombosis (DVT), and indefinite-duration anticoagulation should be considered for patients with unprovoked proximal, un-provoked recurrent, or cancer-associated DVT. In the prospective Out-patient Treatment of Deep Vein Thrombosis in Switzerland (OTIS-DVT) Registry of 502 patients with acute objectively confirmed lower extremity DVT (59% provoked or first distal DVT; 41% unprovoked proximal, unprovoked recurrent, or cancer-associated DVT) from 53 private practices and 11 hospitals, we investigated the planned duration of anticoagulation at the time of treatment initiation. The decision to administer limited-duration anticoagulation therapy was made in 343 (68%) patients with a median duration of 107 (interquartile range 91–182) days for provoked or first distal DVT, and 182 (interquartile range 111–184) days for unprovoked proximal, unprovoked recurrent, or cancer-associated DVT. Among patients with provoked or first distal DVT, anticoagulation was recommended for <3 months in 11%, ≥3 months in 63%, and for an indefinite period in 26%. Among patients with unprovoked proximal, unprovoked recurrent, or cancer-associated DVT, anticoagulation was recommended for <6 months in 22%, 6–12 months in 38%, and for an indefinite period in 40%. Overall, there was more frequent planning of indefinite-duration therapy from hospital physicians as compared with private practice physicians (39% vs. 28%; p=0.019). Considerable inconsistency in planning the duration of anticoagulation therapy mandates an improvement in risk stratification of outpatients with acute DVT.
Characterization of Degenerative Mitral Valve Disease Using Morphologic Analysis of Real-Time Three-Dimensional Echocardiographic Images
Authors: Chandra Sonal , Salgo Ivan S. , Sugeng Lissa , Weinert Lynn , Tsang Wendy , Takeuchi Masaaki , Spencer Kirk T. , O'Connor Anne , Cardinale Michael , Settlemier Scott , Mor-Avi Victor , Lang Roberto M. 
Published: October 2010

Background—
Presurgical planning of mitral valve (MV) repair in patients with Barlow disease (BD) and fibroelastic deficiency (FED) is challenging because of the inability to assess accurately the complexity of MV prolapse. We hypothesized that the etiology of degenerative MV disease (DMVD) could be objectively and accurately ascertained using parameters of MV geometry obtained by morphological analysis of real-time 3D echocardiographic (RT3DE) images.

Methods and Results—

Seventy-seven patients underwent transesophageal RT3DE study: 57 patients with DMVD studied intraoperatively (28 BD, 29 FED classified during surgery) and 20 patients with normal MV who were used as control subjects (NL). MVQ software (Philips) was used to measure parameters of annular dimensions and geometry and leaflet surface area, including billowing volume and height. The Student
t
test and multinomial logistic regression was performed to identify parameters best differentiating DMVD patients from normal as well as FED from BD. Morphological analysis in the DMVD group revealed a progressive increase in multiple parameters from NL to FED to BD, allowing for accurate diagnosis of these entities. The strongest predictors of the presence of DMVD included billowing height and volume. Three-dimensional billowing height with a cutoff value of 1.0 mm differentiated DMVD from NL without overlap, and billowing volume with a cutoff value 1.15 mL differentiated between FED and BD without overlap.

Conclusions—
Morphological analysis as a form of decision support in assessing MV billowing revealed significant quantifiable differences between NL, FED, and BD patients, allowing accurate classification of the etiology of MV prolapse and determination of the anticipated complexity of repair.
Prognostic Value of Coronary Computed Tomographic Angiography in Comparison With Calcium Scoring and Clinical Risk Scores
Authors: Hadamitzky Martin , Distler Roland , Meyer Tanja , Hein Franziska , Kastrati Adnan , Martinoff Stefan , Schömig Albert , Hausleiter Jörg 
Published: October 2010

Background—
Several studies have demonstrated a high accuracy of coronary computed tomography angiography (CCTA) for detection of obstructive coronary artery disease (CAD), whereas some studies have also shown a good prediction of cardiac events. However, it remains to be proven whether CCTA is better predictive of events than conventional risk scores or calcium scoring. Therefore, we compared CCTA with calcium scoring and clinical risk scores for the ability to predict cardiac events.

Methods and Results—

Patients (n=2223) with suspected CAD undergoing CCTA were followed up for a median of 28 months. The end point was the occurrence of cardiac events (cardiac death, nonfatal myocardial infarction, unstable angina requiring hospitalization, and coronary revascularization later than 90 days after CCTA). Patients with obstructive CAD had a significantly higher event rate (2.9% per year; 95% confidence interval, 2.1 to 4.0) than those without obstructive CAD, having an event rate 0.3% per year (95% confidence interval, 0.1 to 0.5; hazard ratio, 13.5; 95% confidence interval, 6.7 to 27.2;
P
<0.001). CCTA had significant incremental predictive value when compared with calcium scoring, both with scores assessing the degree of stenosis (
P
<0.001) and with scores assessing the number of diseased coronary segments (
P
=0.027).

Conclusions—
In patients with suspected CAD, CCTA not only detects coronary stenosis but also improves prediction of cardiac events over and above conventional risk scores and calcium scoring. This may result in a reclassification of cardiovascular risk in a substantial proportion of patients.
Signatures of Nonlinear Dynamics in an Idealized Atmospheric Model
Authors: Kondrashov Dmitri , Kravtsov Sergey , Ghil Michael 
Published: October 2010

Abstract
Signatures of nonlinear dynamics are analyzed by studying the phase-space tendencies of a global baroclinic, quasigeostrophic, three-level (QG3) model with topography. Nonlinear, stochastic, low-order prototypes of the full QG3 model are constructed in the phase space of this model’s empirical orthogonal functions using the empirical model reduction (EMR) approach. The phase-space tendencies of the EMR models closely match the full QG3 model’s tendencies. The component of these tendencies that is not linearly parameterizable is shown to be dominated by the interactions between “resolved” modes rather than by multiplicative “noise” associated with unresolved modes. The method of defining the leading resolved modes and the interactions between them plays a key role in understanding the nature of the QG3 model’s dynamics, whether linear or nonlinear, deterministic or stochastic.
Automatic Minimum and Maximum Alarm Thresholds for Quality Control
Authors: Hasu Vesa , Aaltonen Ari 
Published: October 2010

Abstract
Automated quality control is a necessity for meteorological measurement networks because of an ever-increasing number of measurements. One of the basic quality control tests is to check that the observations fall into a range of acceptable values. This paper proposes an algorithm for the automatic determination of maximum and minimum thresholds for such a test. The automated algorithm results in more statistically justified thresholds compared to the operator-fed values, which some quality control systems normally use. The suggested algorithm is a tuning-free method for the determination of daily minimum and maximum limits. The basis of the algorithm is, first, to collect the essential statistics of observations during their annual cycle and, second, to use these statistics to determine the acceptable observation range. This paper introduces a semirecursive version with lower computational requirements and discusses additional algorithmic details. The algorithm is demonstrated through numerical examples, which show that limits converge to satisfactory values in a few years.
Malignant pleural effusion cells show aberrant glucose metabolism gene expression
Authors: Lin C-C. , Chen L-C. , Tseng V.S. , Yan J-J. , Lai W-W. , Su W-P. , Lin C-H. , Huang C-Y.F. , Su W-C. 
Published: October 2010

Malignant pleural effusion (MPE) accompanying lung adenocarcinoma indicates poor prognosis and early metastasis. This study aimed to identify genes related to MPE formation.Three tissue sample cohorts, seven from healthy lungs, 18 from stage I–III lung adenocarcinoma with adjacent healthy lung tissue and 13 from lung adenocarcinomas with MPE, were analysed by oligonucleotide microarray. The identified genes were verified by quantitative real-time PCR (qRT-PCR), immunohistochemical staining, and immunofluorescence confocal microscopy.20 up- or down-regulated genes with a two-fold change in MPE cancer cells compared to healthy tissues were differentially expressed from early- to late-stage lung cancer. Of 13 genes related to cellular metabolism, aldolase A (ALDOA), sorbitol dehydrogenase (SORD), transketolase (TKT), and tuberous sclerosis 1 (TSC1) were related to glucose metabolism. qRT-PCR validated their mRNA expressions in pleural metastatic samples. Immunohistochemical staining confirmed aberrant TKT, ALDOA, and TSC1 expressions in tumour cells. Immunofluorescence confirmed TKT co-localisation and co-distribution of ALDOA with thyroid transcription factor 1-positive cancer cells. TKT regulated the proliferation, vascular endothelial growth factor secretionin vitroandin vivovascular permeability of cancer cell.Glucose metabolic reprogramming by ALDOA, SORD, TKT and TSC1 is important in MPE pathogenesis.
Role of pulmonary infection in the development of chronic lung disease of prematurity
Authors: Beeton M.L. , Maxwell N.C. , Davies P.L. , Nuttall D. , McGreal E. , Chakraborty M. , Spiller O.B. , Kotecha S. 
Published: October 2010

We studied the role of ante- and post-natal infection in the development of chronic lung disease (CLD) of prematurity.192 newborn infants (61 term and 131 pre-term of <34 weeks gestation: 88 with respiratory distress syndrome, 35 developed CLD and eight died) were recruited. 16S ribosomal RNA (rRNA) genes were identified by PCR of DNA isolated from 840 gastric and lung fluid samples.Ureaplasmaspp. were also cultured.Presence of 16S rRNA genes (OR 1.6, 95% CI 1.2–2.2) andUreaplasmaspp. (OR 3.6, 95% CI 1.7–7.7) was significantly associated with the development of CLD. This association remained if the 16S rRNA genes andUreaplasmaspp. were first identified within the first 3 days of life (OR 2.4 (95% CI 1.4–4.1) and 3.8 (95% CI 1.4–10.0), respectively) or if first identified after 3 days of age (OR 1.7 (95% CI 1.1–2.8) and OR 5.1 (95% CI 1.3–19.8), respectively). Peak lung fluid interleukin (IL)-6 and IL-8 were significantly associated with presence of microbes (p<0.0001 and p = 0.0001, respectively) and development of CLD (p = 0.003 and 0.001, respectively).Both early and late microbial presence in neonatal lung fluid samples was significantly associated with the development of CLD suggesting that both ante- and post-natal infection play a role in the development of CLD.
Discoidin domain receptor 1 regulates bronchial epithelial repair and matrix metalloproteinase production
Authors: Roberts M.E. , Magowan L. , Hall I.P. , Johnson S.R. 
Published: October 2010

Discoidin domain receptor (DDR)1 is an extracellular matrix (ECM)-sensing receptor tyrosine kinase, which is activated by collagen and expressed in bronchial epithelium. DDR1 is responsible for maintaining the normal structure of skin and kidney epithelia and we hypothesised that DDR1 plays a regulatory role in bronchial epithelial integrity by transducing signals from the airway ECM.Effects of DDR1 depletion were studied using RNA interference in primary human bronchial epithelial cells (HBECs) and BEAS-2B cells. The effects of overexpression of DDR1a and DDR1b in BEAS-2B cells were studied using a plasmid vector. We measured the effects on epithelial repair using a scratch wounding model, and levels of matrix metalloproteinases (MMPs) by gelatin zymography (MMP-2 and -9) and ELISA (MMP-7).We showed that knockdown of DDR1 slowed epithelial repair by 50%, which was associated with a reduction in levels of MMP-7, whilst DDR1 overexpression enhanced epithelial repair. DDR1 knockdown reduced proliferation of HBECs, but had no significant effect on adhesion to collagen I or other matrix substrates.These data suggest that ECM signallingviaDDR1 regulates aspects of bronchial epithelial repair, integrity and MMP expression in the airways.
Targeting of c-kit+ haematopoietic progenitor cells prevents hypoxic pulmonary hypertension
Authors: Gambaryan N. , Perros F. , Montani D. , Cohen-Kaminsky S. , Mazmanian M. , Renaud J-F. , Simonneau G. , Lombet A. , Humbert M. 
Published: October 2010

Haematopoietic c-kit+ progenitor cells may contribute to pulmonary vascular remodelling and pulmonary hypertension (PH). Stromal derived factor-1 (SDF-1/CXCL12) and its receptors CXCR4 and CXCR7 have been shown to be critical for homing and mobilisation of haematopoietic c-kit+ progenitor cells in the perivascular niche.We administered AMD3100, a CXCR4 antagonist, and CCX771, a CXCR7 antagonist, to chronic hypoxia exposed mice in order to study the role of c-kit+ progenitor cells in PH. CXCL12, CXCR4 and CXCR7 protein expression, haemodynamic parameters, right ventricular mass, extent of vascular remodelling and perivascular progenitor cell accumulation were studied.Chronic hypoxia-exposed mice showed increased total lung tissue expression of CXCR4, CXCR7 and CXCL12 after development of PH. This was associated with significantly increased right ventricular systolic pressure and evidence of right ventricular hypertrophy, vascular remodelling and perivascular c-kit+/sca-1+ progenitor cell accumulation. CCX771 administration did not abrogate these effects. In contrast, administration of AMD3100, whether alone or combined with CCX771, prevented vascular remodelling, PH and perivascular accumulation of c-kit+/sca-1+ progenitor cells, with a synergistic effect of these agents.This study offers important pathophysiological insights into the role of haematopoietic c-kit+ progenitors in hypoxia-induced vascular remodelling and may have therapeutic implications for PH.
Iron deficiency is common in idiopathic pulmonary arterial hypertension
Authors: Ruiter G. , Lankhorst S. , Boonstra A. , Postmus P.E. , Zweegman S. , Westerhof N. , van der Laarse W.J. , Vonk-Noordegraaf A. 
Published: October 2010

The aims of this study were to assess the prevalence of iron deficiency in idiopathic pulmonary arterial hypertension (IPAH) and investigate whether oral iron supplementation has effects in iron-deficient patients.Iron parameters were measure for all IPAH patients attending our centre (VU University Medical Center, Amsterdam, the Netherlands) between May 2009 and February 2010. Iron data were related to clinical parameters, including 6-min walking distance (6MWD), and haemodynamic parameters measured during right heart catheterisation. In a subset of iron-deficient patients, the uptake of iron from the bowel was studied after administering oral iron for 4 weeks.Iron deficiency was found in 30 (43%) out of 70 patients. 6MWD was reduced in iron-deficient patients compared with iron-sufficient patients (mean±sd390±138versus460±143 m; p<0.05) irrespective of the existence of anaemia. In a subset of 18 patients that received oral iron, ferritin levels were significantly increased, although eight patients only slightly increased their iron storage.This study shows that iron deficiency is frequently present in IPAH and is associated with a lower exercise capacity. The small response to oral iron in 44% of the treated patients suggests impaired iron absorption in these patients.
Tuberculosis contact investigation in low prevalence countries: a European consensus
Authors: Erkens C.G.M. , Kamphorst M. , Abubakar I. , Bothamley G.H. , Chemtob D. , Haas W. , Migliori G.B. , Rieder H.L. , Zellweger J-P. , Lange C. 
Published: October 2010

Contact investigation to identify individuals with tuberculosis and latent infection withMycobacterium tuberculosisis an important component of tuberculosis control in low tuberculosis incidence countries. This document provides evidence-based and best-practice policy recommendations for contact tracing among high- and medium-priority contacts in a variety of settings. It provides a basis for national guidelines on contact investigation and tuberculosis outbreak management, and should support countries and tuberculosis control managers in evaluating and revising national policies. A review of existing guidelines, a literature search, several meetings and consultation with experts were used to formulate and grade recommendations for action during contact investigation.Available tests to identify individuals with latent infection withM. tuberculosisare designed to identify immune response against mycobacterial antigens and have variable predictive value for the likelihood to develop active tuberculosis in different populations. Contact investigation should therefore be limited to situations with a clear likelihood of transmission or to those with a higher probability of developing active tuberculosis, for instance, young children and immunocompromised persons. A risk assessment-based approach is recommended, where the need to screen contacts is prioritised on the basis of the infectiousness of the index case, intensity of exposure and susceptibility of contacts.