bluuebunny/crossref_metadata_2025_split huggingface dataset:
Cannulated Screw Fixation of Refractory Olecranon Stress Fractures With and Without Associated Injuries Allows a Return to Baseball
Authors: Paci James M. , Dugas Jeffrey R. , Guy Jeffrey A. , Cain E. Lyle , Fleisig Glenn S. , Hurst Candice , Wilk Kevin E. , Andrews James R. 
Published: December 2012

Background: An olecranon stress fracture is a rare injury associated with valgus extension overload in baseball players. No long-term outcomes studies have been published documenting the results of surgical fixation of olecranon stress fractures with or without concomitant injuries in baseball players. Hypothesis: Open reduction and internal fixation (ORIF) of an olecranon stress fracture will reliably produce bony union and allow a successful return to the previous level of activity in competitive baseball players. Study Design: Case series; Level of evidence, 4. Methods: Twenty-five patients treated with ORIF for an olecranon stress fracture at least 2 years earlier (range, 2-10.14 years) were retrospectively contacted to complete a telephone survey; 18 of 25 (72%) patients responded. Data were collected to determine the return to play rate, level of arm pain, and overall arm function. Results: All 18 stress fractures went on to successful union; 17 of 18 (94%) athletes returned to baseball at or above their previous level. Average return to play time was 29 weeks. The numeric analog pain score was 0.2 at rest and 0.3 when throwing at the time of follow-up, at an average 6.2 years (range, 2.0-10.14 years) after surgery. The average score at follow-up on the Disabilities of the Arm, Shoulder and Hand Outcome Measure–shortened version ( QuickDASH) was 4.1 (range, 0-27.3). Ten (56%) patients required 13 additional future surgeries on their throwing arm; 7 surgeries in 6 (33%) patients were not related to the index surgery. Six of 18 (33%) patients underwent hardware removal, with 2 (11%) for infection. Conclusion: Open reduction and internal fixation of olecranon stress fractures in competitive baseball players has a high rate of success in returning players to or above their former level of play and allows for good elbow function at an average of 6.2 years postoperatively. However, these patients are at high risk for additional future surgeries on their throwing arm.
Filicide and suicide in a family by paraphenylene diamine poisoning
Authors: Abdelraheem Mohamed Babikir , Elbushra Mohamed , Ali El-Tigani , Ellidir Rashid A , Bushara Amna I , Abdelraheem Waleed B , Zijlstra Eduard E 
Published: December 2012

Paraphenylene diamine (PPD) hair dye poisoning is a common health problem in the Middle East and Indian subcontinent. It is the most common cause of suicide intent especially among females. We hereby present a 27-year-old female who presented with a clinical feature of PPD poisoning due to a suicidal attempt, and she died soon after arrival. She had survived a previous suicide attempt with PPD 4 years before. This time she also intentionally tried to kill her four children using PPD. One child died, one recovered after dialysis for acute kidney injury and the other two survived without any further intervention. This case illustrates that PPD poisoning may be used in filicide and suicide intent and that all patients who committed suicide should be reviewed and assessed by clinical psychiatrist.
Protective effects of vitamin C and selenium supplementation on methomyl-induced tissue oxidative stress in adult rats
Authors: Djeffal Assia , Messarah Mahfoud , Boumendjel Amel , Kadeche Lilia , Feki Abdelfattah El 
Published: December 2012

Methomyl (MET) is used worldwide in agriculture and health programs. Besides its advantages in the agriculture, it causes several toxic effects. The objective of this study was to examine the potential ability of vitamin C and/or selenium (Se), to alleviate the oxidative damage parameters, against MET-induced changes in blood biochemical markers and oxidative damage in liver and kidney of male Wistar rats. The animals were randomized into five groups of eight each: group I served as control rats; group II received MET (8 mg/kg body weight (BW)) in drinking water; group III received both MET and vitamin C (200 mg/kg BW; by intraperitoneal injection); group IV received both MET and Se (0.6 mg/100 g BW). Animals of group V were treated with MET, vitamin C and Se. A significant increase in the levels of hepatic markers enzymes (alanine aminotransferase, aspartate aminotransferase, alkaline phosphatase and lactate dehydrogenase) was determined. Furthermore, renal markers such as urea and creatinine were increased in MET-treated rats. Additionally, serum cholesterol and triglycerides were significantly enhanced. Exposure of rats to MET caused significant increase in malondialdehyde levels, thus causing a drastic alteration in antioxidant defense system, particularly in the activities of catalase and glutathione- S-transferase and glutathione peroxidase. However, simultaneous supplementation with vitamin C and Se restored these parameters partially. In conclusion, the results of the current study revealed that MET-induced toxicity caused perturbations of some biochemical parameters, lipid peroxidation and alterations in the antioxidant enzymes in liver and kidney homogenates. Administration of vitamin C and Se exhibited protective effect by inhibiting MET-induced toxicity in liver and kidney.
The polygenic nature of inhibitors in hemophilia A: results from the Hemophilia Inhibitor Genetics Study (HIGS) Combined Cohort
Authors: Astermark Jan , Donfield Sharyne M. , Gomperts Edward D. , Schwarz John , Menius Erika D. , Pavlova Anna , Oldenburg Johannes , Kessing Bailey , DiMichele Donna M. , Shapiro Amy D. , Winkler Cheryl A. , Berntorp Erik 
Published: December 2012

Key Points
The data demonstrate the complexity of the genetic contribution to inhibitor development in people with hemophilia A. Potentially decisive markers have been identified, indicating the importance of further evaluation of intracellular signaling pathways.
Simvastatin and t-butylhydroquinone suppress KLF1 and BCL11A gene expression and additively increase fetal hemoglobin in primary human erythroid cells
Authors: Macari Elizabeth R. , Schaeffer Emily K. , West Rachel J. , Lowrey Christopher H. 
Published: December 2012

Key PointsSimvastatin and tBHQ suppress KLF1 and BCL11 gene expression and additively increase fetal hemoglobin in primary human erythroid cells. Because both drugs are FDA-approved, these findings could lead to clinical trials in the relatively near future.
Epigenetic silencing of microRNA-193a contributes to leukemogenesis in t(8;21) acute myeloid leukemia by activating the PTEN/PI3K signal pathway
Authors: Li Yonghui , Gao Li , Luo Xufeng , Wang Lili , Gao Xiaoning , Wang Wei , Sun Junzhong , Dou Liping , Li Jingxin , Xu Chengwang , Wang Lixin , Zhou Minhang , Jiang Mengmeng , Zhou Jihao , Caligiuri Michael A. , Nervi Clara , Bloomfield Clara D. , Marcucci Guido , Yu Li 
Published: December 2012

Abstract
t(8;21) is one of the most frequent chromosomal translocations occurring in acute myeloid leukemia (AML) and is considered the leukemia-initiating event. The biologic and clinical significance of microRNA dysregulation associated with AML1/ETO expressed in t(8;21) AML is unknown. Here, we show that AML1/ETO triggers the heterochromatic silencing of microRNA-193a (miR-193a) by binding at AML1-binding sites and recruiting chromatin-remodeling enzymes. Suppression of miR-193a expands the oncogenic activity of the fusion protein AML-ETO, because miR-193a represses the expression of multiple target genes, such as AML1/ETO, DNMT3a, HDAC3, KIT, CCND1, and MDM2 directly, and increases PTEN indirectly. Enhanced miR-193a levels induce G1 arrest, apoptosis, and restore leukemic cell differentiation. Our study identifies miR-193a and PTEN as targets for AML1/ETO and provides evidence that links the epigenetic silencing of tumor suppressor genes miR-193a and PTEN to differentiation block of myeloid precursors. Our results indicated a feedback circuitry involving miR-193a and AML1/ETO/DNMTs/HDACs, cooperating with the PTEN/PI3K signaling pathway and contributing to leukemogenesis in vitro and in vivo, which can be successfully targeted by pharmacologic disruption of the AML1/ETO/DNMTs/HDACs complex or enhancement of miR-193a in t(8;21)–leukemias.
High frequency of GATA2 mutations in patients with mild chronic neutropenia evolving to MonoMac syndrome, myelodysplasia, and acute myeloid leukemia
Authors: Pasquet Marlène , Bellanné-Chantelot Christine , Tavitian Suzanne , Prade Naïs , Beaupain Blandine , LaRochelle Olivier , Petit Arnaud , Rohrlich Pierre , Ferrand Christophe , Van Den Neste Eric , Poirel Hélène A. , Lamy Thierry , Ouachée-Chardin Marie , Mansat-De Mas Véronique , Corre Jill , Récher Christian , Plat Geneviève , Bachelerie Françoise , Donadieu Jean , Delabesse Eric 
Published: December 2012

Key Points
Mutations of key transcription factor in myeloid malignancies.
An RNAi therapeutic targeting Tmprss6 decreases iron overload in Hfe−/− mice and ameliorates anemia and iron overload in murine β-thalassemia intermedia
Authors: Schmidt Paul J. , Toudjarska Iva , Sendamarai Anoop K. , Racie Tim , Milstein Stuart , Bettencourt Brian R. , Hettinger Julia , Bumcrot David , Fleming Mark D. 
Published: December 2012

Key PointsTmprss6 siRNA induces hepcidin and diminishes iron in hemochromatosis or thalassemia mice, improving the anemia seen in the latter model. Manipulation of TMPRSS6 with RNAi therapeutics may be an approach to treating iron overload diseases associated with low hepcidin levels.
Human iNKT and MAIT cells exhibit a PLZF-dependent proapoptotic propensity that is counterbalanced by XIAP
Authors: Gérart Stéphane , Sibéril Sophie , Martin Emmanuel , Lenoir Christelle , Aguilar Claire , Picard Capucine , Lantz Olivier , Fischer Alain , Latour Sylvain 
Published: December 2012

Abstract
Invariant natural killer (iNKT) T cells and mucosal-associated invariant T (MAIT) cells represent peculiar T-lymphocyte subpopulations with innate-like properties that differ from conventional T cells. iNKT are reduced in the primary immunodeficiency caused by mutations in the X-linked inhibitor of apoptosis (XIAP). By studying the mechanism of this depletion, we herein report that iNKT cells exhibit a high susceptibility to apoptosis that is not observed with conventional T cells. Elevated expression of caspases 3 and 7 accounts for the proapoptotic phenotype of iNKT cells, which is inhibited by XIAP although it exerts a moderate effect in conventional T cells. Similarly, MAIT cells exhibit a proapoptotic propensity with elevated expression of activated caspases and are decreased in XIAP-deficient individuals. Knockdown of the transcription factor PLZF/ZBTB-16, which is involved in the effector program of iNKT cells, diminishes their proapoptotic phenotype. Conversely, overexpression of PLZF/ZBTB-16 in conventional T cells leads to a proapoptotic phenotype. Our findings identify a previously unknown pathway of regulation of innate-like T-cell homeostasis depending on XIAP and PLZF. The proapoptotic feature of iNKT cells also gives a reliable explanation of their exhaustion observed in different human conditions including the XIAP immunodeficiency.
Transcriptome analysis reveals upregulation of bitter taste receptors in severe asthmatics
Authors: Orsmark-Pietras Christina , James Anna , Konradsen Jon R. , Nordlund Björn , Söderhäll Cilla , Pulkkinen Ville , Pedroletti Christophe , Daham Kameran , Kupczyk Maciek , Dahlén Barbro , Kere Juha , Dahlén Sven-Erik , Hedlin Gunilla , Melén Erik 
Published: December 2012

The causes of severe childhood asthma are poorly understood. Our aim was to define global patterns of gene expression in children with severe therapy-resistant and controlled asthma.White blood cells were isolated and the global transcriptome profile was characterised using the Affymetrix Human Gene ST 1.0 chip in children with severe, therapy-resistant asthma (n=17), controlled asthma (n=19) and healthy controls (n=18). Receptor expression was studied in separated leukocyte fractions from asthmatic adults (n=12).Overall, 1378 genes were differentially expressed between children with severe/controlled asthma and controls. Three significantly enriched Kyoto Encyclopedia of Genes and Genomes pathways were represented: natural killer cell-mediated cytotoxicity (upregulated in controlled asthma); N-glycan biosynthesis (downregulated in severe asthma); and bitter taste transduction receptors (TAS2Rs) (upregulated in severe asthma). Quantitative PCR experiments confirmed upregulation ofTAS2Rs in severe asthmatics.TAS2Rexpression was replicated in leukocytes from adult asthmatics, in whichTAS2Ragonists also inhibited LPS-induced cytokine release. Significant correlations between expression ofTAS2Rs and clinical markers of asthma severity were found in both adults and children.In conclusion, specific gene expression patterns were observed in children with severe, therapy-resistant asthma. The increased expression of bronchodilatoryTAS2Rs suggests a new target for the treatment of asthma.
Scoring systems using chest radiographic features for the diagnosis of pulmonary tuberculosis in adults: a systematic review
Authors: Pinto Lancelot M. , Pai Madhukar , Dheda Keertan , Schwartzman Kevin , Menzies Dick , Steingart Karen R. 
Published: December 2012

Chest radiography for the diagnosis of active pulmonary tuberculosis (PTB) is limited by poor specificity and reader inconsistency. Scoring systems have been employed successfully for improving the performance of chest radiography for various pulmonary diseases. We conducted a systematic review to assess the diagnostic accuracy and reproducibility of scoring systems for PTB.We searched multiple databases for studies that evaluated the accuracy and reproducibility of chest radiograph scoring systems for PTB. We summarised results for specific radiographic features and scoring systems associated with PTB. Where appropriate, we estimated pooled performance of similar studies using a random effects model.13 studies were included in the review, nine of which were in low tuberculosis (TB) burden settings. No scoring system was based solely on radiographic findings. All studies used systems with various combinations of clinical and radiological features. 11 studies involved scoring systems that were used for making decisions concerning hospital respiratory isolation. None of the included studies reported data on intra- or inter-reporter reproducibility. Upper lobe infiltrates (pooled diagnostic OR 3.57, 95% CI 2.38–5.37, five studies) and cavities (diagnostic OR range 1.97–25.66, three studies) were significantly associated with PTB. Sensitivities of the scoring systems were high (median 96%, IQR 93–98%), but specificities were low (median 46%, IQR 35–50%).Chest radiograph scoring systems appear useful in ruling out PTB in hospitals, but their low specificity precludes ruling in PTB. There is a need to develop accurate scoring systems for people living with HIV and for outpatient settings, especially in high TB burden settings.
Obesity is associated with improved survival in community-acquired pneumonia
Authors: Singanayagam Anika , Singanayagam Aran , Chalmers James D. 
Published: December 2012

Obesity has been identified as a risk factor for adverse outcomes of 2009 H1N1 influenza. However, the impact of obesity on outcomes of infection remains controversial. There are limited data investigating the effect of obesity on outcomes of community-acquired pneumonia (CAP).This prospective observational study included patients presenting with CAP who had body mass index (BMI) measured on admission. Outcome measures included 30-day mortality and need for mechanical ventilation or inotropic support (MV/IS).1079 patients were included, with 21% classified as obese (BMI ≥30 kg·m−2). Obesity was independently associated with reduced 30-day mortality from CAP on multivariate analysis (HR 0.53, 95% CI 0.29–0.98). This was not explained by differences in severity of disease on admission or requirement for MV/IS between obese and nonobese groups. Obese patients had higher median C-reactive protein levels and a higher frequency of sepsis using the systemic inflammatory response syndrome criteria (72.4%versus64.1%; p=0.03), than nonobese patients, suggesting greater systemic inflammation.Obesity was associated with reduced 30-day mortality in patients hospitalised with CAP.
Multicomponent indices to predict survival in COPD: the COCOMICS study
Authors: Marin Jose M. , Alfageme Inmaculada , Almagro Pere , Casanova Ciro , Esteban Cristobal , Soler-Cataluña Juan J. , de Torres Juan P. , Martínez-Camblor Pablo , Miravitlles Marc , Celli Bartolome R. , Soriano Joan B. 
Published: December 2012

Guidelines recommend defining chronic obstructive pulmonary disease (COPD) by airflow obstruction and other factors, but no studies have evaluated the ability of existing multicomponent indices to predict mortality up to 10 years.We conducted a patient-based pooled analysis. Survival analysis and C statistics were used to determine the best COPD index/indices according to several construct variables and by varying time-points. Individual data of 3633 patients from 11 COPD cohorts were collected, totalling the experience of 15 878 person-years.Overall, there were 1245 death events within our cohorts, with a Kaplan–Meier survival of 0.963 at 6 months, which was reduced to 0.432 at 10 years. In all patients, ADO (age, dyspnoea and forced expiratory volume in 1 s), BODE (body mass index, airflow obstruction, dyspnoea and exercise capacity) and e-BODE (BODE plus exacerbations) were the best indices to predict 6-month mortality. The ADO index was the best to predict 12-month (C statistic 0.702), 5-year (C statistic 0.695) and 10-year mortality (C statistic 0.698), and was significantly better than BODE (all p<0.05). The best indices to predict death by C statistics when adjusting by age were e-BODE, BODEx (substitution of exacerbations for exercise capacity) and BODE.No index predicts short-term survival of COPD well. All BODE modifications scored better than ADO after age adjustment. The ADO and BODE indices are overall the most valid multicomponent indices to predict time to death in all COPD patients.
Prevalence and prognosis of unclassifiable interstitial lung disease
Authors: Ryerson Christopher J. , Urbania Thomas H. , Richeldi Luca , Mooney Joshua J. , Lee Joyce S. , Jones Kirk D. , Elicker Brett M. , Koth Laura L. , King Talmadge E. , Wolters Paul J. , Collard Harold R. 
Published: December 2012

The aim of this study was to determine the prevalence, characteristics and outcomes of patients with unclassifiable interstitial lung disease (ILD) and to develop a simple method of predicting disease behaviour.Unclassifiable ILD patients were identified from an ongoing longitudinal cohort. Unclassifiable ILD was diagnosed after a multidisciplinary review did not secure a specific ILD diagnosis. Clinical characteristics and outcomes were compared with idiopathic pulmonary fibrosis (IPF) and non-IPF ILDs. Independent predictors of mortality were determined using Cox proportional-hazards analysis to identify subgroups with distinct disease behaviour.Unclassifiable ILD was diagnosed in 10% of the ILD cohort (132 out of 1370 patients). The most common reason for being unclassifiable was missing histopathological assessment due to a high risk of surgical lung biopsy. Demographic and physiological features of unclassifiable ILD were intermediate between IPF and non-IPF disease controls. Unclassifiable ILD had longer survival rates when compared to IPF on adjusted analysis (hazard ratio 0.62, p = 0.04) and similar survival compared to non-IPF ILDs (hazard ratio 1.54, p = 0.12). Independent predictors of survival in unclassifiable ILD included diffusion capacity of the lung for carbon monoxide (p = 0.001) and a radiological fibrosis score (p = 0.02).Unclassifiable ILD represents approximately 10% of ILD cases and has a heterogeneous clinical course, which can be predicted using clinical and radiological variables.
Pulsus paradoxus
Authors: Hamzaoui Olfa , Monnet Xavier , Teboul Jean-Louis 
Published: December 2012

Systolic blood pressure normally falls during quiet inspiration in normal individuals. Pulsus paradoxus is defined as a fall of systolic blood pressure of >10 mmHg during the inspiratory phase. Pulsus paradoxus can be observed in cardiac tamponade and in conditions where intrathoracic pressure swings are exaggerated or the right ventricle is distended, such as severe acute asthma or exacerbations of chronic obstructive pulmonary disease. Both the inspiratory decrease in left ventricular stroke volume and the passive transmission to the arterial tree of the inspiratory decrease in intrathoracic pressure contribute to the occurrence of pulsus paradoxus. During cardiac tamponade and acute asthma, biventricular interdependence (series and parallel) plays an important role in the inspiratory decrease in left ventricular stroke volume.Early recognition of pulsus paradoxus in the emergency room can help to diagnose rapidly cardiac tamponade. Measurement of pulsus paradoxus is also useful to assess the severity of acute asthma as well as its response to therapy. Recent development of noninvasive devices capable of automatic calculation and display of arterial pressure variation or derived indices should help improve the assessment of pulsus paradoxus at the bedside.
Reliability and validity of 4-metre gait speed in COPD
Authors: Kon Samantha S.C. , Patel Mehul S. , Canavan Jane L. , Clark Amy L. , Jones Sarah E. , Nolan Claire M. , Cullinan Paul , Polkey Michael I. , Man William D-C. 
Published: December 2012

In community-dwelling older adults, usual gait speed over 4 m (4MGS) consistently predicts greater risk of adverse health outcomes. The aims of the present study were to assess the reliability of the 4MGS and the relationship with established health outcome measures in chronic obstructive pulmonary disease (COPD).Test-retest and interobserver reliability of the 4MGS were measured in 80 and 58 COPD patients, respectively. In 586 COPD patients, the 4MGS, as well as forced expiratory volume in 1 s (FEV1), the incremental shuttle walk (ISW), Medical Research Council (MRC) dyspnoea scale and St George’s Respiratory Questionnaire (SGRQ) were measured. Participants were stratified according to “slow” (<0.8 m·s−1) or “normal” 4MGS (≥0.8 m·s−1).Intra-class correlation coefficients for test-retest and interobserver reliability were 0.97 and 0.99, respectively. There was a significant positive correlation between 4MGS with ISW (ρ = 0.78; p<0.001) and a negative correlation with MRC dyspnoea scale and SGRQ (ρ = -0.55 and -0.44; p<0.001 for both). COPD patients with slow 4MGS had significantly reduced ISW and higher MRC dyspnoea scale and SGRQ than those with preserved walking speed, despite similar FEV1% predicted.The 4MGS is reliable in COPD, correlates with exercise capacity, dyspnoea and health-related quality of life, and has potential as a simple assessment tool in COPD.