bluuebunny/crossref_metadata_2025_split huggingface dataset:
SOX‐10 expression in cutaneous myoepitheliomas and mixed tumors
Authors: Naujokas Agne , Charli‐Joseph Yann , Ruben Beth S. , Yeh Iwei , LeBoit Philip E. , McCalmont Timothy H. , Pincus Laura B. 
Published: December 2013

AbstractBackgroundSOX‐10 expression can be demonstrated by immunohistochemistry in salivary gland myoepitheliomas, but its expression in cutaneous myoepitheliomas and in cutaneous mixed tumors with prominent myoepithelial cells has not been studied.MethodsWe assessed the staining pattern of SOX‐10 in five cutaneous myoepitheliomas and six cutaneous mixed tumors with a prominent myoepithelial component among both the myoepithelial cells and cells lining lumens. In addition, we examined the staining of S100, microphthalmia‐associated transcription factor (MiTF), keratin cocktail, HMK903, smooth muscle actin (SMA) and epithelial membrane antigen (EMA).ResultsSOX‐10 positivity was seen in three of five (60%) cutaneous myoepitheliomas and in the myoepithelial cells of all cutaneous mixed tumors. SOX‐10 expression on the cells lining the glandular structures in mixed tumors was variable. All myoepitheliomas and mixed tumors stained positively with S100 and negatively with MiTF. Pan‐keratin, HMK903, SMA and EMA showed variable expression.ConclusionsSOX‐10 is a relatively reliable marker for staining cutaneous myoepitheliomas. Cutaneous myoepitheliomas are notoriously difficult to diagnose, and the addition of SOX‐10 to the repertoire of stains that can label this tumor is of practical utility. These results further support that cutaneous myoepitheliomas and cutaneous mixed tumors exist on a morphologic and immunophenotypic spectrum.
Costs and benefits linked to developments in cognitive control
Authors: Blackwell Katharine A. , Munakata Yuko 
Published: December 2013

AbstractDeveloping cognitive control over one's thoughts, emotions, and actions is a fundamental process that predicts important life outcomes. Such control begins in infancy, and shifts during development from a predominantly reactive form (e.g. retrieving task‐relevant information when needed) to an increasingly proactive form (e.g. maintaining task‐relevant information in anticipation of needing it). While such developments are generally viewed as adaptive, cognitive abilities can also involve trade‐offs, such that the benefits of developing increasingly proactive control may come with associated costs. In two experiments, we test for such cognitive trade‐offs in children who are transitioning to proactive control. We find that proactive control predicts expected benefits in children's working memory, but is also associated with predicted costs in disproportionately slowing children under conditions of distraction. These findings highlight unique advantages and disadvantages of proactive and reactive control, and suggest caution in attempting to alter their balance during development.
Prosthesis Migration after Percutaneous Aortic Valve Implantation
Authors: Sozzi Fabiola B. , Eker Armand , Bourlon Francois , Habib Yacoub , Civaia Filippo , Alexandrescu Clara , Dreyfus Gilles , Dor Vincent 
Published: December 2013

Mini‐AbstractA 91‐year‐old man with severe symptomatic aortic stenosis underwent a transfemoral aortic valve (Edwards Sapien XT23) implantation. Migration of the prosthesis into the left ventricular outflow tract occurred a week after. An urgent successful subsequent open heart surgery for device retrieval and prosthesis replacement was performed.
Psychometric validation of the Spanish version of the Dundee Ready Education Environment Measure applied to dental students
Authors: Tomás I. , Casares‐De‐Cal M. A. , Aneiros A. , Abad M. , Ceballos L. , Gómez‐Moreno G. , Hidalgo J. J. , Llena C. , López‐Jornet P. , Machuca M. C. , Monticelli F. , Palés J. 
Published: December 2013

AbstractAimTo carry out a psychometric evaluation of the Spanish‐language version of the Dundee Ready Education Environment Measure (DREEM) applied to dental students.MethodsA total of 1,391 students from nine Spanish public schools of dentistry responded to the DREEM questionnaire. To analyse the reliability of the DREEM questionnaire, the internal consistency was assessed and a ‘test‐retest’ carried out. Validity was evaluated through analysis of item response rate, floor and ceiling effects, corrected item‐total and item‐subscale correlations and factor structure. A confirmatory factor analysis was performed to analyse the structure of the original DREEM scale.ResultsCronbach's alpha coefficient for the ‘Educational Climate’ (EC) global scale was 0.92. In the subscales, the ‘observed’ Cronbach's alpha coefficients ranged between 0.57 and 0.79 and were higher than the ‘expected’ ones; except for the Social subscale. In the DREEM questionnaire, all of the corrected correlation coefficients between the items and the EC global scale, and the items and their corresponding subscales, were >0.2; except for items 50 and 17. All goodness‐of‐fit indices of confirmatory factor analysis showed acceptable values (close to one or zero, depending on the case), and there was consistency in the results.ConclusionsThe Spanish‐language version of the DREEM questionnaire is a reliable and valid instrument for analysing the EC for dental students and its factor structure is supported by the data. Although our findings indicate that the DREEM may be as culturally independent as was originally stated, more research should be directed at verifying the factor structure in various languages and cultural environments.
Immunophenotypic analysis of T‐acute lymphoblastic leukemia. A CD5‐based ETP‐ALL perspective of non‐ETP T‐ALL
Authors: Chopra Anita , Bakhshi Sameer , Pramanik Suman Kumar , Pandey Ravindra Mohan , Singh Saroj , Gajendra Smeeta , Gogia Ajay , Chandramohan Jagan , Sharma Atul , Kumar Lalit , Seth Rachna , Rai Sandeep , Kumar Rajive 
Published: December 2013

AbstractT‐cell antigens [CD5,CD1a,CD8] define early T‐cell precursor acute lymphoblastic leukemia (ETP‐ALL). To understand immature T‐ALL of which ETP‐ALL is part, we used these antigens to subcategorize non‐ETP T‐ALL for examining expression of myeloid/stem cell antigens (M/S) and clinical features. Using CD5 (+/−) to start categorization, we studied 69 routinely immunophenotyped patients with T‐ALL. CD5− was a homogenous (CD8,CD1a)− M/S+ ETP‐ALL group (n = 9). CD5+ cases were (CD8,CD1a)− pre‐T‐ALL (n = 22) or (CD8,CD1a)+ (n = 38) thymic/cortical T‐ALL; M/S+ 20/22 (90.91%) in former and 22/38 (57.89%) in latter (P = 0.007). ETP‐ and pre‐T‐ALL together (CD1a−,CD5−/+ immature T‐ALL group) were nearly always M/S+ (29/31; 93.55%). In multivariate analysis, only ETP‐ALL predicted poor overall survival (P = 0.02). We conclude (i) CD5 negativity in T‐ALL almost always means ETP‐ALL. CD1a and CD8 negativity, as much as CD5, marks immaturity in T‐ALL, and the CD5+/−/CD1a−/CD8− immature T‐ALL group needs further study to understand the biology of the T‐ALL–myeloid interface. (ii) ETP‐ALL patients may be pre‐T‐ALL if CD2+; CD2+, conversely, CD5−/CD1a−/CD8− pre‐T ALL patients are ETP‐ALL. (iii) Immunophenotypic workup of T‐ALL must not omit CD1a, CD5, CD8 and CD2, and positivity of antigens should preferably be defined as recommended for ETP‐ALL, so that this entity can be better evaluated in future studies of immature T‐ALL, a group to which ETP‐ALL belongs. (iv) ETP‐ALL has poor prognosis.
Zoledronic acid in the management of bone disease as a consequence of multiple myeloma: a review
Authors: Alegre Adrián , Gironella Mercedes , Bailén Alicia , Giraldo Pilar 
Published: December 2013

AbstractBackgroundMultiple myeloma (MM) is a haematological cancer associated with organ damage, essentially bone damage. MM continues to be considered an incurable disease and requires significant therapeutic resources.AimThis manuscript presents a literature review of the utility of zoledronic acid (zoledronate) in the management of bone disease in MM.DiscussionThe clinical benefit of bisphosphonates in the prevention and treatment of bone disease in MM has been demonstrated in the last 10 years. A recent network meta‐analysis has evaluated 20 randomised clinical trials with nearly 7000 patients with myeloma enrolled and confirms the role of these agents in the prevention of pain, bone fractures and other skeletal‐related events (SRE) in MM. However, their role on survival remains unclear. Zoledronate has shown significant reductions in SRE compared with placebo or other agents with acceptable tolerability, and recent studies have shown antitumoural effects for this agent, with a marginal survival benefit demonstrated in MM.ConclusionsZoledronate prevents SRE in MM and presents a marginal survival benefit that deserves further investigation in controlled studies. Current clinical guidelines should be followed regarding the appropriate use of zoledronate in MM, to enhance its effects and to minimise its potential toxicity.
Continuation of deferiprone therapy in patients with mild neutropenia may not lead to a more severe drop in neutrophil count
Authors: El‐Beshlawy Amal M. , El‐Alfy Mohsen S. , Sari Teny T. , Chan Lee L. , Tricta Fernando 
Published: December 2013

AbstractApproximately 6% of patients with thalassemia receiving deferiprone develop neutropenia. Present practice is to monitor absolute neutrophil count (ANC) weekly and to interrupt treatment at the first sign of neutropenia, lest continuation lead to progressive neutrophil reduction. In a 6‐month study evaluating the safety and efficacy of a liquid form of deferiprone in 100 children, ANC was initially checked weekly for all patients. For individuals experiencing mild neutropenia, deferiprone was continued but monitoring was increased to daily until resolution. Therapy was to be suspended only if the episode was prolonged or if it worsened. Four patients experienced single episodes of mild neutropenia, and two others each experienced two episodes. All eight episodes resolved within 4–7 d despite continued therapy. (One patient later developed agranulocytosis and had treatment terminated.) This study showed that not all cases of mild neutropenia during deferiprone therapy develop into agranulocytosis, and suggests that many may not be caused by deferiprone. Transient declines in ANC to levels defined as neutropenic are common even in healthy individuals, particularly children; and it could be that the frequent monitoring of ANC mandated during deferiprone therapy may reveal cases of transient neutropenia that would otherwise have gone undetected and resolved on their own without clinical consequences. In patients with thalassemia, several factors increase the probability of a transient fall in ANC. These findings raise the question of whether deferiprone should be routinely stopped in cases of mild neutropenia, provided that such patients have their ANC monitored more frequently during the neutropenic episode.
Alpha‐thalassaemia and response to hydroxyurea in sickle cell anaemia
Authors: Darbari Deepika S. , Nouraie Mehdi , Taylor James G. , Brugnara Carlo , Castro Oswaldo , Ballas Samir K. 
Published: December 2013

AbstractBackgroundHydroxyurea (HU) reduces vaso‐occlusive crises (VOC) and other complications of sickle cell anaemia (SCA). Alpha‐thalassaemia is a known modifier of SCA. Studies on the efficacy of HU in SCA patients with α‐thalassaemia have yielded varying results.ObjectiveTo determine the effect of α‐thalassaemia in response to HU therapy in the Multicenter Study of Hydroxyurea (MSH) cohort.MethodsWe compared the laboratory parameters and VOC incidence in the MSH cohort stratified by the presence or the absence of α‐thalassaemia.ResultsHydroxyurea showed significant (P = 0.001 for all baseline vs. follow‐up comparisons) treatment effect on red cell indices irrespective of α‐globin gene deletion. The magnitude of the HU‐related changes was similar for mean corpuscular volume (MCV) (no α‐thalassaemia 13 fl and α‐thalassaemia 13 fl) and mean corpuscular haemoglobin (MCH) (no α‐thalassaemia 4 pg and α‐thalassaemia 4 pg) in both groups. Foetal haemoglobin (HbF) and F‐cells also increased significantly with HU treatment in both groups. Total haemoglobin increased after HU treatment in both groups, but the increase was smaller and not statistically significant in patients with α‐thalassaemia. In contrast, HU‐related reduction in VOCs was more pronounced in patients with α‐thalassaemia (VOC incidence rate ratio HU/placebo: 0.63 for α‐thalassaemia and 0.54 for no α‐thalassaemia (P for interaction 0.003).ConclusionHydroxyurea decreases VOCs in SCA patients with and without α‐thalassaemia, and the degree of VOC reduction was more pronounced in the patients with alpha‐thalassaemia. Despite the lower baseline values, changes in standard laboratory parameters such as MCV and HbF percent remain useful in monitoring HU therapy in the presence of α‐thalassaemia.
Detection of alpha(0)‐thalassemia South‐East Asian‐type deletion by droplet digital PCR
Authors: Pornprasert Sakorn , Prasing Watcharee 
Published: December 2013

AbstractBackgroundThe α(0)‐thalassemia South‐East Asian (SEA)‐type deletion is the most common genetic disorder in the Asian population. Couples who are both carriers have a 25% chance of conceiving Bart's hydrops fetalis. Therefore, results from carrier screening and prenatal diagnosis frequently need to be available rapidly. The aim of this study was to implement a droplet digital polymerase chain reaction (ddPCR) for diagnosis of α(0)‐thalassemia SEA‐type deletion.MethodsThe wild‐type α‐globin gene allele and α(0)‐thalassemia SEA allele were quantified in DNA samples of 20 normal individuals, 15 samples with α(0)‐thalassemia SEA trait, and 8 samples with Bart's hydrops fetalis using the ddPCR. The DNA copy number of wild‐type α‐globin gene allele and α(0)‐thalassemia SEA allele was then calculated using the Quantasoft analysis software.ResultsThe mean ± standard deviation (SD) ratio of wild‐type α‐globin gene allele and α(0)‐thalassemia SEA allele among normal individuals, samples with α(0)‐thalassemia SEA trait, and Bart's hydrops fetalis were clearly distinguished with levels of 1.78 ± 0.49, 0.85 ± 0.14, and 0.03 ± 0.03, respectively.ConclusionThe ddPCR may be one alternative technology available for routine clinical diagnosis of α(0)‐thalassemia SEA‐type deletion and prenatal diagnosis of Bart's hydrops fetalis.
Management of erythropoiesis: cross‐sectional study of the relationships between erythropoiesis and nutrition, physical features, and adiponectin in 3519 Japanese people
Authors: Kohno Kei , Narimatsu Hiroto , Shiono Yosuke , Suzuki Ikuko , Kato Yuichi , Fukao Akira , Kubota Isao , Ueno Yoshiyuki , Kayama Takamasa , Kato Takeo 
Published: December 2013

AbstractAlthough erythroid abnormalities (anemia and polycythemia) are commonly observed pathological conditions, not much information about borderline abnormalities is available. In this study, a cross‐sectional study to analyze the relationships between erythropoiesis and nutrition, physical features, and laboratory test findings was conducted in middle‐aged and older men and women. The study included 3519 Japanese people (1579 men and 1940 women), age 40 years and over. Analysis of variance showed that the group with a tendency to anemia was older, had a lower body mass index and diastolic blood pressure, and had higher serum adiponectin and creatinine. Multiple regression analysis showed that adiponectin, triglycerides, and total protein were common factors that affected erythropoiesis in both men and women. Hepatic, renal, and cardiac functions were also factors involved in erythropoiesis in men and in postmenopausal women. In addition, nutrient factors such as alcohol, vitamins, and carbohydrates were also significantly involved in erythropoiesis in men, but there were no significant nutrient factors involved in erythropoiesis in either premenopausal or postmenopausal women. This study showed that factors that influence erythropoiesis differ between men, premenopausal women, and postmenopausal women, and it suggested that appropriately modifying erythropoiesis management for each group of people is essential.
Neurosystems: brain rhythms and cognitive processing
Authors: Cannon Jonathan , McCarthy Michelle M. , Lee Shane , Lee Jung , Börgers Christoph , Whittington Miles A. , Kopell Nancy 
Published: December 2013

AbstractNeuronal rhythms are ubiquitous features of brain dynamics, and are highly correlated with cognitive processing. However, the relationship between the physiological mechanisms producing these rhythms and the functions associated with the rhythms remains mysterious. This article investigates the contributions of rhythms to basic cognitive computations (such as filtering signals by coherence and/or frequency) and to major cognitive functions (such as attention and multi‐modal coordination). We offer support to the premise that the physiology underlying brain rhythms plays an essential role in how these rhythms facilitate some cognitive operations.
Hypothalamic hamartoma: Is the epileptogenic zone always hypothalamic? Arguments for independent (third stage) secondary epileptogenesis
Authors: Scholly Julia , Valenti Maria‐Paola , Staack Anke M. , Strobl Karl , Bast Thomas , Kehrli Pierre , Steinhoff Bernhard J. , Hirsch Edouard 
Published: December 2013

SummaryGelastic seizures associated with hypothalamic hamartomas (HHs) are a clinicoradiologic syndrome presenting with a variety of symptoms, including pharmacoresistant epilepsy with multiple seizure types, electroencephalography (EEG) abnormalities, precocious puberty, behavioral disturbances, and progressive cognitive deterioration. Surgery in adults provides seizure freedom in only one third of patients. The poor results of epilepsy surgery could be explained by an extrahypothalamic epileptogenic zone. The existence of an independent, secondary epileptogenic area with persistent seizures after resection of the presumably primary lesion supports the concept of a “hypothalamic plus” epilepsy. “Hypothalamic plus” epilepsy could be related to either an extrahypothalamic structural lesion (visible on magnetic resonance imaging [MRI] or on neuropathology) or if the former is absent, to a functional alteration with enhanced epileptogenic properties due to a process termed secondary epileptogenesis. We report two patients with gelastic seizures with HH (gelastic seizures isolated or associated with dyscognitive seizures of temporal origin). Both patients underwent two‐step surgery: first an endoscopic resection of the HH, followed at a later time by temporal lobectomy. Both patients became seizure‐free only after the temporal lobectomy. In both cases, neuropathology failed to demonstrate a significant structural lesion in the temporal lobe. To our knowledge, for the first time, these two cases suggest the existence of independent secondary epileptogenesis in humans.
Resecting the epileptogenic tuber: What happens in the long term?
Authors: Shahid Asim 
Published: December 2013

SummaryTuberous sclerosis complex (TSC) is an autosomal dominant, neurocutaneous disorder causing benign noninvasive lesions in multiple organ systems. Cognitive delays and neuropsychiatric disorders such as autism are common. Epilepsy is very common and is associated with single or multiple epileptogenic cortical tubers. More than half of these individuals will become medically refractory over time. For these patients, epilepsy surgery with resection of the epileptogenic tuber or tubers not only can render a select patient population seizure‐free, but also improvesIQand cognitive behaviors over time.
The functional relevance of polyploidization in the skin
Authors: Trakala Marianna , Malumbres Marcos 
Published: December 2013

AbstractCell proliferation and differentiation are tightly coupled through the regulation of the cell division cycle. To preserve specific functional properties in differentiated cells, distinct variants of the basic mitotic cell cycle are used in various mammalian tissues, leading to the formation of polyploid cells. In this issue of Experimental Dermatology, Gandarillas and Freije discuss the evidences for polyploidization in keratinocytes, a process whose physiological relevance is now becoming evident. A better evaluation of these unconventional cell cycles is required not only to improve our understanding of the development and structure of the epidermis but also for future therapies against skin diseases.
Nuclear Raf‐1 kinase regulates the CXCR5 promoter by associating with NFATc3 to drive retinoic acid‐induced leukemic cell differentiation
Authors: Geil Wendy M. , Yen Andrew 
Published: December 2013

Novel functions of signaling molecules have been revealed in studies of cancer stem cells. Retinoic acid (RA) is an embryonic morphogen and stem cell regulator that controls the differentiation of a patient‐derived leukemic cell line, HL‐60, which is composed of progenitor cells with bipotent myelo‐monocytic differentiation capability. RA treatment of HL‐60 cells causes unusually long‐lasting mitogen‐activated protein kinase signaling, with the cells exhibiting the beginning of G0 cell cycle arrest and functional differentiation by 48 h after treatment with RA. This event coincides with the nuclear translocation of Raf‐1, phosphorylated at serine 621. The present study shows how the novel localization of Raf‐1 to the nucleus results in transcriptional changes that contribute to the differentiation of HL‐60 cells induced by RA. We find that nuclear pS621 Raf‐1 associates with NFATc3 near its cognate binding site in the promoter of CXCR5, a gene that must be up‐regulated to drive RA‐induced differentiation. NFATc3 becomes immunoprecipitable with anti‐phosphoserine serum, and CXCR5 is transcriptionally up‐regulated upon RA‐induced differentiation. Inhibiting the pS621 Raf‐1/NFATc3 association with PD98059 inhibits these processes and cripples RA‐induced differentiation. In this novel paradigm for Raf‐1 and RA function, Raf‐1 has a role in driving the nuclear signaling of RA‐induced differentiation of leukemic progenitor cells.Structured digital abstractNFATc3 physically interacts with Raf-1 by coimmunoprecipitation (1, 2)
Increased energy maize production reduces farmland bird diversity
Authors: Sauerbrei Ralf , Ekschmitt Klemens , Wolters Volkmar , Gottschalk Thomas K. 
Published: December 2013

AbstractProducing energy crops as an alternative to fossil fuels in order to reduce CO2 emissions will lead to large‐scale changes in agricultural landscapes. Here, we quantify the potential impact of an increase in maize fields on the diversity of farmland birds by means of high resolution (25 × 25 m) land‐use scenarios. We generated scenarios in which the area of maize production in Germany increases from presently 2.6 to 2.9, 3.6 and 4.3 million ha, corresponding to the energy crop production targets of the German Renewable Energy Act for the years 2020, 2035 and 2050. To test the mitigating potential of conservation measures, each scenario was generated in a standard version and a landscape protection version, with the latter excluding valuable farmland areas from being converted into maize fields. Nine species of farmland birds belonging to the governmental indicator scheme for sustainable land‐use in Germany were modelled for the six nation‐wide scenarios. The models predicted that only the Northern Lapwing and the Little Owl might profit from extended maize production. Despite this, the total number of breeding pairs of the indicator species was predicted to decline by about 0.4 million breeding pairs in the most intensive scenario. Protection of valuable farmland did not mitigate these negative effects in the models. Our findings suggest that increased production of energy crops conflicts with conservation of biological diversity.