bluuebunny/crossref_metadata_2025_split huggingface dataset:
Transthyretin amyloid neuropathy has earlier neural involvement but better prognosis than primary amyloid counterpart: an answer to the paradox?
Authors: Loavenbruck Adam J. , Singer Wolfgang , Mauermann Michelle L. , Sandroni Paola , B. Dyck P. James , Gertz Morie , Klein Christopher J. , Low Phillip A. 
Published: July 2016

ObjectiveTo systematically compare transthyretin with primary amyloid neuropathy to define their natural history and the underlying mechanisms for differences in phenotype and natural history.MethodsAll patients with defined amyloid subtype and peripheral neuropathy who completed autonomic testing and electromyography at Mayo Clinic Rochester between 1993 and 2013 were included. Medical records were reviewed for time of onset of defined clinical features. The degree of autonomic impairment was quantified using the composite autonomic severity scale. Comparisons were made between acquired and inherited forms of amyloidosis.ResultsOne hundred one cases of amyloidosis with peripheral neuropathy were identified, 60 primary and 41 transthyretin. Twenty transthyretin cases were found to have Val30Met mutations; 21 had other mutations. Compared to primary cases, transthyretin cases had longer survival, longer time to diagnosis, higher composite autonomic severity scale scores, greater reduction of upper limb nerve conduction study amplitudes, more frequent occurrence of weakness, and later non‐neuronal systemic involvement. Four systemic markers (cardiac involvement by echocardiogram, weight loss > 10 pounds, orthostatic intolerance, fatigue) in combination were highly predictive of poor survival in both groups.InterpretationThese findings suggest that transthyretin has earlier and greater predilection for neural involvement and more delayed systemic involvement. The degree and rate of systemic involvement is most closely related to prognosis. Ann Neurol 2016;80:401–411
Somatosensory attention identifies both overt and covert awareness in disorders of consciousness
Authors: Gibson Raechelle M. , Chennu Srivas , Fernández‐Espejo Davinia , Naci Lorina , Owen Adrian M. , Cruse Damian 
Published: July 2016

ObjectiveSome patients diagnosed with disorders of consciousness retain sensory and cognitive abilities beyond those apparent from their overt behavior. Characterizing these covert abilities is crucial for diagnosis, prognosis, and medical ethics. This multimodal study investigates the relationship between electroencephalographic evidence for perceptual/cognitive preservation and both overt and covert markers of awareness.MethodsFourteen patients with severe brain injuries were evaluated with an electroencephalographic vibrotactile attention task designed to identify a hierarchy of residual somatosensory and cognitive abilities: (1) somatosensory steady‐state evoked responses, (2) bottom‐up attention orienting (P3a event‐related potential), and (3) top‐down attention (P3b event‐related potential). Each patient was also assessed with a clinical behavioral scale and 2 functional magnetic resonance imaging assessments of covert command following.ResultsSix patients produced only sensory responses, with no evidence of cognitive event‐related potentials. A further 8 patients demonstrated reliable bottom‐up attention‐orienting responses (P3a). No patient showed evidence of top‐down attention (P3b). Only those patients who followed commands, whether overtly with behavior or covertly with functional neuroimaging, also demonstrated event‐related potential evidence of attentional orienting.InterpretationSomatosensory attention‐orienting event‐related potentials differentiated patients who could follow commands from those who could not. Crucially, this differentiation was irrespective of whether command following was evident through overt external behavior, or through covert functional neuroimaging methods. Bedside electroencephalographic methods may corroborate more expensive and challenging methods such as functional neuroimaging, and thereby assist in the accurate diagnosis of awareness. Ann Neurol 2016;80:412–423
Estimation of the diversity between DNA calorimetric profiles, differential melting curves and corresponding melting temperatures
Authors: Chang Chun‐Ling , Fridman Alexander S. , Grigoryan Inessa E. , Galyuk Elena N. , Murashko Oleg N. , Hu Chin‐Kun , Lando Dmitri Y. 
Published: July 2016

AbstractThe Poland–Fixman–Freire formalism was adapted for modeling of calorimetric DNA melting profiles, and applied to plasmid pBR 322 and long random sequences. We studied the influence of the difference (HGC−HAT) between the helix‐coil transition enthalpies of AT and GC base pairs on the calorimetric melting profile and on normalized calorimetric melting profile. A strong alteration of DNA calorimetrical profile with HGC−HAT was demonstrated. In contrast, there is a relatively slight change in the normalized profiles and in corresponding ordinary (optical) normalized differential melting curves (DMCs). For fixed HGC−HAT, the average relative deviation (S) between DMC and normalized calorimetric profile, and the difference between their melting temperatures (Tcal−Tm) are weakly dependent on peculiarities of the multipeak fine structure of DMCs. At the same time, both the deviation S and difference (Tcal−Tm) enlarge with the temperature melting range of the helix‐coil transition. It is shown that the local deviation between DMC and normalized calorimetric profile increases in regions of narrow peaks distant from the melting temperature.
Paraneoplastic pemphigus and myasthenia gravis, associated with inflammatory pseudotumor‐like follicular dendritic cell sarcoma: response to rituximab
Authors: Wang Lifang , Deng Hui , Mao Mei 
Published: July 2016

Key Clinical MessageParaneoplastic pemphigus (PNP) is an autoimmune blistering disease associated with neoplasms. The disease is most commonly of lymphoproliferative origin and presents high mortality. We describe a patient with PNP and myasthenia gravis associated with inflammatory pseudotumor‐like follicular dendritic cell sarcoma, as well as the response to rituximab.
Comparison of tree transpiration under wet and dry canopy conditions in a Costa Rican premontane tropical forest
Authors: Aparecido Luiza Maria Teophilo , Miller Gretchen R. , Cahill Anthony T. , Moore Georgianne W. 
Published: July 2016

AbstractSpatial and temporal variation in wet canopy conditions following precipitation events can influence processes such as transpiration and photosynthesis, which can be further enhanced as upper canopy leaves dry more rapidly than the understory following each event. As part of a larger study aimed at improving land surface modelling of evapotranspiration processes in wet tropical forests, we compared transpiration among trees with exposed and shaded crowns under both wet and dry canopy conditions in central Costa Rica, which has an average 4200 mm annual rainfall. Transpiration was estimated for 5 months using 43 sap flux sensors in eight dominant, ten midstory and eight suppressed trees in a mature forest stand surrounding a 40‐m tower equipped with micrometeorological sensors. Dominant trees were 13% of the plot's trees and contributed around 76% to total transpiration at this site, whereas midstory and suppressed trees contributed 18 and 5%, respectively. After accounting for vapour pressure deficit and solar radiation, leaf wetness was a significant driver of sap flux, reducing it by as much as 28%. Under dry conditions, sap flux rates (Js) of dominant trees were similar to midstory trees and were almost double that of suppressed trees. On wet days, all trees had similarly low Js. As expected, semi‐dry conditions (dry upper canopy) led to higher Js in dominant trees than midstory, which had wetter leaves, but semi‐dry conditions only reduced total stand transpiration slightly and did not change the relative proportion of transpiration from dominant and midstory. Therefore, models that better capture forest stand wet–dry canopy dynamics and individual tree water use strategies are needed to improve accuracy of predictions of water recycling over tropical forests. Copyright © 2016 John Wiley & Sons, Ltd.
Interleaved neuromuscular electrical stimulation: Motor unit recruitment overlap
Authors: Wiest Matheus J. , Bergquist Austin J. , Schimidt Helen L. , Jones Kelvin E. , Collins David F. 
Published: July 2016

ABSTRACTIntroduction: In this study, we quantified the “overlap” between motor units recruited by single pulses of neuromuscular electrical stimulation (NMES) delivered over the tibialis anterior muscle (mNMES) and the common peroneal nerve (nNMES). We then quantified the torque produced when pulses were alternated between the mNMES and nNMES sites at 40 Hz (“interleaved” NMES; iNMES). Methods: Overlap was assessed by comparing torque produced by twitches evoked by mNMES, nNMES, and both delivered together, over a range of stimulus intensities. Trains of iNMES were delivered at the intensity that produced the lowest overlap. Results: Overlap was lowest (5%) when twitches evoked by both mNMES and nNMES produced 10% peak twitch torque. iNMES delivered at this intensity generated 25% of maximal voluntary dorsiflexion torque (11 Nm). Discussion: Low intensity iNMES leads to low overlap and produces torque that is functionally relevant to evoke dorsiflexion during walking. Muscle Nerve 55: 490–499, 2017
Cancer and dementia: an exploratory study of the experience of cancer treatment in people with dementia
Authors: Courtier Nick , Milton Rebecca , King Amanda , Tope Rosie , Morgan Susan , Hopkinson Jane 
Published: July 2016

AbstractObjectivePatients with comorbid cancer and dementia have poorer outcomes than those without dementia. We observe oncology teams managing patients with dementia and memory loss and explore these patients' needs and experiences of outpatient cancer services.MethodsA single site investigation of case study design to examine practices in four clinics using multi‐methods of data collection: retrospective note review, observation, interviews, and recorded consultations. A framework analytic approach identifies themes within and across cases.ResultsThirty‐three clinical encounters with patients with memory loss were observed. Ten consultations were audio‐recorded and 16 individuals interviewed (n = 6 patients‐carer dyads, n = 1 lone patient, and n = 5 staff). Medical records were reviewed for 338 cases. Cancer referrals did not document memory health, so clinicians rely on patient/carer disclosure to identify patients with memory problems. In practice, the problem often remains hidden. Treating teams who do become aware of memory difficulties are unsure how to support patients, but marked memory loss can limit treatment options and preclude radical intent. Carers are key facilitators of successful cancer consultations and management. Their support needs are largely unrecognized.ConclusionsTraining that educates cancer teams on how to identify and support individuals with memory problems before and during treatment and recognize the carer role may facilitate complex cancer care and help reduce inequalities of outcomes.
Exploring stigma as a barrier to cancer service engagement with breast cancer survivors in Kampala, Uganda
Authors: Meacham Elizabeth , Orem Jackson , Nakigudde Gertrude , Zujewski Jo Anne , Rao Deepa 
Published: July 2016

AbstractObjectiveTo understand the role of stigma in the delay of cancer service engagement by women with breast cancer in Kampala, Uganda.BackgroundWomen in Sub‐Saharan African countries are twice as likely to die from cancer as women in high‐income countries, which is largely attributable to late diagnosis. While breast cancer‐related stigma has been identified in Sub‐Saharan Africa, limited research focuses on how stigma impacts the behavior of breast cancer patients in Uganda.MethodsThis qualitative study used a grounded theory approach to examine illness narratives from 20 breast cancer survivors in Uganda, gathered through semistructured interviews.ResultsThematic analysis showed that perceived and internalized stigma associated with breast cancer influenced care engagement throughout illness, delaying engagement and inhibiting treatment completion. Women identified key factors for overcoming stigma including acceptance of diagnosis, social support, and understanding of breast cancer.ConclusionThe growing burden of mortality associated with breast cancer in Uganda can be mitigated by improving early detection and treatment engagement through interventions which account for key psychosocial barriers such as stigma.
Sociodemographic inequalities in barriers to cancer pain management: a report from the American Cancer Society's Study of Cancer Survivors‐II (SCS‐II)
Authors: Stein Kevin D. , Alcaraz Kassandra I. , Kamson Chelsey , Fallon Elizabeth A. , Smith Tenbroeck G. 
Published: July 2016

AbstractObjectiveResearch has increasingly documented sociodemographic inequalities in the assessment and management of cancer‐related pain. Most studies have focused on racial/ethnic disparities, while less is known about the impact of other sociodemographic factors, including age and education. We analyzed data from a large, national, population‐based study of cancer survivors to examine the influence of sociodemographic factors, and physical and mental health comorbidities on barriers to cancer pain management.MethodsThe study included data from 4707 cancer survivors in the American Cancer Society's Study of Cancer Survivors‐II, who reported experiencing pain from their cancer. A multilevel, socioecological, conceptual framework was used to generate a list of 15 barriers to pain management, representing patient, provider, and system levels. Separate multivariable logistic regressions for each barrier identified sociodemographic and health‐related inequalities in cancer pain management, controlling for years since diagnosis, disease stage, and cancer treatment.ResultsTwo‐thirds of survivors reported at least 1 barrier to pain management. While patient‐related barriers were most common, the greatest disparities were noted in provider‐ and system‐level barriers. Specifically, inequalities by race/ethnicity, education, age, and physical and mental health comorbidities were observed.ConclusionFindings indicate survivors who were nonwhite, less educated, older, and/or burdened by comorbidities were most adversely affected. Future efforts in research, clinical practice, and policy should identify and/or implement new strategies to address sociodemographic inequalities in cancer pain management.
“Our people has got to come to terms with that”: changing perceptions of the digital rectal examination as a barrier to prostate cancer diagnosis in African‐Caribbean men
Authors: Seymour‐Smith Sarah , Brown David , Cosma Georgina , Shopland Nicholas , Battersby Steven , Burton Andy 
Published: July 2016

AbstractObjectiveAfrican‐Caribbean men in the United Kingdom in comparison with other ethnicities have the highest incidence rate of prostate cancer. Psychosocial aspects related to screening and presentation impact on men's behavior, with previous studies indicating a range of barriers. This study explores one such barrier, the digital rectal examination (DRE), due to its prominence within UK African‐Caribbean men's accounts.MethodsAfrican‐Caribbean men with prostate cancer (n = 10) and without cancer (n = 10) were interviewed about their perceptions of DRE. A synthetic discursive approach was employed to analyze the data.ResultsFindings illustrate that an interpretative repertoire of homophobia in relation to the DRE is constructed as having an impact upon African‐Caribbean men's uptake of prostate cancer screening. However, the discursive focus on footing and accountability highlight deviations from this repertoire that are built up as pragmatic and orient to changing perceptions within the community.ConclusionsHealth promotion interventions need to address the fear of homophobia and are best designed in collaboration with the community.
Morbidity of curative cancer surgery and suicide risk
Authors: Jayakrishnan Thejus T. , Sekigami Yurie , Rajeev Rahul , Gamblin T. Clark , Turaga Kiran K. 
Published: July 2016

AbstractImportanceCurative cancer operations lead to debility and loss of autonomy in a population vulnerable to suicide death. The extent to which operative intervention impacts suicide risk is not well studied.ObjectiveTo examine the effects of morbidity of curative cancer surgeries and prognosis of disease on the risk of suicide in patients with solid tumors.DesignRetrospective cohort study using Surveillance, Epidemiology, and End Results data from 2004 to 2011; multilevel systematic review.SettingGeneral US population.ParticipantsParticipants were 482 781 patients diagnosed with malignant neoplasm between 2004 and 2011 who underwent curative cancer surgeries.Main outcomes and measuresDeath by suicide or self‐inflicted injury.ResultsAmong 482 781 patients that underwent curative cancer surgery, 231 committed suicide (16.58/100 000 person‐years [95% confidence interval, CI, 14.54‐18.82]). Factors significantly associated with suicide risk included male sex (incidence rate [IR], 27.62; 95% CI, 23.82‐31.86) and age >65 years (IR, 22.54; 95% CI, 18.84‐26.76). When stratified by 30‐day overall postoperative morbidity, a significantly higher incidence of suicide was found for high‐morbidity surgeries (IR, 33.30; 95% CI, 26.50‐41.33) vs moderate morbidity (IR, 24.27; 95% CI, 18.92‐30.69) and low morbidity (IR, 9.81; 95% CI, 7.90‐12.04). Unit increase in morbidity was significantly associated with death by suicide (odds ratio, 1.01; 95% CI, 1.00‐1.03; P = .02) and decreased suicide‐specific survival (hazards ratio, 1.02; 95% CI, 1.00‐1.03, P = .01) in prognosis‐adjusted models.ConclusionsIn this sample of cancer patients in the Surveillance, Epidemiology, and End Results database, patients that undergo high‐morbidity surgeries appear most vulnerable to death by suicide. The identification of this high‐risk cohort should motivate health care providers and particularly surgeons to adopt screening measures during the postoperative follow‐up period for these patients.
Group‐based trajectory modeling of fear of disease recurrence among women recently diagnosed with gynecological cancers
Authors: Manne Sharon L. , Myers‐Virtue Shannon , Kissane David , Ozga Melissa L. , Kashy Deborah A. , Rubin Stephen C. , Rosenblum Norman G. , Heckman Carolyn J. 
Published: July 2016

AbstractObjectiveFear of cancer recurrence is an important clinical phenomenon and is associated with decrements in life domains. The study goals were to characterize patterns of global fear of recurrence (FOR) and 4 domains of fear (health, role, womanhood, and death worry) over time in women who were diagnosed with gynecological cancer and to identify demographic, medical, and psychological predictors of FOR.MethodOne hundred eighteen women participating in the usual care arm of a randomized trial completed the Concerns about Recurrence scale as well as measures of depressive symptoms, cancer‐specific distress, coping, coping efficacy, and social network responses at 4 time points. The majority of the sample was diagnosed with stage 3 ovarian cancer.ResultsGroup‐based trajectory modeling identified subgroups of women with high‐stable (49.1%), high‐decreasing (25.3%), and low‐stable (25.5%) trajectories for global FOR. For role worries, 3 similar group trajectories were identified. For health worries, modeling identified subgroups with high‐decreasing (19.1%) and low‐increasing (80.9%) trajectories. For womanhood worries, modeling identified subgroups with high‐increasing (15.7%) and low‐decreasing (84.2%) trajectories. Young age, metastatic cancer, depression, cancer distress, holding back, and lower coping efficacy were associated with the high‐stable global FOR and at least 1 domain of FOR.ConclusionAlmost half of the women recently diagnosed with gynecological cancer evidence persistently elevated FOR over the 6‐month period postdiagnosis. Psychological interventions to reduce FOR may be more effective if they focus on teaching patients coping skills, as well as greater comfort expressing cancer‐specific concerns to others.
Establishment of Reporter Lines for Detecting Fragile X Mental Retardation (FMR1) Gene Reactivation in Human Neural Cells
Authors: Li Meng , Zhao Huashan , Ananiev Gene E. , Musser Michael T. , Ness Kathryn H. , Maglaque Dianne L. , Saha Krishanu , Bhattacharyya Anita , Zhao Xinyu 
Published: July 2016

AbstractHuman patient-derived induced pluripotent stem cells (hiPSCs) provide unique opportunities for disease modeling and drug development. However, adapting hiPSCs or their differentiated progenies to high throughput assays for phenotyping or drug screening has been challenging. Fragile X syndrome (FXS) is the most common inherited cause of intellectual disability and a major genetic cause of autism. FXS is caused by mutational trinucleotide expansion in the FMR1 gene leading to hypermethylation and gene silencing. One potential therapeutic strategy is to reactivate the silenced FMR1 gene, which has been attempted using both candidate chemicals and cell-based screening. However, molecules that effectively reactivate the silenced FMR1 gene are yet to be identified; therefore, a high throughput unbiased screen is needed. Here we demonstrate the creation of a robust FMR1-Nluc reporter hiPSC line by knocking in a Nano luciferase (Nluc) gene into the endogenous human FMR1 gene using the CRISPR/Cas9 genome editing method. We confirmed that luciferase activities faithfully report FMR1 gene expression levels and showed that neural progenitor cells derived from this line could be optimized for high throughput screening. The FMR1-Nluc reporter line is a good resource for drug screening as well as for testing potential genetic reactivation strategies. In addition, our data provide valuable information for the generation of knockin human iPSC reporter lines for disease modeling, drug screening, and mechanistic studies.
Efficient Regeneration of Human Vα24+ Invariant Natural Killer T Cells and Their Anti-Tumor Activity In Vivo
Authors: Yamada Daisuke , Iyoda Tomonori , Vizcardo Raul , Shimizu Kanako , Sato Yusuke , Endo Takaho A. , Kitahara Genta , Okoshi Momoko , Kobayashi Midori , Sakurai Maki , Ohara Osamu , Taniguchi Masaru , Koseki Haruhiko , Fujii Shin-ichiro 
Published: July 2016

AbstractReprogramming of antigen-specific T lymphocytes into induced pluripotent stem cells (iPSCs) and their subsequent re-differentiation has enabled expansion of functional T lymphocytes in vitro, thus opening up new approaches for immunotherapy of cancer and other diseases. In this study, we have established a robust protocol to reprogram human invariant NKT (Vα24+ iNKT) cells, which have been shown to act as cellular adjuvants and thus exert anti-tumor activity in mice and humans, and to re-differentiate the iNKT cell-derived iPSCs into functional iNKT cells. These iPSC-derived iNKT cells (iPS-Vα24+ iNKT cells) can be activated by ligand-pulsed dendritic cells (DCs) and produce a large amount of interferon-γ upon activation, as much as parental Vα24+ iNKT cells, but exhibit even better cytotoxic activity against various tumor cell lines. The iPS-Vα24+ iNKT cells possess significant anti-tumor activity in tumor-bearing mice and can activate autologous NK cells upon activation by ligand-pulsed DCs in the NOG mouse model in vivo, further extending their therapeutic potential. This study thus provides a first proof of concept for the clinical application of human iPS-Vα24+ iNKT cells for cancer immunotherapy.