bluuebunny/crossref_metadata_2025_split huggingface dataset:
Intestinal obstruction in von Recklinghausen's disease
Authors: Trilling B. , Faucheron J.‐L. 
Published: April 2014

AbstractAimGastrointestinal manifestations occur in up to 25% of patients with neurofibromatosis type 1. This review reports all published cases of acute intestinal obstruction due to neurofibromatosis type 1 and identifies mechanisms of obstruction, the nature of the tumour and the outcome.MethodA systematic review of the literature on acute intestinal obstruction due to neurofibromatosis type 1 was performed by searching the major electronic databases. All relevant references were reviewed for possible inclusion. All the references of the relevant articles were screened for any further articles that were missed in the initial search.ResultsWe identified 25 articles from 1972 to 2013 reporting 25 patients with von Recklinghausen's disease who underwent laparotomy for acute intestinal obstruction. Three further patients were operated on in our institution. The mean age of the patients was 44.2 years and the male/female ratio 15/13. The mechanisms of obstruction were intrinsic obstruction (16), extrinsic obstruction (8) and intussusception (4). Histology was reported to show neurofibroma (19), gastrointestinal stromal tumour (5) and adenocarcinoma (4). Among patients whose outcome was mentioned (17), 10 were asymptomatic after a mean follow‐up of 1.5 years.ConclusionAcute bowel obstruction is a rare manifestation of neurofibromatosis type 1. Surgery is often necessary to treat the complication and to determine the exact nature of the tumour and the prognosis.
Engaging Community Health Centers (CHCs) in Research Partnerships: The Role of Prior Research Experience on Perceived Needs and Challenges
Authors: Beeson Tishra , Jester Michelle , Proser Michelle , Shin Peter 
Published: April 2014

AbstractDespite community health centers’ substantial role in local communities and in the broader safety‐net healthcare system, very limited research has been conducted on community health center research experience, infrastructure, or needs from a national perspective. A national survey of 386 community health centers was conducted in 2011 and 2012 to assess research engagement among community health centers and their perceived needs, barriers, challenges, and facilitators with respect to their involvement in public health and health services research. This paper analyzes the differences between health centers that currently conduct or participate in research and health centers that have no prior research experience to determine whether prior research experience is indicative of different perceived challenges and research needs in community health center settings.
Children's learning of number words in an indigenous farming‐foraging group
Authors: Piantadosi Steven T. , Jara‐Ettinger Julian , Gibson Edward 
Published: April 2014

AbstractWe show that children in the Tsimane', a farming‐foraging group in the Bolivian rain‐forest, learn number words along a similar developmental trajectory to children from industrialized countries. Tsimane' children successively acquire the first three or four number words before fully learning how counting works. However, their learning is substantially delayed relative to children from the United States, Russia, and Japan. The presence of a similar developmental trajectory likely indicates that the incremental stages of numerical knowledge – but not their timing — reflect a fundamental property of number concept acquisition which is relatively independent of language, culture, age, and early education.
The influence of visual and tactile perception on hand control in children with Duchenne muscular dystrophy
Authors: Troise Denise , Yoneyama Simone , Resende Maria Bernadette , Reed Umbertina , Xavier Gilberto Fernando , Hasue Renata 
Published: April 2014

AimTo investigate tactile perception and manual dexterity, with or without visual feedback, in males with Duchenne muscular dystrophy (DMD).MethodForty males with DMD (mean age 9y 8mo, SD 2y 3mo; range 5–14y), recruited from the teaching hospital of the School of Medicine of the University of São Paulo, with disease severity graded as ‘1’ to ‘6’ on the Vignos Scale and ‘1’ on Brooke's Scale, and 49 healthy males (mean age 8y 2mo; range 5–11y; SD 1y 11mo), recruited from a local education center, participated in the study. We assessed tactile perception using two‐point discrimination and stereognosis tests, and manual dexterity using the Pick‐Up test with the eyes either open or closed. Analysis of variance was used to compare groups; a p value of less than 0.05 was considered statistically significant.ResultsMales with DMD exhibited no impairment in tactile perception, as measured by the two‐point discrimination test and the number of objects correctly named in the stereognosis test. Manipulation during stereognosis was statistically slower with both hands (p<0.001), and manual dexterity was much worse in males with DMD when there was no visual feedback (p<0.001).InterpretationMales with DMD exhibited disturbances in manipulation during stereognosis and dexterity tests. Hand control was highly dependent on visual information rather than on tactile perception. Motor dysfunction in males with DMD, therefore, might be related to altered neural control.
Mastery motivation as a predictor of occupational performance following upper limb intervention for school‐aged children with congenital hemiplegia
Authors: Miller Laura , Ziviani Jenny , Ware Robert S , Boyd Roslyn N 
Published: April 2014

AimTo determine the extent to which children's mastery motivation predicts occupational performance outcomes following upper limb intervention (ULI).MethodIn this cohort study, participants received 45 hours of ULI, either in an intensive group‐based or distributed individualized model. The Dimensions of Mastery Questionnaire (DMQ) measured mastery motivation at baseline. Occupational performance outcomes were assessed at baseline and 13 weeks’ post‐intervention using the Canadian Occupational Performance Measure (COPM). Multivariable models determined the contribution of mastery motivation to COPM outcome irrespective of group membership.ResultsForty‐two children with congenital hemiplegia (29 males, 13 females; mean age 7y 8mo [SD 2y 2mo]; range 5y 1mo–12y 8mo; Manual Ability Classification System [MACS] I=20 and II=22; predominant motor type unilateral spastic n=41) participated in the study. Significant gains were seen in COPM performance and satisfaction scores (p<0.001) post‐intervention with no between group differences. Children who had greater persistence with object‐oriented tasks (p=0.02) and better manual ability (p=0.03) achieved higher COPM performance scores at 13 weeks. Children's persistence on object‐oriented tasks was the strongest predictor of COPM satisfaction (p=0.01).InterpretationChildren's persistence with object‐oriented tasks as well as manual abilities needs to be considered when undertaking ULI. Predetermining children's motivational predispositions can assist clinicians to tailor therapy sessions individually based on children's strengths, contributing to effective engagement in ULI.
Diabetes‐specific emotional distress in people with Type 2 diabetes: a comparison between primary and secondary care
Authors: Stoop C. H. , Nefs G. , Pop V. J. , Wijnands‐van Gent C. J. M. , Tack C. J. , Geelhoed‐Duijvestijn P. H. L. M. , Diamant M. , Snoek F. J. , Pouwer F. 
Published: April 2014

AbstractAimsTo compare levels of diabetes distress in people with Type 2 diabetes treated in primary and secondary care and to examine demographic and clinical correlates that may explain potential differences in levels of distress between care settings.MethodsPeople with Type 2 diabetes from 24 primary care practices (n = 774) and three secondary care clinics (n = 526) completed the Problem Areas In Diabetes questionnaire. Data on HbA1c levels and diabetes complications were derived from medical charts. Hierarchical ordinal regression analysis was used to investigate which correlates could explain the potential differences in level of diabetes distress between care settings.ResultsDiabetes distress levels and the prevalence of elevated diabetes distress were considerably lower in the participants treated in primary care (mean (sd) total diabetes distress score 8 (11); 4% of participants with a Problem Areas In Diabetes score ≥ 40) than in secondary care (mean (sd) total diabetes distress score 23 (21); 19% of participants with a Problem Areas In Diabetes score ≥ 40, P < 0.001). In addition to care setting, the following variables were also independently related to diabetes distress: younger age, ethnic minority status, using insulin, having a higher HbA1c level, having a higher BMI and the presence of neuropathy. Other diabetes complications were not independently associated with diabetes distress.ConclusionsIn primary care, lower levels of diabetes distress were reported than in secondary care. The difference in diabetes distress between care settings can be largely, but not fully, explained by specific demographic and clinical characteristics. These results need to be interpreted with caution as they are based on two separate studies, but do call into question the need to screen for diabetes distress in people with Type 2 diabetes in primary care.
The psychosocial impact associated with diabetes‐related amputation
Authors: McDonald S. , Sharpe L. , Blaszczynski A. 
Published: April 2014

AbstractAimsResearch has suggested that the additional impact of a diabetes‐related amputation is associated with poorer physical functioning, poorer psychosocial outcome and greater body image disturbance. However, no study to date has compared patients with diabetes with and without amputation and adequately controlled for additional medical morbidity often found among individuals with an amputation. The aim of this study was to statistically control for any group differences on medical and demographic variables to examine the isolated psychosocial impact of diabetes‐related amputation.MethodsIndividuals with diabetes with an amputation (n = 50) were compared to a control sample (individuals with diabetes without an amputation; n = 240). All participants completed a demographic and medical questionnaire, as well as measures of psychological distress, quality of life and body image.ResultsThe results indicated that, in univariate analyses, depression, physical quality of life and body image disturbance were all poorer in the amputee group. These differences remained for body image disturbance (P = 0.005), but were no longer significant for depression or physical quality of life in multivariate analyses controlling for important demographic and medical variables.ConclusionsThe present study found that the impact of diabetes‐related amputation was significant for body image disturbance. However, it appears that other psychosocial outcomes are better accounted for by medical co‐morbidities common in this group rather than the amputation itself. This research certainly highlights that clinicians must assess for and address all potential medical contributors to psychosocial outcomes, rather than assuming that people will experience poorer outcomes following amputation.
Effectiveness of a group diabetes education programme in under‐served communities in South Africa: a pragmatic cluster randomized controlled trial
Authors: Mash R. J. , Rhode H. , Zwarenstein M. , Rollnick S. , Lombard C. , Steyn K. , Levitt N. 
Published: April 2014

AbstractAimTo evaluate the effectiveness of group education, led by health promoters using a guiding style, for people with Type 2 diabetes in public sector community health centres in Cape Town.MethodsThis was a pragmatic clustered randomized controlled trial with 17 randomly selected intervention and 17 control sites. A total of 860 patients with Type 2 diabetes, regardless of therapy used, were recruited from the control sites and 710 were recruited from the intervention sites. The control sites offered usual care, while the intervention sites offered a total of four monthly sessions of group diabetes education led by a health promoter. Participants were measured at baseline and 12 months later. Primary outcomes were diabetes self‐care activities, 5% weight loss and a 1% reduction in HbA1c levels. Secondary outcomes were self‐efficacy, locus of control, mean blood pressure, mean weight loss, mean waist circumference, mean HbA1c and mean total cholesterol levels and quality of life.ResultsA total of 422 (59.4%) participants in the intervention group did not attend any education sessions. No significant improvement was found in any of the primary or secondary outcomes, apart from a significant reduction in mean systolic (‐4.65 mmHg, 95% CI 9.18 to ‐0.12; P = 0.04) and diastolic blood pressure (‐3.30 mmHg, 95% CI ‐5.35 to ‐1.26; P = 0.002). Process evaluation suggested that there were problems with finding suitable space for group education in these under‐resourced settings, with patient attendance and with full adoption of a guiding style by the health promoters.ConclusionThe reported effectiveness of group diabetes education offered by more highly trained professionals, in well‐resourced settings, was not replicated in the present study, although the reduction in participants' mean blood pressure is likely to be of clinical significance.
Oesophageal function assessed by high‐resolution manometry in patients with diabetes and inadequate glycaemic control
Authors: Roman S. , Marjoux S. , Thivolet C. , Mion F. 
Published: April 2014

AbstractAimsTo describe oesophageal function in people with diabetes and poor glycaemic control using oesophageal high‐resolution manometry and to compare the result between control subjects and patients with gastro‐oesophageal reflux disease.MethodsThe results of oesophageal high‐resolution manometry and a gastric emptying test were reviewed in 20 patients with diabetes. The high‐resolution manometry protocol consisted of 5‐ml swallows of water and multiple swallows of water. Oesophageal motility disorders were classified according to the Chicago classification system. The occurence of multiphasic contractions and intragastric and intrabolus pressures were measured. High‐resolution manometry results were compared between 10 control subjects and 20 patients with gastro‐oesophageal reflux disease. Data were expressed as medians and compared using Mann–Whitney and chi‐squared tests.ResultsOesophageal motility disorders were similarly distributed between the groups. Multiphasic contractions occurred more frequently in patients with diabetes than in those with gastro‐oesophageal reflux disease (60 vs 20% per patient; P<0.01) and were not observed in control subjects. Gastric emptying was delayed in six patients with diabetes and did not correlate with symptoms or oesophageal motility disorders. Intrabolus pressure was higher in patients with diabetes and gastroparesis than in those without (17 vs 10 mmHg; P=0.02) and correlated with intragastric pressure (r=0.46, P<0.01). During multiple swallows of water, oesophageal contractile activity was incompletely inhibited in 83% of patients with diabetes and gastroparesis vs 9% without (P<0.01). Oesophageal function and gastric emptying were not influenced by fasting glycaemia.ConclusionsPatients with gastroparesis might present with impaired inhibition of contractile activity during multiple swallows of water. Increased intrabolus pressure is suggestive of delayed oesophageal clearance as a consequence of gastroparesis.
Nomads no more: early juvenile coho salmon migrants contribute to the adult return
Authors: Bennett Todd R. , Roni Phil , Denton Keith , McHenry Michael , Moses Raymond 
Published: April 2014

AbstractThe downstream movement of coho salmon fry and parr in the fall, as distinct from the spring migration of smolts, has been well documented across the range of the species. In many cases, these fish overwinter in freshwater, but they sometimes enter marine waters. It has long been assumed that these latter fish did not survive to return as adults and were ‘surplus’ to the stream's carrying capacity. From 2004 to 2010, we passive integrated transponder tagged 25,981 juvenile coho salmon in three streams in Washington State to determine their movement, survival and the contribution of various juvenile life histories to the adult escapement. We detected 86 returning adults, of which 32 originated from fall/winter migrants. Half of these fall/winter migrants spent ~1 year in the marine environment, while the other half spent ~2 years. In addition, the median return date for fall/winter migrants was 16 days later than spring migrants. Our results indicated that traditional methods of spring‐only smolt enumeration may underestimate juvenile survival and total smolt production, and also overestimate spring smolt‐to‐adult return (SAR). These are important considerations for coho salmon life cycle models that assume juvenile coho salmon have a fixed life history or use traditional parr‐to‐smolt and SAR rates.
Lineage‐dependent skewing of loss of heterozygosity (LOH) of KRAS gene in a case of juvenile myelomonocytic leukemia
Authors: Tokuda Kiriko , Eguchi‐Ishimae Minenori , Iwabuki Hidehiko , Kawakami Sanae , Tauchi Hisamichi , Ishii Eiichi , Eguchi Mariko 
Published: April 2014

AbstractJuvenile myelomonocytic leukemia (JMML) is a clonal disease arising from abnormal hematopoietic stem cells, although the involvement of lymphoid lineage differs among reported cases. Here, we present a case of JMML with a KRAS G13D mutation. The mutation was detected in various hematopoietic lineages, including T and B lymphocytes and also in lineage− CD34+CD38− hematopoietic stem cells, showing a different percentage of affected cells in each lineage. Single cell‐based analysis of hematopoietic cells revealed the loss of wild‐type KRAS in a significant proportion of G13D‐harboring cells. The percentage of loss of heterozygosity (LOH)/non‐LOH cells showed lineage‐dependent skewing in hematopoietic cells. The loss of the wild‐type KRAS allele may be a common secondary genetic change in KRAS‐related JMML and may affect the differentiation behavior of early JMML progenitors.
Innovative approach for improved rFVIII concentrate
Authors: Morfini Massimo 
Published: April 2014

AbstractThe development of a new recombinant factor VIII was designed and implemented to answer a number of unmet needs of patients affected by hemophilia A. Turoctocog alfa is bioengineered in a specific Chinese hamster ovary clone to present translational and posttranslational characteristics (sulphation, glycosylation) biosimilar to natural circulating forms of FVIII, with the aim to devoid any minimal change which may impact immunogenicity and antigenicity of recombinant protein. Both producer cell line and media are maintained free of any animal or human plasma derivative. Downstream processes of purification are performed by five steps (immunoaffinity chromatography, ion‐exchange chromatography, virus inactivation by means of solvent‐detergent treatment and nanofiltration, and to end with gel filtration), to provide the best possible margin of safety from known and unknown infectious agents. Large clinical trials seem to confirm the expectations placed in Turoctocog alfa in terms of high quality and safety of recombinant FVIII toward the goal of overcoming actual and future challenges of hemophilia therapy.
Anaplastic lymphoma kinase‐positive anaplastic large cell lymphoma: current and future perspectives in adult and paediatric disease
Authors: Eyre Toby A. , Khan Dalia , Hall Georgina W. , Collins Graham P. 
Published: April 2014

AbstractAnaplastic large cell lymphoma (ALCL) is a rare T‐cell lymphoma seen in both adults and children. ALCL is associated with a characteristic chromosomal translocation, t(2;5)(p23;35) which fuses the anaplastic lymphoma kinase (ALK) gene on chromosome 2 with the nucleophosmin (NPM) gene on chromosome 5, resulting in a NPM‐ALK fusion protein, ALK over‐expression and constitutive tyrosine kinase activity. This aggressive lymphoma is more prevalent in males and can present with extranodal involvement (lung, skin and marrow infiltration) and haemophagocytic lymphohistocytosis. The long‐term overall survival is approximately 70–90% in children and over 70% in adults. Staging systems and prognostic risk factors are different in both childhood and adult ALCL. Treatment in adults is typically anthracycline‐based, with autologous stem cell transplantation (ASCT) salvaging patients in relapsed disease. There is evidence for ALL‐like therapy or intensive, pulsed anthracycline‐based induction in children. ASCT, allogeneic SCT and vinblastine maintenance are all considered reasonable options in relapsed childhood disease. The anti‐CD30 immunoconjugate Brentuximab Vedotin and the specific ALK inhibitor Crizotinib are changing the treatment paradigm in ALCL (ALK‐positive or negative) and ALK‐positive ALCL respectively. Both agents have shown encouraging responses in relapsed ALCL. It remains to be seen how these novel agents are used, but it is very possible that they may improve overall responses and survival in both children and adults. This review highlights the presentation, histopathological features, prognostic factors, and evidence‐based treatment approaches in the first line and relapsed setting in ALK‐positive ALCL. The review concludes by discussing the novel approaches using Brentuximab and Crizotinib which are being tested in clinical trials.
Role of paraventricular nucleus‐projecting norepinephrine/epinephrine neurons in acute and chronic stress
Authors: Flak Jonathan N. , Myers Brent , Solomon Matia B. , McKlveen Jessica M. , Krause Eric G. , Herman James P. 
Published: April 2014

AbstractChronic variable stress (CVS) exposure modifies the paraventricular nucleus of the hypothalamus (PVN) in a manner consistent with enhanced central drive of the hypothalamo‐pituitary‐adrenocortical (HPA) axis. As previous reports suggest that post‐stress enhancement of norepinephrine (NE) action contributes to chronic stress regulation at the level of the PVN, we hypothesised that PVN‐projecting NE neurons were necessary for the stress facilitatory effects of CVS. Following intra‐PVN injection of saporin toxin conjugated to a dopamine beta‐hydroxylase (DBH) antibody (DSAP), in rats PVN DBH immunoreactivity was almost completely eliminated, but immunoreactive afferents to other key regions involved in stress integration were spared (e.g. DBH fiber densities were unaffected in the central nucleus of the amygdala). Reductions in DBH‐positive fiber density were associated with reduced numbers of DBH‐immunoreactive neurons in the nucleus of the solitary tract and locus coeruleus. Following 2 weeks of CVS, DSAP injection did not alter stress‐induced adrenal hypertrophy or attenuation of body weight gain, indicating that PVN‐projecting NE [and epinephrine (E)] neurons are not essential for these physiological effects of chronic stress. In response to acute restraint stress, PVN‐targeted DSAP injection attenuated peak adrenocorticotrophic hormone (ACTH) and corticosterone in controls, but only attenuated peak ACTH in CVS animals, suggesting that enhanced adrenal sensitivity compensated for reduced excitatory drive of the PVN. Our data suggest that PVN‐projecting NE/E neurons contribute to the generation of acute stress responses, and are required for HPA axis drive (ACTH release) during chronic stress. However, loss of NE/E drive at the PVN appears to be buffered by compensation at the level of the adrenal.