bluuebunny/crossref_metadata_2025_split huggingface dataset:
Ageing successfully with haemophilia: A multidisciplinary programme
Authors: Boccalandro E. , Mancuso M. E. , Riva S. , Pisaniello D. M. , Ronchetti F. , Santagostino E. , Peyvandi F. , Solimeno L. P. , Mannucci P. M. , Pasta G. 
Published: August 2017

IntroductionPersons with haemophilia (PWH) born before the middle 1970s have spent a substantial part of their lives without the benefits of replacement therapy, that became available on a relative large scale only during the 1970s. As a consequence, this group of PWH, although still relatively young, suffers from musculoskeletal and functional problems that are typical of old people.MethodsWe report herewith the short‐term results of a project based upon a multidisciplinary training programme led by a physiotherapist and an occupational therapist, that was implemented over a period of 12 months in 40 patients with severe or moderate hemophilia A or B born before the middle 1970s and regularly followed‐up at a comprehensive haemophilia treatment centre in Italy. The project was aimed to provide information and skills in order to empower the older PWH carrying physical handicaps and functional limitations that had resulted from the inadequate management in their early ages, and to enable them to cope more efficiently with their crippling disease and prevent further disabilities.Results and ConclusionsThe comparison of the data obtained before and after the 12‐month programme found marginal improvements, but the purpose of this programme was indeed to offer a blueprint for the future. In this respect, the level of satisfaction for the programme was very high and we expect that it will be implemented long‐term by our older PWH.
Subcutaneous diphtheria and tetanus vaccines in children with haemophilia: A pilot study and review of the literature
Authors: Schaefer B. A. , Gruppo R. A. , Mullins E. S. , Tarango C. 
Published: August 2017

IntroductionSubcutaneous (SQ) vaccination has emerged as standard of care in children with severe bleeding disorders to reduce unnecessary factor exposure and avoid provoking an intramuscular bleed, but little is known about comparative immunogenicity to intramuscular (IM) vaccination.AimTo confirm immunogenicity of Diphtheria Tetanus acellular Pertussis (DTaP) vaccines administeredSQto individuals <6 years old with haemophilia.MethodsWe performed a retrospective and prospective pilot study of tetanus and diphtheria antibody titres among patients evaluated at our Haemophilia Treatment Centre between 2015‐2016. Children with haemophilia who had received three to four doses ofDTaP containing vaccine administeredSQwere eligible.ResultsEight children met inclusion criteria. The mean age at the time of diphtheria and tetanus antibody testing was 21.1±17.8 months. All children who receivedSQdiphtheria and tetanus developed a positive antibody titre to both antigens. There was no statistically significant difference in distribution of titre values. The average time between the last dose of vaccine and antibody testing was 6.6±3.9 months amongSQvaccinated subjects. Minor injection site reactions were common withSQvaccines.ConclusionSQadministration of diphtheria and tetanus vaccination appears to be immunogenic in a pilot study of Haemophilia patients and supports this practice as the standard of care for this population.
Prophylaxis use among males with haemophilia B in the United States
Authors: Ullman M. , Zhang Q. C. , Grosse S. D. , Recht M. , Soucie J. M. ,   the Hemophilia Treatment Center Network Investigators
Published: August 2017

IntroductionProphylaxis is considered the optimal treatment for persons with moderate to severe haemophilia (factor activity between 1‐5% of normal and <1% of normal respectively) in countries where safe factor concentrates are available and economically feasible. Historically, prophylactic treatment has not been well studied in the haemophilia B (HB) population due to difficulties in obtaining a sufficiently large sample.AimThis study examines the prevalence of prophylaxis use among a robust sample of persons with HB in the United States and its association with specific demographic and clinical characteristics.MethodsUsing data collected between 1998 and 2011 for the Centers for Disease Control and Prevention's Universal Data Collection project, we analysed data on 2428 males with moderate to severe HB aged 2‐79 years who were seen at 135 federally funded haemophilia treatment centres.ResultsPrevalence of prophylactic treatment in our sample was 35% among children and youth (ages 2‐19) and 14% among adults (age 20 and older). Increased HB prophylaxis use was significantly associated with younger age (<40 years), Hispanic ethnicity, severe disease and self‐infusion, while decreased use was associated with above‐normal body mass index (BMI) in adults. Health care coverage was vital, although type of coverage did not appear to influence access.ConclusionsOur analysis confirms previous reports of lower prevalence of prophylaxis use among individuals with HB compared to those with haemophilia A and adds to the body of knowledge regarding treatment patterns among a historically understudied population.
MiR‐139‐5p inhibits the tumorigenesis and progression of oral squamous carcinoma cells by targeting HOXA9
Authors: Wang Kai , Jin Jun , Ma Tengxiao , Zhai Hongfeng 
Published: August 2017

AbstractOur study sought to clarify the effects of microRNA‐139‐5p (miR‐139‐5p) in the tumorigenesis and progression of oral squamous cell carcinoma (OSCC) by regulating HOXA9. MiR‐139‐5p and HOXA9 expression in OSCC tissues, tumour adjacent tissues, OSCC cells and normal cells were tested by qRT‐PCR. SAS and CAL‐27 cell lines were selected in among four OSCC cell lines and then transfected with miR‐139‐5p mimics, pEGFP‐HOXA9 and cotransfected with miR‐139‐5p mimics + pEGFP‐HOXA9. We used MTT, colony formation, transwell and wound healing assays to analyse cell viability, proliferation, invasion and migration. The target relationship between miR‐139‐5p and HOXA9 was verified by luciferase reporter assay and Western blot, respectively. MiR‐139‐5p was down‐regulated, whereas HOXA9 was up‐regulated in OSCC tissues and cells. The proliferation, invasion and migration ability of SAS and CAL‐27 cells in miR‐139‐5p mimics group were significantly weaker than those in the control group and the miR‐NC group (P < 0.01). MiR‐139‐5p can negatively regulate HOXA9. The proliferation, invasion and migration of SAS and CAL‐27 cells in the miR‐139‐5p mimics + pEGFP‐HOXA9 group were not significantly different from those in the blank control and negative control groups (P > 0.05). Our results indicated that miR‐139‐5p could directly inhibit HOXA9, which might be a potential mechanism in inhibiting the proliferation, invasiveness and migration of OSCC cells.
Adaptive functioning and its associated factors among girl children residing in slum areas of Bhubaneswar, India
Authors: Panigrahi Ansuman , Das Sai C , Sahoo Prabhudarsan 
Published: August 2017

AimAdaptive functioning develops throughout early childhood, and its limitation is a reflection that the child has developmental or emotional problems or even mental retardation. Little is known about the adaptive functioning or developmental status of slum children. The present cross‐sectional study was undertaken during the year 2014 to assess the status of adaptive functioning among girl children aged between 3 and 9 years residing in slum areas of Bhubaneswar and to explore the factors associated with poor adaptive functioning.MethodsStratified multi‐stage cluster random sampling technique was used to select the study population; 256 mother–child pairs from 256 households in selected slum areas were studied. Demographic information was collected, and adaptive functioning was assessed using the modified Vineland Social Maturity Scale. Univariate and multivariate analyses was carried out using Statistical Package for Social Sciences (SPSS) version 21.ResultsOne‐fifth (54, 21%) of the girls sampled had poor adaptive functioning, and 44 (17%) had poor cognitive functioning. Multivariate analysis revealed that the age of the child, parents’ education, presence of stunting in children and attending school/early childhood centre were strong predictors of adaptive functioning in slum children.ConclusionOne‐fifth of girls from slums are developmentally vulnerable; parental education, stunting and early childhood education or exposure to schooling are modifiable factors influencing children's adaptive functioning. Health, education and welfare sectors need to be aware of this so that a multi‐pronged approach can be planned to properly address this issue in one of the most disadvantaged sections of the society.
Complete Genome Sequence of
Sulfuriferula
sp. Strain AH1, a Sulfur-Oxidizing Autotroph Isolated from Weathered Mine Tailings from the Duluth Complex in Minnesota
Authors: Jones Daniel S. , Roepke Elizabeth W. , Hua An An , Flood Beverly E. , Bailey Jake V. 
Published: August 2017

ABSTRACT

We report the closed and annotated genome sequence of
Sulfuriferula
sp. strain AH1. Strain AH1 has a 2,877,007-bp chromosome that includes a partial Sox system for inorganic sulfur oxidation and a complete nitrogen fixation pathway. It also has a single 39,138-bp plasmid with genes for arsenic and mercury resistance.
Neonatal management and outcome after thoracoamniotic shunt placement for fetal hydrothorax
Authors: Witlox Ruben S G M , Klumper Frans J C M , te Pas Arjan B , van Zwet Erik W , Oepkes Dick , Lopriore Enrico 
Published: August 2017

AimTo evaluate the short-term neonatal outcome after fetal thoracoamniotic shunt placement for isolated hydrothorax.MethodsRetrospective evaluation of infants with isolated hydrothorax treated with thoracoamniotic shunt placement at our fetal therapy centre between 2001 and 2016.ResultsIn total 48 fetuses were treated with a thoracoamniotic shunt. All fetuses had signs of hydrops at the time of intervention. Median (IQR) gestational age at shunting was 28.7 (24.4–31.3) weeks. Forty-one of 48 (85%) fetuses were born alive at a median (IQR) gestational age of 34.4 (31.1–36.7) weeks. In one child the course of disease after birth was unknown (this child was excluded from further analyses). After birth, 24/40 (60%) children had signs of pleural effusion and 12/40 (30%) needed a thoracic shunt for continuous pleural drainage. Twenty-one (53%) children required mechanical ventilation, of whom 13 (33%) needed high-frequency ventilation as rescue therapy. Overall 30/40 (75%) infants survived the neonatal period. Neonatal survival rate was significantly higher when infants were born ≥32 weeks’ gestation as compared with <32 weeks: 93% (26/28) versus 33% (4/12), p<0.01.ConclusionPostnatal course of hydropic fetuses treated with thoracoamniotic shunt for isolated hydrothorax is often complicated by respiratory failure and persistent pleural effusions. Neonatal survival is good provided delivery occurs at or after 32 weeks’ gestation.
Perinatal risk factors for neonatal encephalopathy: an unmatched case-control study
Authors: Tann Cally J , Nakakeeto Margaret , Willey Barbara A , Sewegaba Margaret , Webb Emily L , Oke Ibby , Mutuuza Emmanuel Derek , Peebles Donald , Musoke Margaret , Harris Kathryn A , Sebire Neil J , Klein Nigel , Kurinczuk Jennifer J , Elliott Alison M , Robertson Nicola J 
Published: August 2017

ObjectiveNeonatal encephalopathy (NE) is the third leading cause of child mortality. Preclinical studies suggest infection and inflammation can sensitise or precondition the newborn brain to injury. This study examined perinatal risks factor for NE in Uganda.DesignUnmatched case–control study.SettingMulago National Referral Hospital, Kampala, Uganda.Methods210 term infants with NE and 409 unaffected term infants as controls were recruited over 13 months. Data were collected on preconception, antepartum and intrapartum exposures. Blood culture, species-specific bacterial real-time PCR, C reactive protein and placental histology for chorioamnionitis and funisitis identified maternal and early newborn infection and inflammation. Multivariable logistic regression examined associations with NE.ResultsNeonatal bacteraemia (adjusted OR (aOR) 8.67 (95% CI 1.51 to 49.74), n=315) and histological funisitis (aOR 11.80 (95% CI 2.19 to 63.45), n=162) but not chorioamnionitis (aOR 3.20 (95% CI 0.66 to 15.52), n=162) were independent risk factors for NE. Among encephalopathic infants, neonatal case fatality was not significantly higher when exposed to early neonatal bacteraemia (OR 1.65 (95% CI 0.62 to 4.39), n=208). Intrapartum antibiotic use did not improve neonatal survival (p=0.826). After regression analysis, other identified perinatal risk factors (n=619) included hypertension in pregnancy (aOR 3.77), male infant (aOR 2.51), non-cephalic presentation (aOR 5.74), lack of fetal monitoring (aOR 2.75), augmentation (aOR 2.23), obstructed labour (aOR 3.8) and an acute intrapartum event (aOR 8.74).ConclusionsPerinatal infection and inflammation are independent risk factors for NE in this low-resource setting, supporting a role in the aetiological pathway of term brain injury. Intrapartum antibiotic administration did not mitigate against adverse outcomes. The importance of intrapartum risk factors in this sub-Saharan African setting is highlighted.
Can an observational pain assessment tool improve time to analgesia for cognitively impaired older persons? A cluster randomised controlled trial
Authors: Fry Margaret , Chenoweth Lynn , Arendts Glenn 
Published: August 2017

ObjectiveThe primary objective of the study was to measure the impact of an observational pain assessment dementia tool on time from ED arrival to first dose of analgesic medicine.MethodsA multisite cluster randomised controlled trial was conducted to test the Pain Assessment in Advanced Dementia (PAINAD) tool. Patients aged 65 years or older suspected of a long bone fracture were screened for cognitive impairment using the Six-Item Screening (SIS) tool. Patients scoring 4 or less on SIS (intervention sites) were assessed for pain using PAINAD. Control sites, assessed pain using standard methods. The primary outcome was time to first dose of analgesia and was analysed on an intention-to-treat basis with a sensitivity analysis.ResultsWe enrolled 602 patients, of which 323 (54%) were at intervention sites (n=4). The median time to analgesia was 82 min (IQR 45–151 min). There was no statistically significant difference in median time to analgesia for intervention 83 (IQR 48–158 min) and non-intervention 82 min (IQR 41–147 min) sites (p=0.414). After adjusting for age, fracture type, arrival mode and triage category, there remained no significant difference in time to analgesia (HR 0.97, 95% CI 0.80 to 1.17, p=0.74). Of the 602 patients enrolled, 273 actually had cognitive impairment. A sensitivity analysis demonstrated patients at intervention sites received analgesia 13 min sooner (90 vs 103 min, p=0.91).ConclusionUse of the PAINAD was not associated with a shorter time to analgesia, although there was a clinically important but non-significant improvement in the cognitively impaired patient group. Further research is needed to address this clinically important and complex issue.
Reassessing the value of gastroscopy for the investigation of dyspepsia
Authors: Ching Hey-Long , Hale Melissa F , Sidhu Reena , McAlindon Mark E 
Published: August 2017

ObjectiveTo evaluate the diagnostic yield of investigating dyspepsia with oesophagogastroduodenoscopy (OGD) with or without mucosal biopsy.DesignRetrospective service evaluation study.SettingTwo teaching hospitals: The Royal Hallamshire Hospital and Northern General Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, UK.Patients500 patients, 55 years of age and over, who underwent OGD to investigate dyspepsia were included. The study period included a 3-month window. All OGDs were performed on an outpatient basis.InterventionsData were extracted from electronic OGD records within the study period.Main outcome measuresDiagnostic yield provided by endoscopic examination and histological assessment.Results378 patients (75.6%) were reported to have some form of endoscopic abnormality, and 417 patients (83.4%) had biopsies taken. The most common findings at OGD were gastritis (47.2%) and oesophagitis (24.4%). Oesophagogastric malignancy was seen in 1%. Diagnoses made endoscopically or histologically that would not have been appropriately managed by empirical therapies were seen in 16.2%.ConclusionOGD in dyspepsia influences patient management in approximately one-sixth of cases. However, the majority of patients are sufficiently managed withHelicobacter pyloritesting and eradication and/or a trial of proton pump inhibitor therapy. Further non-invasive approaches are needed to identify patients who need endoscopy for biopsy or therapy.
The concordance between component tests and clinical history in British adults with suspected pollen-food syndrome to peanut and hazelnut
Authors: Beck Sarah C , Huissoon Aarnoud P , Collins Donna , Richter Alex G , Krishna Mamidipudi T 
Published: August 2017

BackgroundMild oropharyngeal symptoms to peanut/hazelnut occur in ~30% of patients with pollen-food syndrome (PFS). Component tests are considered a useful adjunct to the diagnosis and may help differentiate PFS from those at a risk of anaphylaxis due to storage protein/lipid transfer protein (LTP) sensitisation.AimsTo assess concordance between component tests and clinical history in suspected PFS to peanut/hazelnut in a specialist clinic.MethodsAdult patients were classified into PFS (group 1, n=69) and PFS with mild systemic symptoms (group 2, n=45) based on clinical history. Specific IgE (sIgE) of ≥0.35 kUA/L was considered positive as per manufacturers’ recommendation. Kappa (κ) inter-rater agreement was calculated for concordance between clinical classification and test profiles.ResultsGroup 1 hazelnut: 85% monosensitised to Cor a1, 12% to storage protein/s or LTP and 3% negative to all components. Group 1 peanut: 41% monosensitised to Ara h8, 44% to storage protein/s or ±LTP and 15% negative to all components. Group 2 hazelnut: 67% monosensitised to Cor a1, 16% sensitised to storage protein/s and 17% negative to all components. Group 2 peanut: 19% monosensitised to Ara h8, 62% sensitised to storage protein/s and/or LTP and 19% negative to all components.SIgE to Ara h8 and Cor a1 were greater in group 1 versus group 2: (median (IQR) kUA/L; hazelnut: 12.1 (7.8-25.2) vs 2.4 (0.36-6.3), p<0.001; peanut: 2.4 (0.10-21.1) vs 0.3 (0-3), p<0.01)).ConclusionConcordance between component tests and clinical history for adults with PFS was good for hazelnut (κ=0.63) but poor for peanut (κ=−0.12). Food challenges are warranted in discordant cases for an accurate diagnosis.
Assessment of orientation practices for ethics consultation at Harvard Medical School-affiliated hospitals
Authors: Zaidi Danish , Kesselheim Jennifer C 
Published: August 2017

BackgroundFew studies have been conducted to assess the quality of orientation practices for ethics advisory committees that conduct ethics consultation. This survey study focused on several Harvard teaching hospitals, exploring orientation quality and committee members’ self-evaluation in the American Society of Bioethics and Humanities (ASBH) ethics consultation competencies.MethodsWe conducted a survey study that involved 116 members and 16 chairs of ethics advisory committees, respectively (52% and 62.5% response rates). Predictor variables included professional demographics, duration on committees and level of training. Outcome variables included familiarity with and preparedness in the ASBH competencies and satisfaction with orientations. We hypothesised that responses would be associated with both the aforementioned predictors and whether or not participants had encountered the ASBH competencies in training.ResultsA majority of respondents found their orientation curricula to be helpful (62%), although a significant portion of respondents did not receive any orientation (24%) or were unsatisfied with their orientation (14%). Familiarity with ASBH competencies was a statistically significant predictor of respondents’ self-evaluation in particular categories (54% had heard of the competencies). Standard educational materials were reported as offered during orientation, such as readings (50%) and case studies (41%); different medium resources were less evidenced such as videos on ethics consultation (19%).ConclusionsInstitutions should re-evaluate orientation practices for ethics committee members that perform ethics consultation. Integrating ASBH competencies and useful methods into a resourceful pedagogy will help improve both member satisfaction with orientation and preparation in consultation.
Medical students’ perceptions of professional misconduct: relationship with typology and year of programme
Authors: Zulkifli Juliana , Noel Brad , Bennett Deirdre , O’Flynn Siun , O’Tuathaigh Colm 
Published: August 2017

AimTo examine the contribution of programme year and demographic factors to medical students’ perceptions of evidence-based classification categories of professional misconduct.MethodsStudents at an Irish medical school were administered a cross-sectional survey comprising 31 vignettes of professional misconduct, which mapped onto a 12-category classification system. Students scored each item using a 5-point Likert scale, where 1 represents the least severe form of misconduct and 5 the most severe.ResultsOf the 1012 eligible respondents, 561 students completed the survey, providing a response rate of 55%. Items pertaining to disclosure of conflict of interest were ranked as the least severe examples of professional misconduct, and this perception was highest among finalyear students. While ratings of severity declined for items related to ‘inappropriate conduct not in relation to patient’ and ‘inappropriate use of social media’ between years 1 and 3, ratings for both categories increased again among clinical cycle (fourth and final year) students.ConclusionsIncreased clinical exposure during years 4 and 5 of the undergraduate programme was associated with better recognition of the importance of selected professional domains. Disclosure of conflict of interest is identified as an area of medical professionalism that requires greater emphasis for students who are at the point of transition from student to doctor.