bluuebunny/crossref_metadata_2025_split huggingface dataset:
Outcomes of splenectomy in patients with common variable immunodeficiency (CVID): a survey of 45 patients
Authors: Wong G K , Goldacker S , Winterhalter C , Grimbacher B , Chapel H , Lucas M , Alecsandru D , McEwen D , Quinti I , Martini H , Schmidt R E , Ernst D , Espanol T , Vidaller A , Carbone J , Fernandez-Cruz E , Lougaris V , Plebani A , Kutukculer N , Gonzalez-Granado L I , Contreras R , Kiani-Alikhan S , Ibrahim M A A , Litzman J , Jones A , Gaspar H B , Hammarstrom L , Baumann U , Warnatz K , Huissoon A P 
Published: November 2012

Summary
Splenectomy has been used in patients with common variable immunodeficiency disorders (CVID), mainly in the context of refractory autoimmune cytopenia and suspected lymphoma, but there are understandable concerns about the potential of compounding an existing immunodeficiency. With increasing use of rituximab as an alternative treatment for refractory autoimmune cytopenia, the role of splenectomy in CVID needs to be re-examined. This retrospective study provides the largest cohesive data set to date describing the outcome of splenectomy in 45 CVID patients in the past 40 years. Splenectomy proved to be an effective long-term treatment in 75% of CVID patients with autoimmune cytopenia, even in some cases when rituximab had failed. Splenectomy does not worsen mortality in CVID and adequate immunoglobulin replacement therapy appears to play a protective role in overwhelming post-splenectomy infections. Future trials comparing the effectiveness and safety of rituximab and splenectomy are needed to provide clearer guidance on the second-line management of autoimmune cytopenia in CVID.
Assessing and addressing cardiovascular risk in adults with Turner syndrome
Authors: Turtle Emma J. , Sule Ashish A. , Bath Louise E. , Denvir Martin , Gebbie Ailsa , Mirsadraee Saeed , Webb David J. 
Published: November 2012

SummaryTurner syndrome (TS), the result of a structurally abnormal or absent X chromosome, occurs in one in 2 000 live born females. The phenotype is highly variable, but short stature and gonadal dysgenesis are usually present. The main objective in adults with TS is health surveillance, but TS still causes a reduction in life expectancy of up to 13 years, with cardiovascular disease, congenital or acquired, as the major cause of an early death. While it has been established that all women with TS should undergo in‐depth cardiovascular examination at diagnosis, advice on the cardiovascular management of women with TS is limited. Here, we provide a summary of our current practice within a multidisciplinary team, supported by our expertise in various aspects of cardiovascular risk management, and the evidence from research where it is available, with the aim of providing optimal support to our patients with TS.BackgroundA dedicated Adult Turner Clinic was established in South East Scotland in 2002. This gynaecology‐led clinic serves a population of roughly 1·2 million and, currently, reviews around 50 women with TS annually. Referrals for adult care come from paediatrics or general practice. Following a series of individual case discussions regarding the management of more complex cardiovascular problems, we have assembled a dedicated multidisciplinary group to determine a timely cardiovascular screening strategy, a basis for specialist referral, and appropriate hypertension management. This team now includes a paediatric endocrinologist, gynaecologist, cardiologist (with an interest in inherited disorders), vascular radiologist and hypertension specialist.Here, we review the literature on cardiovascular disease in women with TS and, make recommendations, based on relatively limited high‐quality evidence, together with our experience, on the optimal timing of cardiovascular screening.
ABC3 Part II: a review of the new criteria for evaluating cervical cytology in England
Authors: Blanks R. G. 
Published: November 2012

R.G. Blanks  ABC3 Part II: a review of the new criteria for evaluating cervical cytology in EnglandThe new Achievable Standards, Benchmarks for Reporting, and Criteria for Evaluating Cervical Cytopathology, 3rd edn (ABC3) includes radical changes in the criteria for evaluating cervical cytology. First, they include a new mission statement ‘the objective of cervical screening is to reduce cervical cancer incidence and mortality by screening with a high sensitivity for the detection of CIN2 or worse, whilst maintaining a high specificity’.Second, the original four performance measurement criteria where laboratories were examined further if they were below the 10th or above the 90th percentile has been changed to three and laboratories are only mandatorily examined if they fall below the 5th or above the 95th percentile. The old criteria related to the percentage of samples that were inadequate, the percentage of all adequate samples reported as moderate dyskaryosis or worse (equivalent to high‐grade squamous intraepithelial lesion or cancer), the percentage of adequate samples reported as mild dyskaryosis or borderline (equivalent to low‐grade squamous intraepithelial lesion or atypical squamous/glandular cells) and the positive predictive value. The new criteria are percentage of inadequate samples, positive predictive value and a new measure termed referral value. These changes mean that far fewer laboratories will require mandatory examination.Third, a raft of optional performance measures have been introduced to help laboratories examine their annual statistical return to the Department of Health in comparison with other laboratories. These measures have been designed to produce a more uniform national programme, and to help laboratories decide whether they are maximizing the benefit of screening while minimizing the harm, which is the goal of all screening programmes. This review examines in detail the new criteria and explains in more detail some of the thinking behind them.
Insulin resistance: a risk marker for disease and disability in the older person
Authors: Krentz A. J. , Viljoen A. , Sinclair A. 
Published: November 2012

AbstractClinical metabolic studies have demonstrated that insulin action declines progressively with age in humans. In addition to its close association with Type 2 diabetes, which reduces life expectancy in older people, age‐related insulin resistance is implicated in pathogenesis of several highly prevalent disorders for which ageing is a major risk factor. These include atherosclerotic cardiovascular disease, dementia, frailty and cancer. Accordingly, insulin resistance may be viewed as biomarker of age‐related ill health and reduced lifespan. The rapidly rising number of older people, coupled with a high prevalence of insulin resistance resulting from obesity and sedentary lifestyles, presents unprecedented public health and societal challenges. Studies of centenarians have shown that preserved whole‐body sensitivity to insulin is associated with longevity. The mechanisms through which insulin action is associated with age‐related diseases remain unclear. Changes in body composition, i.e. sarcopenia and excess adiposity, may be more potent than age per se. Moreover, the impact of insulin resistance has been difficult to disentangle from the clustering of vascular risk factors that co‐segregate with the insulin resistance–hyperinsulinaemia complex. Potentially modifiable mediators of age‐related changes in insulin sensitivity include alterations in adipocytokines, impaired skeletal myocyte mitochondrial function and brown fat activity. The hypothesis that improving or maintaining insulin sensitivity preserves health and extends lifespan merits further evaluation. Practical non‐pharmacological interventions directed against age‐related insulin resistance remain underdeveloped. Novel metabolically active pharmacological agents with theoretical implications for some age‐related disorders are entering clinical trials. However, recent adverse experiences with the thiazolidinediones suggest the need for a cautious approach to the use of insulin sensitizing drugs in older people. This could be particularly important in the absence of diabetes where the risk to benefit analysis may be less favourable.
VEGF‐dependent continuous angiogenesis in the median eminence of adult mice
Authors: Morita S. , Ukai S. , Miyata S. 
Published: November 2012

AbstractBrain vasculature forms the blood–brain barrier (BBB) that restricts the movement of molecules between the brain and blood, but the capillary of the median eminence (ME) lacks the BBB for secretion of adenohypophysial hormone‐releasing peptides. In the present study, we aimed to elucidate whether continuous angiogenesis occurs in the ME of adult mice. By using a mitotic marker, bromodeoxyuridine (BrdU), we demonstrated that new endothelial cells were born continuously in the ME of adults. Prominent expression of NG2, platelet‐derived growth factor receptor B (PDGFRB), and delta‐like ligand 4 was observed at pericytes of adults, although the expression of these angiogenesis‐associated proteins has been shown to be at low or trace levels in adult mature capillary. In addition, vascular endothelial growth factor (VEGF), a key regulator of angiogenesis, was expressed highly in the nervous parenchyma of the ME. Expression of VEGF receptor 2 (VEGFR2) was observed at endothelial cells in the external zone and at somatodendrites in the internal zone. Finally, a VEGFR‐ and PDGFR‐associated tyrosine kinase inhibitor, SU11248, significantly decreased the number of BrdU‐positive proliferating endothelial cells and parenchyma cells. In conclusion, the present study demonstrates VEGF‐dependent continuous angiogenesis in the ME of adult mouse brains under normal conditions, which provides new insight into our understanding of neurosecretion in the ME.
Adolescent gain in positive valence of a socially relevant stimulus: engagement of the mesocorticolimbic reward circuitry
Authors: Bell Margaret R. , De Lorme Kayla C. , Figueira Rayson J. , Kashy Deborah A. , Sisk Cheryl L. 
Published: November 2012

AbstractA successful transition from childhood to adulthood requires adolescent maturation of social information processing. The neurobiological underpinnings of this maturational process remain elusive. This research employed the male Syrian hamster as a tractable animal model for investigating the neural circuitry involved in this critical transition. In this species, adult and juvenile males display different behavioral and neural responses to vaginal secretions, which contain pheromones essential for expression of sexual behavior in adulthood. These studies tested the hypothesis that vaginal secretions acquire positive valence over adolescent development via remodeling of neural circuits underlying sexual reward. Sexually naïve adult, but not juvenile, hamsters showed a conditioned place preference for vaginal secretions. Differences in behavioral response to vaginal secretions between juveniles and adults correlated with a difference in the vaginal secretion‐induced neural activation pattern in mesocorticolimbic reward circuitry. Fos immunoreactivity increased in response to vaginal secretions in the medial amygdala and ventral tegmental dopaminergic cells of both juvenile and adult males. However, only in adults was there a Fos response to vaginal secretions in non‐dopaminergic cells in interfascicular ventral tegmental area, nucleus accumbens core and infralimbic medial prefrontal cortex. These results demonstrate that a socially relevant chemosensory stimulus acquires the status of an unconditioned reward during adolescence, and that this adolescent gain in social reward is correlated with experience‐independent engagement of specific cell groups in reward circuitry.
Malaysian government dentists' experience, willingness and barriers in providing domiciliary care for elderly people
Authors: Othman Akmal Aida , Yusof Zamros , Saub Roslan 
Published: November 2012

ObjectiveTo assess Malaysian government dentists' experience, willingness and barriers in providing domiciliary care for elderly people.Material and methodsA descriptive cross‐sectional study was conducted using a self‐administered postal questionnaire targeting government dentists working in the Ministry of Health in Peninsular Malaysia.ResultsSeven hundred and eleven out of 962 dentists responded with a response rate of 74.0%. Only 36.1% of the dentists had experience in providing domiciliary care for elderly people in the past 2 years with mean number of visit per year of 1. Younger dentists below the age of 30 and those with confidence in providing the service were the most willing to undertake domiciliary care for elderly patients (OR = 13.5, p < 0.05). The 3 most reported barriers were patient's complex medical history (74.1%), patient's poor attitude towards oral health service (67.5%), and dentist's unfavourable working condition (64.4%).ConclusionThe majority of Malaysian government dentists had not been involved in providing domiciliary care for elderly patients. Apart from overcoming the barriers, other recommendations include improving undergraduate dental education, education for elderly people and carers, improving dentist's working condition, and introducing domiciliary financial incentive for dentist.
Use of pharmacokinetic modelling to individualize FFP dosing in factor V deficiency
Authors: Shakhnovich V. , Daniel J. , Wicklund B. , Kearns G. , Neville K. 
Published: November 2012

SummaryTherapy with fresh frozen plasma (FFP) confers serious risks, such as contraction of blood‐borne viruses, allergic reaction, volume overload and development of alloantibodies. The aim of this study was to apply principles of pharmacokinetic (PK) modelling to individual factor content of FFP to optimize individualized dosing, while minimizing potential risks of therapy. We used PK modelling to successfully target individual factor replacement in an 8‐month‐old patient receiving FFP for treatment of a severe congenital factor V (FV) deficiency. The model fit for the FV activity vs. time data was excellent (r = 0.98) and the model accurately predicted FV activity during the intraoperative and postoperative period. Accurate PK modelling of individual factor activity in FFP has the potential to provide better targeted therapy, enabling clinicians to more precisely dose patients requiring coagulation products, while avoiding wasteful and expensive product overtreatment, minimizing potentially life‐threatening complications due to undertreatment and limiting harmful product‐associated risks.
Prognostic value of microsatellite instability in sporadic locally advanced rectal cancer following neoadjuvant radiotherapy
Authors: Du Changzheng , Zhao Jun , Xue Weicheng , Dou Fangyuan , Gu Jin 
Published: November 2012

AimsThis study was conducted to investigate the clinicopathological significance and prognostic value of microsatellite instability (MSI) in locally advanced rectal cancer (LARC) following neoadjuvant radiotherapy.Methods and resultsA total of 316 consecutive patients with LARC who underwent neoadjuvant radiotherapy and curative surgery were included retrospectively. Microsatellite instability in pretreatment biopsy tissue was assessed using the pentaplex panel of mononucleotides. Twenty‐five tumours (7.9%) were assessed as high‐frequency MSI (MSI‐H) and 291 were low‐frequency MSI (MSI‐L; n = 42) or microsatellite stable (MSS; n = 249). There were no significant differences in terms of gender, age, tumour location or pretreatment serum carcinoembryonic antigen between the MSI‐H and MSI‐L + MSS groups. Microsatellite instability was not associated statistically with pathological stage, radiation‐induced tumour regression or downstaging. No significant difference was found in disease‐free survival (DFS) between the two groups but, within the subgroup of ypN0 stage, patients with MSI‐H tumours presented a significantly improved DFS compared with those with MSI‐L or MSS tumours (100% versus 79.8%, P < 0.05), whereas no DFS improvement was observed for patients with MSI‐H tumours in the ypN + subgroup.ConclusionsMicrosatellite instability could not predict a histopathological response to neoadjuvant radiotherapy, but was a good prognostic marker for patients without lymph node metastasis after neoadjuvant radiotherapy.
In vitro digestibility of flaxseed (Linum usitatissimum L.) protein: effect of seed mucilage, oil and thermal processing
Authors: Marambe Harsha K. , Shand Phyllis J. , Wanasundara Janitha P. D. 
Published: November 2012

SummaryFlaxseed (Linum usitatissimum L.) protein is reported to release physiologically important amino acids and bioactive peptides during gastrointestinal (GI) digestion. The effect of seed mucilage and oil and thermal processing on the in vitro protein digestibility (IPD) of flaxseed protein was assessed under simulated GI digestion. Protein in ground whole flaxseed that contained both mucilage and oil had the lowest digestibility (12.61%). Baking and boiling before size reduction significantly (P < 0.05) improved the IPD (31.77% and 28.04%, respectively). Further increase in IPD occurred when mucilage (51.00%) and both mucilage and oil (66.79%) were removed. Isolated flax protein had a similar IPD value (68.00%) as the mucilage and oil–removed flaxseed. The polypeptide of approximately 13 kDa showed resistance to GI digestion compared with other polypeptides of all these treated seeds. Removal of oil and mucilage as well as thermal treatment enhanced protein digestibility of flaxseed.
Manipulation of volatile compound transformation in durian wine by nitrogen supplementation
Authors: Lee Pin‐Rou , Toh Mingzhan , Yu Bin , Curran Philip , Liu Shao‐Quan 
Published: November 2012

SummaryThis study evaluated the impact of added diammonium phosphate (DAP) and a L‐leucine‐L‐phenylalanine mixture (Leu+Phe) on durian wine fermentation by Saccharomyces cerevisiae var. bayanus EC‐1118. Changes in yeast cell population, oBrix, sugars, organic acids and pH were similar in all fermentations, regardless of nitrogen supplementation at a concentration of 150 mg N L−1. The supplementation of Leu+Phe accelerated the utilisation and reduced the formation of volatile sulphur compounds such as diethyl disulphide, ethyl thioacetate and 2‐(ethylthio)ethanol, which were 64.3%, 26.0% and 48.4% lower than the control, respectively. Nevertheless, the supplementation of Leu+Phe heightened the production of isoamyl alcohol, 2‐phenylethyl alcohol and their corresponding esters, especially isoamyl acetate was 1.6 times and 2‐phenylethyl acetate was 26.5 times higher than the control. The supplementation with DAP exerted negligible effects on the volatile composition. The results of this study suggest that the addition of specific amino acids may be a novel approach to manipulating durian wine flavour by suppressing or accentuating the formation of certain aroma compounds.
Rheological and sensory attributes of cream caramel desserts containing fructooligosaccharides as substitute sweeteners
Authors: Protonotariou Styliani V. , Karali Euagelia , Evageliou Vasiliki , Yanniotis Stavros , Mandala Ioanna 
Published: November 2012

SummaryThe objectives of this research were to substitute (10–50%) sucrose with fructooligosaccharides (FOS) in cream caramel dessert and to study the rheological and sensory characteristics of the gels produced. Small deformation experiments showed that the substitution of sucrose with FOS led to a decrease in the solid character of the gels. Moreover, samples in which sucrose was substituted with 20% and 30% FOS exhibited lower gel strength, and sample containing 30% FOS showed more than three times lower gel strength than that with 100% sucrose. However, all samples, albeit fluids, already had a substantial gel‐like character at high temperature. According to large deformation experiments, the strength and rigidity of all samples increased with storage time. The values for both parameters were greater for the samples containing 100% and 90% sucrose. Triangle tests were performed for sensory evaluation. Panellists did not find significant differences in terms of sweetness when sucrose was substituted up to 30%. Storage was significant only for the sample with 100% sucrose, as the sensory characteristics of the sample with 10% FOS were not affected.
Evaluation of NFKB1A variants in patients with knee osteoarthritis
Authors: Hulin‐Curtis S. L. , Sharif M. , Bidwell J. L. , Perry M. J. 
Published: November 2012

SummaryA key feature of osteoarthritis (OA) is articular cartilage loss mediated by numerous catabolic factors including pro‐inflammatory cytokines. Cytokine expression is modulated by the nuclear factor κB (NF‐κB) family of transcription factors that are in turn, regulated by the inhibitor of NF‐κB IκBα encoded by NFKB1A. We examined eight, previously reported common germline polymorphisms to determine whether NFKB1A variants are associated with knee OA. Eight common single‐nucleotide polymorphisms (SNPs) across the NFKB1A gene were genotyped in 189 cases with knee OA and 197 healthy controls. Allele, genotype and haplotype frequencies were compared between case and control groups and stratified according to gender due to the increased prevalence of female OA. Serum concentrations of four biochemical markers elevated in OA were compared with genotype for each knee OA case. None of the SNPs showed an association with knee OA; however, stratification of the data for gender showed an increased frequency of the rs8904 variant allele in the female knee OA case group (P = 0.02). Six common haplotypes were identified (H1–H6). H6 was marginally more prevalent in the knee OA group (P = 0.05). The rs8904 variant was associated with increased levels of hyaluronan (HA), a marker of synovial inflammation at 12 and 24 months compared to baseline levels. The nearby rs696 variant demonstrated increased levels of C‐reactive protein (CRP) at 12 months and HA at 12 and 24 months. A reduction in CRP levels at 12 months was observed for the rs2233419 variant. These findings provide evidence for the association of NFKB1A variants and knee OA.
Venous thromboembolism prophylaxis audit in two Queensland hospitals
Authors: Phillips N. M. , Heazlewood V. J. 
Published: November 2012

AbstractBackgroundVenous thromboembolism (VTE) represents a major public health problem in Australia and worldwide, contributing to hundreds of thousands of deaths each year.AimTo assess adherence to recommended guidelines in a range of clinical settings.MethodsRetrospective, observational study of 955 medical (M), surgical (S) and orthopaedic (O) patient charts of all M, S and O patients admitted during March 2011. Patients on warfarin were excluded from the analysis. Appropriate or inappropriate prophylaxis was assessed according to high, medium and low risk stratification. Patient risk stratification for VTE, suitability of prophylaxis given, adverse events and length of stay were recorded.ResultsNine hundred and thirteen eligible patients were assessed, 54% male, mean age 57 ± 21 years. Regarding the 372 M patients, 235 (63%) were on appropriate prophylaxis, compared with 84% (273/326) S and 78% (168/215) O patients (M to S, P < 0.0001; M to O, P = 0.0002; S to O, P = 0.113). High risk prevalence was 56% in M, 9% in S and 12% in O patients (P < 0.0001). Nine confirmed or possible VTE events occurred (seven M, with five of these on inappropriate prophylaxis). All three bleeding events (one fatal) were in M patients, two of whom had appropriate prophylaxis. Average length of stay was 4.1 ± 5.0, 2.1 ± 3.3 and 2.1 ± 3.8 days (P < 0.001) for M, S and O patients respectively.ConclusionBetter adherence to prophylaxis guidelines is required, especially in M patients where the prevalence of high‐risk VTE is greater.