Characterising the nutritional status of children with primary ciliary dyskinesia.

INTRODUCTION: Primary ciliary dyskinesia (PCD) is a rare, heterogeneous genetic disorder where impaired mucociliary clearance is caused by dysfunctional motile cilia leading to bronchiectasis. There is limited evidence characterising the nutritional status of children with PCD, although lower body mass index (BMI) z-score has been associated with worse lung function (FEV1). METHODS: All children (n = 43) with PCD, aged <16 years, from a single tertiary centre were prospectively enrolled. Information on clinical phenotype and nutritional status including bioelectrical impedance spectroscopy (BIS) phase-angle was collected. RESULTS: There was a weak positive association between height-for-age z-score (HAZ) and FEV1 z-score (n = 28, r = 0.4, p = 0.049). Those with a low fat free mass index (<-2 z scores) had a lower BMI z score (-1.3 ± 1.2 vs. 0.8 ± 0.7, p = 0.0002). BIS phase angle identified more patients at nutritional risk than using moderate malnutrition cut-offs of either HAZ or BMI ≤ -2 z scores alone (21% vs. 4.6% vs. 6.9% respectively). PCD patients had a higher incidence of vitamin D insufficiency (<50 nmoL/L) (54%) and deficiency (<30 nmoL/L) (26%) than healthy children. CONCLUSIONS: We have characterised the nutritional phenotype of a cohort of children with PCD. Monitoring vitamin D levels is important in PCD patients. There is a weak association between lung function and nutritional status, and measures of BIS phase-angle. The use of BIS phase-angle may allow for early identification of at risk children and may therefore be of benefit for nutritional assessments in the clinical setting. These findings will help inform a future nutritional intervention strategy in children with PCD.

Bioimpedance spectroscopy measurements of phase angle and height for age are predictive of outcome in children following surgery for congenital heart disease.

OBJECTIVE: Children with congenital heart disease (CHD) are often growth restricted (low weight- and/or height-for-age) which may increase risk of poor post operative resilience. Bioelectrical impedance spectroscopy (BIS) has been used to determine body composition in different clinical settings and has been shown to mark differences in nutritional state and clinical outcome. In disease conditions were fluid is not normally distributed it is proposed that raw impedance values and BIS derived phase-angle may serve as prognostic indicators of clinical outcome. We sought to describe the relationship between nutritional status, phase-angle and post-operative outcomes in children with congenital heart disease. DESIGN: Single centre prospective cohort study. SETTING: Paediatric Intensive Care Unit (PICU), Southampton Children's Hospital. PATIENTS: 122 children with CHD following cardiac surgery (March 2015-April 2016). Outcome variables included growth, mechanical-ventilation, PICU length of stay (PICU-LOS) and phase-angle at 50 Hz. MEASUREMENTS AND MAIN RESULTS: BIS measurements were taken before and on the day of surgery (day 0), day 2 post-operatively and on discharge from hospital. Pre-operative moderate malnutrition defined as height-for-age-z-score (HAZ) ≤-2 was observed in 28.5% of infants and 20.6% of children. Regression analysis was used to investigate the relationship between phase-angle, HAZ and clinical outcomes. Moderate-malnutrition (HAZ ≤-2) was associated with an increased PICU-LOS (odds ratios (OR) with 95% confidence interval: 1.8; 1.1-2.7, p = 0.008) whilst a low phase-angle (≤2.7° on day 2 was associated with longer PICU-LOS (OR 7.8; 2.7-22.45, p < 0.001)); When the model was adjusted for age, known risk factors and length of surgery, HAZ ≤-2 and phase-angle ≤2.7° on day 2 were associated with longer PICU-LOS (p = 0.001 and p = 0.04 respectively) and together explained 81.7% of the variability in PICU-LOS. CONCLUSIONS: Moderate malnutrition (HAZ ≤-2) in infants and children undergoing cardiac surgery is associated with longer PICU-LOS. Post-operative measures of BIS phase angle may further improve our ability to identifying hose children with an increased risk of prolonged PICU-LOS compared to using pre-operative anthropometry alone.

Botulism challenge studies of a modified atmosphere package for fresh mussels: inoculated pack studies.

A series of botulism challenge studies were performed to determine the possibility of production of botulinum toxin in mussels (Mytilus edulis) held under a commercial high-oxygen (60 to 65% O(2)), modified atmosphere packaging (MAP) condition. Spore mixtures of six strains of nonproteolytic Clostridium botulinum were introduced into mussel MAP packages receiving different packaging buffers with or without the addition of lactic acid bacteria. Dye studies and package flipping trials were conducted to ensure internalization of spores by packed mussels. Inoculated mussel packages were stored at normal (4°C) and abusive (12°C) temperatures for 21 and 13 days, respectively, which were beyond the packaged mussels' intended shelf life. Microbiological and chemical analyses were conducted at predetermined intervals (a total of five sampling times at each temperature), including total aerobic plate counts, C. botulinum counts, lactic acid bacterial counts, package headspace gas composition, pH of packaging buffer and mussel meat, and botulinum toxin assays of packaging buffer and mussel meat. Results revealed that C. botulinum inoculated in fresh mussels packed under MAP packaging did not produce toxin, even at an abusive storage temperature and when held beyond their shelf life. No evidence was found that packaging buffers or gas composition influenced the lack of botulinum toxin production in packed mussels.

Total hip replacement for the treatment of acute femoral neck fractures: results from the National Joint Registry of England and Wales at 3-5 years after surgery.

INTRODUCTION: This paper describes, for the first time, the outcomes of patients undergoing total hip replacement for acute fractured neck of femur (#NOF) as recorded by the National Joint Registry of England and Wales (NJR). METHODS: In the NJR we identified 1,302 of 157,232 Hospital Episode Statistics linked patients who had been recorded as having a total hip replacement for acute #NOF between April 2003 and November 2008. RESULTS: The revision rate at five years for fully uncemented components was 4.1% (95% confidence interval [CI]: 2.2-7.3%), for hybrid it was 2.2% (95% CI: 0.9%-5.3%) and for fully cemented components 0.9% (95% CI: 0.4-2.0%). Five-year revision rates were increased for those whose operations were performed via a posterior versus a lateral approach. The Kaplan-Meier estimate of 30-day mortality was 1.4% (95% CI: 1.0-2.4%), which is over double the 30-day mortality rate for total hip replacement identified by the Office for National Statistics. The mean length of stay was also increased for those undergoing total hip replacements for #NOF compared with non-emergency indications. CONCLUSIONS: Our data suggest that total hip replacements for acute #NOF give comparable results with total hip replacements for other indications.

Case report: spontaneous coronary artery dissection during elective caesarean section under spinal anaesthesia.

A 37-year-old woman, otherwise healthy, had an acute myocardial infarction associated with acute left ventricular failure during an elective caesarean section under spinal anaesthesia. Emergency coronary catheterisation postoperatively revealed a probable dissection distally in the D1 branch of the left anterior descending artery. We review the recent literature and case reports on spontaneous coronary artery dissection, and discuss its recommended management.

The genetic characterisation of stripe rust resistance in the German wheat cultivar Alcedo.

Stripe rust resistance in the German winter wheat cv. Alcedo has been described as durable, the resistance having remained effective when grown extensively in Germany and Eastern Europe between 1975 and 1989. Genetic characterisation of field resistance in a cross between Alcedo and the stripe rust susceptible UK winter wheat cv. Brigadier identified two major QTL in Alcedo located on the long arms of chromosomes 2D (QPst.jic-2D) and 4B (QPst.jic-4B). Stripe rust resistance was evaluated by measuring the extent of fungal growth, percentage infection (Pi) and the necrotic/chlorotic response of the plant to infection, infection type (IT). Both QPst.jic-2D and QPst.jic-4B contributed significantly to the reduction in stripe rust infection (Pi), with QPst.jic-2D explaining up to 36.20% and QPst.jic-4B 28.90% of the phenotypic variation measured for Pi. Both QTL were identified by the IT phenotypic scores, with QPst.jic-2D in particular being associated with a strong necrotic phenotype (low IT), QPst.jic-2D explaining up to 53.10% of IT phenotypic variation and QPst.jic-4B 22.30%. In addition, two small effect QTL for field stripe rust resistance were identified in Brigadier, QPst.jic-1B on the long arm of chromosome 1B and QPst.jic-5A on the short arm of chromosome 5A. The influence of QPst.jic-1B was primarily seen with the Pi phenotype, contributing up to 13.10% of the explained phenotypic variation. QPst.jic-5A was only detected using an approximate multiple-QTL model and selecting markers linked to the major effect QTL, QPst.jic-2D and QPst.jic-4B as co-factors. Seedling stripe rust resistance was also mapped in the cross, which confirmed the location of Yr17 from Brigadier to the short arm of chromosome 2A. A seedling expressed QTL was also located in Alcedo that mapped to the same location as the field stripe rust resistance QPst.jic-2D.

Cognitive-behavioural therapy for adolescents with bulimic symptomatology: the acceptability and effectiveness of internet-based delivery.

BACKGROUND: The evidence base for the treatment of adolescents with bulimia nervosa (BN) is limited. AIMS: To assess the feasibility, acceptability, and clinical outcomes of a web-based cognitive-behavioural (CBT) intervention for adolescents with bulimic symptomatology. METHOD: 101 participants were recruited from eating disorders clinics or from beat, a UK-wide eating disorders charity. The programme consisted of online CBT sessions ('Overcoming Bulimia Online'), peer support via message boards, and email support from a clinician. Participants' bulimic symptomatology and service utilisation were assessed by interview at baseline and at three and six months. Participants' views of the treatment package were also determined. RESULTS: There were significant improvements in eating disorder symptoms and service contacts from baseline to three months, which were maintained at six months. Participants' views of the intervention were positive. CONCLUSIONS: The intervention has the potential for use as a first step in the treatment of adolescents with bulimic symptomatology.

QTL identification and microphenotype characterisation of the developmentally regulated yellow rust resistance in the UK wheat cultivar Guardian.

Yellow rust (causal agent: Puccinia striiformis f.sp. tritici) resistance in the UK wheat cultivar Guardian is developmentally regulated, resistance increasing as the plant matures. Yellow rust resistance was assessed under field conditions on plants after ear emergence to ensure maximum expression of resistance. Three quantitative trait loci (QTL) for yellow rust resistance were identified, being located on chromosomes 1B (QPst.jic-1B), 2D (QPst.jic-2D) and 4B (QPst.jic-4B). The largest resistance effect, QPst.jic-1B located to the same position on the long arm of chromosome 1B as the known durable source of yellow rust resistance, Yr29. Microscopic studies were carried out to determine what effect the resistance in Guardian had on the development of P. striiformis f.sp. tritici. While the adult plant resistance in Guardian did not prevent germinated urediniospores from establishing an effective infection site, the growth of hyphae within flag leaf tissue was significantly inhibited, slowing the development of microcolonies. 3,3-diaminabenzadine (DAB) and trypan blue staining indicated that this inhibition of hyphal growth was not associated with hydrogen peroxide accumulation or extensive plant cell death.

More realistic soil cleanup standards with dual-equilibrium desorption.

The desorption of contaminants from soils/sediments is one of the most important processes controlling contaminant transport and environmental risks. None of the currently adopted desorption models can accurately quantify desorption at relatively low concentrations; these models often overestimate the desorption and thus the risks of hydrophobic organic chemicals, such as benzene and chlorinated solvents. In reality, desorption is generally found to be biphasic, with two soil-phase compartments. A new dual-equilibrium desorption (DED) model has been developed to account for the biphasic desorption. This model has been tested using a wide range of laboratory and field data and has been used to explain key observations related to underground storage tank plumes. The DED model relates the amount of a chemical sorbed to the aqueous concentration, with simple parameters including octanol-water partition coefficient, solubility, and fractional organic carbon; thus, it is the only biphasic model, to date, that is based on readily available parameters. The DED model can be easily incorporated into standard risk and transport models. According to this model, many regulatory standards of soils and sediments could be increased without increasing the risks.

An ethics core curriculum for Australasian medical Schools.

Teaching ethics incorporates teaching of knowledge as well as skills and attitudes. Each of these requires different teaching and assessment methods. A core curriculum of ethics knowledge must address both the foundations of ethics and specific ethical topics. Ethical skills teaching focuses on the development of ethical awareness, moral reasoning, communication and collaborative action skills. Attitudes that are important for medical students to develop include honesty, integrity and trustworthiness, empathy and compassion, respect, and responsibility, as well as critical self-appraisal and commitment to lifelong education.

Influence of plant development and environment on transgene expression in potato and consequences for insect resistance.

Clonal replicates of different transformed potato plants expressing transgene constructs containing the constitutive Cauliflower Mosaic Virus (CaMV) 35S promoter, and sequences encoding the plant defensive proteins snowdrop lectin (Galanthus nivalis agglutinin; GNA), and bean chitinase (BCH) were propagated in tissue culture. Plants were grown to maturity, at first under controlled environmental conditions, and later in the glasshouse. For a given transgene product, protein accumulation was found to vary between the different lines of clonal replicates (where each line was derived from a single primary transformant plant), as expected. However, variability was also found to exist within each line of clonal replicates, comparable to the variation of mean expression levels observed between the different clonal lines. Levels of GNA, accumulated in different parts of a transgenic potato plant, also showed variation but to a lesser extent than plant-plant variation in expression. With the majority of the clonal lines investigated, accumulation of the transgene product was found to increase as the potato plant developed, with maximum levels found in mature plants. The variation in accumulation of GNA among transgenic plants within a line of clonal replicates was exploited to demonstrate that the enhanced resistance towards larvae of the tomato moth, Lacanobia oleracea L., caused by expression of this protein in potato, was directly correlated with the level of GNA present in the plants, and that conditions under which the plants were grown affect the levels of GNA expression and subsequent levels of insect resistance.

Plant transformation technology. Developments and applications.

Plant transformation has its roots in the research on Agrobacterium that was being undertaken in the early 1980s. The last two decades have seen significant developments in plant transformation technology, such that a large number of transgenic crop plants have now been released for commercial production. Advances in the technology have been due to development of a range of Agrobacterium-mediated and direct DNA delivery techniques, along with appropriate tissue culture techniques for regenerating whole plants from plant cells or tissues in a large number of species. In addition, parallel developments in molecular biology have greatly extended the range of investigations to which plant transformation technology can be applied. Research in plant transformation is concentrating now not so much on the introduction of DNA into plant cells, but rather more on the problems associated with stable integration and reliable expression of the DNA once it has been integrated.

Biomedicine, genetics and disability: reflections on nursing and a philosophy of holism.

This article critically explores the notion of those sociopolitical spaces that are 'disability', 'holism' and 'genetics', arguing from the perspectives of someone who identifies as having a disability. Medical genetics is seen to reflect the ideology and dominant biomedical reductionist thought. In contrast with this, it is proposed that disability and health are inherently social. A nursing approach is seen to recognize the social and holistic nature of the human person and to present a critical reflection on the reductionistic applications of medical genetics.

Economic rationalism and the cost efficiency of hospital chaplaincy: an Australian study.

Health care reform is also occurring in Australia and effects hospital chaplaincy programs. "Economic rationalism" is the philosophic foundation of this effort and its contrast with the values inherit in hospital chaplaincy are highlighted. Selected research results from the Australian system are described and the authors offer a perspective on the cost efficiency of hospital chaplaincy.

Childhood abuse and sexual revictimization in a female Navy recruit sample.

To examine effects of childhood abuse on adult rape, 1,887 female Navy recruits were surveyed. Overall 35% of recruits had been raped and 57% had experienced childhood physical abuse (CPA) and/or childhood sexual abuse (CSA). Controlling for CPA, rape was significantly (4.8 times) more likely among women who had experienced CSA than among women who had not. In contrast, CPA (controlling for CSA) was unrelated to likelihood of adult rape. Alcohol problems and number of sex partners were examined as mediators. Although both variables predicted rape, their effects were independent of the effects of CSA. Finally, despite ethnic group differences in the prevalence of victimization, the predictors of rape did not differ significantly across ethnic groups.

The social nature of disability, disease and genetics: a response to Gillam, Persson, Holtug, Draper and Chadwick.

The dominance of the biomedically informed view of disability, genetics, and diagnosis is explored. An understanding of the social nature of disability and genetics, especially in terms of oppression, adds a richer dimension to an understanding of ethical issues pertaining to genetics. This is much wider than the limited question of whether or not such technology discriminates. Instead, it is proposed that such technology will perpetuate the oppression and control of people with disability, especially if the knowledge of people with disability is not utilised in bioethical debates.

Digestive proteolytic activity in larvae of tomato moth, Lacanobia oleracea; effects of plant protease inhibitors in vitro and in vivo.

Three distinct digestive protease activities, with strongly alkaline pH optima, were identified in the gut of tomato moth (Lacanobia oleracea) larvae, and characterised using specific synthetic substrates and inhibitors. These were; a trypsin-like activity, a chymotrypsin-like activity specific for substrates and inhibitors containing more than one amino acid residue, and an elastase-like activity, accounting for 40%, 30% and 20% of overall proteolysis respectively. The protease activities differed in their sensitivities to inhibition by different plant protein protease inhibitors (PIs), as estimated by I(50) values. Soya bean Kunitz trypsin inhibitor (SKTI) was the only plant PI tested to inhibit all three digestive protease activities at concentrations <40 &mgr;g/ml (approx. 5x10(-6)M). Incorporation of SKTI into a potato leaf-based artificial diet at 2% of total protein, decreased larval survival and growth (by approx. 33% and 40% respectively after 21 days) and retarded development (by approx. 2 days). However, when SKTI was expressed in transgenic potato plants at approx. 0.5% of total protein, only marginal effects on L. oleracea larvae were observed, which decreased with time. Whilst the presence of SKTI in artificial diet increased endogenous larval trypsin-like activity by up to four-fold, no effects on this activity were observed in larvae feeding on transgenic plants.

Expression and characterisation of single-chain antibody fragments produced in transgenic plants against the organic herbicides atrazine and paraquat.

Single-chain antibody fragments (scAbs), which have a human C-kappa constant domain and a hexa-histidine tail attached to the carboxy terminus of the single-chain Fv (ScFv) fragments to facilitate purification, have been raised against the herbicides paraquat and atrazine and expressed in transgenic Nicotiana tabacum cv. Samsun NN. Prior to purification, the anti-atrazine scAb is expressed as up to 0.014% of soluble leaf protein and has a binding profile in ELISA, against an atrazine-bovine serum albumin (BSA) conjugate, similar to that of the scAb produced in Escherichia coli. Competition ELISA has shown that the plant-derived scAb also recognises free atrazine. Following antibody affinity purification to isolate dimers, the affinity for immobilised antigen approaches that of the parental monoclonal antibody. This was confirmed by surface plasmon resonance analysis. The purified scAb also recognises related triazine herbicides. When isolated from cell-suspension cultures, the anti-paraquat scAb binds to a paraquat conjugate in a concentration-dependent manner, with a profile similar to the parental monoclonal antibody. This is the first demonstration that functional scAbs against organic pollutants can be produced in transgenic plants and that the scAbs may be appropriate for the development of immunoassay-based detection systems.