Consumer Acceptability and Sensory Profile of Three New Celery (Apium graveolens) Hybrids and Their Parental Genotypes.

Celery is a stalky green vegetable that is grown and consumed globally and used in many cuisines for its distinctive taste and flavour. Previous investigations identified the aroma composition of celery and profiled its sensory characteristics using a trained panel; however, evaluation of the sensory characteristics of celery combined with a consumer panel, where consumer preferences and acceptability are determined, is novel. In this study, three parental genotypes (12, 22 and 25) and three new hybrids (12x22, 22x12 and 25x12) were presented to a trained sensory panel (n = 12) for profiling and a consumer panel (n = 118), where liking and preference were assessed. Celery samples were analysed by SPME GC-MS and significant differences in aroma composition between all samples were identified, causing significant differences in the sensory profile. Furthermore, significant differences in attributes assessed for liking (appearance, aroma, texture and overall) were identified. Consumer segmentation identified three groups of consumers exhibiting differences in the hedonic reaction to the samples. Sweet and bitter taste along with overall flavour were identified as drivers of liking. Hybrid 25x12 was found to be the hybrid that exhibited high intensities for most of the attributes assessed.

Investigating the Relationship of Genotype and Geographical Location on Volatile Composition and Sensory Profile of Celery (Apium graveolens).

Numerous varieties of celery are grown in multiple countries to maintain supply, demand and availability for all seasons; thus, there is an expectation for a consistent product in terms of taste, flavour, and overall quality. Differences in climate, agronomy and soil composition will all contribute to inconsistencies. This study investigated the volatile and sensory profile of eight celery genotypes grown in the UK (2018) and Spain (2019). Headspace analysis determined the volatile composition of eight genotypes, followed by assessment of the sensory profile using a trained panel. Significant differences in the volatile composition and sensory profile were observed; genotype and geographical location both exerted influences. Two genotypes exhibited similar aroma composition and sensory profile in both locations, making them good candidates to drive breeding programmes aimed at producing varieties that consistently display these distinctive sensory properties. Celery samples harvested in the UK exhibited a higher proportion of sesquiterpenes and phthalides, whereas samples harvested in Spain expressed a higher aldehyde and ketone content. Studying the relationship between growing environment and genotype will provide information to guide growers in how to consistently produce a high-quality crop.

The effects of elevated temperature and PCO2 on the energetics and haemolymph pH homeostasis of juveniles of the European lobster, Homarus gammarus.

Regulation of extracellular acid-base balance, while maintaining energy metabolism, is recognised as an important aspect when defining an organism's sensitivity to environmental changes. This study investigated the haemolymph buffering capacity and energy metabolism (oxygen consumption, haemolymph [l-lactate] and [protein]) in early benthic juveniles (carapace length <40 mm) of the European lobster, Homarus gammarus, exposed to elevated temperature and PCO2 At 13°C, H. gammarus juveniles were able to fully compensate for acid-base disturbances caused by the exposure to elevated seawater PCO2  at levels associated with ocean acidification and carbon dioxide capture and storage (CCS) leakage scenarios, via haemolymph [HCO3-] regulation. However, metabolic rate remained constant and food consumption decreased under elevated PCO2 , indicating reduced energy availability. Juveniles at 17°C showed no ability to actively compensate haemolymph pH, resulting in decreased haemolymph pH particularly under CCS conditions. Early benthic juvenile lobsters at 17°C were not able to increase energy intake to offset increased energy demand and therefore appear to be unable to respond to acid-base disturbances due to increased PCO2 at elevated temperature. Analysis of haemolymph metabolites suggests that, even under control conditions, juveniles were energetically limited. They exhibited high haemolymph [l-lactate], indicating recourse to anaerobic metabolism. Low haemolymph [protein] was linked to minimal non-bicarbonate buffering and reduced oxygen transport capacity. We discuss these results in the context of potential impacts of ongoing ocean change and CCS leakage scenarios on the development of juvenile H. gammarus and future lobster populations and stocks.

Potential predatory and legitimate biomedical journals: can you tell the difference? A cross-sectional comparison.

BACKGROUND: The Internet has transformed scholarly publishing, most notably, by the introduction of open access publishing. Recently, there has been a rise of online journals characterized as 'predatory', which actively solicit manuscripts and charge publications fees without providing robust peer review and editorial services. We carried out a cross-sectional comparison of characteristics of potential predatory, legitimate open access, and legitimate subscription-based biomedical journals. METHODS: On July 10, 2014, scholarly journals from each of the following groups were identified - potential predatory journals (source: Beall's List), presumed legitimate, fully open access journals (source: PubMed Central), and presumed legitimate subscription-based (including hybrid) journals (source: Abridged Index Medicus). MEDLINE journal inclusion criteria were used to screen and identify biomedical journals from within the potential predatory journals group. One hundred journals from each group were randomly selected. Journal characteristics (e.g., website integrity, look and feel, editors and staff, editorial/peer review process, instructions to authors, publication model, copyright and licensing, journal location, and contact) were collected by one assessor and verified by a second. Summary statistics were calculated. RESULTS: Ninety-three predatory journals, 99 open access, and 100 subscription-based journals were analyzed; exclusions were due to website unavailability. Many more predatory journals' homepages contained spelling errors (61/93, 66%) and distorted or potentially unauthorized images (59/93, 63%) compared to open access journals (6/99, 6% and 5/99, 5%, respectively) and subscription-based journals (3/100, 3% and 1/100, 1%, respectively). Thirty-one (33%) predatory journals promoted a bogus impact metric - the Index Copernicus Value - versus three (3%) open access journals and no subscription-based journals. Nearly three quarters (n = 66, 73%) of predatory journals had editors or editorial board members whose affiliation with the journal was unverified versus two (2%) open access journals and one (1%) subscription-based journal in which this was the case. Predatory journals charge a considerably smaller publication fee (median $100 USD, IQR $63-$150) than open access journals ($1865 USD, IQR $800-$2205) and subscription-based hybrid journals ($3000 USD, IQR $2500-$3000). CONCLUSIONS: We identified 13 evidence-based characteristics by which predatory journals may potentially be distinguished from presumed legitimate journals. These may be useful for authors who are assessing journals for possible submission or for others, such as universities evaluating candidates' publications as part of the hiring process.

Breastfeeding for procedural pain in infants beyond the neonatal period.

BACKGROUND: Randomised controlled trials (RCTs) show that breastfeeding newborn infants during painful procedures reduces pain. Mechanisms are considered to be multifactorial and include sucking, skin-to-skin contact, warmth, rocking, sound and smell of the mother, and possibly endogenous opiates present in the breast milk. OBJECTIVES: To determine the effect of breastfeeding on procedural pain in infants beyond the neonatal period (first 28 days of life) up to one year of age compared to no intervention, placebo, parental holding, skin-to-skin contact, expressed breast milk, formula milk, bottle feeding, sweet-tasting solutions (e.g. sucrose or glucose), distraction, or other interventions. SEARCH METHODS: We searched the following databases to 18 February 2016: the Cochrane Central Register of Controlled Trials (CENTRAL) (the Cochrane Library), MEDLINE including In-Process & Other Non-Indexed Citations (OVID), Embase (OVID), PsycINFO (OVID), and CINAHL (EBSCO); the metaRegister of Controlled Trials (mRCT), ClinicalTrials.gov (clinicaltrials.gov), and the World Health Organization International Clinical Trials Registry Platform (WHO ICTRP) (apps.who.int/trialsearch/) for ongoing trials. SELECTION CRITERIA: We included RCTs and quasi-RCTs involving infants aged 28 days postnatal to 12 months and receiving breastfeeding while undergoing a painful procedure. Comparators included, but were not limited to, oral administration of water, sweet-tasting solutions, expressed breast or formula milk, no intervention, use of pacifiers, positioning, cuddling, distraction, topical anaesthetics, and skin-to-skin care. Procedures included, but were not limited to: subcutaneous or intramuscular injection, venipuncture, intravenous line insertion, heel lance, and finger lance. We applied no language restrictions. DATA COLLECTION AND ANALYSIS: We used standard methodological procedures expected by Cochrane. Two review authors independently considered trials for inclusion in the review, assessed risk of bias, and extracted data. The main outcome measures were behavioural or physiological indicators and composite pain scores, as well as other clinically important outcomes reported by the authors of included studies. We pooled data for the most comparable outcomes and where data from at least two studies could be included. We used mean difference (MD) with 95% confidence interval (CI), employing a random-effects model for continuous outcomes measured on the same scales. For continuous outcomes measured on different scales, we pooled standardised mean differences (SMDs) and associated 95% CIs. For dichotomous outcomes, we planned to pool events between groups across studies using risk ratios (RRs) and 95% CIs. However, as insufficient studies reported dichotomous outcomes, we did not pool such events. We assessed the evidence using GRADE and created a 'Summary of findings' table. MAIN RESULTS: We included 10 studies with a total of 1066 infants. All studies were conducted during early childhood immunisation. As the breastfeeding intervention cannot be blinded, we rated all studies as being at high risk of bias for blinding of participants and personnel. We assessed nine studies as being at low risk of bias for incomplete outcome data. In addition, we rated nine studies as high risk for blinding of outcome assessment. We scored risk of bias related to random sequence generation, allocation concealment, and selective reporting as unclear for the majority of the studies due to lack of information.Our primary outcome was pain. Breastfeeding reduced behavioural pain responses (cry time and pain scores) during vaccination compared to no treatment, oral water, and other interventions such as cuddling, oral glucose, topical anaesthetic, massage, and vapocoolant. Breastfeeding did not consistently reduce changes in physiological indicators, such as heart rate. We pooled data for duration of cry from six studies (n = 547 infants). Breastfeeding compared to water or no treatment resulted in a 38-second reduction in cry time (MD -38, 95% CI -50 to -26; P < 0.00001). The quality of the evidence according to GRADE for this outcome was moderate, as most infants were 6 months or younger, and outcomes may be different for infants during their 12-month immunisation. We pooled data for pain scores from five studies (n = 310 infants). Breastfeeding was associated with a 1.7-point reduction in standardised pain scores (SMD -1.7, 95% CI -2.2 to -1.3); we considered this evidence to be of moderate quality as data were primarily from infants younger than 6 months of age. We could pool heart rate data following injections for only two studies (n = 186); we considered this evidence to be of low quality due to insufficient data. There were no differences between breastfeeding and control (MD -3.6, -23 to 16).Four of the 10 studies had more than two study arms. Breastfeeding was more effective in reducing crying duration or pain scores during vaccination compared to: 25% dextrose and topical anaesthetic cream (EMLA), vapocoolant, maternal cuddling, and massage.No included studies reported adverse events. AUTHORS' CONCLUSIONS: We conclude, based on the 10 studies included in this review, that breastfeeding may help reduce pain during vaccination for infants beyond the neonatal period. Breastfeeding consistently reduced behavioural responses of cry duration and composite pain scores during and following vaccinations. However, there was no evidence that breastfeeding had an effect on physiological responses. No studies included in this review involved populations of hospitalised infants undergoing other skin-breaking procedures. Although it may be possible to extrapolate the review results to this population, further studies of efficacy, feasibility, and acceptability in this population are warranted.

Energy metabolism and cellular homeostasis trade-offs provide the basis for a new type of sensitivity to ocean acidification in a marine polychaete at a high-CO2 vent: adenylate and phosphagen energy pools versus carbonic anhydrase.

Species distributions and ecology can often be explained by their physiological sensitivity to environmental conditions. Whilst we have a relatively good understanding of how these are shaped by temperature, for other emerging drivers, such as PCO2  we know relatively little. The marine polychaete Sabella spallanzanii increases its metabolic rate when exposed to high PCO2  conditions and remains absent from the CO2 vent of Ischia. To understand new possible pathways of sensitivity to CO2 in marine ectotherms, we examined the metabolic plasticity of S. spallanzanii exposed in situ to elevated PCO2  by measuring fundamental metabolite and carbonic anhydrase concentrations. We show that whilst this species can survive elevated PCO2  conditions in the short term, and exhibits an increase in energy metabolism, this is accompanied by a significant decrease in carbonic anhydrase concentration. These homeostatic changes are unlikely to be sustainable in the longer term, indicating S. spallanzanii may struggle with future high PCO2  conditions.

Effectiveness of quality improvement strategies on the management of diabetes: a systematic review and meta-analysis.

BACKGROUND: The effectiveness of quality improvement (QI) strategies on diabetes care remains unclear. We aimed to assess the effects of QI strategies on glycated haemoglobin (HbA(1c)), vascular risk management, microvascular complication monitoring, and smoking cessation in patients with diabetes. METHODS: We identified studies through Medline, the Cochrane Effective Practice and Organisation of Care database (from inception to July 2010), and references of included randomised clinical trials. We included trials assessing 11 predefined QI strategies or financial incentives targeting health systems, health-care professionals, or patients to improve management of adult outpatients with diabetes. Two reviewers independently abstracted data and appraised risk of bias. FINDINGS: We reviewed 48 cluster randomised controlled trials, including 2538 clusters and 84,865 patients, and 94 patient randomised controlled trials, including 38,664 patients. In random effects meta-analysis, the QI strategies reduced HbA(1c) by a mean difference of 0·37% (95% CI 0·28-0·45; 120 trials), LDL cholesterol by 0·10 mmol/L (0·05-0.14; 47 trials), systolic blood pressure by 3·13 mm Hg (2·19-4·06, 65 trials), and diastolic blood pressure by 1·55 mm Hg (0·95-2·15, 61 trials) versus usual care. We noted larger effects when baseline concentrations were greater than 8·0% for HbA(1c), 2·59 mmol/L for LDL cholesterol, and 80 mm Hg for diastolic and 140 mm Hg for systolic blood pressure. The effectiveness of QI strategies varied depending on baseline HbA(1c) control. QI strategies increased the likelihood that patients received aspirin (11 trials; relative risk [RR] 1·33, 95% CI 1·21-1·45), antihypertensive drugs (ten trials; RR 1·17, 1·01-1·37), and screening for retinopathy (23 trials; RR 1·22, 1·13-1·32), renal function (14 trials; RR 128, 1·13-1·44), and foot abnormalities (22 trials; RR 1·27, 1·16-1·39). However, statin use (ten trials; RR 1·12, 0·99-1·28), hypertension control (18 trials; RR 1·01, 0·96-1·07), and smoking cessation (13 trials; RR 1·13, 0·99-1·29) were not significantly increased. INTERPRETATION: Many trials of QI strategies showed improvements in diabetes care. Interventions targeting the system of chronic disease management along with patient-mediated QI strategies should be an important component of interventions aimed at improving diabetes management. Interventions solely targeting health-care professionals seem to be beneficial only if baseline HbA(1c) control is poor. FUNDING: Ontario Ministry of Health and Long-term Care and the Alberta Heritage Foundation for Medical Research (now Alberta Innovates--Health Solutions).

Roles of crustacean hyperglycaemic hormone in ionic and metabolic homeostasis in the Christmas Island blue crab, Discoplax celeste.

There is a growing body of evidence implicating the involvement of crustacean hyperglycaemic hormone (CHH) in ionic homeostasis in decapod crustaceans. However, little is known regarding hormonally influenced osmoregulatory processes in terrestrial decapods. As many terrestrial decapods experience opposing seasonal demands upon ionoregulatory physiologies, we reasoned that these would make interesting models in which to study the effect of CHH upon these phenomena. In particular, those (tropical) species that also undergo seasonal migrations might be especially informative, as we know relatively little regarding the nature of CHHs in terrestrial decapods, and hormonally mediated responses to seasonal changes in metabolic demands might also be superimposed or otherwise integrated with those associated with ionic homeostasis. Using Discoplax celeste as a model crab that experiences seasonal extremes in water availability, and exhibits diurnal and migratory activity patterns, we identified two CHHs in the sinus gland. We biochemically characterised (cDNA cloning) one CHH and functionally characterised (in terms of dose-dependent hyperglycaemic responses and glucose-dependent negative feedback loops) both CHHs. Whole-animal in situ branchial chamber (22)NaCl perfusion experiments showed that injection of both CHHs increased gill Na(+) uptake in a seasonally dependent manner, and (51)Cr-EDTA clearance experiments demonstrated that CHH increased urine production by the antennal gland. Seasonal and salinity-dependent differences in haemolymph CHH titre further implicated CHH in osmoregulatory processes. Intriguingly, CHH appeared to have no effect on gill Na(+)/K(+)-ATPase or V-ATPase activity, suggesting unknown mechanisms of this hormone's action on Na(+) transport across gill epithelia.

Effect of physical training on airway inflammation in animal models of asthma: a systematic review.

BACKGROUND: There is little data on the effect of exercise on markers of airway inflammation in human asthmatics. The main objective of this review is to determine the effects of physical training on markers of airway inflammation in animal models of asthma. METHODS: A peer reviewed search was applied to Medline, Embase, Web of Science, Cochrane, and DARE databases. Data extraction was performed in a blinded fashion. RESULTS: From the initial 2336 studies, a total of 10 studies were selected for the final analysis. All were randomized controlled trials with low to moderate intensity training on ovalbumin-sensitized mice. In the exercised group of mice, there was a reduction in BAL eosinophils and Th-2 cytokines, no change in Th-1 cytokines, an increase in IL-10, and a reversal of airway remodeling. The data was not pooled owing to significant heterogeneity between studies, and a funnel plot test for publication bias was not performed because there were few studies reporting on any one outcome measure. The asthma models differed between studies in age and gender of mice, as well as in timing of physical training after sensitization. The risk of bias was unclear for some studies though this may not influence outcome measures. The accuracy of data extracted from graphics is unknown. CONCLUSIONS: Physical training improves airway inflammation in animal asthma models.

Effect of physical training on airway inflammation in bronchial asthma: a systematic review.

BACKGROUND: The majority of the global population cannot afford existing asthma pharmacotherapy. Physical training as an airway anti-inflammatory therapy for asthma could potentially be a non-invasive, easily available, affordable, and healthy treatment modality. However, effects of physical training on airway inflammation in asthma are currently inconclusive. The main objective of this review is to summarize the effects of physical training on airway inflammation in asthmatics. METHODS: A peer reviewed search was applied to Medline, Embase, Web of Science, Cochrane, and DARE databases. We included all observational epidemiological research studies and RCTs. Studies evaluating at least one marker of airway inflammation in asthmatics after a period of physical training were selected. Data extraction was performed in a blinded fashion. We decided a priori to avoid pooling of the data in anticipation of heterogeneity of the studies, specifically heterogeneity of airway inflammatory markers studied as outcome measures. RESULTS: From the initial 2635 studies; 23 studies (16 RCTs and 7 prospective cohort studies) were included. Study sizes were generally small (median sample size = 30). There was a reduction in C-reactive protein, malondialdehyde, nitric oxide, sputum cell counts and IgE in asthmatics with physical training. Mixed results were observed after training for fractional excretion of nitric oxide and bronchial hyperresponsiveness. The data was not pooled owing to significant heterogeneity between studies, and a funnel plot tests for publication bias were not performed because there were less than 10 studies for almost all outcome measures. Physical training intervention type, duration, intensity, frequency, primary outcome measures, methods of assessing outcome measures, and study designs were heterogeneous. CONCLUSION: Due to reporting issues, lack of information and heterogeneity there was no definite conclusion; however, some findings suggest physical training may reduce airway inflammation in asthmatics.

Consolidated standards of reporting trials (CONSORT) and the completeness of reporting of randomised controlled trials (RCTs) published in medical journals.

BACKGROUND: An overwhelming body of evidence stating that the completeness of reporting of randomised controlled trials (RCTs) is not optimal has accrued over time. In the mid-1990s, in response to these concerns, an international group of clinical trialists, statisticians, epidemiologists, and biomedical journal editors developed the CONsolidated Standards Of Reporting Trials (CONSORT) Statement. The CONSORT Statement, most recently updated in March 2010, is an evidence-based minimum set of recommendations including a checklist and flow diagram for reporting RCTs and is intended to facilitate the complete and transparent reporting of trials and aid their critical appraisal and interpretation. In 2006, a systematic review of eight studies evaluating the "effectiveness of CONSORT in improving reporting quality in journals" was published. OBJECTIVES: To update the earlier systematic review assessing whether journal endorsement of the 1996 and 2001 CONSORT checklists influences the completeness of reporting of RCTs published in medical journals. SEARCH METHODS: We conducted electronic searches, known item searching, and reference list scans to identify reports of evaluations assessing the completeness of reporting of RCTs. The electronic search strategy was developed in MEDLINE and tailored to EMBASE. We searched the Cochrane Methodology Register and the Cochrane Database of Systematic Reviews using the Wiley interface. We searched the Science Citation Index, Social Science Citation Index, and Arts and Humanities Citation Index through the ISI Web of Knowledge interface. We conducted all searches to identify reports published between January 2005 and March 2010, inclusive. SELECTION CRITERIA: In addition to studies identified in the original systematic review on this topic, comparative studies evaluating the completeness of reporting of RCTs in any of the following comparison groups were eligible for inclusion in this review: 1) Completeness of reporting of RCTs published in journals that have and have not endorsed the CONSORT Statement; 2) Completeness of reporting of RCTs published in CONSORT-endorsing journals before and after endorsement; or 3) Completeness of reporting of RCTs before and after the publication of the CONSORT Statement (1996 or 2001). We used a broad definition of CONSORT endorsement that includes any of the following: (a) requirement or recommendation in journal's 'Instructions to Authors' to follow CONSORT guidelines; (b) journal editorial statement endorsing the CONSORT Statement; or (c) editorial requirement for authors to submit a CONSORT checklist and/or flow diagram with their manuscript. We contacted authors of evaluations reporting data that could be included in any comparison group(s), but not presented as such in the published report and asked them to provide additional data in order to determine eligibility of their evaluation. Evaluations were not excluded due to language of publication or validity assessment. DATA COLLECTION AND ANALYSIS: We completed screening and data extraction using standardised electronic forms, where conflicts, reasons for exclusion, and level of agreement were all automatically and centrally managed in web-based management software, DistillerSR(®). One of two authors extracted general characteristics of included evaluations and all data were verified by a second author. Data describing completeness of reporting were extracted by one author using a pre-specified form; a 10% random sample of evaluations was verified by a second author. Any discrepancies were discussed by both authors; we made no modifications to the extracted data. Validity assessments of included evaluations were conducted by one author and independently verified by one of three authors. We resolved all conflicts by consensus.For each comparison we collected data on 27 outcomes: 22 items of the CONSORT 2001 checklist, plus four items relating to the reporting of blinding, and one item of aggregate CONSORT scores. Where reported, we extracted and qualitatively synthesised data on the methodological quality of RCTs, by scale or score. MAIN RESULTS: Fifty-three publications reporting 50 evaluations were included. The total number of RCTs assessed within evaluations was 16,604 (median per evaluation 123 (interquartile range (IQR) 77 to 226) published in a median of six (IQR 3 to 26) journals. Characteristics of the included RCT populations were variable, resulting in heterogeneity between included evaluations. Validity assessments of included studies resulted in largely unclear judgements. The included evaluations are not RCTs and less than 8% (4/53) of the evaluations reported adjusting for potential confounding factors.   Twenty-five of 27 outcomes assessing completeness of reporting in RCTs appeared to favour CONSORT-endorsing journals over non-endorsers, of which five were statistically significant. 'Allocation concealment' resulted in the largest effect, with risk ratio (RR) 1.81 (99% confidence interval (CI) 1.25 to 2.61), suggesting that 81% more RCTs published in CONSORT-endorsing journals adequately describe allocation concealment compared to those published in non-endorsing journals. Allocation concealment was reported adequately in 45% (393/876) of RCTs in CONSORT-endorsing journals and in 22% (329/1520) of RCTs in non-endorsing journals. Other outcomes with results that were significant include: scientific rationale and background in the 'Introduction' (RR 1.07, 99% CI 1.01 to 1.14); 'sample size' (RR 1.61, 99% CI 1.13 to 2.29); method used for 'sequence generation' (RR 1.59, 99% CI 1.38 to 1.84); and an aggregate score over reported CONSORT items, 'total sum score' (standardised mean difference (SMD) 0.68 (99% CI 0.38 to 0.98)). AUTHORS' CONCLUSIONS: Evidence has accumulated to suggest that the reporting of RCTs remains sub-optimal. This review updates a previous systematic review of eight evaluations. The findings of this review are similar to those from the original review and demonstrate that, despite the general inadequacies of reporting of RCTs, journal endorsement of the CONSORT Statement may beneficially influence the completeness of reporting of trials published in medical journals. Future prospective studies are needed to explore the influence of the CONSORT Statement dependent on the extent of editorial policies to ensure adherence to CONSORT guidance.

Burden and severity of disease of aromatic L-amino acid decarboxylase deficiency: a systematic literature review.

OBJECTIVE: The objective was to investigate the severity of aromatic L-amino acid decarboxylase deficiency (AADCd) as reported in the published literature and to collate evidence of the clinical manifestations of AADCd, and the impact of the disease on patients, caregivers, and healthcare systems. METHODS: Published articles reporting severity of disease or disease impact were eligible for inclusion in this review. Articles were searched in MEDLINE, EMBASE, Cochrane CENTRAL, TRIP medical, and CRD databases in October 2021. The quality of the included studies was investigated using a modified version of the grading system of the Centre for Evidence-Based Medicine (CEBM). Descriptive data of the literature was extracted and a narrative synthesis of the results across studies was conducted. This review is reported according to the PRISMA reporting guidelines for systematic reviews. RESULTS: The search identified 970 unique reports, of which 59 met eligibility criteria to be included in the review. Of these, 48 included reports provided details on the clinical manifestations of AADCd. Two reports explored the disease impact on patients, while four described the impact on caregivers. Five reports assessed the impact on healthcare systems. Individuals with AADCd experience very severe clinical manifestations regardless of motor milestones achieved, and present with a spectrum of other complications. Individuals with AADCd present with very limited function, which, in combination with additional complications, substantially impact the quality-of-life of individuals and their caregivers. The five studies which explore the impact on the healthcare system reported that adequate care of individuals with AADCd requires a vast array of medical services and supportive therapies. CONCLUSIONS: Irrespective of the ambulatory status of individuals, AADCd is a debilitating disease that significantly impacts quality-of-life for individuals and caregivers. It impacts the healthcare system due to the need for complex coordinated activities of a multidisciplinary specialist team.

Outcomes of Femoral Arterial Catheterisation in Neonates: A Retrospective Cohort Study.

BACKGROUND: To review the outcome of all femoral arterial catheter (FAC) insertions in a single, large neonatal unit over a 12 year period, we will describe the incidence of harms arising from FAC insertion and to identify risk factors associated with ischaemic injury. METHODS: Retrospective survey of data relating to all episodes of FAC insertion in a single neonatal intensive care unit over a 12 year period up to 2020. RESULTS: 146 FACs were inserted into 139 babies with a median (interquartile range) gestation and birth weight of 27 (24 to 37) weeks and 1092 (682 to 2870) g. Impaired limb perfusion occurred in 32 (22%). This was transient and recovered with no injury in 26 of the 32. There was an increased risk of impaired limb perfusion in babies with lower weight at the time of insertion; from 5.7% in babies over 3000 g to 34.7% in babies under 1000 g (relative risk 6.1 (1.5 to 24.6)). Six babies (4%) had ischaemic injury. Risk factors for ischaemic injury included weight below 1000 g (four cases), pre-existing partial arterial obstruction (two cases), concerns about limb perfusion prior to FAC insertion (two cases) and a delay in removing the FAC after recognition of the poor perfusion (five cases). Two clinicians inserted 71 (50%) FACs and had no associated injuries. CONCLUSIONS: FAC can be used in neonates, although there is a risk of ischaemic injury, particularly in very small babies. Our data can be used to inform decisions about patient selection for this procedure.

Widespread gaps in the quality of care for primary biliary cholangitis in UK.

OBJECTIVE: Primary biliary cholangitis (PBC) is a progressive, autoimmune, cholestatic liver disease affecting approximately 15 000 individuals in the UK. Updated guidelines for the management of PBC were published by The European Association for the Study of the Liver (EASL) in 2017. We report on the first national, pilot audit that assesses the quality of care and adherence to guidelines. DESIGN: Data were collected from 11 National Health Service hospitals in England, Wales and Scotland between 2017 and 2020. Data on patient demographics, ursodeoxycholic acid (UDCA) dosing and key guideline recommendations were captured from medical records. Results from each hospital were evaluated for target achievement and underwent χ2 analysis for variation in performance between trusts. RESULTS: 790 patients' medical records were reviewed. The data demonstrated that the majority of hospitals did not meet all of the recommended EASL standards. Standards with the lowest likelihood of being met were identified as optimal UDCA dosing, assessment of bone density and assessment of clinical symptoms (pruritus and fatigue). Significant variations in meeting these three standards were observed across UK, in addition to assessment of biochemical response to UDCA (all p<0.0001) and assessment of transplant eligibility in high-risk patients (p=0.0297). CONCLUSION: Our findings identify a broad-based deficiency in 'real-world' PBC care, suggesting the need for an intervention to improve guideline adherence, ultimately improving patient outcomes. We developed the PBC Review tool and recommend its incorporation into clinical practice. As the first audit of its kind, it will be used to inform a future wide-scale reaudit.

Long-term adherence of human brain cells in vitro is enhanced by charged amine-based plasma polymer coatings.

Advances in cellular reprogramming have radically increased the use of patient-derived cells for neurological research in vitro. However, adherence of human neurons on tissue cultureware is unreliable over the extended periods required for electrophysiological maturation. Adherence issues are particularly prominent for transferable glass coverslips, hindering imaging and electrophysiological assays. Here, we assessed thin-film plasma polymer treatments, polymeric factors, and extracellular matrix coatings for extending the adherence of human neuronal cultures on glass. We find that positive-charged, amine-based plasma polymers improve the adherence of a range of human brain cells. Diaminopropane (DAP) treatment with laminin-based coating optimally supports long-term maturation of fundamental ion channel properties and synaptic activity of human neurons. As proof of concept, we demonstrated that DAP-treated glass is ideal for live imaging, patch-clamping, and optogenetics. A DAP-treated glass surface reduces the technical variability of human neuronal models and enhances electrophysiological maturation, allowing more reliable discoveries of treatments for neurological and psychiatric disorders.

Case series of atypical high flow arterio-venous malformations in children.

Arteriovenous malformations (AVM) constitute a clinically significant form of vascular malformations in children. We present three cases of paediatric AVMs that demonstrated unusual features of high flow and significant shunting of blood without a clearly demonstrable nidus. Venous malformations were associated with the lesions. A discussion of the concepts underlying AVM physiology and how these case reports contradict these is included.

An evaluation of the completeness of safety reporting in reports of complementary and alternative medicine trials.

BACKGROUND: Adequate reporting of safety in publications of randomized controlled trials (RCTs) is a pre-requisite for accurate and comprehensive profile evaluation of conventional as well as complementary and alternative medicine (CAM) treatments. Clear and concise information on the definition, frequency, and severity of adverse events (AEs) is necessary for assessing the benefit-harm ratio of any intervention. The objectives of this study are to assess the quality of safety reporting in CAM RCTs; to explore the influence of different trial characteristics on the quality of safety reporting. METHODS: Survey of safety reporting in RCTs published in 2009 across 15 widely used CAM interventions identified from the Cochrane Collaboration's CAM Field specialized register of trials. Primary outcome measures, the adequacy of reporting of AEs; was defined and categorized according to the CONSORT for harms extension; the percentage of words devoted to the reporting of safety in the entire report and in the results section. RESULTS: Two-hundred and five trials were included in the review. Of these, 15% (31/205) reported that no harms were observed during the trial period. Of the remaining 174 trials reporting any safety information, only 21% (36/174) had adequate safety reporting.For all trials, the median percentage of words devoted to the reporting of safety in the results section was 2.6. Moreover, 69% (n = 141) of all trials devoted a lesser or equal percentage of words to safety compared to author affiliations. Of the predictor variables used in regression analysis, multicenter trials had more words devoted to safety in the results section than single centre trials (P = 0.045). CONCLUSIONS: An evaluation of safety reporting in the reports of CAM RCTs across 15 different CAM interventions demonstrated that the reporting of harms was largely inadequate. The quality of reporting safety information in primary reports of CAM randomized trials requires improvement.

Investigating the Relationship of Genotype and Climate Conditions on the Volatile Composition and Sensory Profile of Celery (Apium graveolens).

Apium graveolens is a biennial crop grown across the globe for its stalks, leaves and seed and is known for its distinct flavour and strong taste. Various extraction methods on fresh and dried celery and its essential oil are reported in the literature examining the aroma profile of this crop and demonstrating that its volatile composition is determined by variables including cultivar, season, geographical location and agronomic practices. This study investigated the volatile and sensory profile of eight celery genotypes grown over two years (2018 and 2020) in the same location in the UK. Solid-phase-micro-extraction followed by gas chromatography-mass spectrometry were used to determine the volatile compounds present in these genotypes and sensory evaluation using a trained panel to assess the sensory profile of fresh celery. Significant differences (p < 0.05) in the volatile composition and sensory profile were observed and influenced by both genotype and harvest year. Two genotypes exhibited similar aroma composition and sensory profile between the years. Celery samples harvested in 2018, which possessed air temperatures that were considerably warmer than in 2020, exhibited higher proportions of sesquiterpenes and phthalides and we hypothesise that the higher proportions were generated as a response to heat stress. Studying the relationship between the genotype and the environment will provide clear information to guide growers in how to consistently produce a higher quality crop.

Investigating the factors that influence the aroma profile of Apium graveolens: A review.

Celery (Apium graveolens) is a regularly consumed vegetable, providing strong, distinct flavours to dishes as well as health benefits. Constituents of the aroma profile of celery include a range of volatile compounds (terpenes, phthalides and aldehydes) that contribute to its characteristic odour and flavour. Vast amount of research has been completed on the aroma profile of celery. However, there is limited information stating the cultivar, origin and geographical location, despite that research on a plethora of other crops has indicated that these are key factors driving crop performance and quality attributes. This paper characterises the underlying biochemistry that determines the aroma profile of celery, whilst investigating the genetic and environmental influences leading to its variation. We make recommendations for minimum standards (MIAPAE: Minimum Information About a Plant Aroma Experiment) that should be adopted by the scientific community prior to publication of data relating to flavour and aroma characterisation of crops.

Influence of harvest maturity on the aroma quality of two celery (Apium graveolens) genotypes.

Celery is a fibrous horticultural vegetable grown globally and widely consumed due to its health benefits, distinct flavours and culinary versatility. Currently, few datasets examine its aroma development across maturity which could help guide growers towards optimising harvest times whilst identifying potential consequences of harvesting outside commercial maturity. Freeze-dried celery of two genotypes, selected for biochemical and sensory differences, were harvested at three time-points and investigated using solid-phase microextraction gas chromatography/mass spectrometry (SPME GC/MS) and gas chromatography/olfactometry (GC/O). Both maturity and genotype showed significant (P < 0.05) interactions between compounds, and harvest stage exhibited greater impact upon aroma quality than plant genotype. Thus, indicating that agronomic practice is key in determining crop quality. Monoterpenes, sesquiterpenes and phthalides begun to decrease once commercial maturity was reached, whereas alcohols were more prominent in post-mature celery. GC/O results confirmed the importance of phthalides to mature celery aroma and aroma differences caused by genotype.