Data Resource: The National Pupil Database (NPD)

Introduction: The National Pupil Database (NPD) is a record-level administrative data resource curated by the UK government's Department for Education that is used for funding purposes, school performance tables, policy making, and research. Processes Data are sourced from schools, exam awarding bodies, and local authorities who collect data on an on-going basis and submit to the Department for Education either termly or yearly. Data contents NPD contains child-level and school-level data on all pupils in state schools in England (6.6 million in the 2016/17 academic year). The primary module is the census, which has information on characteristics and school enrolment. Other modules include alternative provision, exam attainment, absence and exclusions. Data from children's social care are also available on children referred for support and those who become looked after. Children's records are linkable across different modules and across time using a nationally unique, anonymised child-level identifier. Linkage to external datasets has also been accomplished using child-level identifiers. Conclusions The NPD is an especially valuable data resource for researchers interested in the educational experience and outcomes of children and young people in England. Although limited by the fact that children in private schools or who are home schooled are not included, it provides a near-complete picture of school trajectories and outcomes for the majority of children. Linkage to other datasets can enhance analyses and provide answers to questions that would otherwise be costly, time consuming and difficult to find

A tandem duplication within the fibrillin 1 gene is associated with the mouse tight skin mutation.

Mice carrying the Tight skin (Tsk) mutation have thickened skin and visceral fibrosis resulting from an accumulation of extracellular matrix molecules. These and other connective tissue abnormalities have made Tskl + mice models for scleroderma, hereditary emphysema, and myocardial hypertrophy. Previously we localized Tsk to mouse chromosome 2 in a region syntenic with human chromosome 15. The microfibrillar glycoprotein gene, fibrillin 1 (FBN1), on human chromosome 15q, provided a candidate for the Tsk mutation. We now demonstrate that the Tsk chromosome harbors a 30- to 40-kb genomic duplication within the Fbn1 gene that results in a larger than normal in-frame Fbn1 transcript. These findings provide hypotheses to explain some of the phenotypic characteristics of Tskl + mice and the lethality of Tsk/Tsk embryos

Spin States of Homochiral and Heterochiral Isomers of [Fe(PyBox)2]2+ Derivatives

The following iron(II) complexes of 2,6-bis(oxazolinyl)pyridine (PyBox; LH) derivatives are reported: [Fe(LH)2][ClO4]2 (1); [Fe((R)-LMe)2][ClO4]2 ((R)-2; LMe=2,6-bis{4-methyloxazolinyl}pyridine); [Fe((R)-LPh)2][ClO4]2 ((R)-3) and [Fe((R)-LPh)((S)-LPh)][ClO4]2 ((RS)-3; LPh=2,6-bis{4-phenyloxazolinyl}pyridine); and [Fe((R)-LiPr)2][ClO4]2 ((R)-4) and [Fe((R)-LiPr)((S)-LiPr)][ClO4]2 ((RS)-4; LiPr=2,6-bis{4-isopropyloxazolinyl}pyridine). Solid (R)-3⋅MeNO2 exhibits an unusual very gradual, but discontinuous thermal spin-crossover with an approximate Tmath formula of 350 K. The discontinuity around 240 K lies well below Tmath formula , and is unconnected to a crystallographic phase change occurring at 170 K. Rather, it can be correlated with a gradual ordering of the ligand conformation as the temperature is raised. The other solid compounds either exhibit spin-crossover above room temperature (1 and (RS)-3), or remain high-spin between 5–300 K [(R)-2, (R)-4 and (RS)-4]. Homochiral (R)-3 and (R)-4 exhibit more twisted ligand conformations and coordination geometries than their heterochiral isomers, which can be attributed to steric clashes between ligand substituents [(R)-3]; or, between the isopropyl substituents of one ligand and the backbone of the other ((R)-4). In solution, (RS)-3 retains its structural integrity but (RS)-4 undergoes significant racemization through ligand redistribution by 1H NMR. (R)-4 and (RS)-4 remain high-spin in solution, whereas the other compounds all undergo spin-crossover equilibria. Importantly, Tmath formula for (R)-3 (244 K) is 34 K lower than for (RS)-3 (278 K) in CD3CN, which is the first demonstration of chiral discrimination between metal ion spin states in a molecular complex

Identification and functional characterization of an N-terminal oligomerization domain for polycystin-2*

Autosomal dominant polycystic kidney disease (ADPKD), the most common inherited cause of kidney failure, is caused by mutations in either PKD1 (85%) or PKD2 (15%). The PKD2 protein, polycystin-2 (PC2 or TRPP2), is a member of the transient receptor potential (TRP) superfamily and functions as a non-selective calcium channel. PC2 has been found to form oligomers in native tissues suggesting that it may form functional homo- or heterotetramers with other subunits, similar to other TRP channels. Our experiments unexpectedly revealed that PC2 mutant proteins lacking the known C-terminal dimerization domain were still able to form oligomers and co-immunoprecipitate full-length PC2, implying the possible existence of a proximal dimerization domain. Using yeast two-hybrid and biochemical assays, we have mapped an alternative dimerization domain to the N terminus of PC2 (NT2-1-223, L224X). Functional characterization of this domain demonstrated that it was sufficient to induce cyst formation in zebrafish embryos and inhibit PC2 surface currents in mIMCD3 cells probably by a dominant-negative mechanism. In summary, we propose a model for PC2 assembly as a functional tetramer which depends on both C- and N-terminal dimerization domains. These results have significant implications for our understanding of PC2 function and disease pathogenesis in ADPKD and provide a new strategy for studying PC2 function

Family care giving for Aboriginal peoples during end-of-life: findings from the Northern Territory

Abstract Although there is an extensive literature on the needs and experience of family caregivers for seriously ill people, there is a distinct lack of research articles on the specific topic of Aboriginal family caregivers. The lack of available information is a particular concern in view of the fact that a national survey on the social impact of caring for terminally ill people in Australia indicates that the experience for Indigenous Australian carers is fraught with challenges of distance, social isolation, poverty, and overcrowding, as well as different cultural needs. Thus, in order to begin to address the gap in knowledge the present article provides findings on family care giving for Aboriginal peoples from an Australian National Health and Medical Research Council two year study conducted in the Northern Territory

Parents’ experiences of health visiting for children with Down syndrome

© MA Healthcare Limited.Children with Down syndrome have an increased likelihoodof experiencing serious health conditions. Health visitors canhave an important role in monitoring and promoting healthand development for young children with Down syndrome.This study aimed to explore parents’ experiences of healthvisiting services for children with Down syndrome. Twentyfour parents of children with Down syndrome aged 0–5 yearscompleted a brief questionnaire about the number and natureof visits from health visitors in the previous 12 months andtheir support needs. Some parents commented that otherprofessionals met the needs of their child, whereas others saidthat they would like more advice and support from healthvisitors. A further exploration of broader health serviceprovision, including health visiting, for young children withDown syndrome is needed.Peer reviewedFinal Accepted Versio

Identification and characterisation of enteroaggregative Escherichia coli subtypes associated with human disease

Enteroaggregative E. coli (EAEC) are a major cause of diarrhoea worldwide. Due to their heterogeneity and carriage in healthy individuals, identification of diagnostic virulence markers for pathogenic strains has been difficult. In this study, we have determined phenotypic and genotypic differences between EAEC strains of sequence types (STs) epidemiologically associated with asymptomatic carriage (ST31) and diarrhoeal disease (ST40). ST40 strains demonstrated significantly enhanced intestinal adherence, biofilm formation, and pro-inflammatory interleukin-8 secretion compared with ST31 isolates. This was independent of whether strains were derived from diarrhoea patients or healthy controls. Whole genome sequencing revealed differences in putative virulence genes encoding aggregative adherence fimbriae, E. coli common pilus, flagellin and EAEC heat-stable enterotoxin 1. Our results indicate that ST40 strains have a higher intrinsic potential of human pathogenesis due to a specific combination of virulence-related factors which promote host cell colonization and inflammation. These findings may contribute to the development of genotypic and/or phenotypic markers for EAEC strains of high virulence

Growth in ataxia telangiectasia

BACKGROUND: Ataxia telangiectasia (A-T) is a DNA repair disorder that affects multiple body systems. Neurological problems and immunodeficiency are two important features of this disease. At this time, two main severity groups are defined in A-T: classic (the more severe form) and mild. Poor growth is a common problem in classic A-T. An objective of this study was to develop growth references for classic A-T. Another objective was to compare growth patterns in classic A-T and mild A-T with each other and with the general population, using the CDC growth references. A final objective was to examine the effects of chronic infection on height. RESULTS: We found that classic A-T patients were smaller overall, and suffered from height and weight faltering that continued throughout childhood and adolescence. When compared to the CDC growth references, the median heights and weights for both male and female patients eventually fell to or below the 3rd centile on the CDC charts. Height faltering was more pronounced in females. Birthweight was lower in the classic A-T group compared to mild A-T and the general population, whereas birth length was not. Finally, we investigated height and BMI faltering in relation to number of infections and found no association. CONCLUSIONS: Classic A-T appears to affect growth in utero. Although children appear to grow well in very early life, faltering begins early, and is unrelenting

Nanoparticles within WWTP sludges have minimal impact on leachate quality and soil microbial community structure and function

One of the main pathways by which engineered nanoparticles (ENPs) enter the environment is through land application of waste water treatment plant (WWTP) sewage sludges. WWTP sludges, enriched with Ag and ZnO ENPs or their corresponding soluble metal salts during anaerobic digestion and subsequently mixed with soil (targeting a final concentration of 1400 and 140 mg/kg for Zn and Ag, respectively), were subjected to 6 months of ageing and leaching in lysimeter columns outdoors. Amounts of Zn and Ag leached were very low, accounting for <0.3% and <1.4% of the total Zn and Ag, respectively. No differences in total leaching rates were observed between treatments of Zn or Ag originally input to WWTP as ENP or salt forms. Phospholipid fatty acid profiling indicated a reduction in the fungal component of the soil microbial community upon metal exposure. However, overall, the leachate composition and response of the soil microbial community following addition of sewage sludge enriched either with ENPs or metal salts was very similar