Residential school placements for children and young people with intellectual disabilities: their use and implications for adult social care

Out of area residential placements are associated with a range of poor outcomes for adults with intellectual disabilities and behaviours that challenge. In recent years there has been an increased drive to reduce such placements at as early a stage as possible. In this context the current review collates research and policy regarding use of residential schools for children and young people with intellectual disabilities and transition from these settings to adult services. The review highlights that relatively little is known about both use of, and transition from, residential schooling for children and young people with intellectual disabilities in the UK. Thirteen articles are identified: 7 examining the child or families’ experiences before placement, 4 examining outcomes during the placement, and 4 examining the process of transitioning from the placement and longer term outcomes. The methodological quality of articles was often limited. A lack of control groups, independent samples, or adequate sample sizes was particularly notable. Results are discussed in relation to factors that lead to a child’s placement in a residential school, children and families’ experiences of the placement, and outcomes following placement, including the transition process. A number of research priorities are highlighted based on gaps in the literature. Examples of alternative forms of support from clinical practice are provided, with recognition that a multi-element model is likely to be needed to provide high quality support to this group of young people

Positive behavioural support for adults with intellectual disabilities and behaviour that challenges: an initial exploration of the economic case

Background: Since the closure of long-stay hospitals, positive behavioural support and person centred approaches have been recognised and encouraged to support people with intellectual disabilities in the community. Method and materials: A small before-after study was conducted to explore the economic case for a positive behavioural support service for five adults with intellectual disabilities and behaviour that challenges in a small local authority in England. Evidence from a Delphi exercise was used as comparator. Results: The service was effective in improving the outcomes (behaviours that challenge, activity engagement, community participation) at a total cost of services of £2,296 per week. By maintaining persons with less severe challenges in the community (£9 to £164 per week) and those with more severe behavioural needs in less service-intensive residential accommodations (£1,213 to £3,696 per week), the service may potentially reduce public services cost in the long term. Conclusions: The positive behavioural support service may have the potential to improve outcomes and lower cost of care in the long term

Ti-6Al-4V microstructural orientation at different length scales as a function of scanning strategies in Electron Beam Melting in additive manufacturing

Additive manufacturing has been around for many years, yet the underlying physics of thermal gradients, local pressure environment, and other non-steady state manufacturing conditions are not fully understood. A Multi-University Research Initiative (MURI) is currently ongoing to measure liquid/solid and solid/solid interface stabilities in AM Ti-6Al-4V. Samples were produced with different beamscanning strategies in order to study the role of thermal gradients on the resulting microstructure. The motivation is to determine which beam-scanning strategy leads to desired grain size and texture. Orientation at different length scales (from mm to nm) can be quantified and compared with a combination of techniques including Precession Electron Diffraction (PED), Electron Backscatter Diffraction (EBSD) and Neutron diffraction. This new information will help predict properties of additively manufactured parts

Addressing obesity in Roma communities: : A community readiness approach

YesParticipation in community programmes by the Roma community is low whilst this community presents with high risk of poor health and low levels of wellbeing. To improve rates of participation in programmes compatibility must be achieved between implementation efforts and levels of readiness in the community. The Community Readiness Model (CRM) is a widely used toolkit which provides an indication of how prepared and willing a community is to take action on specific issues. We present findings from a CRM assessment for the Eastern European Roma community in Bradford, UK on issues related to nutrition and obesity. We interviewed key respondents identified as knowledgeable about the Roma community using the CRM. This approach applies a mixed methodology incorporating readiness scores and qualitative data. A mean community readiness score was calculated enabling researchers to place the community in one of nine possible stages of readiness. Interview transcripts were analysed using a qualitative framework analysis to generate contextual information. An overall score consistent with vague awareness was achieved, which indicates a low level of community readiness. This score suggests there will be a low likelihood of participation in currently available nutrition and obesity programmes. To our knowledge this is the first study to apply the CRM in the Roma community for any issue. We present the findings for each of the six dimensions that make up the CRM together with salient qualitative findings.Better Start Bradfor

Elevation of gangliosides in four brain regions from Parkinson’s disease patients with a GBA mutation

A number of genetic risk factors have been identified over the past decade for Parkinson's Disease (PD), with variants in GBA prominent among them. GBA encodes the lysosomal enzyme that degrades the glycosphingolipid, glucosylceramide (GlcCer), with the activity of this enzyme defective in Gaucher disease. Based on the ill-defined relationship between glycosphingolipid metabolism and PD, we now analyze levels of various lipids by liquid chromatography/electrospray ionization-tandem mass spectrometry in four brain regions from age- and sex-matched patient samples, including idiopathic PD, PD patients with a GBA mutation and compare both to control brains (n = 21 for each group) obtained from individuals who died from a cause unrelated to PD. Of all the glycerolipids, sterols, and (glyco)sphingolipids (251 lipids in total), the only lipid class which showed significant differences were the gangliosides (sialic acid-containing complex glycosphingolipids), which were elevated in 3 of the 4 PD-GBA brain regions. There was no clear correlation between levels of individual gangliosides and the genetic variant in Gaucher disease [9 samples of severe (neuronopathic), 4 samples of mild (non-neuronopathic) GBA variants, and 8 samples with low pathogenicity variants which have a higher risk for development of PD]. Most brain regions, i.e. occipital cortex, cingulate gyrus, and striatum, did not show a statistically significant elevation of GlcCer in PD-GBA. Only one region, the middle temporal gyrus, showed a small, but significant elevation in GlcCer concentration in PD-GBA. We conclude that changes in ganglioside, but not in GlcCer levels, may contribute to the association between PD and GBA mutations

Dissociating Slow Responses From Slow Responding

Increased Intra-Subject Variability (ISV) is a candidate endophenotype of ADHD. ISV's relationship with response speed is highly relevant for ADHD as patients are highly variable but typically no slower than controls. This brief report addresses the relationship between variability and speed by employing dimensional analyses for differentiated performance measures, with a particular focus on the ex-Gaussian measures, across relevant ADHD studies and in young healthy adults (N = 70). For both patients with ADHD and healthy adults, we found that reaction time standard deviation and mean reaction time were strongly correlated, thus failing to dissociate, but ex-Gaussian tau (tau) shared only little variance with Gaussian mu (mu), thus dissociating slow responses (tau) from response speed or-if given-slow responding (mu). Our results highlight the utility of employing the ex-Gaussian measures to disentangle ISV and speed, particularly for ADHD data as patients make more slow responses but are not overall slower than typical controls

Reduced cerebral vascularization in experimental neuronopathic Gaucher disease.

The glycosphingolipidosis, Gaucher disease, in which a range of neurological manifestations occur, results from a deficiency of acid β-glucocerebrosidase, with subsequent accumulation of β-glucocerebroside, its upstream substrates, and the non-acylated congener β-glucosylsphingosine. However, the mechanisms by which end-organ dysfunction arise are poorly understood. Here, we report strikingly diminished cerebral microvascular density in a murine model of disease, and provide a detailed analysis of the accompanying cerebral glycosphingolipidome in these animals, with marked elevations of β-glucosylsphingosine. Further in vitro studies confirmed a concentration-dependent impairment of endothelial cytokinesis upon exposure to quasi-pathological concentrations of β-glucosylsphingosine. These findings support a premise for pathogenic disruption of cerebral angiogenesis as an end-organ effect, with potential for therapeutic modulation in neuronopathic Gaucher disease. Copyright © 2017 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd

Experiences in running a complex electronic data capture system using mobile phones in a large-scale population trial in southern Nepal.

The increasing availability and capabilities of mobile phones make them a feasible means of data collection. Electronic Data Capture (EDC) systems have been used widely for public health monitoring and surveillance activities, but documentation of their use in complicated research studies requiring multiple systems is limited. This paper shares our experiences of designing and implementing a complex multi-component EDC system for a community-based four-armed cluster-Randomised Controlled Trial in the rural plains of Nepal, to help other researchers planning to use EDC for complex studies in low-income settings. We designed and implemented three interrelated mobile phone data collection systems to enrol and follow-up pregnant women (trial participants), and to support the implementation of trial interventions (women's groups, food and cash transfers). 720 field staff used basic phones to send simple coded text messages, 539 women's group facilitators used Android smartphones with Open Data Kit Collect, and 112 Interviewers, Coordinators and Supervisors used smartphones with CommCare. Barcoded photo ID cards encoded with participant information were generated for each enrolled woman. Automated systems were developed to download, recode and merge data for nearly real-time access by researchers. The systems were successfully rolled out and used by 1371 staff. A total of 25,089 pregnant women were enrolled, and 17,839 follow-up forms completed. Women's group facilitators recorded 5717 women's groups and the distribution of 14,647 food and 13,482 cash transfers. Using EDC sped up data collection and processing, although time needed for programming and set-up delayed the study inception. EDC using three interlinked mobile data management systems (FrontlineSMS, ODK and CommCare) was a feasible and effective method of data capture in a complex large-scale trial in the plains of Nepal. Despite challenges including prolonged set-up times, the systems met multiple data collection needs for users with varying levels of literacy and experience

Therapy development for the mucopolysaccharidoses : updated consensus recommendations for neuropsychological endpoints

Neurological dysfunction represents a significant clinical component of many of the mucopolysaccharidoses (also known as MPS disorders). The accurate and consistent assessment of neuropsychological function is essential to gain a greater understanding of the precise natural history of these conditions and to design effective clinical trials to evaluate the impact of therapies on the brain. In 2017, an International MPS Consensus Panel published recommendations for best practice in the design and conduct of clinical studies investigating the effects of therapies on cognitive function and adaptive behavior in patients with neuronopathic mucopolysaccharidoses. Based on an International MPS Consensus Conference held in February 2020, this article provides updated consensus recommendations and expands the objectives to include approaches for assessing behavioral and social-emotional state, caregiver burden and quality of life in patients with all mucopolysaccharidoses

Protocol of the Low Birth Weight South Asia Trial (LBWSAT), a cluster-randomised controlled trial testing impact on birth weight and infant nutrition of Participatory Learning and Action through women's groups, with and without unconditional transfers of fortified food or cash during pregnancy in Nepal.

BACKGROUND: Low birth weight (LBW, < 2500 g) affects one third of newborn infants in rural south Asia and compromises child survival, infant growth, educational performance and economic prospects. We aimed to assess the impact on birth weight and weight-for-age Z-score in children aged 0-16 months of a nutrition Participatory Learning and Action behaviour change strategy (PLA) for pregnant women through women's groups, with or without unconditional transfers of food or cash to pregnant women in two districts of southern Nepal. METHODS: The study is a cluster randomised controlled trial (non-blinded). PLA comprises women's groups that discuss, and form strategies about, nutrition in pregnancy, low birth weight and hygiene. Women receive up to 7 monthly transfers per pregnancy: cash is NPR 750 (~US$7) and food is 10 kg of fortified sweetened wheat-soya Super Cereal per month. The unit of randomisation is a rural village development committee (VDC) cluster (population 4000-9200, mean 6150) in southern Dhanusha or Mahottari districts. 80 VDCs are randomised to four arms using a participatory 'tombola' method. Twenty clusters each receive: PLA; PLA plus food; PLA plus cash; and standard care (control). Participants are (mostly Maithili-speaking) pregnant women identified from 8 weeks' gestation onwards, and their infants (target sample size 8880 birth weights). After pregnancy verification, mothers may be followed up in early and late pregnancy, within 72 h, after 42 days and within 22 months of birth. Outcomes pertain to the individual level. Primary outcomes include birth weight within 72 h of birth and infant weight-for-age Z-score measured cross-sectionally on children born of the study. Secondary outcomes include prevalence of LBW, eating behaviour and weight during pregnancy, maternal and newborn illness, preterm delivery, miscarriage, stillbirth or neonatal mortality, infant Z-scores for length-for-age and weight-for-length, head circumference, and postnatal maternal BMI and mid-upper arm circumference. Exposure to women's groups, food or cash transfers, home visits, and group interventions are measured. DISCUSSION: Determining the relative importance to birth weight and early childhood nutrition of adding food or cash transfers to PLA women's groups will inform design of nutrition interventions in pregnancy. TRIAL REGISTRATION: ISRCTN75964374 , 12 Jul 2013