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"Oliver, Chris"
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Cook with Jamie : my guide to making you a better cook
A celebrity cook demystifies a range of culinary techniques and food-serving practices, from stocking a kitchen and mastering food-preparation basics to selecting groceries and matching wines with dishes, in a book with more than one hundred and seventy-five recipes.
Sleep disorders in rare genetic syndromes: a meta-analysis of prevalence and profile
Background
Sleep disorders are common in people with intellectual disability (ID) and autism, with growing evidence of diverse sleep profiles across ID associated genetic syndromes. Documenting the prevalence and profile of specific sleep disorders in syndromes will quantify syndrome-driven ‘risk’, inform prognosis and enhance understanding of aetiology of sleep disorders.
Method
Following PRISMA guidelines for meta-analysis, we searched Ovid PsycINFO, Ovid MEDLINE, Ovid Embase, Web of Science and PubMed Central with use of syndrome-specific keywords and 60 sleep-related search terms. We screened and extracted papers that reported sleep disorder prevalence data for five or more individuals within a genetic syndrome, and applied quality criteria to produce a quality-effects prevalence model of six types of sleep disorder across nineteen syndromes. Relative risk estimates were calculated for the prevalence of each sleep disorder in each syndrome.
Results
Two hundred and seventy three papers were identified, generating 463 prevalence estimates for Angelman, CHARGE, Cornelia de Lange, Down, fragile X, Prader–Willi, Rett, Smith–Magenis and Williams syndromes, mucopolysaccharidoses (MPS disorders), neurofibromatosis and tuberous sclerosis complex. Prevalence estimates were higher in genetic syndromes than published equivalents for typically developing individuals, with few exceptions. Between-syndrome differences for some disorders were evident; sleep-disordered breathing was most prevalent in MPS disorders (72–77%), while excessive daytime sleepiness was highest in Smith–Magenis syndrome (60%). Conversely, insomnia, which was reported at a higher rate than TD estimates in all syndromes except fragile X, was not associated with specific genetic risk. This suggests insomnia could emerge because of the individual’s environment or associated developmental delay, rather than any specific genetic syndromes.
Limitations
Due to the broad scope of the meta-analysis, only syndromes previously identified as reporting preliminary sleep research were included. Other syndromes may also experience elevated prevalence rates of specific types of sleep disorder. Only English language papers were included.
Conclusions
Differing prevalence rates between types of sleep disorder suggest differing causal mechanisms, such as cranio-facial morphology in Down and Prader–Willi syndromes and the build-up of mucopolysaccharides in MPS disorders. Priorities for clinical assessment and intervention for sleep disorders are discussed.
Journal Article
The New Mutants. Demon bear
The visionary talents of writer Chris Claremont and legendary illustrator Bill Sienkiewicz bring the Demon Bear that has haunted Danielle Moonstar's dreams to horrifying life! It took her parents, and now it has returned for Dani - and only the combined efforts of her fellow New Mutants can stop it from finishing the job! Sink your teeth into a true classic! Then, Dani's nightmare returns years later as San Francisco - and her new team X-Force, come under attack from a similarly unholy ursine!
Book
Introducing RDA : a guide to the basics after 3R
Since Oliver's guide was first published in 2010, thousands of LIS students, records managers, and catalogers and other library professionals have relied on its clear, plainspoken explanation of RDA: Resource Description and Access as their first step towards becoming acquainted with the cataloging standard.
eBook
What you need to be warm
During the coldest season, when the world feels scary -- what do you remember about being warm? Baked potatoes. Trust. A kettle on the stove. Blankets. A smile. And, most of all, the reassurance that you belong. In his powerful and moving poem, featuring illustrations from thirteen extraordinary artists, bestselling author and UNHCR Goodwill Ambassador Neil Gaiman draws together many different memories to answer the question, 'what do you need to be warm?'
Book
Persistence and predictors of self-injurious behaviour in autism: a ten-year prospective cohort study
Background
Self-injurious behaviours, such as head banging, hair pulling, skin picking and scratching, are common in individuals with autism. Despite high prevalence rates, there is a paucity of longitudinal research to refine models of risk and mechanism and inform service planning. In this longitudinal study, we investigated self-injury in a cohort of individuals with autism over 10 years to identify behavioural and demographic characteristics associated with persistent self-injury.
Methods
Carers of 67 individuals with autism completed questionnaires relating to the presence of self-injury and relevant risk markers at
T
1
(mean [SD] age in years 13.4 [7.7]) and
T
3
(mean [SD] age in years 23.9 [7.7]) 10 years later. Forty-six of these also took part at
T
2
(3 years after initial participation). Analysis assessed demographic and behavioural risk markers for self-injury, as well as the predictive value of items assessed at
T
1
and
T
2.
Results
Self-injury was persistent in 44% of individuals over the 10-year period, with behavioural characteristics of impulsivity (
p
< .001) and overactivity (
p
= .002), identified as risk markers for persistence. A predictive model of self-injury was derived from LASSO analysis, with baseline impulsivity, interest and pleasure, stereotyped behaviour, social communication and adaptive functioning predicting self-injury over 10 years.
Conclusions
In this unique longitudinal investigation into the persistence of self-injury in a non-clinical sample of individuals with autism over a 10 year period, we have identified a novel, robust and stable profile of behavioural characteristics associated with persistent self-injury. Findings support an early intervention strategy targeted towards individuals identified to be at a higher risk of developing self-injurious behaviour.
Journal Article
The development of early social cognitive skills in neurogenetic syndromes associated with autism: Cornelia de Lange, fragile X and Rubinstein–Taybi syndromes
Background
Cornelia de Lange (CdLS), Fragile X (FXS) and Rubinstein–Taybi syndromes (RTS) evidence unique profiles of autistic characteristics. To delineate these profiles further, the development of early social cognitive abilities in children with CdLS, FXS and RTS was compared to that observed in typically developing (TD) and autistic (AUT) children.
Methods
Children with CdLS (
N
= 22), FXS (
N
= 19) and RTS (
N
= 18), completed the Early Social Cognition Scale (ESCogS). Extant data from AUT (
N
= 19) and TD (
N
= 86) children were used for comparison.
Results
Similar to AUT children, children with CdLS, FXS and RTS showed an overall delay in passing ESCogS tasks. Children with CdLS showed a similar degree of delay to AUT children and greater delay than children with FXS and RTS. The CdLS, FXS and RTS groups did not pass tasks in the same sequence observed in TD and AUT children. Children with CdLS (
p
= 0.04), FXS (
p
= 0.02) and RTS (
p
= 0.04) performed better on tasks requiring understanding simple intentions in others significantly more than tasks requiring joint attention skills.
Conclusions
An underlying mechanism other than general cognitive delay may be disrupting early social cognitive development in children with CdLS, FXS and RTS. Factors that may disrupt early social cognitive development within these syndromes are discussed.
Journal Article
Caregivers’ experience of sleep management in Smith–Magenis syndrome: a mixed-methods study
Background
Smith–Magenis syndrome (SMS) is a rare genetic syndrome associated with a unique profile of early morning waking and daytime sleepiness. Children with SMS evidence high rates of self-injury and aggression and have a preference for adult over peer attention, with strong motivation to interact with a particular caregiver. In addition, people with SMS have lower adaptive functioning skills relative to cognitive abilities and demonstrate high levels of impulsivity. Taken together, these factors may result in individuals being awake overnight requiring vigilant caregiver supervision. Despite these complexities, no study has described the strategies caregivers take to keep their children with SMS safe overnight or considered the impact of these experiences on caregivers or the wider family.
Methods
The current study used a mixed-methods approach to consider sleep management strategies and challenges for caregivers of people with SMS at different ages. Caregivers completed an international online survey about sleep management and related difficulties, use of interventions and access to services and support. Semi-structured interviews were conducted with 14 caregivers in the UK to increase understanding of caregiver experiences and priorities for change in the UK context. Interviews were transcribed verbatim and coded using thematic analysis.
Results
Evidence from the online survey (n = 40) revealed wide-ranging impacts of poor sleep on the person with SMS and the wider family. Only 5% of caregivers reported that the sleep problems had no impact on their child, and 76% reported a moderately or extremely significant impact on themselves. For some individual caregivers, sleep management difficulties improved over time whereas for others no change was reported. Weekly respite emerged as the ideal provision for 49% of caregivers, although only 14% had access to this. The majority of caregivers (54%) received no respite. Thematic analysis of qualitative interviews revealed interactions between aspects of the behavioural phenotype of SMS which may contribute to complex and unusual presentations in relation to sleep management and safety.
Conclusions
Caregivers’ priorities for sleep management and support were delineated, with key implications for services in terms of the use of SMS-sensitive strategies and respite provision.
Journal Article