Intellectual and motor performance, quality of life and psychosocial adjustment in children with cystinosis

Cystinosis is a rare multisystemic progressive disorder mandating lifelong medical treatment. Knowledge on the intellectual and motor functioning, health-related quality of life and psychosocial adjustment in children with cystinosis is limited. We have investigated nine patients (four after renal transplantation) at a median age of 9.7years (range 5.3-19.9years). Intellectual performance (IP) was analysed with the Wechsler Intelligence Scale for Children-III (seven children) and the Kaufman Assessment Battery for Children (two children). Motor performance (MP) was evaluated using the Zurich Neuromotor Assessment Test, and quality of life (QOL) was studied by means of the Netherlands Organization for Applied Scientific Research Academical Medical Center Child Quality of Life Questionnaire. Psychosocial adjustment was assessed by the Child Behavior Checklist. The overall intelligence quotient (IQ) of our patient cohort (median 92, range 71-105) was significantly lower than that of the healthy controls (p = 0.04), with two patients having an IQ < 85. Verbal IQ (93, range 76-118) was significantly higher than performance IQ (90, range 68-97; p = 0.03). The MP was significantly below the norm for pure motor, pegboard and static balance, as well as for movement quality. The patients' QOL was normal for six of seven dimensions (exception being positive emotions), whereas parents reported significant impairment in positive emotions, autonomy, social and cognitive functions. Significant disturbance was noted in terms of psychosocial adjustment. Based on the results from our small patient cohort, we conclude that intellectual and motor performance, health-related QOL and psychosocial adjustment are significantly impaired in children and adolescents with cystinosi

Strategies to support the growth and evolution of nursing and midwifery education in Tasmania

There is a prediction of significant nursing workforce shortfalls across Australia. Tasmania, as an island state of Australia, is more sensitive to such a shortage due to the existing challenges associated with pre-service and postgraduate nurse education, training, recruitment and retention. This paper describes strategies adopted by the School of Nursing (SoN), University of Tasmania to ensure the preparation and sustainability of the registered nurse workforce that is pivotal for state-wide healthcare. Due to lower levels of educational attainment amongst Tasmanian school leavers, the SoN has developed alternative pathway programs that offer entry for students who do not meet standardised entry. Staged progression to pedagogically sound online teaching platforms, pre-requisite limitations and uncoupling subject contents are strategies that have been incorporated in new undergraduate curriculum. The SoN’s postgraduate program includes a suite of 24 specialisations including a post-registration midwifery specialisation and students have the flexible option to exit the program at a graduate certificate, graduate diploma or finish at a master’s level. Partnerships with local industry, nursing associations, healthcare consumers, other health disciplines and students ensure that the SoN is responsive to sector needs and regulatory authority requirements to graduate qualified registered nurses serving in highly-demanding healthcare environments

Daylight duration and time allocation of children and adolescents

This study explores the allocation of time, particularly to sleep, among children and adolescents in response to daily daylight variation. Utilising a dataset of over 50,000 time-use diaries from two Australian cohorts spanning 16 years and employing an individual fixed effects estimator, we uncover a substantial causal impact of daily daylight duration on sleep patterns. Our findings reveal that days with longer daylight hours are associated with a decrease in total sleep duration, primarily driven by a later sleep onset time. Additionally, longer daylight hours correspond to reduced time spent on personal care and media activities, with increased dedication to school and physical activities. Furthermore, we identify socio-demographic factors moderating these effects, such as older age and weekend days exerting a stronger influence on sleep duration, while females and children of unemployed mothers exhibit a subtle impact. These insights contribute to our understanding of how environmental factors shape daily routines and offer implications for designing schedules that promote positive developmental outcomes in young individuals

Unfolded protein response is activated in Lewy body dementias

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Comparative efficacy, cognitive effects and acceptability of electroconvulsive therapies for the treatment of depression: Protocol for a systematic review and network meta-analysis

Introduction There have been important advances in the use of electroconvulsive therapy (ECT) to treat major depressive episodes. These include variations to the type of stimulus the brain regions stimulated, and the stimulus parameters (eg, stimulus duration/pulse width). Our aim is to investigate ECT types using a network meta-analysis (NMA) approach and report on comparative treatment efficacy, cognitive side effects and acceptability. Method We will conduct a systematic review to identify randomised controlled trials that compared two or more ECT protocols to treat depression. This will be done using the following databases: Embase, MEDLINE PubMed, Web of Science, Scopus, PsycINFO, Cochrane CENTRAL and will be supplemented by personal contacts with researchers in the field. All authors will be contacted to provide missing information. Primary outcomes will be symptom severity on a validated continuous clinician-rated scale of depression, cognitive functioning measured using anterograde verbal recall, and acceptability calculated using all-cause drop-outs. Secondary outcomes will include response and remission rates, autobiographical memory following a course of ECT, and anterograde visuospatial recall. Bayesian random effects hierarchical models will compare ECT types. Additional meta-regressions may be conducted to determine the impact of effect modifiers and patient-specific prognostic factors if sufficient data are available. Discussion This NMA will facilitate clinician decision making and allow more sophisticated selection of ECT type according to the balance of efficacy, cognitive side effects and acceptability. Ethics This systematic review and NMA does not require research ethics approval as it will use published aggregate data and will not collect nor disclose individually identifiable participant data. PROSPERO registration number CRD42022357098