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Aim: We examined key features of two outcome measures for social dysfunction and autism spectrum disorder traits, the Social Responsiveness Scale, Second Edition (SRS-2) and the Social Skills Improvement System - Rating Scales (SSIS-RS), in children with neurofibromatosis type 1 (NF1). The aim of the study was to provide objective evidence as to which behavioural endpoint should be used in clinical trials.Method: Cross-sectional behavioural and demographic data were pooled from four paediatric NF1 tertiary referral centres in Australia and the United States (N=122; 65 males, 57 females; mean age [SD] 9y 2mo [3y], range 3-15y).Results: Distributions of SRS-2 and SSIS-RS scores were unimodal and both yielded deficits, with a higher proportion of severely impaired scores on the SRS-2 (16.4%) compared to the SSIS-RS (8.2%). Pearson's product-moment correlations revealed that both questionnaires were highly related to each other (r=-0.72, p<0.001) and to measures of adaptive social functioning (both p<0.001). Both questionnaires were significantly related to attention-deficit/hyperactivity disorder symptoms, but only very weakly associated with intelligence.Interpretation: The SRS-2 and SSIS-RS capture social dysfunction associated with NF1, suggesting both may be suitable choices for assessing social outcomes in this population in a clinical trial. However, careful thought needs to be given to the nature of the intervention when selecting either as a primary endpoint.What This Paper Adds: The Social Responsiveness Scale, Second Edition yielded a large deficit relative to population norms. The Social Skills Improvement System - Rating Scales yielded a moderate deficit relative to population norms. Both scales were highly correlated, suggesting that they are measuring a unitary construct. [ABSTRACT FROM AUTHOR]

Aim: To compare hospital admission patterns after the first year of life in Australian children with developmental disabilities and children with no known disability, according to maternal country of birth and Indigenous status.Method: This was a retrospective cohort study using linked data across health, disability, and hospital admission databases. The study investigated 656 174 children born in Western Australia between 1983 and 2008 with a total of 1 091 834 records of hospital admissions.Results: Children with no known disability born to Indigenous mothers had the highest rate of hospital admissions compared to children of non-Indigenous mothers. Children of foreign-born mothers from low-income countries had the highest rate of hospital admissions if disability was present. Children with cerebral palsy (CP) with or without associated intellectual disability had the highest rate of hospital admissions among children with developmental disability, especially if mothers were foreign-born.Interpretation: Children with CP and intellectual disability, particularly from minority backgrounds (Indigenous Australian and foreign-born mothers), were at higher risk of being admitted to hospital after the first year of life.What This Paper Adds: Hospital admissions in Australian children with and without disabilities differ according to maternal country of birth. Hospital admission rates in children without a developmental disability were greatest for Australian-born Indigenous children. Disabled Australian-born children of foreign-born mothers from low-income countries had the highest hospital admission rates. Hospital admission risk was greatest for Australian-born children with cerebral palsy, especially if mothers were foreign-born. [ABSTRACT FROM AUTHOR]

Aim: To investigate the impact of socio-economic disadvantage on indicators of cerebral palsy (CP) severity - motor impairment, intellectual disability, and the presence of severe comorbidities - in children with CP in Australia.Method: Data from the Australian Cerebral Palsy Register were analysed. Socio-economic disadvantage was assessed using maternal age, maternal country of birth, and a measure of neighbourhood socio-economic status (SES) at the time of the child's birth. Descriptive bivariate analysis, trend analysis, risk ratios, and mediation analysis were undertaken to examine the impact of disadvantage on the indicators of CP severity.Results: A socio-economic gradient was demonstrated with an increasing proportion of children with non-ambulant status, at least moderate intellectual disability, and the presence of severe comorbidities (having epilepsy, functional blindness, bilateral deafness, and/or no verbal communication) with decreasing neighbourhood SES, adolescent motherhood, and maternal minority ethnicity.Interpretation: In Australia, socio-economic disadvantage at birth impacts adversely on CP severity at age 5 years. By identifying that socio-economically disadvantaged children with CP are at greater risk of more severe functional outcomes, we can inform targeted interventions at the family and neighbourhood level to reduce these inequities for children with CP.What This Paper Adds: Socio-economic disadvantage is associated with increased severity of cerebral palsy functional outcomes. This encompasses low neighbourhood socio-economic status, adolescent motherhood, and maternal minority ethnicity. [ABSTRACT FROM AUTHOR]

Aim: To investigate trends in birth prevalence of cerebral palsy (CP) overall and by gestational age, and examine the distribution of motor type, spastic topography, and severity using Australian CP Register data from 1995 to 2009.Method: Prenatal and perinatal CP data were collated from state/territory CP registers. Birth prevalence estimates per 1000 live births and per 1000 neonatal survivors (NNS) were calculated in five epochs. Data from three state registers with population-level ascertainment were used to investigate birth prevalence trends by gestational age using Poisson regression. Distribution of motor type, spastic topography, and moderate to severe disability (IQ≤50 and/or Gross Motor Function Classification System levels III-V) were evaluated within birthweight categories.Results: Birth prevalence of CP varied across population-level states but within each state declined significantly over time (p<0.05). Birth prevalence per 1000 neonatal survivors declined amongst children born before 28 weeks (South Australia, Victoria p<0.001) and those born at or after 37 weeks (Victoria p<0.001, Western Australia p<0.002). Across Australia the percentage of children with bilateral spastic CP declined amongst those born less than 1000g. The percentage of children with moderate to severe disability decreased (48%-34%, p<0.001).Interpretation: Birth prevalence of CP declined. Encouragingly, the percentage of children with CP whose disability was moderate to severe also decreased.What This Paper Adds: Birth prevalence of cerebral palsy (CP) differed but declined across Australian states (1995-2009). Australian CP birth prevalence declined significantly amongst children born before 28 weeks and those born at or after 37 weeks. The percentage of children with moderate to severe disability decreased. [ABSTRACT FROM AUTHOR]

Aim: To describe health-related quality of life of Australian children and adolescents with Down syndrome and compare it with norm-referenced data.Method: A cross-sectional survey was conducted with parents of 75 children and adolescents (43 males, 32 females) with Down syndrome aged 5 to 18 years (mean age 13y 2mo, SD 4y 8mo). The proxy-report KIDSCREEN-27 questionnaire was administered and five dimensions of health-related quality of life were measured. Data were analysed descriptively and compared with normative data.Results: Total group mean scores for psychological well-being, autonomy and parent relation, and school environment dimensions were within normal threshold values, whereas mean scores for physical well-being, and social support and peers dimensions, were poorer. For participants with Down syndrome aged 8 to 18 years, the difference with normative data for proxy-reported physical well-being, psychological well-being, and social support and peers dimensions favoured typically developing children. Adolescents (13-18y) with Down syndrome scored poorer on all dimensions than children (5-12y) with Down syndrome.Interpretation: Our findings assist a better understanding of the lived experiences of children and adolescents with Down syndrome, as perceived by their parents, and suggest aspects of health that could be influenced to optimize their quality of life.What This Paper Adds: Proxy-reported psychological well-being and autonomy were within the normal range for children with Down syndrome. Physical well-being and social support scores were significantly lower than normative data. Proxy-reported scores for adolescents with Down syndrome were consistently poorer than for children with Down syndrome and the differences were clinically important. [ABSTRACT FROM AUTHOR]

Aim: To determine whether intrathecal baclofen (ITB) therapy improves performance and performance satisfaction in goal areas identified by patients' parents.Method: This study formed part of an ongoing multicentre national audit involving six paediatric ITB pump implant centres across Australia. The Canadian Occupational Performance Measure was the primary outcome measure utilized at baseline, 6 months, and 12 months after pump implants in paediatric patients receiving ITB therapy for the first time between 31st December 2009 and 31st December 2014.Results: Twenty-five children had goals identified (mean age 11y 1mo), 19 had a diagnosis of cerebral palsy and 22 were at Gross Motor Function Classification System level IV, V, or equivalent. Strong evidence for an improvement in goal performance (2.33, 95% CI 1.70, 2.96, p<0.001) and performance satisfaction scores (3.08, 95% CI 2.28, 3.88, p<0.001) were demonstrated at 6 months, compared to baseline. The differences were clinically significant and were sustained to 12 months.Interpretation: ITB therapy in paediatric patients with hypertonia results in clinically significant improvements in average performance and performance satisfaction scores.What This Paper Adds: The most commonly identified goals of parents of children treated with intrathecal (ITB) therapy were: improving ease of dressing, positioning, and transfers. ITB therapy is effective in improving performance and performance satisfaction in children with hypertonia. Score improvements are mainly evident within the first 6 months of therapy. [ABSTRACT FROM AUTHOR]

Aim: To identify a cohort of children with cerebral palsy (CP) from hospital data; determine the proportion that participated in standardized educational testing and attained a score within the normal range; and describe the relationship between test results and motor symptoms.Method: This population-based retrospective cohort study used data from New South Wales, Australia. We linked hospital data for children younger than 16 years of age admitted between 1st July 2000 and 31st March 2014 to education data from 2009 to 2014. Hospital diagnosis codes were used to identify a cohort of children with CP (n=3944) and describe their motor symptoms. Educational outcomes in the CP cohort were compared with those among children without CP.Results: Of those with educational data (n=1770), 46% were exempt from reading assessment because of intellectual or functional disability, 7% were absent or withdrawn from testing and 47% participated in testing. About 30% of all children with educational data had test scores in the normal range. The proportion was greatest among those with hemiplegia (>40%) and lowest among those with tetraplegia (<10%).Interpretation: One-third of children with CP participated in standardized testing and achieved a result in the normal range. The proportions were lower in children with more severe motor symptoms.What This Paper Adds: From 2009 to 2014, most Australian children with cerebral palsy (CP) attended a mainstream school. The rate of disability-related exemption from standardized educational testing was almost 50%. Thirty per cent of children with CP achieved educational scores in the normal range. [ABSTRACT FROM AUTHOR]