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Background Children with severe disability often develop osteoporosis and have an increased risk of fracture. In Rett syndrome, the prevalence of fracture is four times greater than in the general population, and the femur is commonly affected. This study used qualitative methods to investigate the regaining of mobility within 12 months following femoral fracture in Rett syndrome and parent caregiver experiences. Methods Caregivers ( n = 14) of cases registered with the Australian Rett Syndrome Database with a daughter with Rett syndrome were recruited if their daughter sustained a femoral fracture between 2009 and 2014. Median (interquartile range) age at fracture was 11 years and 4 months (8 years and 8 months to 22 years and 3 months). Qualitative methods were used to investigate parent/caregiver experiences and their daughter's recovery following fracture. Themes in the interview data were identified with thematic analysis. Results Operative management was chosen for those walking independently and non-operative management for most of the remaining. All who walked independently and one of the four who required assistance recovered pre-operative walking skills within 6 months. Themes identified by caregivers related to the complexities of pain recognition, the caregiver emotional journey and later rebuilding of relationships with service providers. Conclusions Those who walked with assistance were vulnerable to loss of this skill. Difficulties in pain recognition increased time to diagnose fracture, and the acute episode was associated with heightened caregiver stress. Service providers can use family-centred practice models to support the strengthening of family functioning following this acute event that is surprisingly common in those with severe disability. [ABSTRACT FROM AUTHOR]

Objective To characterise changing risk factors of preterm birth in Western Australia between 1984 and 2006. Design Population-based study. Setting Western Australia. Population All non-Aboriginal women giving birth to live singleton infants between 1984 and 2006. Methods Multinomial, multivariable regression models were used to assess antecedent profiles by preterm status and labour onset types (spontaneous, medically indicated, prelabour rupture of membranes [ PROM]). Population attributable fraction ( PAF) estimates characterized the contribution of individual antecedents as well as the overall contribution of two antecedent groups: pre-existing medical conditions (including previous obstetric history) and pregnancy complications. Main outcome measure Antecedent relationships with preterm birth, stratified by labour onset type. Results Marked increases in maternal age and primiparous births were observed. A four-fold increase in the rates of pre-existing medical complications over time was observed. Rates of pregnancy complications remained stable. Multinomial regression showed differences in antecedent profiles across labour onset types. PAF estimates indicated that 50% of medically indicated preterm deliveries could be eliminated after removing six antecedents from the population; estimates for PROM and spontaneous preterm reduction were between 10 and 20%. Variables pertaining to previous and current obstetric complications (previous preterm birth, previous caesarean section, pre-eclampsia and antepartum haemorrhage) were the most influential predictors of preterm birth and adverse labour onset ( PROM and medically indicated). Conclusions Preterm antecedent profiles have changed markedly over the 23 years studied. Some changes may be attributable to true change, others to advances in surveillance and detection. Still others may signify change in clinical practice. [ABSTRACT FROM AUTHOR]

Background There is a lack of literature describing dental admissions in children particularly very young children. This paper describes dental and oral cavity admissions and associated factors in children under two years of age using total-population databases. Methods The data used for this study were extracted from population-based databases which are linkable with midwives' data collected on all births in Western Australia. Children born from 1980 to 1998 inclusive ( n= 459 831) were followed until two years of age including data on deaths, hospital admissions, birth defects and intellectual disability. Dental admissions (by ICD-9 category) and associated factors were investigated. Results There were 1513 dental admissions occurring in 1459 of the children up to the age of two years. Children were most frequently admitted under ICD-9 category 521, which includes a hospital admission for dental caries (39% of all oral cavity admissions), followed by ICD-9 category 528 (29%), which includes diseases of the oral soft tissues. Univariate analysis indicated that those with intellectual disability (OR 2.10, 95%CI 1.40-3.16), birth defect (1.74, 1.45-2.09), residing in a region without fluoridated water (2.15, 1.72-2.69) being male (1.14, 1.03-1.26), those from rural areas (2.29, 2.07-2.54) and Indigenous children (4.45, 3.91-5.05) were significantly more likely to have had a dental admission. Conclusion Using total-population data allowed us to describe the admissions in children under two years and associated factors while able to identify children with intellectual disability or birth defects. [ABSTRACT FROM AUTHOR]

Background This study aimed to explore the factors that predict functioning in families with a child with Down syndrome using a mixed methods design. The quantitative component examined the effect of maladaptive and autism-spectrum behaviours on the functioning of the family while the qualitative component explored the impact of having a child with Down syndrome on family holidays, family activities and general family functioning. Methods Participants in this study were 224 primary caregivers of children with Down syndrome aged 4-25 years (57.1% male; 42.9% female) currently residing in Western Australia (74.0% in metropolitan Perth and 26.0% in rural Western Australia). Results Maladaptive and autism-spectrum behaviour were associated with poorer family functioning. Mean total scores on the measures of family functioning and marital adjustment were comparable to that of families of typically developing children. Consistent with the quantitative findings, normality was the most common theme to emerge in the qualitative data. Child problem behaviours were also identified by parents/carers as having a negative impact on the family. Conclusions This study has implications for the development of programs to support families with a child with Down syndrome and may dispel some of the myths surrounding the impact of intellectual disability on the family. [ABSTRACT FROM AUTHOR]

Background: The aim of this study was to compare dental hospital admissions in a total state birth population of Indigenous and non-Indigenous children aged under five years in Western Australia. Methods: Midwives' notification data were linked to databases of deaths, admissions, birth defects and intellectual disability. Births during 1980-1995 were followed until five years of age (n = 383 665). Dental admissions were classified by ICD-9 principal diagnosis categories. Results: There were 738 dental admissions for 665 children aged up to five years of Indigenous mothers (n = 20 921). Indigenous children comprised 6.3% of all children having a dental admission in this age group; 3.2% of children with Indigenous mothers had a dental admission compared with 2.7% of non-Indigenous children. Overall, 8.7% (n = 58) of Indigenous children with a dental admission had a birth defect and 5.5% (n = 23) had an intellectual disability (compared to 8.8% and 3.2%). Indigenous children were four times more likely to be diagnosed with oral soft tissue diseases than non-Indigenous children, and less likely to be categorized as having diseases of the dental hard tissues. Indigenous children were more likely to have a longer dental admission. Conclusions: These analyses provide important findings regarding hospital admissions for Indigenous children. Admissions for disorders of the soft tissues are more common in Indigenous children. [ABSTRACT FROM AUTHOR]

Introduction Government early intervention services for children with intellectual disability (ID) in Western Australia have adopted the model of family-centred care. The aim of this study was to evaluate how well it was being practised, to describe the pattern of service utilization and to identify factors influencing parental perceptions of family-centred care. Methods The study included children aged 0–6 years with ID, who were registered clients of Disability Services Commission, Western Australia. Parents completed a postal survey questionnaire about the frequency and type of services received and their perceptions of services using the Measure of Processes of Care (MPOC-56) questionnaire. Mean scores for the five MPOC domains were compared using anova against the independent variables of child age group, child diagnostic group, service type and frequency, place of residence, family and demographic variables. Significant variables in each domain were then entered into multivariate analyses. Results Of 292 eligible families, 165 (59%) returned a completed questionnaire. While over 50% of children had contact with occupational, speech and physical therapists at least once per month, less than 20% of children had at least annual contact with either psychology or dental services. Families rated their satisfaction highest for ‘respectful and supportive care’ and lowest for ‘providing general information’. Individual item analyses indicated less satisfaction with ‘co-ordinated and comprehensive care’. Higher means were associated with more frequent contact with occupational therapy. Conclusion Overall respondents reported early intervention services for young children with ID in Western Australia provided satisfactory family-centred care by means of the 56-item MPOC. The frequency of contact with allied health professionals was positively associated with parental ratings of family-centred care. The study indicates under-servicing in dental care and psychology services. [ABSTRACT FROM AUTHOR]

Objectives To determine whether children who have child maltreatment allegation or substantiation have a higher rate of general hospital admissions and injury related admissions when compared to other children and to investigate other types of admissions, such as mental health, infections and admissions due to external causes. Study design A prospective matched case-control study of children born in Western Australia between 1990 and 2005 using de-identified record linked Child Protection and Hospital Morbidity data. Rates of prior hospital admissions for cases versus controls were calculated, and conditional logistic regression was used to estimate the effect of hospital admission rate on the risk of child maltreatment allegation and substantiated allegation. Results Children with child maltreatment allegations and substantiations had higher mean prior admission rates compared to controls. Higher rates of general admissions and admissions for injuries, infections, mental and behavioural disorders, and external causes of morbidity, were associated with a markedly increased risk of child maltreatment allegations and substantiation. Conclusions The hospital system plays not only an important role both in the surveillance of maltreatment-related injuries and conditions but also in the role of prevention in the referral of families who may need support and assistance in ensuring the health and safety of their children. This research highlights the importance of moving to electronic patient records in identifying children who have high rates of admissions and the types of conditions they have previously presented with, particularly for injuries, mental and behavioural disorders and external causes of admissions. [ABSTRACT FROM AUTHOR]

Aims: To describe the hospitalisation history in the first five years of life for all children born in Western Australia (WA) between 1983 and 1992 and diagnosed with intellectual disability (ID). Methods: Unit record linkage of the WA Midwives Collection, WA Intellectual Disability Database, and the WA Hospital Morbidity Dataset provided the population database of WA born children with and without ID. Affected children were divided into those co-affected with autism spectrum disorders (ASD), and those whose ID had or had no known biomedical cause. Those without a biomedical cause were further subdivided into mild-moderate and severe categories. Results: On average, ID affected children were more likely than non-affected children to be admitted to hospital (RR: 1.64; 95% CI 1.6 to 1.7), on more occasions (5.3 versus 2.2 admissions), for longer (29.6 versus 8.3 days), and for a larger range of clinical diagnoses. The only exception was the group of children co-diagnosed with ASD whose hospitalisation profile resembled more that of non-affected children. Conclusions: This total population study is unique because of the availability of the system of linkable population registers and administrative health databases in WA. The results indicated that this vulnerable population of children with ID has substantial medical needs. This paper points to the need for authorities to develop supportive programmes for this population especially in the current climate of de-medicalisation of ID. More research is not only needed on the welfare of the affected children but also on the impact of the substantial medical and other needs of affected children on the rest of their immediate and extended families. [ABSTRACT FROM AUTHOR]

Despite the demands it places on individuals, families and the community, intellectual disability (ID) is a neglected area of public health. Accurate estimates of prevalence are sparse and range from 0.5 to 3.0%. The cause of the condition is unknown in at least 50% of cases. This paper describes the Intellectual Disability Exploring Answers (IDEA) database set up in Western Australia to provide an infrastructure for research and to facilitate the planning of service provision for people with ID. Since 1953 a database for ID has been maintained in Western Australia, a state with a population of 1.95 million in an area of 2.52 million km 2 . The current IDEA database aims to obtain ongoing population-based ascertainment of ID from providers of clinical and educational services, with the potential for linkage to a network of other state databases. The average prevalence of ID for children born in Western Australia over the years 1983–1996 was 15.2 per 1000 live births, with 50% ascertained only through the education system. During this time period 60% of cases were male. Of children with an ID born in Western Australia in 1980–1999 and surviving to 1 year, 30.1% had a birth defect, and the prevalence ratio of birth defects in this group compared to the population with no birth defects was 6.5 (CI 6.3–6.8). [ABSTRACT FROM AUTHOR]

Objective: To investigate the prevalence of intellectual disability in Western Australia (WA), its causes, prevention, and trends over time.Methodology: Data from an administrative database of intellectual disability in WA were used to report on the trends in intellectual disability in childhood.Results: The prevalence of intellectual disability was 8.3 per 1000 live births in 1980-90. For half the cases, there was no known cause for the intellectual disability. Down syndrome accounted for 14 to 15% of all cases. Since the introduction of newborn screening, no WA-born child participating in the screening program has been diagnosed with intellectual disability as a result of either phenylketonuria or congenital hypothyroidism. The rate of autism spectrum disorders rose from three to six per 10 000 in the 1980-83 WA birth cohort to 10-13 per 10 000 for the 1989-92 cohort.Conclusions: Recent linkage of this administrative database to the WA Maternal and Child Health Research Data Base provides a unique opportunity for more detailed investigation of intellectual disability and its risk factors in a large, well-ascertained population of children. [ABSTRACT FROM AUTHOR]

A response from the author of the article "Are preterm birth rates influenced by changes in risk factors for preterm birth in Western Australia 1984-2006?" in the 2013 issue is presented.

Objective: To describe trends, age-specific patterns, and factors influencing hospitalizations for 5 rare craniofacial anomalies (CFAs)., Methods: Data on livebirths (1983-2010; n = 721 019) including rare CFA (craniofacial microsomia, mandibulofacial dysostosis, Pierre Robin sequence, Van der Woude syndrome, and frontonasal dysplasia), episodes of death, and demographic and perinatal factors were identified from the Western Australian Register of Developmental Anomalies, Death Registrations and Midwives Notification System. Information on incident craniofacial and noncraniofacial related admissions, length of hospital stay, and intensive care and emergency-related admissions were identified using principal diagnosis and procedural codes were extracted from the Hospital Morbidity Data Collection and linked to other data sources. Associations of hospitalizations by age groups as well as demographic and perinatal factors were expressed as incidence rate ratio (IRR)., Results: The incident hospitalizations were 3 times as high for rare CFA (IRR 3.22-3.72) throughout childhood into adolescence than those without. Children with rare CFA had 3-4 times as many potentially preventable hospitalizations until 18 years of age than those without. Specifically, respiratory infections (IRR 2.13-2.35), ear infections (IRR 7.92-26.28), and oral health-related conditions contributed for most noncraniofacial admissions until the adolescence period. A greater incidence of noncraniofacial related hospitalizations was observed among Indigenous children, births with intrauterine growth restrictions, and families with high socioeconomic disadvantage., Conclusions: Throughout childhood, individuals with rare CFA had greater hospital service use, specifically for potentially preventable conditions, than those without. These population-level findings can inform new preventive strategies and early disease management targeted toward reducing preventable hospitalizations., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Understanding hospital service use among children with a diagnosis of craniosynostosis (CS) is important to improve services and outcomes. This study aimed to describe population-level trends, patterns, and factors influencing hospitalizations for craniosynostosis in Western Australia. Data on live births (1990-2010; n = 554,624) including craniosynostosis, episodes of death, demographic, and perinatal factors were identified from the midwives, birth defects, hospitalizations, and death datasets. Information on craniosynostosis and non-craniosynostosis-related admissions, cumulative length of hospital stay (cLoS), intensive care unit, and emergency department-related admissions were extracted from the hospitalization dataset and linked to other data sources. These associations were examined using negative binomial regression presented as annual percent change and associations of hospitalizations by age groups, demographic, and perinatal factors were expressed as incidence rate ratio (IRR). We found an increasing trend in incident hospitalizations but a marginal decline in cLoS for craniosynostosis over the observed study period. Perinatal conditions, feeding difficulties, nervous system anomalies, respiratory, and other infections contributed to majority of infant non-CS-related admissions.Respiratory infections accounted for about twice the number of admissions for individuals with CS (IRRs 1.94-2.34) across all observed age groups. Higher incidence of non-CS hospitalizations was observed among females, with associated anomalies, to families with highest socioeconomic disadvantage and living in remote areas of the state.   Conclusion: Marginal reduction in the cLoS for CS-related admissions observed over the 21-year period are potentially indicative of improved peri-operative care. However, higher incidence of respiratory infection-related admissions for syndromic synostosis is concerning and requires investigation., (© 2023. The Author(s).)

Background: Children with congenital heart defects (CHDs) are at higher risk of developing an intellectual disability. However, severity of intellectual disabilities among this group of children are largely unknown. Our objective was to determine the risk of intellectual disability (ID), ID severity, and autism among children with CHDs., Methods: We conducted a retrospective cohort study of singleton live births in Western Australia (n = 20,592) between 1983 and 2010. Children with CHDs were identified from the Western Australian Register for Developmental Anomalies (n = 6563) and infants without CHDs were randomly selected from state birth records (n = 14,029). Children diagnosed with ID before 18 years were identified by linkage to statewide Intellectual Disability Exploring Answers database. Odds ratios (OR) and 95% confidence intervals (CI) were calculated from logistic regression models for all CHDs combined and by CHD severity adjusting for potential confounders., Results: Of 20,592 children, 466 (7.1%) with CHDs and 187 (1.3%) without CHDs had an ID. Compared to children without CHDs, children with any CHD had 5.26 times (95% CI 4.42, 6.26) the odds of having an ID and 4.76 times (95% CI 3.98, 5.70) the odds of having mild/moderate ID. Children with any CHD had 1.76 times the odds of having autism (95% CI 1.07, 2.88), and 3.27 times the odds of having an unknown cause of ID (95% CI 2.65, 4.05) compared to children without CHD. The risk of having autism (aOR 3.23, 95% CI 1.11, 9.38), and unknown cause of ID (aOR 3.45, 95% CI 2.09, 5.70) was greatest for children with mild CHD., Conclusions: Children with CHDs were more likely to have an ID or autism. Future research should elucidate underlying etiology of ID in children with CHDs., (© 2023. The Author(s).)

Objective: To describe birth prevalence of rare craniofacial anomalies and associations with antenatal and perinatal factors., Study Design: All live and stillbirths in Western Australia between 1980 and 2010 were identified from the Western Australian Birth Registrations and the Midwives Notification System (also provides information on antenatal and perinatal factors). Rare craniofacial anomalies (craniosynostosis, craniofacial microsomia, and others [Pierre Robin, Van der Woude, and Treacher Collins syndrome]) were ascertained from the Western Australian Register of Developmental Anomalies and linked to other data sources. Trends in prevalence, adjusted for sex and Indigenous status, were investigated by Poisson regression and presented as annual percent change (APC). Strengths of association of related factors were assessed using multivariable log-binomial regression adjusted for sex, Indigenous status, birth year, socioeconomic disadvantage, and remoteness and reported as risk ratios with 95% CIs., Results: There was a temporal increase in prevalence of metopic synostosis (APC 5.59 [2.32-8.96]) and craniofacial microsomia (Goldenhar syndrome) (APC 4.43 [1.94-6.98]). Rare craniofacial anomalies were more likely among infants born preterm, as twins or greater-order multiples, with growth restriction, to older parents, to mothers undertaking fertility treatments, and with pre-existing medical conditions, specifically epilepsy, diabetes, or hypothyroidism. Prenatal identification of rare craniofacial anomalies was uncommon (0.6%)., Conclusions: Our findings indicate a steady increase over time in prevalence of metopic synostosis and craniofacial microsomia (Goldenhar syndrome). Possible associations of fertility treatments and pre-existing maternal medical conditions with rare craniofacial anomalies require further investigation., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Introduction: Individuals with Rett syndrome (RTT) experience impaired gross motor skills, limiting their capacity to engage in physical activities and participation in activities. There is limited evidence of the effectiveness of supported physical activity interventions. This study aims to evaluate the effects of a telehealth-delivered physical activity programme on physical activity, sedentary behaviour and quality of life in RTT., Methods and Analysis: This is a multicentre study, conducted in Australia, Denmark and Israel. It is a randomised waitlist-controlled trial comparing an intervention to support physical activity with usual care. Participants are children and adults with RTT, recruited from the Australian Rett Syndrome Database, the Danish Center for Rett Syndrome and the Rett Syndrome Association of Israel. The intervention duration is 12 weeks, including fortnightly telephone contact to plan, monitor and develop individual activity programmes. Outcomes are measured at baseline, at 13 weeks and then at 25 weeks. The primary outcomes are sedentary behaviour assessed with an activPAL accelerometer and the number of daily steps measured with a StepWatch Activity Monitor. Secondary outcomes include sleep, behaviour and quality of life. Caregiver experiences will be assessed immediately after the intervention using a satisfaction questionnaire. Group differences for each outcome will be evaluated with analysis of covariance, adjusting for baseline values on an intention-to-treat basis., Ethics and Dissemination: Ethics approval has been obtained in Western Australia from the Child and Adolescent Health Services (RGS3371), in Denmark from the Capital Region Ethics Committee (H-19040514) and in Israel from the Ariel University Institutional Review Board (AU-HEA-ML-20190331). Manuscripts on the development of the intervention from pilot work and the results of the intervention will be submitted to peer-reviewed journals. Results will be presented at conferences and consumer forums. We will develop an online resource documenting the physical activity programme and available supporting evidence., Trial Registration Number: NCT04167059; Pre-results., Competing Interests: Competing interests: JD is employed at Telethon Kids Institute where she is program head of Child Disability. The protocol for this study was developed for this grant received from Rettsyndrome.org. The authors do not have competing interests to declare in relation to this study., (© Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Positive health outcomes have been observed following gastrostomy insertion in children with intellectual disability, which is being increasingly used at younger ages to improve nutritional intake. This study investigated the effect of gastrostomy insertion on survival of children with severe intellectual disability., Methods: We used linked disability and health data of children and adolescents who were born in Western Australia between 1983 and 2009 to compare survival of individuals with severe intellectual disability by exposure to gastrostomy status. For those born in 2000-2009, we employed propensity score matching to adjust for confounding by indication. Effect of gastrostomy insertion on survival was compared by pertinent health and sociodemographic risk factors., Results: Compared with children born in the 1980s-1990s, probability of survival following first gastrostomy insertion for those born in 2000-2009 was higher (2 years: 94% vs. 83%). Mortality risk was higher in cases than that in their matched controls (hazard ratio 2.9, 95% confidence interval 1.1, 7.3). The relative risk of mortality (gastrostomy vs. non-gastrostomy) may have differed by sex, birthweight and time at first gastrostomy insertion. Respiratory conditions were a common immediate or underlying cause of death among all children, particularly among those undergoing gastrostomy insertion., Conclusions: Whilst gastrostomy insertion was associated with lower survival rates than children without gastrostomy, survival improved with time, and gastrostomy afforded some protection for the more vulnerable groups, and earlier use appears beneficial to survival. Specific clinical data that may be used to prioritise the need for gastrostomy insertion may be responsible for the survival differences observed., (© 2020 MENCAP and International Association of the Scientific Study of Intellectual and Developmental Disabilities and John Wiley & Sons Ltd.)

Quantification of stillbirth risk has potential to support clinical decision-making. Studies that have attempted to quantify stillbirth risk have been hampered by small event rates, a limited range of predictors that typically exclude obstetric history, lack of validation, and restriction to a single classifier (logistic regression). Consequently, predictive performance remains low, and risk quantification has not been adopted into antenatal practice. The study population consisted of all births to women in Western Australia from 1980 to 2015, excluding terminations. After all exclusions there were 947,025 livebirths and 5,788 stillbirths. Predictive models for stillbirth were developed using multiple machine learning classifiers: regularised logistic regression, decision trees based on classification and regression trees, random forest, extreme gradient boosting (XGBoost), and a multilayer perceptron neural network. We applied 10-fold cross-validation using independent data not used to develop the models. Predictors included maternal socio-demographic characteristics, chronic medical conditions, obstetric complications and family history in both the current and previous pregnancy. In this cohort, 66% of stillbirths were observed for multiparous women. The best performing classifier (XGBoost) predicted 45% (95% CI: 43%, 46%) of stillbirths for all women and 45% (95% CI: 43%, 47%) of stillbirths after the inclusion of previous pregnancy history. Almost half of stillbirths could be potentially identified antenatally based on a combination of current pregnancy complications, congenital anomalies, maternal characteristics, and medical history. Greatest sensitivity is achieved with addition of current pregnancy complications. Ensemble classifiers offered marginal improvement for prediction compared to logistic regression.

Objective: To examine the frequency of hospital admissions before and after gastrostomy insertion in children with severe intellectual disability., Study Design: We conducted a retrospective cohort study using linked health administrative and disability data from Western Australia (WA) and New South Wales (NSW). Children born between 1983 and 2009 in WA and 2002 and 2010 in NSW who had a gastrostomy insertion performed (n = 673 [WA, n = 325; NSW, n = 348]) by the end of 2014 (WA) and 2015 (NSW) were included. Conditional Poisson regression models were used to evaluate the age-adjusted effect of gastrostomy insertion on acute hospitalizations for all-cause, acute lower respiratory tract infections (LRTI), and epilepsy admissions., Results: The incidence of all-cause hospitalizations declined at 5 years after procedure (WA cohort 1983-2009: incidence rate ratio, 0.70 [95% CI, 0.60-0.80]; WA and NSW cohort 2002-2010: incidence rate ratio, 0.63 [95% CI, 0.45-0.86]). Admissions for acute LRTI increased in the WA cohort and remained similar in the combined cohort. Admissions for epilepsy decreased 4 years after gastrostomy in the WA cohort and were generally lower in the combined cohort. Fundoplication seemed to decrease the relative incidence of acute LRTI admissions in the combined cohort., Conclusions: Gastrostomy was associated with health benefits including reduced all-cause and epilepsy hospitalizations, but was not protective against acute LRTI. These decreases in hospitalizations may reflect improved delivery of nutrition and medications., (Crown Copyright © 2019. Published by Elsevier Inc. All rights reserved.)

Objective: To describe the long-term neurodevelopmental and cognitive outcomes for children born preterm., Study Design: In this retrospective cohort study, information on children born in Western Australia between 1983 and 2010 was obtained through linkage to population databases on births, deaths, and disabilities. For the purpose of this study, disability was defined as a diagnosis of intellectual disability, autism, or cerebral palsy. The Kaplan-Meier method was used to estimate the probability of disability-free survival up to age 25 years by gestational age. The effect of covariates and predicted survival was examined using parametric survival models., Results: Of the 720 901 recorded live births, 12 083 children were diagnosed with disability, and 5662 died without any disability diagnosis. The estimated probability of disability-free survival to 25 years was 4.1% for those born at gestational age 22 weeks, 19.7% for those born at 23 weeks, 42.4% for those born at 24 weeks, 53.0% for those born at 25 weeks, 78.3% for those born at 28 weeks, and 97.2% for those born full term (39-41 weeks). There was substantial disparity in the predicted probability of disability-free survival for children born at all gestational ages by birth profile, with 5-year estimates of 4.9% and 10.4% among Aboriginal and Caucasian populations, respectively, born at 24-27 weeks and considered at high risk (based on low Apgar score, male sex, low sociodemographic status, and remote region of residence) and 91.2% and 93.3%, respectively, for those at low risk (ie, high Apgar score, female sex, high sociodemographic status, residence in a major city)., Conclusions: Apgar score, birth weight, sex, socioeconomic status, and maternal ethnicity, in addition to gestational age, have pronounced impacts on disability-free survival., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Objectives: Our overall aim was to evaluate the Western Australian Intellectual Disability Exploring Answers (IDEA) surveillance system. The primary objective was to evaluate the attributes of the system. The secondary objective was to provide recommendations to data custodians and stakeholders to strengthen the system., Method: The IDEA system was evaluated using process observation, interviews and secondary data analysis of system attributes: usefulness, simplicity, data quality, acceptability, representativeness, timeliness and stability. 2001 US Centers for Disease Control and Prevention guidelines were used., Results: We found that the IDEA system was useful, simple, flexible, acceptable, representative, timely and stable. We compared individuals from the IDEA system (n=10 593) with those with cerebral palsy and intellectual disability (ID) (n=582) from another surveillance system. Of the 582 with cerebral palsy and ID, 501 (86.1%) were in the IDEA system and 81 (13.9%) were not. In total, 0.7% of cases (81/10674) with ID were not identified in the IDEA system. There were little differences in cases that were not identified in the IDEA system between Indigenous status, sex and place of residence., Conclusions: The strengths of the IDEA system include having a high data quality resource contributing to national and international data on ID, strong government support and a dedicated management team. Output from studies linking to IDEA data have had major contributions to the international literature about ID. However, limited resources have prevented it from realising its full potential in relation to translational activities. The IDEA system is a valuable resource to address the needs of people living with ID., Competing Interests: Competing interests: JB is employed to work on the IDEA system. HL is the data custodian of the IDEA system., (© Author(s) (or their employer(s)) 2019. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Importance: The origins and development of autism spectrum disorder (ASD) remain unresolved. No individual-level study has provided estimates of additive genetic, maternal, and environmental effects in ASD across several countries., Objective: To estimate the additive genetic, maternal, and environmental effects in ASD., Design, Setting, and Participants: Population-based, multinational cohort study including full birth cohorts of children from Denmark, Finland, Sweden, Israel, and Western Australia born between January 1, 1998, and December 31, 2011, and followed up to age 16 years. Data were analyzed from September 23, 2016 through February 4, 2018., Main Outcomes and Measures: Across 5 countries, models were fitted to estimate variance components describing the total variance in risk for ASD occurrence owing to additive genetics, maternal, and shared and nonshared environmental effects., Results: The analytic sample included 2 001 631 individuals, of whom 1 027 546 (51.3%) were male. Among the entire sample, 22 156 were diagnosed with ASD. The median (95% CI) ASD heritability was 80.8% (73.2%-85.5%) for country-specific point estimates, ranging from 50.9% (25.1%-75.6%) (Finland) to 86.8% (69.8%-100.0%) (Israel). For the Nordic countries combined, heritability estimates ranged from 81.2% (73.9%-85.3%) to 82.7% (79.1%-86.0%). Maternal effect was estimated to range from 0.4% to 1.6%. Estimates of genetic, maternal, and environmental effects for autistic disorder were similar with ASD., Conclusions and Relevance: Based on population data from 5 countries, the heritability of ASD was estimated to be approximately 80%, indicating that the variation in ASD occurrence in the population is mostly owing to inherited genetic influences, with no support for contribution from maternal effects. The results suggest possible modest differences in the sources of ASD risk between countries.

An increased prevalence of autism spectrum disorder (ASD) among children of immigrant backgrounds has been observed but clinical profiles are rarely compared. Diagnostic data from children with ASD notified to the Western Australian Register for Autism Spectrum Disorders were analysed according to maternal-race ethnicity and country of birth. A total of 4776 children aged between 0 and 18 years diagnosed with ASD from 1999 to 2017 were included. Those born to immigrant mothers from lower income countries were younger at the time of diagnosis, had an increased risk of intellectual disability and poorer presentations in the social and communication domains. Further work is required to understand environmental influences that may affect children born to immigrant mothers and to improve monitoring and assessments.

Background: To compare the prevalence of preterm birth, post term birth, intra-uterine growth restriction and distribution of Apgar scores in offspring of foreign-born women in Western Australia with that of their Australian-born non-Indigenous and Indigenous counterparts., Methods: A population-based linked data study, involving 767,623 singleton births in Western Australia between 1980 and 2010 was undertaken. Neonatal outcomes included preterm birth, post term births, intra-uterine growth restriction (assessed using the proportion of optimal birth weight) and low Apgar scores. These were compared amongst foreign-born women from low, lower-middle, upper middle and high income countries and Australian-born non-Indigenous and Indigenous women over two different time periods using multinomial logistic regression adjusted for covariates., Results: Compared with Australian born non-Indigenous women, foreign-born women from low income countries were at some increased risk of extreme preterm (aRRR 1.59, 95% CI 0.87, 2.89) and very early preterm (aRRR 1.63, 95% CI 0.92, 2.89) births during the period from 1980 to 1996. During the period from 1997 to 2010 they were also at some risk of extreme preterm (aRRR 1.42, 95% CI 0.98, 2.04) very early preterm (aRRR 1.34, 95% CI 1.11, 1.62) and post term birth (aRRR 1.93, 95% CI 0.99, 3.78). During this second time period, other adverse outcomes for children of foreign-born women from low income and middle income countries included increases in severe (aRRR 1.69, 95% CI 1.30, 2.20; aRRR 1.72, 95% CI 1.53, 1.93), moderate (aRRR 1.54, 95% CI 1.32, 1.81; aRRR 1.59, 95% CI 1.48, 1.70) and mild (aRRR 1.28, 95% CI 1.14, 1.43; aRRR 1.31, 95% CI 1.25, 1.38) IUGR compared to children of Australian-born non-Indigenous mothers. Uniformly higher risks of adverse outcomes were also demonstrated for infants of Indigenous mothers., Conclusions: Our findings illustrate the vulnerabilities of children born to foreign women from low and middle-income countries. The need for exploratory research examining mechanisms contributing to poorer birth outcomes following resettlement in a developed nation is highlighted. There is also a need to develop targeted interventions to improve outcomes for these women and their families.

Season of birth has been hypothesized to be a risk factor for autism spectrum disorder (ASD). However, the evidence has been mixed and limited due to methodological challenges. We examine ASD birth trends for 5,464,628 births across 5 countries. ASD birth prevalence data were obtained from the International Collaboration for Autism Registry Epidemiology database, including children born in Denmark, Finland, Norway, Sweden, and Western Australia. Empirical mode decomposition and cosinor modeling were used to assess seasonality. We show seasonal variation in ASD births for the countries of Finland and Sweden. There was a modest increase in risk for children born in the fall and a modest decrease in risk for children born in the spring. Solar radiation levels around conception and the postnatal period were inversely correlated with seasonal trends in ASD risk. In the first multinational study of birth seasonality of ASD, there was evidence supporting the presence of seasonal trends in Finland and Sweden. The observations that risk was highest for fall births (i.e., conceived in the winter) and lowest for spring births (i.e., conceived in the summer), and sunlight levels during critical neurodevelopmental periods explained much of the seasonal trends, are consistent with the hypothesis that a seasonally fluctuating risk factor may influence risk of ASD.

The largest group of recipients of pediatric gastrostomy have neurological impairment with intellectual disability (ID). This study investigated trends in first gastrostomy insertion according to markers of disadvantage and ID etiology. Linked administrative and health data collected over a 32-year study period (1983-2014) for children with ID born between 1983 and 2009 in Western Australia were examined. The annual incidence rate change over calendar year was calculated for all children and according to socioeconomic status, geographical remoteness, and Aboriginality. The most likely causes of ID were identified using available diagnosis codes in the linked data set. Of 11,729 children with ID, 325 (2.8%) received a first gastrostomy within the study period. The incidence rate was highest in the 0-2 age group and there was an increasing incidence trend with calendar time for each age group under 6 years of age. This rate change was greatest in children from the lowest socioeconomic status quintile, who lived in regional/remote areas or who were Aboriginal. The two largest identified groups of ID were genetically caused syndromes (15.1%) and neonatal encephalopathy (14.8%).Conclusion: Gastrostomy is increasingly used in multiple neurological conditions associated with ID, with no apparent accessibility barriers in terms of socioeconomic status, remoteness, or Aboriginality. What is Known: • The use of gastrostomy insertion in pediatrics is increasing and the most common recipients during childhood have neurological impairment, most of whom also have intellectual disability (ID). What is New: • Nearly 3% of children with ID had gastrostomy insertion performed, with the highest incidence in children under 3 years of age. • Gastrostomy use across different social groups was equitable in the Australian setting.

Low Apgar score has been associated with higher risk for several neurological and psychiatric disorders, including cerebral palsy and intellectual disability. Studies of the association between Apgar score and autism spectrum disorder (ASD) have been inconsistent. We aimed to investigate (1) the association between low Apgar score at 5 min and risk for ASD, and (2) the modifying effects of gestational age and sex on this association in the largest multinational database of ASD. We included prospective data from 5.5 million individuals and over 33,000 cases of ASD from Norway, Sweden, Denmark and Western Australia who were born between 1984 and 2007. We calculated crude and adjusted risk ratios (RR) with 95% confidence intervals (95% CIs) for the associations between low Apgar score and ASD. All analyses for ASD were repeated for autistic disorder (AD). We used interaction terms and stratified analysis to investigate the effects of sex, gestational age, and birth weight on the association. In fully adjusted models, low Apgar scores (1-3) (RR, 1.42; 95% CI, 1.16-1.74), and intermediate Apgar scores (4-6) (RR, 1.50; 95% CI, 1.36-1.65) were associated with a higher RR of ASD than optimal Apgar score (7-10). The point estimates for low (RR, 1.88; 95% CI, 1.41-2.51) and intermediate Apgar score (RR, 1.54; 95% CI, 1.32-1.81) were larger for AD than for ASD. This study suggests that low Apgar score is associated with higher risk of ASD, and in particular AD. We did not observe any major modifying effects of gestational age and sex, although there seems to be substantial confounding by gestational age and birth weight on the observed association.

The association between a single interpregnancy interval (IPI) and birth outcomes has not yet been explored using matched methods. We modeled the odds of preterm birth, being small for gestational age, and having low birth weight in a second, live-born infant in a cohort of 192,041 sibling pairs born in Western Australia between 1980 and 2010. The association between IPI and birth outcomes was estimated from the interaction between birth order and IPI (with 18-23 months as the reference category), using conditional logistic regression. Matched analysis showed the odds of preterm birth were higher for siblings born following an IPI of <6 months (adjusted interaction odds ratio = 1.22, 95% confidence interval: 1.06, 1.38) compared with those born after an IPI of 18-23 months. There were no significant differences for IPIs of <6 months for other outcomes (small for gestational age or low birth weight). This is the first study to use matched analyses to investigate the association between a single IPI on birth outcomes. IPIs of <6 months were associated with increased odds of preterm birth in second-born infants, although the association is likely smaller than previously estimated by unmatched studies.

Objectives: To examine whether children conceived using assisted reproductive technology (ART) have a higher risk of intellectual disability (ID) compared with non-ART-conceived children and describe known causes of ID in these groups., Methods: We linked ID and ART data from population-based registers in Western Australia. Our cohort included live births from 1994 to 2002 ( n = 210 627) with at least 8 years of follow-up. The prevalence of ID was compared between ART- and non-ART-conceived children, and risk of ID was estimated using Poisson regression with robust SEs. We also stratified by plurality and gestation at delivery., Results: Children conceived using ART had a small increased risk of ID (risk ratio 1.58; 95% confidence interval 1.19-2.11) even when analyses were restricted to singleton births (risk ratio 1.56; 95% confidence interval 1.10-2.21). The risk of ID was more than doubled for those born very preterm, for severe ID, and after intracytoplasmic sperm injection (ICSI) treatments. Children conceived using ICSI had a greater risk of ID than those conceived using in vitro fertilization and were more likely to have a known genetic cause for ID (27.6% vs 12.9% in vitro fertilization and 11.9% non-ART)., Conclusions: The risk of ID was increased in children born after ART in Western Australia from 1994 to 2002. More recent cohorts should be examined to assess the impact of important changes in ART clinical practice. Our results are particularly pertinent because multiple embryo transfers are routinely performed in many countries, increasing the risk of preterm birth, and ICSI use rates are high., Competing Interests: POTENTIAL CONFLICT OF INTEREST: Dr Hart is a part owner and shareholder of an in vitro fertilization company, has received travel grants and honoraria from pharmaceutical manufacturers of gonadotrophins, and is on the medical advisory board of pharmaceutical companies that manufacture gonadotrophins; the other authors have indicated they have no potential conflicts of interest to disclose., (Copyright © 2018 by the American Academy of Pediatrics.)

Objective: Gastrostomy insertion in pediatrics is usually used in children with complex needs and severe disability. The accessibility and acceptance of the procedure is increasing but population-based occurrence data are lacking and there is limited understanding of its use in clinical subgroups., Methods: This birth cohort study investigated the trends in first gastrostomy insertion among a pediatric population born between 1983 and 2009 in Western Australia using linked administrative and health data collected over a 32-year period (1983-2014). Indications were identified using diagnosis codes from linked hospitalization data and grouped according to a refined classification system. Age and birth cohort patterns of first gastrostomy use, over calendar year and age respectively, were described., Results: Of the 690,688 children born between 1983 and 2009, 466 underwent a gastrostomy insertion. Overall, the prevalence was approximately 7 cases per 10,000 births. New gastrostomy insertions were increasingly performed in children during the preschool years over calendar years and in successive birth cohorts. Children with a neurological disorder constituted the largest group receiving gastrostomy (n = 372; 79.8) including 325 (87.4%) with comorbid intellectual disability., Conclusions: New gastrostomy insertion among children who require long-term enteral feeding support increased over the study period. The procedure is most often performed in the context of severe neurological disability, including intellectual disability, and offers families potential for long-term home-based management of feeding difficulties.

Objectives: To investigate how well intellectual disability (ID) can be ascertained using hospital morbidity data compared with a population-based data source., Design, Setting and Participants: All children born in 1983-2010 with a hospital admission in the Western Australian Hospital Morbidity Data System (HMDS) were linked with the Western Australian Intellectual Disability Exploring Answers (IDEA) database. The International Classification of Diseases hospital codes consistent with ID were also identified., Main Outcome Measures: The characteristics of those children identified with ID through either or both sources were investigated., Results: Of the 488 905 individuals in the study, 10 218 (2.1%) were identified with ID in either IDEA or HMDS with 1435 (14.0%) individuals identified in both databases, 8305 (81.3%) unique to the IDEA database and 478 (4.7%) unique to the HMDS dataset only. Of those unique to the HMDS dataset, about a quarter (n=124) had died before 1 year of age and most of these (75%) before 1 month. Children with ID who were also coded as such in the HMDS data were more likely to be aged under 1 year, female, non-Aboriginal and have a severe level of ID, compared with those not coded in the HMDS data. The sensitivity of using HMDS to identify ID was 14.7%, whereas the specificity was much higher at 99.9%., Conclusion: Hospital morbidity data are not a reliable source for identifying ID within a population, and epidemiological researchers need to take these findings into account in their study design., Competing Interests: Competing interests: None declared., (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Background: Australian Aboriginal children have increased infant and childhood mortality compared with Caucasian children, but their mortality related to congenital heart defects (CHDs) throughout life is unknown., Methods: We conducted a retrospective cohort study using data on 8,110 live born, singleton infants with CHDs born January 1980 to December 2010 from the Western Australian Register of Developmental Anomalies. Vital status was determined from death and medical records. Data for infants with chromosomal anomalies (except Down syndrome) were excluded. Kaplan-Meier Product-Limit estimates and 95% confidence intervals (CIs) were computed by Aboriginality. Hazard ratios (HRs) and 95% CIs were calculated from multivariable Cox-Proportional Hazard Regression models., Results: Aboriginal children had lower survival than Caucasians for all CHDs combined but most notably during the neonatal period for functional single ventricle (50.0% vs. 86.1%; p = 0.015) and during the postneonatal period for tetralogy of Fallot (87.0% vs. 97.4%; p = 0.021) and atrioventricular septal defect (60.0% vs. 94.6%; p = 0.010). After adjusting for covariates except remoteness and socioeconomic status (SES), Aboriginal children with all CHDs combined (HR = 1.4; 95% CI, 1.0-1.9), with transposition of the great arteries (HR = 4.3; 95% CI, 1.0-18.9) or functional single ventricle (HR = 8.6; 95% CI, 1.3-57.9) had increased risk of mortality compared with Caucasian children. When remoteness and SES were included, the risks were not statistically significant., Conclusion: Long-term survival was lower for Aboriginal children with CHDs, and Aboriginal children with specific CHD phenotypes had increased risk of mortality throughout life. Increased risk may be due to SES and environmental factors. Birth Defects Research (Part A), 2016. © 2016 Wiley Periodicals, Inc. Birth Defects Research (Part A) 106:1016-1031, 2016. © 2016 Wiley Periodicals, Inc., (© 2016 Wiley Periodicals, Inc.)

To investigate the prevalence of intellectual disability (ID) and/or autism spectrum disorders (ASDs) in Western Australia (WA).A cohort of children born from 1983 to 2010 in WA with an ID and/or ASD were identified using the population-based IDEA (Intellectual Disability Exploring Answers) database, which ascertains cases through the Disability Services Commission (DSC) as well as education sources. Information on race, gender, mother's residence at birth and deaths was obtained through linkage to the Midwives Notification System and the Mortality Register. Diagnostic information on the cause of ID was obtained through review of medical records where available and children were classified as biomedical cause, ASD, or unknown cause.An overall prevalence of ID of 17.0/1000 livebirths (95% CI: 16.7, 17.4) showed an increase from the 10-year previous prevalence of 14.3/1000. The prevalence for mild or moderate ID was 15.0 (95% CI: 14.6, 15.3), severe ID was 1.2 (95% CI: 1.1, 1.3), and unknown level of ID was 0.9 (95% CI: 0.8, 1.0)/1000 livebirths. The prevalence for Aboriginal children was 39.0/1000 compared with 15.7/1000 for non-Aboriginal children, giving a prevalence ratio of 2.5 (95% CI: 2.4, 2.6). Prevalence of all ASD was 5.1/1000 of which 3.8/1000 had ASD and ID.The prevalence of ID has risen in WA over the last 10 years with most of this increase due to mild or moderate ID. Whilst the prevalence of ASD has also increased over this time this does not fully explain the observed increase. Aboriginal children are at a 2.5-fold risk of ID but are less likely to be accessing disability services.

Background: Mothers of a child with autism spectrum disorder have more psychiatric disorders after the birth of their child. This might be because they have more psychiatric disorders before the birth, or the increase could be related to the burden of caring for their child., Aims: We aimed to calculate the incidence of a psychiatric diagnosis in women with no psychiatric history after the birth of their eldest child with autism spectrum disorder compared to women with no child with autism spectrum disorder or intellectual disability and no psychiatric history., Methods: By linking datasets from Western Australian population-based registries, we calculated the incidence of a psychiatric disorder in mothers of children with autism spectrum disorder and compared to mothers of children with no autism spectrum disorder or intellectual disability. Negative binomial regression using STATA 13 was used for all analyses., Results: Apart from alcohol and substance abuse, mothers of children with autism spectrum disorder had higher incidences of all categories of psychiatric disorders than other mothers., Conclusion and Implications: The increase of psychiatric disorders in mothers of children with autism spectrum disorder and no psychiatric history compared to similar mothers with no child with autism spectrum disorder or intellectual disability might be due to a pre-existing genetic disposition coupled with an environmental trigger provided by the challenges of raising their children with autism spectrum disorder. In addition, the increased burden borne by the mothers of children with autism spectrum disorder might result in a higher incidence of psychiatric disorders in mothers who are not genetically disposed., (© The Author(s) 2015.)

Background: Previous research shows that maternal mental illness is an important risk factor for child maltreatment. This study aims to quantify the relationship between maternal mental health and risk of child maltreatment according to the different types of mental health diagnoses., Methods: The study used a retrospective cohort of children born in Western Australia between 1990 and 2005, with deidentified linked data from routine health and child protection collections., Results: Nearly 1 in 10 children (9.2%) of mothers with a prior mental health contact had a maltreatment allegation. Alternatively, almost half the children with a maltreatment allegation had a mother with a mental health contact. After adjusting for other risk factors, a history of mental health contacts was associated with a more than doubled risk of allegations (HR=2.64, 95% CI 2.50 to 2.80). Overall, all mental health diagnostic groups were associated with an increased risk of allegations. The greatest risk was found for maternal intellectual disability, followed by disorders of childhood and psychological development, personality disorders, substance-related disorders, and organic disorders. Maltreatment allegations were substantiated at a slightly higher rate than for the general population., Conclusions: Our study shows that maternal mental health is an important factor in child protection involvement. The level of risk varies across diagnostic groups. It is important that mothers with mental health issues are offered appropriate support and services. Adult mental health services should also be aware and discuss the impact of maternal mental health on the family and children's safety and well-being., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/)

Background: The caesarean delivery rate in the developed world has been increasing. It is not well understood how caesarean delivery rates have changed by gestational age at birth in Western Australia, particularly in relation to the introduction of the early-term delivery guidelines in Australia in 2006., Methods: Data from the Western Australian Midwives Notification System were used to identify 193,136 singletons born to primiparous women at 34-42 weeks' gestation during 1995-2010. Caesarean delivery rates were calculated by gestational age group (34-36 weeks, 37-38 weeks, and 39-42 weeks) and stratified into pre-labour and in-labour caesarean delivery. The average annual percent change (AAPC) for the caesarean delivery rates was calculated using joinpoint regression. Log-binomial regression was used to estimate the risk of having a caesarean delivery while adjusting for maternal and antenatal factors., Results: Caesarean delivery rates rose steadily from 1995 to 2005 (AAPC = 5.9%, [95% confidence interval (CI) 4.9, 6.9]), but stabilised since then (AAPC = 0.9%, [95% CI -1.9, 3.8]). The rate of in-labour caesarean deliveries rose consistently from 1995 to 2010 across all gestational age groups. The pre-labour caesarean delivery rate rise was most dominant at 37-38 weeks' gestation from 1995 to 2005 (AAPC = 6.8%, [95% CI 5.4, 8.2]), but declined during 2006-10 (AAPC = -4.5, [95% CI -6.7, -2.3]), while at the same time the rate at 39-42 weeks rose slightly., Conclusions: The rise in pre-labour caesarean deliveries during 1995-2005 occurred predominantly at 37-38 weeks' gestation, but declined again from 2006 to 2010. This suggests that the recently developed Australian early-term delivery guidelines may have had some success in reducing early-term deliveries in Western Australia., (© 2015 John Wiley & Sons Ltd.)

People with intellectual disabilities are at a higher risk for experiencing behavioral, emotional, and psychiatric problems in comparison with the general population. People with Down syndrome have been reported as experiencing fewer behavioral problems than others with intellectual disability, although still at a greater level than the non-intellectually disabled population, except for depression and Alzheimer disease. The aim of this study was to describe the trajectories of subscales of behavior, including depressive symptoms, communication disturbance, anxiety, disruptiveness, and social relating abilities, for young adults with Down syndrome.Families of young adults with Down syndrome living in Perth, Western Australia, participated in a questionnaire study over 8 years, 2004 (n = 255), 2009 (n = 191), and 2011 (n = 188). Questionnaires collected information about young person characteristics and family functioning. The parent-completed Developmental Behavior Checklist-Adult (DBC-A) and Developmental Behavior Checklist-Primary Carer Version (DBC-P) were used to measure emotional and behavioral problems. These measures include the following subscales: disruptive, communication and anxiety disturbances, self-absorbed, antisocial, depressive, and social relating.DBC score declined from 2004 to 2011 reflecting an improvement in behavior in the self-absorbed (coeff -0.011, 95% confidence interval (CI) -0.031, -0.008), anxiety (coef -0.009 95%CI -0.129, -0.006), communication disturbances (coeff -0.008, 95% CI -0.012, -0.005) and disruptive/antisocial behavior (coeff -0.013, 95% CI -0.016, -0.009) subscales. Subscales for depressive symptoms and social relating problems decreased less (coeff -0.003, 95% CI -0.007, -0.0001) (coeff -0.003 95% CI -0.007, 0.001). Young people who were lower functioning were reported as exhibiting significantly more behavioral problems across every subscale when compared with those who were higher functioning.Behavior of young adults with Down syndrome improves over time but depressive symptoms and social relating behavior problems persist into adulthood. It is possible that those with persistent depressive symptoms are at a high risk for developing depressive illness in adulthood. Identifying young people with Down syndrome who are at risk for developing depression in adult life has implications for prevention and early treatment.

Mothers of a child with intellectual disability (ID) have more psychiatric disorders after the birth of their child than other mothers. However, it is unclear if this is because they have more psychiatric disorders before the birth or if the increase is related to the burden of caring for the child. We aimed to calculate the rate of new psychiatric disorders in mothers after the birth of their eldest child with ID born between 1983 and 2005 and to compare these with rates in women with a child with no ID or autism spectrum disorder (ASD) born during the same period. By linking data from Western Australian population-based registries, we selected women with no psychiatric history who survived the birth of their live-born child (N = 277,559) and compared rates of psychiatric disorders for women with a child with ID and women without a child with or ASD. Negative binomial regression with STATA 12 was used for all analyses. Mothers of children with mild-moderate ID of unknown cause had around two to three and a half times the rate of psychiatric disorders of mothers of children without ID or ASD. Mothers of children with Down syndrome and no pre-existing psychiatric disorder showed resilience and had no impairments in their mental health. Interventions and services are needed for mothers of other children with ID to improve their mental health. Further research is implicated to explore the mental health of mothers of children with ID and a pre-existing psychiatric disorder., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

Introduction: Mothers of children with intellectual disability or autism spectrum disorder (ASD) have poorer health than other mothers. Yet no research has explored whether this poorer health is reflected in mortality rates or whether certain causes of death are more likely. We aimed to calculate the hazard ratios for death and for the primary causes of death in mothers of children with intellectual disability or ASD compared to other mothers., Methods: The study population comprised all mothers of live-born children in Western Australia from 1983-2005. We accessed state-wide databases which enabled us to link socio-demographic details, birth dates, diagnoses of intellectual disability or ASD in the children and dates and causes of death for all mothers who had died prior to 2011. Using Cox Regression with death by any cause and death by each of the three primary causes as the event of interest, we calculated hazard ratios for death for mothers of children intellectual disability or ASD compared to other mothers., Results and Discussion: During the study period, mothers of children with intellectual disability or ASD had more than twice the risk of death. Mothers of children with intellectual disability were 40% more likely to die of cancer; 150% more likely to die of cardiovascular disease and nearly 200% more likely to die from misadventure than other mothers. Due to small numbers, only hazard ratios for cancer were calculated for mothers of children with ASD. These mothers were about 50% more likely to die from cancer than other mothers. Possible causes and implications of our results are discussed., Conclusion: Similar studies, pooling data from registries elsewhere, would improve our understanding of factors increasing the mortality of mothers of children with intellectual disability or ASD. This would allow the implementation of informed services and interventions to improve these mothers' longevity.

Background: Infants born moderate to late preterm are twice as likely to be rehospitalised within the first few weeks following discharge from the birth admission. It is not understood how rehospitalisation risk changes with age or how risks have changed over time., Methods: A retrospective birth cohort study of all live, singleton births in Western Australia 1 January 1980-31 December 2010, without congenital anomalies, followed to 18 years of age. Rehospitalisation rates for gestational age categories (<28, 28-31, 32-33, 34-36, 37-38 and ≥42 weeks) were compared with term births (39-41 weeks) using negative binomial regression. To assess whether rehospitalisation risk changed with age or over time, analyses were conducted for different age intervals and for 5-year birth cohorts., Results: Rehospitalisation rates were higher up to 18 years for all preterm and early term categories including early term (37-38 weeks) [130.2/1000 person-years at risk (pyr); 95% confidence interval 129.1, 131.4]; late preterm (34-36 weeks) (164.2/1000 pyr; 161.1, 167.4), and post-term (≥42 weeks) (115.3/1000 pyr; 111.7, 119.0) compared with term births (109.1/1000 pyr; 108.5, 109.7). The effect of gestational age on rehospitalisation was highest during the first year of life and declined by adolescence [e.g. 34-36 weeks: rate ratio = 2.10 (2.04, 2.15) for 29 days-1 year; 1.14 (1.11, 1.18) for 12-18 years]. The risk of rehospitalisation up to 1 year of age has declined since 1980, except for those born <32 weeks., Conclusions: Rehospitalisation risk is greater for singleton children born at all gestational ages compared with those born full term. This effect of gestational age on rehospitalisation is highest in the first year post-discharge, but has almost disappeared by adolescence., (© 2014 John Wiley & Sons Ltd.)

Aim: To investigate the relationship between functioning and post-school day occupation for young adults with Down syndrome., Methods: Families of young people with Down syndrome (n = 269) aged 15-30 years in 2009 were recruited from the population-based Down syndrome 'Needs Opinion Wishes' database in Western Australia. Questionnaires were mailed to participating families and involved two parts, young person characteristics and family functioning; 203 were returned (75%). Of those families who returned questionnaires, 164 (80.8%) of their young adults had left school. Participation in post-school day occupations was the main outcome and included; open employment, training, sheltered employment or alternatives to employment (ATE)., Results: Young adults were reported as participating in open employment (n = 42), training (n = 17), sheltered employment (n = 64) or ATE (n = 41) post-school. Those who reported better functioning in self-care, community and communication skills were more likely to be in open employment and/or attending Technical and Further Education compared with those attending sheltered employment and/or ATE after adjusting for age, gender and rural/metropolitan regions. Current health as measured by visits to a general practitioner (GP) and hospitalizations revealed a weak relationship with post-school day occupations, with increasing likelihood of participating in open employment or training with increasing hospitalizations and GP visits., Conclusions: Our analysis shows that functioning in activities of daily living was related to post-school day occupation. Current health status and behaviour were found to have a weak relationship with post-school day occupation adjusting for functioning in the final model., (© 2013 John Wiley & Sons Ltd.)

Objective: Children with Down syndrome, the most common genetic cause of intellectual disability, are prone to multiple and varied health-related problems. This study describes patterns of hospitalisations for children and young people with Down syndrome in Western Australia., Methods: Birth records were linked to the Western Australian population-based Intellectual Disability database to identify all children born with Down syndrome in Western Australia between 1 January, 1983 and 31 December, 1999. These records were linked to the Hospital Morbidity Data System to provide information on all hospitalisations up to 31 December, 2004. Hospitalisation data, coded using ICD-9CM or ICD-10 (v0.5) were grouped into clinically relevant categories using the primary diagnosis. Rates of hospital admission for all and specific diagnoses were expressed in 1000-person-years at-risk and median age at first admission and length of stay were calculated., Results: Of the 405 children, 395 had one or more hospital admissions, totalling 3786 admissions for all children and an estimated 39.5 person-years in hospital. On average, children were admitted 9.7 times, with an estimated rate of 757.2 admissions per 1000pyr (95% CI: 680, 843). A quarter of all admissions occurred in the first year of life. The average hospital length of stay was 3.8 days (95% CI: 3.7, 4.1). Upper respiratory tract conditions affected the most children (58.5%) and accounted for 12.1% of all admissions. Other disorders which affected a high percentage of children were ear/hearing conditions (50.6%), disorders of the oral cavity (38.0%) and lower respiratory tract conditions (37.5%). Overall, children with Down syndrome were hospitalised at a rate five times (95% CI = 4.3-6.2) that of the general population., Conclusion: Children with Down syndrome are at increased risk of morbidity for varied causes underlining the importance of comprehensive and targeted primary care for this group.

Objective: To characterise changing risk factors of preterm birth in Western Australia between 1984 and 2006., Design: Population-based study., Setting: Western Australia., Population: All non-Aboriginal women giving birth to live singleton infants between 1984 and 2006., Methods: Multinomial, multivariable regression models were used to assess antecedent profiles by preterm status and labour onset types (spontaneous, medically indicated, prelabour rupture of membranes [PROM]). Population attributable fraction (PAF) estimates characterized the contribution of individual antecedents as well as the overall contribution of two antecedent groups: pre-existing medical conditions (including previous obstetric history) and pregnancy complications., Main Outcome Measure: Antecedent relationships with preterm birth, stratified by labour onset type., Results: Marked increases in maternal age and primiparous births were observed. A four-fold increase in the rates of pre-existing medical complications over time was observed. Rates of pregnancy complications remained stable. Multinomial regression showed differences in antecedent profiles across labour onset types. PAF estimates indicated that 50% of medically indicated preterm deliveries could be eliminated after removing six antecedents from the population; estimates for PROM and spontaneous preterm reduction were between 10 and 20%. Variables pertaining to previous and current obstetric complications (previous preterm birth, previous caesarean section, pre-eclampsia and antepartum haemorrhage) were the most influential predictors of preterm birth and adverse labour onset (PROM and medically indicated)., Conclusions: Preterm antecedent profiles have changed markedly over the 23 years studied. Some changes may be attributable to true change, others to advances in surveillance and detection. Still others may signify change in clinical practice., (© 2013 The Authors BJOG An International Journal of Obstetrics and Gynaecology © 2013 RCOG.)

Aim: The aim of this study was to examine the association between maternal alcohol use disorder and intellectual disability in children., Method: All mothers with an International Classification of Diseases (ICD) 9 and/or 10 alcohol-related diagnosis, a proxy for alcohol use disorder, recorded on the Western Australian health, mental health, and drug and alcohol data sets were identified through the Western Australian Data Linkage Unit (n=5614 non-Aboriginal; n=2912 Aboriginal). A comparison cohort of mothers without an alcohol-related diagnosis was frequency matched on maternal age within maternal Aboriginal status and year of birth of their children. Linkage with the Western Australian Midwives Notification System (1983-2001) identified all births to these mothers (n=10 664 and 7907 respectively). Linkage to the Western Australian Intellectual Disability Database and Register of Developmental Anomalies identified cases of intellectual disability with no identified genetic origin (intellectual disability) (n=1487) and fetal alcohol syndrome (n=66). Odds ratios (ORs) and 95% confidence intervals (CIs) for intellectual disability were calculated using logistic regression incorporating generalized estimating equations and used to estimate population-attributable fractions., Results: At least 3.8% (95% CI 2.84-4.89%) of cases of intellectual disability could be avoided by preventing maternal alcohol use disorder: 1.3% (95% CI 0.81-1.86%) in non-Aboriginal and 15.6% (95% CI 10.85-20.94%) in Aboriginal children. We observed a three-fold increase in the adjusted odds of intellectual disability in children of mothers with an alcohol-related diagnosis recorded during pregnancy (non-Aboriginal OR 2.89, 95% CI 1.62-5.18; Aboriginal OR 3.12, 95% CI 2.13-4.56), with a net excess proportion of 3.7% and 5.5% respectively. One-third (32%) of children diagnosed with fetal alcohol syndrome had intellectual disability., Interpretation: Maternal alcohol use disorder is the leading known risk factor for intellectual disability with no identified genetic origin., (© The Authors. Developmental Medicine & Child Neurology © 2012 Mac Keith Press.)

Background: Publicly insured women usually have a different demographic background to privately insured women, which is related to poor neonatal outcomes after birth. Given the difference in nature and risk of preterm versus term births, it would be important to compare adverse neonatal outcomes after preterm birth between these groups of women after eliminating the demographic differences between the groups., Methods: The study population included 3085 publicly insured and 3380 privately insured, singleton, preterm deliveries (32-36 weeks gestation) from Western Australia during 1998-2008. From the study population, 1016 publicly insured women were matched with 1016 privately insured women according to the propensity score of maternal demographic characteristics and pre-existing medical conditions. Neonatal outcomes were compared in the propensity score matched cohorts using conditional log-binomial regression, adjusted for antenatal risk factors. Outcomes included Apgar scores less than 7 at five minutes after birth, time until establishment of unassisted breathing (>1 minute), neonatal resuscitation (endotracheal intubation or external cardiac massage) and admission to a neonatal special care unit., Results: Compared with infants of privately insured women, infants of publicly insured women were more likely to receive a low Apgar score (ARR = 2.63, 95% CI = 1.06-6.52) and take longer to establish unassisted breathing (ARR = 1.61, 95% CI = 1.25-2.07), yet, they were less likely to be admitted to a special care unit (ARR = 0.84, 95% CI = 0.80-0.87). No significant differences were evident in neonatal resuscitation between the groups (ARR = 1.20, 95% CI = 0.54-2.67)., Conclusions: The underlying reasons for the lower rate of special care admissions in infants of publicly insured women compared with privately insured women despite the higher rate of low Apgar scores is yet to be determined. Future research is warranted in order to clarify the meaning of our findings for future obstetric care and whether more equitable use of paediatric services should be recommended.

Background: As well as being highly comorbid conditions, autism spectrum disorders (ASD) and intellectual disability (ID) share a number of clinically-relevant phenomena. This raises questions about similarities and overlap in diagnosis and aetiological pathways that may exist for both conditions., Aims: To examine maternal conditions and perinatal factors for children diagnosed with an ASD, with or without ID, and children with ID of unknown cause, compared with unaffected children., Methods: The study population comprised all live singleton births in Western Australia (WA) between January 1984 and December 1999 (N = 383,153). Univariate and multivariate multinomial logistic regression models were applied using a blocked modelling approach to assess the effect of maternal conditions, sociodemographic factors, labour and delivery characteristics and neonatal outcomes., Results: In univariate analyses mild-moderate ID was associated with pregnancy hypertension, asthma, urinary tract infection, some types of ante-partum haemorrhage, any type of preterm birth, elective C-sections, breech presentation, poor fetal growth and need for resuscitation at birth, with all factors showing an increased risk. Severe ID was positively associated with poor fetal growth and need for resuscitation, as well as any labour or delivery complication. In the multivariate analysis no maternal conditions or perinatal factors were associated with an increased risk of ASD without ID. However, pregnancy hypertension and small head circumference were associated with a reduced risk (OR = 0.64, 95% CI: 0.43, 0.94; OR = 0.58, 95% CI: 0.34, 0.96, respectively). For ASD with ID, threatened abortion before 20 weeks gestation and poor fetal growth were associated with an increased risk., Conclusion: Findings show that indicators of a poor intrauterine environment are associated with an elevated risk of ID, while for ASD, and particularly ASD without ID, the associations are much weaker. As such, these findings highlight the importance of accounting for the absence or presence of ID when examining ASD, if we are to improve our understanding of the causal pathways associated with these conditions.

Aim: The purpose of this study was to describe qualitatively the experience of parenting for mothers of a child with Down syndrome and to explore what if any was the role of spirituality and organized religion in this experience., Method: A homogenous sample of eight mothers of children between 7 and 12 years of age with Down syndrome was recruited through a population-based source of families of children with Down syndrome in Western Australia. In-depth interviews were used to explore the mother's experience of parenting and to examine the role of spirituality and organized religion in their personal experience of mothering., Results: In this study, stressful life events recounted by the mothers included initial acceptance, developmental behaviour of the child, functionality of the child, health conditions and financial stress. Overall spirituality was described as a stronger and more dynamic source of support than organized religion in coping with stressors and life's challenges associated with raising a child with Down syndrome., Conclusion: Findings from this study revealed that being a mother to a child with Down syndrome can best be described as a mosaic of experiences, emotions and a journey of self growth. Both spirituality and organized religion to a greater or lesser extent were useful in mediating stress and supporting mothers particularly during challenging life events in the course of their journey with their child with Down syndrome.

Prevalence statistics for autism spectrum disorders (ASD) vary widely across geographical boundaries. Some variation can be explained by diagnostic methods, case ascertainment and age at diagnosis. This study compared prevalence statistics for two distinct geographical regions, Denmark and Western Australia, both of which have had population-based registers and consistent classification systems operating over the past decade. Overall ASD prevalence rates were higher in Denmark (68.5 per 10,000 children) compared with Western Australia (51.0 per 10,000 children), while the diagnosis of childhood autism was more prevalent in Western Australia (39.3 per 10,000 children) compared with Denmark (21.8 per 10,000 children). These differences are probably caused by local phenomena affecting case ascertainment but influence from biological or geographical factors may exist.

Background: Research findings investigating the sociodemographics of autism spectrum disorder (ASD) have been inconsistent and rarely considered the presence of intellectual disability (ID)., Methods: We used population data on Western Australian singletons born from 1984 to 1999 (n = 398,353) to examine the sociodemographic characteristics of children diagnosed with ASD with or without ID, or ID without ASD compared with non-affected children., Results: The profiles for the four categories examined, mild-moderate ID, severe ID, ASD without ID and ASD with ID varied considerably and we often identified a gradient effect where the risk factors for mild-moderate ID and ASD without ID were at opposite extremes while those for ASD with ID were intermediary. This was demonstrated clearly with increased odds of ASD without ID amongst older mothers aged 35 years and over (odds ratio (OR) = 1.69 [CI: 1.18, 2.43]), first born infants (OR = 2.78; [CI: 1.67, 4.54]), male infants (OR = 6.57 [CI: 4.87, 8.87]) and increasing socioeconomic advantage. In contrast, mild-moderate ID was associated with younger mothers aged less than 20 years (OR = 1.88 [CI: 1.57, 2.25]), paternal age greater than 40 years (OR = 1.59 [CI: 1.36, 1.86]), Australian-born and Aboriginal mothers (OR = 1.60 [CI: 1.41, 1.82]), increasing birth order and increasing social disadvantage (OR = 2.56 [CI: 2.27, 2.97]). Mothers of infants residing in regional or remote areas had consistently lower risk of ASD or ID and may be linked to reduced access to services or under-ascertainment rather than a protective effect of location., Conclusions: The different risk profiles observed between groups may be related to aetiological differences or ascertainment factors or both. Untangling these pathways is challenging but an urgent public health priority in view of the supposed autism epidemic.

Purpose:  To describe leisure participation for school-aged children with Down syndrome and to investigate how factors, classified by the World Health Organisation's International Classification of Functioning, Disability and Health, influence their leisure participation., Method:  Families in Western Australia with a child aged 5-18 years with Down syndrome were surveyed in a population-based study (n = 208) in 2004., Results:  One-third of parents reported that their child with Down syndrome had no friends although half reported two or more friends. Factors affecting number of friendships included the child's functional ability, behavioural issues and parent's availability of time. Those children with higher functional independence scores in daily tasks were more likely to have two or more friends than those with lower functional independence scores (OR: 1.02, 95% CI 1.01-1.04 for a single point increase in WeeFIM score). All children participated in predominantly solitary and sedentary leisure activities., Conclusions:  Leisure participation was affected by complex factors both within and external to the child with Down syndrome. Further investigation of the relevance of these factors to leisure may enable more satisfying and meaningful participation in leisure for school-aged children with Down syndrome.