Cohort profile: Scotland's record-linkage e-cohorts of people with intellectual disabilities, and autistic people (SCIDA).

PURPOSE: To investigate health, mortality and healthcare inequalities experienced by people with intellectual disabilities, and autistic people, and their determinants; an important step towards identifying and implementing solutions to reduce inequalities. This paper describes the cohorts, record-linkages and variables that will be used. PARTICIPANTS: Scotland's Census, 2011 was used to identify Scotland's citizens with intellectual disabilities, and autistic citizens, and representative general population samples with neither. Using Scotland's community health index, the Census data (demography, household, employment, long-term conditions) were linked with routinely collected health, death and healthcare data: Scotland's register of deaths, Scottish morbidity data 06 (SMR06: cancer incidence, mortality, treatments), Prescribing Information System (identifying asthma/chronic obstructive pulmonary disease; angina/congestive heart failure/hypertension; peptic ulcer/reflux; constipation; diabetes; thyroid disorder; depression; bipolar disorders; anxiety/sleep; psychosis; attention deficit hyperactivity disorder; epilepsy; glaucoma), SMR01 (general/acute hospital admissions and causes, ambulatory care sensitive admissions), SMR04 (mental health admissions and causes), Scottish Care Information-Diabetes Collaboration (diabetic care quality, diabetic outcomes), national bowel screening programme and cervical screening. FINDINGS TO DATE: Of the whole population, 0.5% had intellectual disabilities, and 0.6% were autistic. Linkage was successful for >92%. The resultant e-cohorts include: (1) 22 538 people with intellectual disabilities (12 837 men and 9701 women), 4509 of whom are children <16 years, (2) 27 741 autistic people (21 390 men and 6351 women), 15 387 of whom are children <16 years and (3) representative general population samples with neither condition. Very good general health was reported for only 3389 (15.0%) people with intellectual disabilities, 10 510 (38.0%) autistic people, compared with 52.4% general population. Mental health conditions were reported for 4755 (21.1%) people with intellectual disabilities, 3998 (14.4%) autistic people, compared with 4.2% general population. FUTURE PLANS: Analyses will determine the extent of premature mortality, causes of death, and avoidable deaths, profile of health conditions and cancers, healthcare quality and screening and determinants of mortality and healthcare.

Mortality in 787,666 school pupils with and without autism: A cohort study.

LAY ABSTRACT: There are few studies on the deaths of children and young people with autism; some studies on children and adults combined suggest that those with autism may have higher death rates than other people. More children are diagnosed with autism than in the past, suggesting that there are now more children with milder autism who have the diagnosis than in the past, so studies in the past might not apply to the current generation of children and young people diagnosed with autism. We examined the rates of death in children and young people in Scotland using recorded information in Scotland's annual pupil census, linked to the National Records of Scotland deaths register, between 2008 and 2015. In total, 9754 (1.2%) out of 787,666 pupils had autism. Six pupils with autism died in the study period, compared with 458 other pupils. This was equivalent to 16 per 100,000 for pupils with autism and 13 per 100,000 pupils without autism; hence, the rate of death was very similar. In the pupils with autism, the most common causes of death were diseases of the nervous system, whereas they were from external causes in the comparison pupils. The autism group had some deaths due to epilepsy which might have been prevented by good quality care. We cautiously conclude that the death rate in the current generation of children and young adults with autism is no higher than for other children, but that even in this high-income country, some deaths could be prevented by high quality care.

Respiratory-associated deaths in people with intellectual disabilities: a systematic review and meta-analysis.

OBJECTIVE: To review and synthesise evidence on rates of respiratory-associated deaths and associated risk factors in the intellectual disability population. DESIGN: Systematic review and meta-analysis. DATA SOURCES: Embase, CINAHL, ISI Web of Science (all databases including Medline) and PsychINFO were searched for studies published between 1st January 1985 and 27th April 2020 and examined study and outcome quality. Reference lists and Google Scholar were also hand searched. RESULTS: We identified 2295 studies, 17 were included in the narrative synthesis and 10 studies (11 cohorts) in the meta-analysis. Data from 90 302 people with intellectual disabilities and 13 808 deaths from all causes in people with intellectual disabilities were extracted. Significantly higher rates of respiratory-associated deaths were found among people with intellectual disabilities (standardised mortality ratio(SMR): 10.86 (95% CI: 5.32 to 22.18, p<0.001) compared with those in the general population, lesser rates for adults with ID (SMR: 6.53 (95% CI: 4.29 to 9.96, p<0.001); and relatively high rates from pneumonia 26.65 (95% CI: 5.63 to 126.24, p<0.001). The overall statistical heterogeneity was I2=99.0%. CONCLUSION: Premature deaths due to respiratory disorders are potentially avoidable with improved public health initiatives and equitable access to quality healthcare. Further research should focus on developing prognostic guidance and validated tools for clinical practice to mitigate risks of respiratory-associated deaths. PROSPERO REGISTRATION NUMBER: CRD42020180479.

Rates and causes of mortality among children and young people with and without intellectual disabilities in Scotland: a record linkage cohort study of 796 190 school children.

OBJECTIVES: To investigate mortality rates and causes in children and young people with intellectual disabilities. DESIGN: Retrospective cohort; individual record linkage between Scotland's annual pupil census and National Records of Scotland death register. SETTING: General community. PARTICIPANTS: Pupils receiving local authority-funded schooling in Scotland, 2008 to 2013, with an Additional Support Need due to intellectual disabilities, compared with other pupils. MAIN OUTCOME MEASURES: Deaths up to 2015: age of death, age-standardised mortality ratios (age-SMRs); causes of death including cause-specific age-SMRs; avoidable deaths as defined by the UK Office of National Statistics. RESULTS: 18 278/947 922 (1.9%) pupils had intellectual disabilities. 106 died over 67 342 person-years (crude mortality rate=157/100 000 person-years), compared with 458 controls over 3 672 224 person-years (crude mortality rate=12/100 000 person-years). Age-SMR was 11.6 (95% CI 9.6 to 14.0); 16.6 (95% CI 12.2 to 22.6) for female pupils and 9.8 (95% CI 7.7 to 12.5) for male pupils. Most common main underlying causes were diseases of the nervous system, followed by congenital anomalies; most common all-contributing causes were diseases of the nervous system, followed by respiratory system; most common specific contributing causes were cerebral palsy, pneumonia, respiratory failure and epilepsy. For all contributing causes, SMR was 98.8 (95% CI 69.9 to 139.7) for congenital anomalies, 76.5 (95% CI 58.9 to 99.4) for nervous system, 63.7 (95% CI 37.0 to 109.7) for digestive system, 55.3 (95% CI 42.5 to 72.1) for respiratory system, 32.1 (95% CI 17.8 to 57.9) for endocrine and 14.8 (95% CI 8.9 to 24.5) for circulatory system. External causes accounted for 46% of control deaths, but the SMR for external-related deaths was still higher (3.6 (95% CI 2.2 to 5.8)) for pupils with intellectual disabilities. Deaths amenable to good care were common. CONCLUSION: Pupils with intellectual disabilities were much more likely to die than their peers, and had a different pattern of causes, including amenable deaths across a wide range of disease categories. Improvements are needed to reduce amenable deaths, for example, epilepsy-related and dysphagia, and to support families of children with life-limiting conditions.

Do social support and eating family meals together play a role in promoting resilience to bullying and cyberbullying in Scottish school children?

This study investigates if cyberbullying is associated with wellbeing independently of traditional bullying and if social support and eating family meals together promotes resilience by buffering adolescents against the consequences of both types of bullying. Data for 5286 eleven, thirteen and fifteen year olds participating in the cross-sectional 2018 Scottish Health Behaviour in School-aged Children study were analysed. Adolescent self-report measures were used to assess traditional bullying, cyberbullying, classmate and teacher support and frequency of family meals together. Psychological wellbeing was assessed with the 5-item World Health Organization Wellbeing index. Analyses were conducted separately by gender with multilevel models, adjusting for sociodemographic factors. Resilience to bullying and cyberbullying was operationalised using statistical interactions. For both genders, cyberbullying and traditional bullying measures were associated with reduced wellbeing and all social support indicators were associated with increased wellbeing. In models containing both bullying measures, frequent traditional bullying victimisation was associated with a 7.2 (95% CI: 3.4-10.1) reduction in wellbeing score for boys and a 7.2 (95% CI: 4.5-10.0) reduction for girls, while cyberbullying was associated with 10.5 (95% CI: 5.8-15.1) reduction in wellbeing score for boys and 11.1 (95% CI: 6.7-15.5) reduction for girls. For both genders adjusting for classmate support explained away the relationships between traditional bullying and wellbeing, but cyberbullying was associated negatively with wellbeing independent of social support. Only one of 12 interaction tests provided any evidence of resilience. Cyberbullying was associated with a 7.8 (95% CI: 0.2-15.4) reduction in wellbeing score for girls who ate with their family every day, and 17.3 (95% CI: 10.5-24.1) reduction for girls who ate with their families less than weekly. In conclusion, cyberbullying is a strong, albeit rare, threat to adolescent wellbeing. Social support is important for wellbeing, but its ability to buffer adolescents against the consequences of bullying may be limited.

Effect of Pregnancy on Ventricular and Aortic Dimensions in Repaired Tetralogy of Fallot.

BACKGROUND: The aim was to assess whether cardiovascular adaptation to pregnancy in women with repaired tetralogy of Fallot (TOF) adversely affects hemodynamic stability, in particular with respect to right ventricular (RV) dilatation, pulmonary regurgitation, or aortic root dilatation. METHODS AND RESULTS: This was a retrospective cohort study of women with repaired TOF with paired cardiovascular magnetic resonance scans before and after their first pregnancy (baseline RV end systolic volume index 49 mL/m2 and RV end diastolic volume index 118 mL/m2) matched with a comparison group of nulliparous women with TOF. Cases were matched for age at baseline cardiovascular magnetic resonance scan, time between follow-up of cardiovascular magnetic resonance scans, QRS duration, RV ejection fraction, and indexed RV end systolic and diastolic volume at baseline. Effect of pregnancy and time on parameters was assessed using mixed-effects modelling. Nineteen women with repaired TOF who had completed their first pregnancy were identified and matched with 38 nulliparous women. We observed no deleterious effects of pregnancy on RV volumes, aortic dimensions, or exercise data. There was an effect of pregnancy observed in both left ventricular end diastolic volume and left ventricular stroke volume, consistent with a sustained small increase in left ventricular stroke volume attributed to pregnancy (53-55 mL/m2). CONCLUSIONS: Women with repaired TOF and with mild-to-moderate RV dilatation considering pregnancy can be reassured that pregnancy is unlikely to cause deterioration in their cardiovascular status. We recommend that women are routinely assessed and followed up before and after pregnancy and that prepregnancy counseling is tailored to their individual clinical status.

Interstudy reproducibility of right ventricular volumes, function, and mass with cardiovascular magnetic resonance.

BACKGROUND: Cardiovascular magnetic resonance (CMR) has shown excellent results for interstudy reproducibility in the assessment of left ventricular (LV) parameters. However, interstudy reproducibility data for the more complex-shaped right ventricle in a large study group have not yet been reported. We sought to determine the interstudy reproducibility of measurements of right ventricular (RV) volumes, function, and mass with CMR and compare it with correspondent LV values. METHODS: Sixty subjects (47 men; 20 healthy volunteers, 20 patients with heart failure, 20 patients with ventricular hypertrophy) underwent 2 CMR studies for assessment of RV measurements with a minimum time interval between each study. RESULTS: The overall interstudy reproducibility (range between groups) for the RV was 6.2% (4.2%-7.8%) for end-diastolic volume, 14.1% (8.1%-18.1%) for end-systolic volume, 8.3% (4.3%-10.4%) for ejection fraction (EF), and 8.7% (7.8%-9.4%) for RV mass. RV reproducibility was not as good as for the LV for all measures in all 3 groups, but this was only statistically significant for EF (P <.01). CONCLUSIONS: CMR showed good interstudy reproducibility for RV function parameters in healthy subjects, patients with heart failure, and patients with hypertrophy, which suggests that CMR is reliable for serial RV assessment. These data can be used to power sample sizes for longitudinal research studies of RV volume and function. The reproducibility values were similar to, but generally lower than, the reproducibility values for the LV in the same study population, which indicates that sample sizes for RV studies are in general larger than those for LV studies.