Academic achievement at ages 11 and 16 in children born with congenital anomalies in England: A multi-registry linked cohort study.

BACKGROUND: Children born with major congenital anomalies (CAs) have lower academic achievement compared with their peers, but the existing evidence is restricted to a number of specific CAs. OBJECTIVES: To investigate academic outcomes at ages 11 and 16 in children with major isolated structural CAs and children with Down or Turner syndromes. METHODS: This population-based cohort study linked data on approximately 11,000 school-aged children born with major CAs in 1994-2004 registered by four regional CA registries in England with education data from the National Pupil Database (NPD). The comparison group was a random sample of children without major CAs from the background population recorded in the NPD that were frequency matched (5:1) to children with CAs by birth year, sex and geographical area. RESULTS: Overall, 71.9%, 73.0% and 80.9% of children with isolated structural CAs achieved the expected attainment level at age 11 compared to 78.3%, 80.6% and 86.7% of the comparison group in English language, Mathematics and Science, respectively. Children with nervous system CAs as a whole had the lowest proportion who achieved the expected attainment at age 11. At age 16, 46.9% of children with CAs achieved the expected level compared to 52.5% of their peers. Major CAs were associated with being up to 9% (95% confidence interval [CI] 8%, 11%) and 12% (95% CI 9%, 14%) less likely to achieve expected levels at ages 11 and 16, respectively, after adjustment for socioeconomic deprivation. CONCLUSIONS: Although many children with isolated CAs achieved the expected academic level at ages 11 and 16, they were at higher risk of underachievement compared to their peers. These stark yet cautiously encouraging results are important for counselling parents of children with specific CAs and also highlight the possible need for special education support to reduce potential academic difficulties.

Risk factors for mortality in infancy and childhood in children with major congenital anomalies: A European population-based cohort study.

BACKGROUND: Preterm birth and young maternal age are known risk factors for infant and childhood mortality. There is limited knowledge of the impact of these risk factors in children born with major congenital anomalies (CAs), who have inherently higher risks of death compared with other children. OBJECTIVES: To investigate the risk factors for mortality up to age 10 years in children born with specific major CAs. METHODS: This population-based cohort study involved 150,198 livebirths from 1995 to 2014 in 13 European CA registries linked to mortality data. Cox proportional hazards models estimated the association of gestational age, maternal age and child's sex with death <1 year and 1-9 years for the whole cohort and by CA subgroup. Hazard ratios (HR) from each registry were pooled using multivariate meta-analysis. RESULTS: Preterm birth had a dose-response association with mortality; compared with infants born at 37+ weeks gestation, those born at <28, 28-31 and 32-36 weeks had 14.88 (95% CI 12.57, 17.62), 8.39 (95% CI 7.16, 9.85) and 3.88 (95% CI 3.40, 4.43) times higher risk of death <1 year, respectively. The corresponding risks at 1-9 years were 4.99 (95% CI 2.94, 8.48), 3.09 (95% CI 2.28, 4.18) and 2.04 (95% CI 1.69, 2.46) times higher, respectively. Maternal age <20 years (versus 20-34 years) was a risk factor for death <1 year (HR 1.30, 95% CI 1.09, 1.54) and 1-9 years (HR 1.58, 95% CI 1.19, 2.10). Females had 1.22 (95% CI 1.07, 1.39) times higher risk of death between 1 and 9 years than males. CONCLUSION: Preterm birth was associated with considerably higher infant and childhood mortality in children with CAs, comparable to estimates reported elsewhere for the background population. Additional risk factors included young maternal age and female sex. Information on risk factors could benefit clinical care and guide counselling of parents following CA diagnoses.

Educational achievement of children with selected major congenital anomalies and associated factors: a Finnish registry-based study.

BACKGROUND: Children with major congenital anomalies may be at risk of poor educational outcomes. We aimed to evaluate the educational achievement of children born with major congenital anomalies compared with children without major congenital anomalies in relation to sociodemographic factors. METHODS: We performed a registry-based study including 401 544 children in Finland, graduates of the compulsory school who applied to secondary education. We used health data from the Finnish Register of Congenital Malformations for children born from 1995 to 2002 linked with education data from the Finnish Ministry of Education and Culture. We used generalized linear regression to compare the mean grade differences of children with specific major congenital anomalies and 'All anomalies' subgroup (major congenital anomalies, chromosomal syndromes, and multiple anomalies) with reference children. RESULTS: Children with major congenital anomalies were less likely to apply for further education than reference children (88.0% vs. 96.8%; odds ratio = 4.13; 95% confidence interval, 3.92-4.36). For most non-chromosomal congenital anomalies, children born with congenital anomalies had similar educational achievement to the reference children. For the 'All anomalies' subgroup, children with congenital anomalies had lower educational achievement than reference children. Among children with congenital anomalies, male sex, lower maternal educational levels and younger maternal age were associated with lower educational achievement. CONCLUSIONS: For children applying to further education, most non-chromosomal congenital anomalies were not associated with lower educational achievement. Nevertheless, efforts are needed to improve educational achievement in children with major congenital anomalies associated with maternal sociodemographic background.

Causes of death in children with congenital anomalies up to age 10 in eight European countries.

BACKGROUND: Congenital anomalies (CAs) increase the risk of death during infancy and childhood. This study aimed to evaluate the accuracy of using death certificates to estimate the burden of CAs on mortality for children under 10 years old. METHODS: Children born alive with a major CA between 1 January 1995 and 31 December 2014, from 13 population-based European CA registries were linked to mortality records up to their 10th birthday or 31 December 2015, whichever was earlier. RESULTS: In total 4199 neonatal, 2100 postneonatal and 1087 deaths in children aged 1-9 years were reported. The underlying cause of death was a CA in 71% (95% CI 64% to 78%) of neonatal and 68% (95% CI 61% to 74%) of postneonatal infant deaths. For neonatal deaths the proportions varied by registry from 45% to 89% and by anomaly from 53% for Down syndrome to 94% for tetralogy of Fallot. In children aged 1-9, 49% (95% CI 42% to 57%) were attributed to a CA. Comparing mortality in children with anomalies to population mortality predicts that over 90% of all deaths at all ages are attributable to the anomalies. The specific CA was often not reported on the death certificate, even for lethal anomalies such as trisomy 13 (only 80% included the code for trisomy 13). CONCLUSIONS: Data on the underlying cause of death from death certificates alone are not sufficient to evaluate the burden of CAs on infant and childhood mortality across countries and over time. Linked data from CA registries and death certificates are necessary for obtaining accurate estimates.

Ten-year survival of children with trisomy 13 or trisomy 18: a multi-registry European cohort study.

OBJECTIVE: To investigate the survival to 10 years of age of children with trisomy 13 (T13) and children with trisomy 18 (T18), born 1995-2014. DESIGN: Population-based cohort study that linked mortality data to data on children born with T13 or T18, including translocations and mosaicisms, from 13 member registries of EUROCAT, a European network for the surveillance of congenital anomalies. SETTING: 13 regions in nine Western European countries. PATIENTS: 252 live births with T13 and 602 with T18. MAIN OUTCOME MEASURES: Survival at 1 week, 4 weeks and 1, 5 and 10 years of age estimated by random-effects meta-analyses of registry-specific Kaplan-Meier survival estimates. RESULTS: Survival estimates of children with T13 were 34% (95% CI 26% to 46%), 17% (95% CI 11% to 29%) and 11% (95% CI 6% to 18%) at 4 weeks, 1 and 10 years, respectively. The corresponding survival estimates were 38% (95% CI 31% to 45%), 13% (95% CI 10% to 17%) and 8% (95% CI 5% to 13%) for children with T18. The 10-year survival conditional on surviving to 4 weeks was 32% (95% CI 23% to 41%) and 21% (95% CI 15% to 28%) for children with T13 and T18, respectively. CONCLUSIONS: This multi-registry European study found that despite extremely high neonatal mortality in children with T13 and T18, 32% and 21%, respectively, of those who survived to 4 weeks were likely to survive to age 10 years. These reliable survival estimates are useful to inform counselling of parents after prenatal diagnosis.

Temporal and geographical variations in survival of children born with congenital anomalies in Europe: A multi-registry cohort study.

BACKGROUND: Congenital anomalies are a major cause of perinatal, neonatal and infant mortality. OBJECTIVES: The aim was to investigate temporal changes and geographical variation in survival of children with major congenital anomalies (CA) in different European areas. METHODS: In this population-based linkage cohort study, 17 CA registries members of EUROCAT, the European network for the surveillance of CAs, successfully linked data on 115,219 live births with CAs to mortality records. Registries estimated Kaplan-Meier survival at 28 days and 5 years of age and fitted Cox's proportional hazards models comparing mortality at 1 year and 1-9 years of age for children born during 2005-2014 with those born during 1995-2004. The hazard ratios (HR) from each registry were combined centrally using a random-effects model. The 5-year survival conditional on having survived to 28 days of age was calculated. RESULTS: The overall risk of death by 1 year of age for children born with any major CA in 2005-2014 decreased compared to 1995-2004 (HR 0.68, 95% confidence interval [CI] 0.53, 0.89). Survival at 5 years of age ranged between registries from 97.6% to 87.0%. The lowest survival was observed for the registry of OMNI-Net (Ukraine) (87.0%, 95% CI 86.1, 87.9). CONCLUSIONS: Survival of children with CAs improved for births in 2005-2014 compared with 1995-2004. The use of CA registry data linked to mortality data enables investigation of survival of children with CAs. Factors such as defining major CAs, proportion of terminations of pregnancy for foetal anomaly, source of mortality data and linkage methods are important to consider in the design of future studies and in the interpretation of the results on survival of children with CAs.

Survival of children with rare structural congenital anomalies: a multi-registry cohort study.

BACKGROUND: Congenital anomalies are the leading cause of perinatal, neonatal and infant mortality in developed countries. Large long-term follow-up studies investigating survival beyond the first year of life in children with rare congenital anomalies are costly and sufficiently large standardized cohorts are difficult to obtain due to the rarity of some anomalies. This study aimed to investigate the survival up to 10 years of age of children born with a rare structural congenital anomaly in the period 1995-2014 in Western Europe. METHODS: Live births from thirteen EUROCAT (European network for the epidemiological surveillance of congenital anomalies) population-based registries were linked to mortality records. Survival for 12,685 live births with one of the 31 investigated rare structural congenital anomalies (CAs) was estimated at 1 week, 4 weeks and 1, 5 and 10 years of age within each registry and combined across Europe using random effects meta-analyses. Differences between registries were evaluated for the eight rare CAs with at least 500 live births. RESULTS: Amongst the investigated CAs, arhinencephaly/holoprosencephaly had the lowest survival at all ages (58.1%, 95% Confidence Interval (CI): 44.3-76.2% at 1 week; 47.4%, CI: 36.4-61.6% at 1 year; 35.6%, CI: 22.2-56.9% at 10 years). Overall, children with rare CAs of the digestive system had the highest survival (> 95% at 1 week, > 84% at 10 years). Most deaths occurred within the first four weeks of life, resulting in a 10-year survival conditional on surviving 4 weeks of over 95% for 17 out of 31 rare CAs. A moderate variability in survival between participating registries was observed for the eight selected rare CAs. CONCLUSIONS: Pooling standardised data across 13 European CA registries and the linkage to mortality data enabled reliable survival estimates to be obtained at five ages up to ten years. Such estimates are useful for clinical practice and parental counselling.

Ten-Year Survival of Children With Congenital Anomalies: A European Cohort Study.

OBJECTIVES: To investigate the survival up to age 10 for children born alive with a major congenital anomaly (CA). METHODS: This population-based linked cohort study (EUROlinkCAT) linked data on live births from 2005 to 2014 from 13 European CA registries with mortality data. Pooled Kaplan-Meier survival estimates up to age 10 were calculated for these children (77 054 children with isolated structural anomalies and 4011 children with Down syndrome). RESULTS: The highest mortality of children with isolated structural CAs was within infancy, with survival of 97.3% (95% confidence interval [CI]: 96.6%-98.1%) and 96.9% (95% CI: 96.0%-97.7%) at age 1 and 10, respectively. The 10-year survival exceeded 90% for the majority of specific CAs (27 of 32), with considerable variations between CAs of different severity. Survival of children with a specific isolated anomaly was higher than in all children with the same anomaly when those with associated anomalies were included. For children with Down syndrome, the 10-year survival was significantly higher for those without associated cardiac or digestive system anomalies (97.6%; 95% CI: 96.5%-98.7%) compared with children with Down syndrome associated with a cardiac anomaly (92.3%; 95% CI: 89.4%-95.3%), digestive system anomaly (92.8%; 95% CI: 87.7%-98.2%), or both (88.6%; 95% CI: 83.2%-94.3%). CONCLUSIONS: Ten-year survival of children born with congenital anomalies in Western Europe from 2005 to 2014 was relatively high. Reliable information on long-term survival of children born with specific CAs is of major importance for parents of these children and for the health care professionals involved in their care.

Academic achievement and needs of school-aged children born with selected congenital anomalies: A systematic review and meta-analysis.

Children with congenital anomalies have poorer intellectual and cognitive development compared to their peers, but evidence for academic achievement using objective measures is lacking. We aimed to summarize and synthesize evidence on academic outcomes and special education needs (SEN) of school-aged children born with selected major structural congenital anomalies. Electronic databases (MEDLINE, EMBASE, Scopus, PsycINFO, CINAHL, ProQuest Natural Science and Education Collections), reference lists and citations for 1990-2020 were systematically searched. We included original-research articles on academic achievement in children with non-syndromic congenital anomalies that involved school test results, standardized tests and/or SEN data. Random-effects meta-analyses were performed to estimate pooled mean test scores in mathematics and/or reading where possible and pooled odds ratios (ORs) for SEN in children with severe congenital heart defects (CHDs) and children with orofacial clefts (OFCs). Thirty-nine eligible studies (n = 21,066 children) were synthesized narratively. Sixteen studies were included in meta-analyses. Children with non-syndromic congenital anomalies were at a higher risk of academic underachievement than controls across school levels. Children with severe CHD (pooled OR = 2.32, 95% CI: 1.90, 2.82), and children with OFC (OR = 1.38 (95% CI: 1.20, 1.57), OR = 3.07 (95% CI: 2.65, 3.56), and OR = 3.96 (95% CI: 3.31, 4.72) for children with cleft lip, cleft palate and cleft lip/palate, respectively) had significantly higher ORs for SEN than controls. Children with non-syndromic congenital anomalies underperform academically and have higher SEN rates compared to their peers. Early monitoring and development of differential SEN are important to promote academic progress in these children.

Long-term survival of children born with congenital anomalies: A systematic review and meta-analysis of population-based studies.

BACKGROUND: Following a reduction in global child mortality due to communicable diseases, the relative contribution of congenital anomalies to child mortality is increasing. Although infant survival of children born with congenital anomalies has improved for many anomaly types in recent decades, there is less evidence on survival beyond infancy. We aimed to systematically review, summarise, and quantify the existing population-based data on long-term survival of individuals born with specific major congenital anomalies and examine the factors associated with survival. METHODS AND FINDINGS: Seven electronic databases (Medline, Embase, Scopus, PsycINFO, CINAHL, ProQuest Natural, and Biological Science Collections), reference lists, and citations of the included articles for studies published 1 January 1995 to 30 April 2020 were searched. Screening for eligibility, data extraction, and quality appraisal were performed in duplicate. We included original population-based studies that reported long-term survival (beyond 1 year of life) of children born with a major congenital anomaly with the follow-up starting from birth that were published in the English language as peer-reviewed papers. Studies on congenital heart defects (CHDs) were excluded because of a recent systematic review of population-based studies of CHD survival. Meta-analysis was performed to pool survival estimates, accounting for trends over time. Of 10,888 identified articles, 55 (n = 367,801 live births) met the inclusion criteria and were summarised narratively, 41 studies (n = 54,676) investigating eight congenital anomaly types (spina bifida [n = 7,422], encephalocele [n = 1,562], oesophageal atresia [n = 6,303], biliary atresia [n = 3,877], diaphragmatic hernia [n = 6,176], gastroschisis [n = 4,845], Down syndrome by presence of CHD [n = 22,317], and trisomy 18 [n = 2,174]) were included in the meta-analysis. These studies covered birth years from 1970 to 2015. Survival for children with spina bifida, oesophageal atresia, biliary atresia, diaphragmatic hernia, gastroschisis, and Down syndrome with an associated CHD has significantly improved over time, with the pooled odds ratios (ORs) of surviving per 10-year increase in birth year being OR = 1.34 (95% confidence interval [95% CI] 1.24-1.46), OR = 1.50 (95% CI 1.38-1.62), OR = 1.62 (95% CI 1.28-2.05), OR = 1.57 (95% CI 1.37-1.81), OR = 1.24 (95% CI 1.02-1.5), and OR = 1.99 (95% CI 1.67-2.37), respectively (p < 0.001 for all, except for gastroschisis [p = 0.029]). There was no observed improvement for children with encephalocele (OR = 0.98, 95% CI 0.95-1.01, p = 0.19) and children with biliary atresia surviving with native liver (OR = 0.96, 95% CI 0.88-1.03, p = 0.26). The presence of additional structural anomalies, low birth weight, and earlier year of birth were the most commonly reported predictors of reduced survival for any congenital anomaly type. The main limitation of the meta-analysis was the small number of studies and the small size of the cohorts, which limited the predictive capabilities of the models resulting in wide confidence intervals. CONCLUSIONS: This systematic review and meta-analysis summarises estimates of long-term survival associated with major congenital anomalies. We report a significant improvement in survival of children with specific congenital anomalies over the last few decades and predict survival estimates up to 20 years of age for those born in 2020. This information is important for the planning and delivery of specialised medical, social, and education services and for counselling affected families. This trial was registered on the PROSPERO database (CRD42017074675).

Socio-economic inequalities in mortality in children with congenital heart disease: A systematic review and meta-analysis.

BACKGROUND: The impact of socio-economic status (SES) on congenital heart disease (CHD)-related mortality in children is not well established. OBJECTIVES: We aimed to systematically review and appraise the existing evidence on the association between SES (including poverty, parental education, health insurance, and income) and mortality among children with CHD. DATA SOURCES: Seven electronic databases (Medline, Embase, Scopus, PsycINFO, CINAHL, ProQuest Natural, and Biological Science Collections), reference lists, citations, and key journals were searched. STUDY SELECTION AND DATA EXTRACTION: We included articles reporting original research on the association between SES and mortality in children with CHD if they were full papers published in the English language and regardless of (a) timing of mortality; (b) individual or area-based measures of SES; (c) CHD subtype; (d) age at ascertainment; (e) study period examined. Screening for eligibility, data extraction, and quality appraisal were performed in duplicate. SYNTHESIS: Meta-analyses were performed to estimate pooled ORs for in-hospital mortality according to health insurance status. RESULTS: Of 1388 identified articles, 28 met the inclusion criteria. Increased area-based poverty was associated with increased odds/risk of postoperative (n = 1), neonatal (n = 1), post-discharge (n = 1), infant (n = 1), and long-term mortality (n = 2). Higher parental education was associated with decreased odds/risk of neonatal (n = 1) and infant mortality (n = 5), but not with long-term mortality (n = 1). A meta-analysis of four US articles showed increased unadjusted odds of in-hospital mortality in those with government/public versus private health insurance (OR 1.40, 95% CI 1.24, 1.56). The association between area-based income and CHD-related mortality was conflicting, with three of eight articles reporting significant associations. CONCLUSION: This systematic review provides evidence that children of lower SES are at increased risk of CHD-related mortality. As these children are over-represented in the CHD population, interventions targeting socio-economic inequalities could have a large impact on improving CHD survival.

What helped and hindered implementation of an intervention package to reduce smoking in pregnancy: process evaluation guided by normalization process theory.

BACKGROUND: Smoking in pregnancy causes harm to mother and baby. Despite evidence from trials of what helps women quit, implementation in the real world has been hard to achieve. An evidence-based intervention, babyClear©, involving staff training, universal carbon monoxide monitoring, opt-out referral to smoking cessation services, enhanced follow-up protocols and a risk perception tool was introduced across North East England. This paper presents the results of the qualitative analyses, reporting acceptability of the system changes to staff, as well as aids and hindrances to implementation and normalization of this complex intervention. METHODS: Process evaluation was used to complement an effectiveness study. Interviews with maternity and smoking cessation services staff and observations of training were undertaken. Normalization Process Theory (NPT) was used to frame the interview guides and analysis. NPT is an empirically-derived theory, developed by sociologists, that uses four concepts to understand the process of routinising new practices. RESULTS: Staff interviews took place across eight National Health Service trusts at a time of widespread restructuring in smoking cessation services. Principally interviewees worked in maternity (n = 63) and smoking cessation services (n = 35). Five main themes, identified inductively, influenced the implementation: 1) initial preparedness of the organisations; 2) staff training; 3) managing partnership working; 4) resources; 5) review and planning for sustainability. CONCLUSIONS: NPT was used to show that the babyClear© package was acceptable to staff in a range of organisations. Illustrated in Themes 1, 2 & 3, staff welcomed ways to approach pregnant women about their smoking, without damaging their professional relationship with them. Predicated on producing individual behaviour change in women, the intervention does this largely through reorganising and standardising healthcare systems that are required to implement best practice guidelines. Changing organisational systems requires belief and commitment from staff, so that they set up and maintain practical adjustments to their practice and are reflective about adapting themselves and the work context as new challenges are encountered. The ongoing challenge is to identify and maintain the elements of the intervention package which are essential for its effectiveness and how to tailor them to local circumstances and resources without compromising its core ingredients.

Projected number of children with isolated spina bifida or down syndrome in England and Wales by 2020.

Children with major congenital anomalies often require lifelong access to health and social care services. Estimating future numbers of affected individuals can aid health and social care planning. This study aimed to estimate the number of children aged 0-15 years living with spina bifida or Down syndrome in England and Wales by 2020. Cases of spina bifida and Down syndrome born during 1998-2013 were identified from the Northern Congenital Abnormality Survey and the National Down Syndrome Cytogenetic Register, respectively. The number of infants born with spina bifida during 1998-2019 were estimated by applying the average prevalence rate in the North of England to actual and projected births in England and Wales. Poisson regression was performed to estimate the number of infants born with Down syndrome in England and Wales during 1998-2013 and 2004-2019. The numbers of children aged 0-15 living with spina bifida or Down syndrome in 2014 and in 2020 were then estimated by multiplying year- and age-specific survival estimates by the number of affected births. An estimated 956 children with isolated spina bifida, 623 children with spina bifida and hydrocephalus and 11,592 children with Down syndrome aged 0-15 years will be living in England and Wales by 2020, increases of 7.2%, 12.0% and 12.7% since 2014, respectively. Due to improvements in survival, an increase in population size and changes in maternal age distribution at delivery, we anticipate further increases in the number of children living with spina bifida or Down syndrome by 2020.

Evaluation of a complex healthcare intervention to increase smoking cessation in pregnant women: interrupted time series analysis with economic evaluation.

OBJECTIVES: To evaluate the effectiveness of a complex intervention to improve referral and treatment of pregnant smokers in routine practice, and to assess the incremental costs to the National Health Service (NHS) per additional woman quitting smoking. DESIGN: Interrupted time series analysis of routine data before and after introducing the intervention, within-study economic evaluation. SETTING: Eight acute NHS hospital trusts and 12 local authority areas in North East England. PARTICIPANTS: 37 726 records of singleton delivery including 10 594 to mothers classified as smoking during pregnancy. INTERVENTIONS: A package of measures implemented in trusts and smoking cessation services, aimed at increasing the proportion of pregnant smokers quitting during pregnancy, comprising skills training for healthcare and smoking cessation staff; universal carbon monoxide monitoring with routine opt-out referral for smoking cessation support; provision of carbon monoxide monitors and supporting materials; and an explicit referral pathway and follow-up protocol. MAIN OUTCOME MEASURES: Referrals to smoking cessation services; probability of quitting smoking during pregnancy; additional costs to health services; incremental cost per additional woman quitting. RESULTS: After introduction of the intervention, the referral rate increased more than twofold (incidence rate ratio=2.47, 95% CI 2.16 to 2.81) and the probability of quitting by delivery increased (adjusted OR=1.81, 95% CI 1.54 to 2.12). The additional cost per delivery was £31 and the incremental cost per additional quit was £952; 31 pregnant women needed to be treated for each additional quitter. CONCLUSIONS: The implementation of a system-wide complex healthcare intervention was associated with significant increase in rates of quitting by delivery.

Predicting the prevalence of cerebral palsy by severity level in children aged 3 to 15 years across England and Wales by 2020.

AIM: To estimate the number of children living with cerebral palsy (CP) in England and Wales in 2013 by severity, and to extrapolate this figure to 2020. METHOD: Data from the North of England Collaborative Cerebral Palsy Survey for births during the period 1991 to 2000 were restricted to individuals aged at or above 3 years to estimate the prevalence of CP and to calculate 15-year survival by severity according to the number of severe impairments and lifestyle assessment score. The number of 3- to 15-year-olds with CP of different severity in England and Wales was estimated in 2013 and 2019 using actual and nationally projected births. RESULTS: Cumulative survival estimates up to the age of 16 years in children with CP differ significantly by severity, ranging between 97 per cent and 100 per cent for children with non-severe CP, and between 64 per cent and 67 per cent for those with the most severe CP. By the end of 2013, the estimated number of children aged 3 to 15 years living with CP in England and Wales will be about 20 500 rising to approximately 22 100 by 2020, a 7.5 per cent increase. INTERPRETATION: Owing to an increasing population, the number of children living with CP in England and Wales will increase by 2020. This will have significant implications for health and social care service planning.

Risk estimates of recurrent congenital anomalies in the UK: a population-based register study.

BACKGROUND: Recurrence risks for familial congenital anomalies in successive pregnancies are known, but this information for major structural anomalies is lacking. We estimated the absolute and relative risks of recurrent congenital anomaly in the second pregnancy for women with a history of a congenital anomaly in the first pregnancy, for all major anomaly groups and subtypes. METHODS: Population-based register data on 18,605 singleton pregnancies affected by major congenital anomaly occurring in 872,493 singleton stillbirths, live births and terminations of pregnancy for fetal anomaly were obtained from the Northern Congenital Abnormality Survey, North of England, UK, for 1985-2010. Absolute risks (ARs) and relative risks (RRs) for recurrent congenital anomaly (overall, from a similar group, from a dissimilar group) in the second pregnancy were estimated by history of congenital anomaly (overall, by group, by subtype) in the first pregnancy. RESULTS: The estimated prevalences of congenital anomaly in first and second pregnancies were 275 (95% CI 270-281) and 163 (95% CI 159-168) per 10,000 respectively. For women whose first pregnancy was affected by congenital anomaly, the AR of recurrent congenital anomaly in the second pregnancy was 408 (95% CI 365-456) per 10,000, 2.5 (95% CI 2.3-2.8, P < 0.0001) times higher than for those with unaffected first pregnancies. For similar anomalies, the recurrence risk was considerably elevated (RR = 23.8, 95% CI 19.6-27.9, P < 0.0001), while for dissimilar anomalies the increase was more modest (RR = 1.4, 95% CI 1.2-1.6, P = 0.001), although the ARs for both were 2%. CONCLUSIONS: Absolute recurrence risks varied between 1 in 20 and 1 in 30 for most major anomaly groups. At pre-conception and antenatal counselling, women whose first pregnancy was affected by a congenital anomaly and who are planning a further pregnancy may find it reassuring that, despite high relative risks, the absolute recurrence risk is relatively low.

Associations between human exposure to polybrominated diphenyl ether flame retardants via diet and indoor dust, and internal dose: A systematic review.

The aim of this review was to identify and appraise the current international evidence of associations between concentrations of polybrominated diphenyl ethers (PBDEs) in humans and their indoor dusts and food. We systematically searched Medline, Embase, Web of Science and Scopus (up to Jan 2015), using a comprehensive list of keywords, for English-language studies published in peer-reviewed journals. We extracted information on study design, quality, participants, sample collection methods, adjustments for potential confounders and correlations between PBDE concentrations in internal and external matrices. Of 131 potential articles, 17 studies met the inclusion criteria and were included in the narrative synthesis. We concluded that three key factors influenced correlations between external and internal PBDE exposure; half-life of individual congeners in the human body; proximity and interaction between PBDE source and study subject; and time of study relative to phase out of PBDE technical products. Internal dose of Penta-BDE technical mix congeners generally correlated strongly with dust. The exception was BDE-153 which is known to have higher persistence in human tissues. Despite the low bioaccessibility and short half-life of BDE-209, its high loading in dusts gave strong correlations with body burden where measured. Correlations between PBDE concentrations in duplicate diet and body burden were not apparent from the included studies. Whether dust or diet is the primary exposure source for an individual is tied to the loading of PBDE in dust or food items and the amounts ingested. Simple recommendations such as more frequent hand washing may reduce PBDE body burden.

Measuring self-reported quality of life in 8- to 11-year-old children born with gastroschisis: Is the KIDSCREEN questionnaire acceptable?

BACKGROUND: Children born with gastroschisis have a good prognosis but require surgical correction and long-term follow up. There has been little research on the impact of gastroschisis on the child's health-related quality of life (QoL). The aim was to assess face and content validity of the KIDSCREEN-52 questionnaire as a measurement of self- and proxy-reported QoL in children born with gastroschisis and to evaluate self-reported QoL in these children compared with the reference population. METHODS: In this cross-sectional exploratory study, we used the validated KIDSCREEN-52 questionnaire and individual interview with 8- to 11-year-old children born with gastroschisis who were identified from the Northern Congenital Abnormality Survey. Self-reported QoL scores were compared with age-matched UK norms by using the two-sample t test. RESULTS: Ten children (median age 9.6 years, interquartile range 8.3-11.0) and their parents participated. Children found KIDSCREEN a helpful tool to explore their feelings and that it covered life aspects important to them. Parents believed that all priority areas were represented and that it was straightforward for their children to complete. In nine KIDSCREEN domains, children with gastroschisis had similar QoL scores to those in the reference population, and in one (psychological well-being) the mean score was significantly better (p = 0.03). All children described their health as good/very good or excellent; eight said they would not like to change anything about their body. CONCLUSION: The KIDSCREEN questionnaire has adequate face and content validity as a measure of QoL in children with gastroschisis and is acceptable to both children and parents.

Pregestational diabetes is associated with adverse outcomes in twin pregnancies: a regional register-based study.

INTRODUCTION: The incidence of pregnancies complicated by twinning and diabetes is increasing in the UK. This is a worrying trend as both diabetes and twin gestations are associated with a high risk of adverse pregnancy outcomes. The few studies that have specifically addressed how twinning and pregestational diabetes in the same pregnancy may affect outcome have reported conflicting results. MATERIAL AND METHODS: We analyzed data on 27 women with a twin pregnancy and pregestational diabetes (54 babies) and 6407 women with a twin pregnancy without diabetes (12 814 babies) from the Northern Survey of Twin and Multiple Pregnancy during 1998-2010. A composite adverse pregnancy outcome (comprising fetal loss before 24 weeks, termination of pregnancy, stillbirth, infant death or any major congenital anomaly), extended perinatal mortality (stillbirths and neonatal deaths) and major congenital anomaly were the main outcome measures. Adjusted rate ratios were estimated using generalized estimating equations for Poisson regression controlling for potential confounders. RESULTS: Mothers with twin pregnancies with diabetes were older (p = 0.001) and had higher body mass indices (p < 0.0001) than those without diabetes. Their twins were more likely to be delivered earlier (p = 0.026), be delivered by cesarean section (80.4% vs. 49.7%; p < 0.0001), be large-for-gestational-age (p < 0.0001) and require admission to a special care baby unit (p < 0.0001). Pregestational diabetes was associated with significantly increased rates of the composite adverse outcome and major congenital anomalies in twins (adjusted rate ratios 2.66, 95% confidence interval 1.14-6.20 and adjusted rate ratios 3.51, 95% confidence interval 1.31-9.40, respectively). CONCLUSION: Maternal pregestational diabetes in twin pregnancies is associated with a significantly increased risk of an adverse pregnancy outcome.

Self-reported quality of life of young children with conditions from early infancy: a systematic review.

CONTEXT: There is little consistency in the use of instruments for measuring self-reported quality of life (QoL) in young children. OBJECTIVE: To systematically review studies of self-reported QoL in children aged <12 years with congenital health conditions, and to examine the agreement between self- and proxy-reports. DATA SOURCES: Literature databases (MEDLINE, EMBASE, Web of Science, PsychINFO) were systematically searched, reference lists of eligible studies were scanned. STUDY SELECTION: We included studies published in English between January 1989 and June 2013 which used validated instruments to assess self-reported QoL in children aged <12 years with a distinct congenital health condition identified in early infancy. DATA EXTRACTION: We extracted data on study design, objective, sample characteristics, QoL assessment instrument, statistical techniques and results. RESULTS: From 403 full-text articles assessed for eligibility, 50 studies underwent detailed review, and 37 were included in a narrative synthesis. Children's self-reported QoL was assessed by using a variety of generic and/or condition-specific instruments, with the Pediatric Quality of Life Inventory being the most frequently used (25% [9 studies]). Regardless of the condition or the instrument used, children often reported QoL similar to the reference population, except for lower scores in the physical functioning/health domain. There were differences between younger and older age groups according to QoL domain. The child's perception of QoL differed from that of his or her parents, in particular for subjective domains such as emotional functioning, and these differences were age related. The main limitation of the review resulted from the lack of published studies on self-reported QoL in young children, in particular, lacking both self-reports and proxy reports. Existing studies demonstrated wide variability in the QoL instruments used and approaches to statistical analyses, lack of information about the formation of the study sample (response rate; comparison of responders and nonresponders) and low sample sizes in the age group of interest. CONCLUSIONS: The reviewed studies demonstrated that, even for younger children, both child and parent perspectives are essential to understanding the impact of a condition on a child's QoL.