Detection and investigation of temporal clusters of congenital anomaly in Europe: seven years of experience of the EUROCAT surveillance system.

Detection and investigation of congenital anomaly clusters is one part of surveillance to detect new or changing teratogenic exposures in the population. The EUROCAT (European Surveillance of Congenital Anomalies) cluster monitoring system and results are described here. Monitoring was conducted annually from 2007 to 2013 for 18 registries covering an annual birth population up to 0.5 million births. For each registry and 72 anomaly subgroups, the scan "moving window" technique was used to detect clusters in time occurring within the last 2 years based on estimated date of conception. Registries conducted preliminary investigations using a standardised protocol to determine whether there was cause for concern, and expert review was used at key points. 165 clusters were detected, a rate of 3.4% of all 4823 cluster tests performed over 7 years, more than expected by chance. Preliminary investigations of 126 new clusters confirmed that 35% were an unusual aggregation of cases, while 56% were explained by data quality or diagnostic issues, and 9% were not investigated. For confirmed clusters, the registries' course of action was continuing monitoring. Three confirmed clusters continued to grow in size for a limited period in subsequent monitoring. This system is best suited to early detection of exposures which are sudden, widespread and/or highly teratogenic, and was reassuring in demonstrating an absence of a sustained exposure of this type. Such proactive monitoring can be run efficiently without overwhelming the surveillance system with false positives, and serves an additional purpose of data quality control.

Risk factors for congenital heart disease: The Baby Hearts Study, a population-based case-control study.

We investigated the role of maternal environmental factors in the aetiology of congenital heart disease (CHD). A population-based case-control study (242 CHD cases, 966 controls) was conducted using an iPad questionnaire for mother with linkage to maternity and first trimester prescription records. Risk of CHD was associated with low maternal education (OR adjusted for confounders 1.59; 95% confidence interval [CI], 1.02-2.49), pregestational diabetes (OR 4.04; 95% CI 1.00-16.28), self-reported maternal clotting disorders (adjOR 8.55, 95%CI 1.51-48.44), prescriptions for the anticlotting medication enoxaparin (adjOR 3.22, 95%CI 1.01-10.22) and self-reported vaginal infections (adjOR 1.69, 95%CI 1.01-2.80). There was no strong support for the hypothesis that periconceptional folic acid supplements have a protective effect, but there was a protective effect of frequent consumption of folate rich fruits (adjOR 0.64, 95%CI 0.47-0.89). Compared to the most common pre-pregnancy dietary pattern, CHD risk was associated with a poor diet low in fruit and vegetables (adjOR 1.56, 95%CI 1.05-2.34). Mothers of cases reported more pregnancy related stress (adjOR 1.69; 95% CI 1.22-2.34) and multiple stressors (adjOR 1.94, 95%CI 0.83-4.53). We found no supportive evidence for CHD risk being associated with obesity, smoking, depression or antidepressant use in this population. Our findings add to the previous evidence base to show potential for public health approaches to help prevent CHD in future by modifying environmental factors. Independent confirmation should be sought regarding elevated CHD risk associated with maternal blood clotting disorders and their treatment, since we are the first to report this.

Reliability and validity of the Child Health QuestionnairePF-50 for European children with cerebral palsy.

OBJECTIVE: To evaluate the psychometric performance of the Child Health Questionnaire (CHQ) in children with cerebral palsy (CP). METHOD: 818 parents of children with CP, aged 8-12 from nine regions of Europe completed the CHQ (parent form 50 items). Functional abilities were classified using the five-level Gross Motor Function Classification Scheme (Levels I-III as ambulant; Level IV-V as nonambulant CP). RESULTS: Ceiling effects were observed for a number of subscales and summary scores across all Gross Motor Function Classification System levels, whilst floor effects occurred only in the physical functioning scale (Level V CP). Reliability was satisfactory overall. Confirmatory factor analysis (CFA) revealed a seven-factor structure for the total sample of children with CP but with different factor structures for ambulant and nonambulant children. CONCLUSION: The CHQ has limited applicability in children with CP, although with judicious use of certain domains for ambulant and nonambulant children can provide useful and comparable data about child health status for descriptive purposes.

The health of children with cerebral palsy and stress in their parents.

TITLE: The health of children with cerebral palsy and stress in their parents. AIM: This paper is a report of a study conducted to describe the health of children with cerebral palsy and investigate predictors of stress in their parents. BACKGROUND: Children with severe cerebral palsy tend to have poorer health than their able-bodied peers, and their parents are more likely to be stressed and have poorer health. METHOD: A cross-sectional survey with home visits using standard questionnaires was administered to parents in 2004-05. A total of 102/199 (51%) children and parents participated. The children were compared with a normative sample. RESULTS: Children with cerebral palsy had poorer physical health, and 79% of parents reported that their child had moderate to severe pain. Their poorer health, in comparison with the normal sample and measured by the Child Health Questionnaire, was related to feeding problems and seizures, general health perceptions to intellectual and feeding impairment, and family activities with severe motor, intellectual and feeding impairment. Poorer psychological well-being on the hyperactivity domain of the Strengths & Difficulties Questionnaire was related to feeding difficulties, on the prosocial domain to more severe forms of all child impairments, and on the social impairment scale to intellectual impairment. Children with psychological problems had statistically significantly increased odds (OR = 7.2, 95% CIs 2.6-20.3) of having parents with high stress. CONCLUSION: Children with cerebral palsy and associated impairments are at higher risk of poorer health and family well-being. A family-centred approach to the care of children with cerebral palsy and their families is essential to ensure both receive adequate care and support.

The health of children and young people with cerebral palsy: a longitudinal, population-based study.

BACKGROUND: Cerebral palsy (CP) is a chronic condition about which little is known in relation to the long term stability of and factors influencing health. OBJECTIVES: To describe the health status of 4-17 year olds with ambulant CP, compare with the general population and identify factors predicting change in health over time. DESIGN: A longitudinal, clinical survey. SETTING: A regional hospital-based Gait Analysis Laboratory. PARTICIPANTS: Those aged 4-17 years and able to walk at least 10m independently were identified from a case register of people with CP. A total of 184 subjects took part (38% of all eligibles in the region); 154 (84%) returned for a second assessment on average 2.5 years later. METHODS: The Child Health Questionnaire (Parent-form-50) was completed by 184 parents at time 1, and 156 at time 2. RESULTS: Children and young people with CP have significantly poorer health across a number of domains when compared to children in the general child population. Over time improvements occurred in behaviour (p=0.01), family activities (p<0.001) and physical functioning (p=0.05). Linear regression showed that gross motor function (p<0.001) and cerebral palsy subtype (p<0.05) were associated with changes in physical functioning; age was associated with changes in behaviour (p=0.007) and family activities (p=0.01); and communication ability was significantly associated with changes in family activities (p=0.005). CONCLUSIONS: Children and young people with CP have poorer health than their able bodied peers but relatively stable health over 2.5 years. Where change occurred, it was for the better.

To what extent do children with cerebral palsy participate in everyday life situations?

The aims of the study are to describe participation of children with cerebral palsy in everyday life situations, to investigate the relationship between participation (primary outcome variable) with child and parent characteristics (independent variables) and to compare the frequency of participation (secondary outcome variable) of children with cerebral palsy with children without disabilities. A cross-sectional survey of parents of children with cerebral palsy in Northern Ireland was undertaken in families' homes using standard questionnaires. Children with cerebral palsy born between 31/8/1991 and 1/4/1997 were identified from a case register of people with the condition. A total of 102 parents opted in (51% response rate). Questionnaires included the Life Habits Questionnaire (Life-H) to measure difficulties in participation and The Frequency of Participation Questionnaire (FPQ), to measure frequency of participation with comparative data for children without disability. Overall, children with cerebral palsy participated less often than their non-disabled peers across a number of lifestyle and cultural pursuits. Among the 102 children with cerebral palsy, participation in 'relationships' was the least disrupted area of everyday life and aspects of 'school', 'personal care' and 'mobility' were the most disrupted. Children with cerebral palsy and severe co-impairments were significantly less likely to experience higher levels of participation in most areas of everyday life when compared to children with cerebral palsy and no severe co-impairments. Child physical and psychological well-being did not influence participation although higher parenting stress was significantly related to lower child participation in 'community activities'. Participation is an important health outcome for children with cerebral palsy and should be incorporated in routine clinical practice. Professionals have a role to play both at the level of addressing individual child and family needs as well as influencing legislation and policy to ensure improved access to services and local communities.

Use of the child health questionnaire in children with cerebral palsy: a systematic review and evaluation of the psychometric properties.

OBJECTIVE: To review the psychometric performance of the Child Health Questionnaire (CHQ) in samples of children with cerebral palsy (CP). METHOD: Four search terms were applied to five databases in a search for papers published between 1993 and January 2007. RESULTS: A total of 13 papers were identified, providing data on 1229 unique children aged 2-18 years old. Three studies reported on the reliability of the CHQ (internal consistency), whilst six studies provided evidence on various dimensions of validity (concurrent; discriminant and item discriminant validity). CONCLUSIONS: This review identified a number of psychometric issues that need to be addressed. These include the assessment of additional types of reliability; an examination of the factor structure of the CHQ within the CP population; and the development of normative data using substantial representative samples, particularly in Europe. Until these issues are addressed, researchers utilizing the CHQ in children with CP should be cautious about its interpretation.