The association of maternal polyunsaturated fatty acids during pregnancy with social competence and problem behaviours at 7 years of age: The MEFAB cohort.

BACKGROUND: The prenatal exposure to maternal n-6 and n-3 polyunsaturated fatty acids (PUFAs) might influence the development of social competence and internalizing and externalizing behaviours of the child, because of the numerous functions of PUFAs within the nervous system. METHODS: To analyse the association of selected maternal PUFAs (i.e., AA, EPA, DHA, total n-6, total n-3, and the n-6:n-3 ratio) measured during gestation with childhood social competence and problem behaviours, we examined 311 mother-child pairs from the Maastricht Essential Fatty Acid Birth (MEFAB) cohort. For each woman, PUFA-specific changes in relative concentrations were calculated by identifying the best-fitting curve of PUFA concentration by linear splines of gestational age. The associations of changes in maternal PUFAs in early and late pregnancy with childhood social competence, total problems, internalizing and externalizing behaviours, measured with the Child Behaviour Checklist 4/18 at age 7, were investigated with linear regression analyses adjusted for maternal and children's socio-demographic characteristics. RESULTS: In late gestation (i.e., from gestational week 30), an increase in AA was associated with higher social competence, while a decrease in total n-6 was associated with lower externalizing behaviours. No other significant associations were found. DISCUSSION: In this prospective study, increasing maternal AA and decreasing total n-6 were associated with improved social competence and externalizing behaviours, respectively, in 7-year old children. Nonetheless, the clinical significance of the identified associations is modest and further investigations are warranted to clarify the relationship between maternal AA and total n-6 during pregnancy and childhood social and behavioural development.

Wrist Arthroscopy in Juvenile Idiopathic Arthritis: A Review of Current Literature and Future Implications.

Introduction Juvenile Idiopathic Arthritis (JIA) is the most common rheumatological condition in children and frequently affects the wrist. The roles for wrist arthroscopy and arthroscopic synovectomy (AS) in JIA are unclear. Our aim was to find the current evidence supporting its use. Methods Systematic literature review of relevant publications from 1990 to present in the Cochrane Library, Clinical Knowledge Summaries, DynaMed, PEMSoft, NICE Guidance, MEDLINE, EMBASE, and PubMed. Results We found no publications detailing the use of arthroscopy or AS specifically in patients with JIA involving the wrist. There is evidence that AS reduces pain, improves function, and induces remission in patients with rheumatoid arthritis resistant to medical management. Discussion and Conclusion Although there is paucity in evidence for the use of AS in the wrists of patients with JIA, studies suggest it to be safe and effective, and could be applied to patients with refractive JIA. It is possible that early identification of patients suffering from JIA with extensive joint destruction and little symptoms could benefit from AS, delaying joint destruction and preserving function.

Genome-wide data reveal novel genes for methotrexate response in a large cohort of juvenile idiopathic arthritis cases.

Clinical response to methotrexate (MTX) treatment for children with juvenile idiopathic arthritis (JIA) displays considerable heterogeneity. Currently, there are no reliable predictors to identify non-responders: earlier identification could lead to a targeted treatment. We genotyped 759 JIA cases from the UK, the Netherlands and Czech Republic. Clinical variables were measured at baseline and 6 months after start of the treatment. In Phase I analysis, samples were analysed for the association with MTX response using ordinal regression of ACR-pedi categories and linear regression of change in clinical variables, and identified 31 genetic regions (P<0.001). Phase II analysis increased SNP density in the most strongly associated regions, identifying 14 regions (P<1 × 10(-5)): three contain genes of particular biological interest (ZMIZ1, TGIF1 and CFTR). These data suggest a role for novel pathways in MTX response and further investigations within associated regions will help to reach our goal of predicting response to MTX in JIA.

Intrauterine environment and cognitive development in young twins.

Intrauterine factors important for cognitive development, such as birth weight, chorionicity and umbilical cord characteristics were investigated. A total of 663 twin pairs completed the Wechsler Intelligence Scale for Children-Revised and scores were available for Performance, Verbal and Total Intelligence Quotient (IQ). The intrauterine factors examined were birth weight, placental weight and morphology, cord knots, cord length and cord insertion. IQ scores for the varying levels of the intrauterine markers adjusting for gender and gestational age were calculated. The heritability of IQ and the association between IQ and intrauterine environment were examined. Twins with lower birth weight and cord knots had lower IQ scores. The aetiology of IQ is largely distinct from that of birth weight and cord knots, and non-shared environment may influence the observed relationships.

Young people's satisfaction of transitional care in adolescent rheumatology in the UK.

BACKGROUND: To examine the quality of transitional health care from the perspectives of young people with juvenile idiopathic arthritis (JIA) and their parents. METHODS: Adolescents with JIA and their parents were recruited from 10 major UK rheumatology centres. Satisfaction with health-care delivery was measured prior to, and 12 months after, the implementation of a structured and co-ordinated programme of transitional care using self-completed questionnaires designed for this study. RESULTS: Of 359 families invited to participate, 308 (86%) adolescents with JIA and 303 (84%) parents/guardians accepted. A fifth of adolescents had persistent oligoarthritis. Median age was 14.2 (11-18) years with median disease duration of 5.7 (0-16) years. Young people and their parents rated provider characteristics more important than aspects of the physical environment or process issues. Staff honesty and knowledge were rated as the most essential aspects of best practice. Prior to implementing the programme of transitional care, parents rated service delivery for all items significantly worse than best practice. Overall satisfaction improved 12 months after entering the programme. However, while parent satisfaction improved for 70.4% of items, significant improvements were only observed for three (13.6%) items rated by adolescents. CONCLUSION: The perceived quality of health care for young people with JIA and their parents was significantly lower than what they would like. Satisfaction with many aspects of care during transition from paediatric to adult services can be improved through the implementation of a structured, co-ordinated programme of transitional care.

Development and preliminary validation of the 'Mind the Gap' scale to assess satisfaction with transitional health care among adolescents with juvenile idiopathic arthritis.

BACKGROUND: To develop a scale to assess satisfaction with transitional health care among adolescents with a chronic illness and their parents. METHODS: The 'Mind the Gap' scale was developed using evidence from a previous needs assessment, in three stages: (1) definition of the construct; (2) design of the scale items, response options and instructions; (3) full administration of the scale, item analysis and dimensionality analysis. The scale was administered to 308 adolescents with juvenile idiopathic arthritis (JIA) and 303 parents/guardians, prior to and 12 months after the implementation of an evaluation of a structured and co-ordinated programme of transitional care. The patient population involved adolescents with JIA and their parents recruited from 10 major UK rheumatology centres. RESULTS: A total of 301 (97.7%) adolescents and 286 (95.0%) parents chose to complete the questionnaire, with median item completion rates of 100.0% (0-100%) for both adolescents and parents thus confirming feasibility. Face and content validity were confirmed. Factor analyses revealed a three-factor structure which explained 49.5% and 56.1% of the variation in adolescent and parent scores respectively. The internal consistency of each subscale ('management of environment', 'provider characteristics' and 'process issues') was indicated by Cronbach's alphas of 0.71, 0.89 and 0.89 for adolescents, respectively, and 0.83, 0.91 and 0.92 for parents respectively. Cronbach's alphas for the entire scales were 0.91 and 0.94 for the adolescent and parent forms respectively. CONCLUSION: These preliminary results report the potential of the 'Mind the Gap' scale in evaluating transitional care for adolescents with JIA. In view of the generic nature of transitional care reflected in the scale, this scale has wider potential for use with adolescents with other chronic illness in view of the generic nature of transition. This development is particularly timely in the context of transitional care developments in the UK and further validation of the scale is in progress.

The impact of a coordinated transitional care programme on adolescents with juvenile idiopathic arthritis.

OBJECTIVE: There is an extensive evidence base for the need of transitional care, but a paucity of robust outcome data. The aim of the study was to determine whether the quality of life of adolescents with juvenile idiopathic arthritis (JIA) could be improved by a co-ordinated, evidence-based programme of transitional care. METHODS: Adolescents with JIA aged 11, 14 and 17 yrs and their parents were recruited from 10 rheumatology centres in the UK. Data were collected at baseline, 6 and 12 months including core outcome variables. The primary outcome measure was health-related quality of life (HRQL): Juvenile Arthritis Quality of Life Questionnaire (JAQQ). Secondary outcome measures included: knowledge, satisfaction, independent health behaviours and pre-vocational experience. RESULTS: Of the 359 families invited to participate, 308 (86%) adolescents and 303 (84%) parents accepted. A fifth of them had persistent oligoarthritis. Median disease duration was 5.7 (0-16) yrs. Compared with baseline values, significant improvements in JAQQ scores were reported for adolescent and parent ratings at 6 and 12 months and for most secondary outcome measures with no significant deteriorations between 6 and 12 months. Continuous improvement was observed for both adolescent and parent knowledge with significantly greater improvement in the younger age groups at 12 months (P = 0.002). CONCLUSIONS: This study represents the first objective evaluation of an evidence-based transitional care programme and demonstrates that such care can potentially improve adolescents' HRQL.

Growing up and moving on in rheumatology: parents as proxies of adolescents with juvenile idiopathic arthritis.

OBJECTIVE: To examine agreement about physical health, functional ability, and health-related quality of life (HRQOL) between adolescents with juvenile idiopathic arthritis (JIA) and their parents. METHODS: The study group comprised 303 adolescent-parent dyads who completed individual questionnaires, including the Childhood Health Assessment Questionnaire with visual analog scales for pain and general well-being, and the Juvenile Arthritis Quality of Life Questionnaire. Agreement was determined using the Bland and Altman method. RESULTS: Approximately half of the adolescent-parent dyads showed clinically acceptable agreement for pain, general well-being, functional disability, and HRQOL. Where discrepancies occurred, there were similar numbers of parental overestimation and underestimation, with the exception that parents rated functional ability worse than did adolescents. Parents were also consistent with respect to overestimation or underestimation, irrespective of the health domain in question. Agreement was associated with better disease-related outcome variables, but was not significantly influenced by demographic factors. Agreement between adolescents and parents was dependent on the level of disease outcome and the health domain under scrutiny, and was less for moderate disease outcomes (as compared with mild or severe) and less visible phenomena (e.g., pain, global well-being). CONCLUSION: There is a wide variation in agreement between adolescents with JIA and their parents that is dependent on which health-related variable is under scrutiny. Proxy report is likely to be valid for adolescents with JIA at either the mild or severe end of the spectrum and/or for the visible manifestations of the disease. Consideration of both adolescent and parent-proxy reports is therefore important in future research.

Health-related quality of life in adolescents with juvenile idiopathic arthritis.

OBJECTIVE: To describe the health-related quality of life (HRQOL) of adolescents with juvenile idiopathic arthritis (JIA), and to examine the usefulness of the Juvenile Arthritis Quality of Life Questionnaire (JAQQ) in a UK context. It was hypothesized that HRQOL would decrease with worsening disease and disability. METHODS: Patients with JIA ages 11, 14, and 17 years were recruited from 10 major rheumatology centers. HRQOL was measured using the JAQQ. Other data were core outcome variables including the Childhood Health Assessment Questionnaire, demographic characteristics, arthritis-related knowledge, and satisfaction with health care. RESULTS: Questionnaires were completed by 308 adolescents. One-fifth had persistent oligoarthritis. Median disease duration was 5.7 years (range <1-16 years). The JAQQ was shown to have good psychometric properties when used in the UK, but was not without limitations. HRQOL of adolescents with JIA was less than optimal, particularly in the domains of gross motor and systemic functioning. Items most frequently rated as adolescents' biggest psychological problems were "felt frustrated" and "felt depressed," rated by 30.2% and 23.4%, respectively. These were particularly problematic for the 17-year-olds, with 39% reporting frustration as one of their biggest problems and 63.6% reporting depression. Variation in the adolescent JAQQ scores was explained by functional disability, pain, and disease activity. CONCLUSION: JIA can have a significant adverse effect on the HRQOL of adolescents. The JAQQ is a useful tool to assess the HRQOL of UK adolescents with JIA, but there is need for improved measures that incorporate developmentally appropriate issues.

Growing up and moving on. A multicentre UK audit of the transfer of adolescents with juvenile idiopathic arthritis from paediatric to adult centred care.

OBJECTIVE: To assess the provisions made for the transfer of adolescents with juvenile idiopathic arthritis to adult rheumatology clinics in the UK and the impact of a transitional care programme. METHODS: An audit of the documentation of the provisions made for transfer in 10 centres participating in a controlled trial of transitional care. Each centre conducted a retrospective case note audit of the recent patients transferred to adult care before and 12-24 months after the start of the trial. Demographic details, age when transition was first discussed, age at transfer, transitional issues, multidisciplinary team involvement, adolescent self advocacy, and readiness were documented. RESULTS: There were improvements at follow up in documentation of transitional issues, disease specific educational needs, adolescent readiness, and parental needs with the exception of dental care, dietary calcium, and home exercise programmes. The age at which the concept of an independent clinic visit was introduced was lower (mean (SD): 16.8 (1.06) v 15.8 (1.46) years, p = 0.01) but there were no other changes in age related transitional milestones. Significantly more participants had preparatory visits to the adult clinic, had a transition plan, and had joint injections while awake at follow up. CONCLUSIONS: The improvement in documentation suggests that involvement in the research project increased awareness of transitional issues. The difficulty of changing policy into practice was highlighted, with room for improvement, particularly at the paediatric/adult interface. The reasons for this are likely to be multiple, including resources and lack of specific training.

Growing up and moving on in rheumatology: a multicentre cohort of adolescents with juvenile idiopathic arthritis.

OBJECTIVES: To define the transitional care workload of a multicentre cohort of adolescents with juvenile idiopathic arthritis (JIA) including disease, self-advocacy and vocational issues prior to the implementation of a transitional care programme. METHODS: Data were collected using questionnaires completed by senior clinicians, patients and parents in 10 UK paediatric rheumatology centres. Entry criteria for patients included a confirmed diagnosis of JIA for at least 6 months and an age of 11, 14 or 17 yr. RESULTS: Of 359 families invited to participate, 308 (85.79%) adolescents with JIA and 303 parents/guardians accepted. Of these, 19.5% had persistent oligoarthritis. Despite their imminent transfer to adult care, ongoing transitional issues were identified in the 17-yr-old cohort: 55.8% were still seeing the rheumatologists with their parent, 20% were not self-medicating, 68.5% had not had intra-articular injections under local anaesthetic and 14% had received no careers counselling. This age group also had significant disease-related issues; 54.6% had moderate to severe functional disability, 67.5% were still on disease-modifying anti-rheumatic drugs and, as a group, they had significantly greater pain than younger patients. CONCLUSIONS: This study has objectively identified the transitional care workload facing paediatric and adult rheumatologists in terms of disease-related, self-advocacy and vocational issues. Outcome data following the implementation of a coordinated transitional care programme are awaited.

Transitional care for adolescents with juvenile idiopathic arthritis: a Delphi study.

OBJECTIVE: To identify the ideal programme of transitional care for adolescents with juvenile idiopathic arthritis (JIA) as perceived by users and providers, and to examine the feasibility of achieving this within a UK National Health Service context. METHODS: A modified two-stage Delphi study was undertaken with rheumatology health professionals, young people with JIA (aged 12-25 yr) and their parents. Participants were presented with statements about transitional care and asked to rate (i) the extent to which these constituted best practice, and (ii) their feasibility. RESULTS: Second-round questionnaires were completed by 83 individuals, representing an overall response rate of 90%. Items strongly agreed to constitute best practice and highly feasible included: 'addressing young people's psychosocial and educational/vocational needs'; 'using an individualized approach'; 'providing honest explanations of the adolescent's condition and health-care'; 'providing opportunities for adolescents to express opinions and make informed decisions'; 'having continuity in health personnel'; and 'giving adolescents the option of being seen by professionals without their parents'. However, providing adolescent-focused environments, professionals knowledgeable in transitional care and opportunities for young people with JIA to meet similar others were seen as feasible in only a few hospitals. CONCLUSIONS: There is considerable agreement as to the most important elements of transitional care. Those that are easily achievable should be undertaken in all hospitals that care for adolescents with JIA. However, not all elements identified were perceived as easily feasible; further research is required to determine how to implement these elements.

User perspectives of transitional care for adolescents with juvenile idiopathic arthritis.

OBJECTIVES: To gain insight into the transitional needs of adolescents with juvenile idiopathic arthritis (JIA) and to examine how these needs may be addressed within a structured programme of transitional care. METHODS: A qualitative study using focused group discussions was performed. Groups comprised (i) adolescents with JIA aged 12-18 yr, (ii) young adults with JIA aged 19-30 yr, (iii) parents of adolescents with JIA, and (iv) parents of young adults with JIA. RESULTS: Transitional needs included aspects of participants' physical, social, psychological and vocational lives. Participants (n = 55) called for developmentally appropriate care based upon shared decision-making, continuity of health professionals, and wider access to information and community services. Suggestions for improved care included individualized assessment of patient's holistic needs and increased transfer preparation. CONCLUSIONS: These results provide a useful guide to transitional care and suggest an approach that is adolescent-focused and evidence-based.

Unmet education and training needs of rheumatology health professionals in adolescent health and transitional care.

OBJECTIVES: To determine the perceived education and training needs of health professionals involved in transitional care for adolescents with juvenile idiopathic arthritis (JIA). METHODS: Two distinct questionnaires to identify transitional issues in JIA were distributed to key health professionals (n = 908) and clinical personnel involved in the implementation of a transitional care programme (n = 22). RESULTS: The first survey was completed by 263 professionals. Education needs were reported by 114 (43%) of health professionals. Transition issues and informational resources were the most frequently reported areas of need. The second survey was completed by 22 clinical personnel who rated 'lack of training', 'lack of teaching materials geared towards adolescents' and 'limited clinic time' as the main barriers to providing developmentally appropriate care to adolescents. CONCLUSION: Unmet education and training needs of health care professionals exist in key areas of transitional care and provide useful directions for the development of future training programmes.

Developing a programme of transitional care for adolescents with juvenile idiopathic arthritis: results of a postal survey.

OBJECTIVES: To explore the transitional needs of adolescents with juvenile idiopathic arthritis (JIA), as perceived by a range of professionals, and to examine how these needs may be addressed within a structured programme of transitional care. METHODS: Postal surveys (n = 1670) were distributed to key professionals employed in health, social support, education and vocation. RESULTS: Surveys were completed by 478 individuals. The majority of respondents (91%) were currently active in the care of adolescents with JIA. Planning for transitional care was perceived to be important for both adolescents and parents and to require multidisciplinary involvement. Respondents rated a wide range of resources to be important in supporting adolescents, including self-medication teaching packages and social skills training. A number of barriers to providing transitional care were identified, including inadequate resources, coordination and training. CONCLUSION: Transitional care in the context of JIA is perceived as necessary by a wide variety of professionals.

Nailfold capillary microscopy in healthy children and in childhood rheumatic diseases: a prospective single blind observational study.

OBJECTIVES: To develop an objective method of nailfold capillaroscopy (NFC), applicable to a wide age range of paediatric patients. To compare the morphological characteristics of the nailfold capillaries in different rheumatology patient groups and controls. METHODS: A colour digital video camera attached to a stereomicroscope was used to capture nailfold capillary images. Computerised image processing was used to analyse and store data. Subsequent quantitative and qualitative morphological analysis was performed in the following paediatric patient and control groups: 18 children with connective tissue diseases (CTD: juvenile dermatomyositis, systemic sclerosis, and undifferentiated connective tissue disease), eight with systemic lupus erythematosus, nine with primary Raynaud's disease, three with primary vasculitis, 15 with juvenile idiopathic arthritis, 17 healthy children and 20 healthy adults. Images were analysed by a single assessor who was unaware of the patient details. RESULTS: The NFC technique was simple to perform and gave reproducible results, although some intra- and intersubject variation was noted. Capillary density and width was age related, with younger children having fewer and wider capillaries than older children and adults. Linear capillary density was significantly higher in healthy adults (mean (SD) 8.6 (1.6) capillaries/mm) compared with healthy children (HC 6.9 (0.9) capillaries/mm). The group with CTD had the most abnormal findings, with lower linear density (4.9 (1.7) capillaries/mm) and increased capillary loop width (10.7 (7.3) mm) compared with HC (3.5 (1.7) mm). In addition, 11/18 (61%) patients in the CTD group had more than two definitely abnormal capillaries in at least two nailfolds, an abnormality not seen in other subjects. Two qualitative measures, the degree of avascularity and general disarrangement of capillary pattern, were more commonly observed in the CTD group than in HC. The proportion of tortuous capillaries did not differ significantly between study groups. CONCLUSIONS: This study is unique in measuring objective quantitative and qualitative parameters of the nailfold vasculature across a wide spectrum of age and disease. Differences in capillary morphology and frequency in children with CTD compared with other paediatric diseases and healthy controls were demonstrated. In the clinical situation, an assessment of the general degree of disarrangement may offer a fast tool for assessment of the nailfold vasculature which correlates well with NFC data.

Development and assessment of a World Wide Web site for systemic lupus erythematosus patient information.

Patient education is an important component of the management of chronic diseases such as SLE. We have investigated the value of the World Wide Web as a medium for delivery of SLE patient information. Volunteers recruited from the clinic and from the website completed interviews and questionnaires aimed at defining their information needs. A new website was then established and its impact on users tested using knowledge questionnaires. The new website was used extensively (20-30 users each day) over the 24 month period of study until April 2001. A total of 510 participants completed an online questionnaire that showed that for some users it was their first use of the internet to gather lupus information, but the majority (58.9%) accessed it at least monthly for this purpose. We also found that, while most users (56.9%) found current disease information was at an appropriate level, 37.5% thought it was too basic. Knowledge questionnaires from 42 participants before and after using the site showed a significant rise in users' knowledge of the areas covered by the site. As far as we are aware this study is the first to show that a patient-oriented website can have a positive effect on disease knowledge. The relative ease with which good quality information can be disseminated via the web suggests that this medium is likely to be less costly and perhaps more educationally effective than printed information, and so is likely to become a primary vehicle for patient education. The website tested can be found at: www.rheumatology.bham.ac.uk/lupus/intro.html.

The use of multidisciplinary assessment and scientific measurement in advanced juvenile idiopathic arthritis can categorise gait deviations to guide treatment.

BACKGROUND: It is difficult to identify the range of gait deviations associated with juvenile idiopathic arthritis (JIA) using simple clinical observations. AIMS: To use objective gait analysis to accurately describe biomechanical gait abnormalities in JIA and to search for common patterns, which may subsequently serve as a basis for therapeutic intervention. METHODS: Children with persistent polyarticular arthritis and symmetrical joint involvement were referred to the Gait Analysis Laboratory and independently assessed by a multidisciplinary team. Gait analysis was performed using an in-house Visual Vector System and the Novel PEDAR in-shoe plantar pressure measurement system. Clinical groupings were based on the extent of joint restriction: minimal (group A), and moderate-severe (with supinatory foot deformity (group B), or with pronatory foot deformity (group C)). Gait analysis enabled classification of each subject into one of four gait patterns: either near normal (pattern I) or one of three adaptive patterns defined by the predominant abnormality--lower limb pain (pattern II), lower limb deformity (pattern III), or a combination of pain and deformity of the lower limb (pattern IV). RESULTS: Of the 15 subjects assessed as part of this study, seven were placed into clinical group A, six into group B, and two into group C. All the subjects with gait patterns I and II were found in clinical group A. Both subjects from clinical group C exhibited gait pattern III. All subjects from clinical group B and the remainder from group A exhibited a mixture of gait patterns III and IV. CONCLUSION: Despite the initial clinical observations it was not always possible to predict the resultant gait pattern. Scientific gait analysis allowed a clear distinction to be made between primary and secondary gait deviations, and accurate targeting of physiotherapy and orthotic interventions to suit each individual. Prospective quantitative analysis in a larger sample is under way to support the clinical effectiveness of these findings.

Remotely sensed habitat diversity predicts butterfly species richness and community similarity in Canada.

Although there is no shortage of potential explanations for the large-scale patterns of biological diversity, the hypothesis that energy-related factors are the primary determinants is perhaps most extensively supported, especially in cold-temperate regions. By using unusually high-resolution biodiversity and environmental data that have not previously been available, we demonstrate that habitat heterogeneity, as measured by remotely sensed land cover variation, explains Canadian butterfly richness better than any energy-related variable we measured across spatial scales. Although species-richness predictability declines with progressively smaller quadrat sizes, as expected, we demonstrate that most variability (>90%) in butterfly richness may be explained by habitat heterogeneity with secondary contributions from climatic energy. We also find that patterns of community similarity across Canada are strongly related to patterns of habitat composition but not to differences in energy-related factors. Energy should still be considered significant but its main role may be through its effects on within-habitat diversity and perhaps, indirectly, on the sorts of habitats that may be found in a region. Effects of sampling intensity and spatial autocorrelation do not alter our findings.