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Biomedical subjects

J Wray

Publications and source records attributed to J Wray.

At least 19 recordsLinked to original sources
PubMed2005

Predictors of poor mid-term health related quality of life after primary isolated coronary artery bypass grafting surgery.

OBJECTIVE: To assess the determinants of poor mid-term health related quality of life (HRQoL) at one year after primary isolated coronary artery bypass grafting (CABG). METHODS: 463 patients who underwent primary isolated CABG for multivessel disease and came for their annual follow up at the outpatient clinic during one year at Harefield Hospital, Middlesex, were approached to participate in the present study. Prospective clinical data were collected as part of the clinical care of the patients and were retrospectively analysed when the patients consented to participate in the study at their outpatient visit. After their consent they were given three HRQoL assessment questionnaires. Scores, together with clinical data, were analysed by both univariate and multivariate analyses with regard to poor HRQoL outcome. RESULTS: 437 (94.4%) patients consented to participate in the study and filled in the HRQoL questionnaires. Ten variables were identified in the univariate analysis as potential predictors of poor scores of the physical element of HRQoL; however, only three variables-gastrointestinal problems, congestive heart failure, and type D personality trait-predicted poor physical scores independently. Eleven variables were identified in the univariate analysis as potential predictors of poor scores of the mental element of HRQoL; however, only three variables-peripheral vascular disease, infective complications, and type D personality trait-predicted poor physical scores independently. CONCLUSION: Preoperative gastrointestinal problems, preoperative congestive heart failure, and type D personality trait were independent predictors of the poor physical component of HRQoL. Peripheral vascular disease, infective complications, and type D personality trait were independent predictors of the poor mental component of HRQoL. Interestingly, patients with type D personality were more than twice as likely to have poor physical HRQoL and more than five times as likely to have poor mental HRQoL.

Adult↗
PubMed2004

Metabolic and neuropsychological phenotype in women heterozygous for ornithine transcarbamylase deficiency.

We compared neurocognitive indices with clinical status, mutation analysis, and urea synthetic capacity in 19 women heterozygous for ornithine transcarbamylase deficiency. Although as a group, these women had average IQ scores, they displayed a specific neuropsychological phenotype with significant strengths in verbal intelligence, verbal learning, verbal memory, and reading, and significant weaknesses in fine motor dexterity/speed and nonsignificant weaknesses in nonverbal intelligence, visual memory, attention/executive skills, and math. This suggests selective vulnerability of white matter and better preservation of gray matter. When the group was divided into symptomatic and asymptomatic subgroups, based on either clinical history or residual urea synthetic capacity, the asymptomatic subgroup outperformed the symptomatic subgroup on all tested domains of neuropsychological functioning. Furthermore, the amount of residual urea synthetic capacity was predictive of several end point cognitive measures. There was no correlation between neonatal versus late-onset mutation or between normal or abnormal allopurinol challenge and neuropsychological outcome. In sum, we identified a specific metabolic and neurocognitive phenotype in women heterozygous for ornithine transcarbamylase deficiency. The findings support the importance of maintaining meticulous metabolic control in children with urea cycle disorders, because even mildly symptomatic subjects demonstrate cognitive deficits.

Adult↗
PubMed2004

Quality of life and coping following minimally invasive direct coronary artery bypass (MIDCAB) surgery.

Minimally invasive direct coronary artery bypass (MIDCAB) surgery has been shown to be a promising technique for surgical treatment of single or double vessel disease. However, little is known about quality of life, mood state or coping in this group of patients. The records of 55 consecutive patients who underwent MIDCAB surgery at Harefield Hospital between April 1999 and May 2001 were reviewed. In order to assess quality of life, mood state and coping, patients were contacted by telephone to conduct a semi-structured interview and were subsequently sent four questionnaires. The measures used were the Hospital Anxiety and Depression Scale, the Short Form Health Survey, the WHOQoL-BREF and the COPE. Forty-eight patients were contacted by telephone, forty-four of whom returned the completed questionnaires. Overall ratings of quality of life were excellent for the majority of patients, and rates of anxiety and depression were lower than previously found following coronary artery bypass surgery. It is concluded that following MIDCAB surgery quality of life and mood state outcomes are encouraging. However, a prospective, longitudinal study is now required to further elucidate the relationship between quality of life, mood state and coping and to identify predictive factors for physical and psychological outcome following this new surgical technique.

Adaptation, Psychological↗
PubMed2003

Maternal phenylketonuria in Western Australia: pregnancy outcomes and developmental outcomes in offspring.

OBJECTIVE: To examine the outcomes of phenylketonuric (PKU) pregnancies in Western Australia including birth characteristics and cognitive and behavioural outcomes in offspring. METHODS: A cross-sectional study of women and their offspring who were identified from the Western Australian Maternal PKU Program (WAMPKUP) from 1991 to 2000 was carried out. Cognitive assessments (K-BIT or Griffiths scales) were conducted on women and their children, and behavioural assessments (CBCL) were conducted on the children. RESULTS: Thirty pregnancies by nine women were registered on the WAMPKUP between 1991 and 2000. There were 16 live births, with one preterm delivery at 32 weeks. There were no congenital abnormalities. Five of the nine mothers and their nine children (aged 18 months-10 years) participated in developmental assessments. A linear relationship was shown between lower maternal IQ scores and later attainment of metabolic control in pregnancy (rs = -0.828; P = 0.01). There was significant correlation between lower offspring IQ scores and later attainment of metabolic control in pregnancy (rs = -0.734; P = 0.02). Correlation between maternal and offspring cognitive scores was not significant. Four of nine (44%) children rated in the clinical range for behavioural problems. Compared to children with no behavioural difficulties, these children had lower cognitive abilities (P = 0.05) and maternal metabolic control during pregnancy was poor (P = 0.05). CONCLUSIONS: Poor metabolic control in pregnancy is associated with poorer cognitive outcomes and increased behavioural difficulties in offspring of mothers with PKU. The results have implications for the implementation of appropriate dietary measures before conception in PKU pregnancies, and indicate a need for the establishment of multidisciplinary teams to follow up individuals with PKU to communicate the importance of pregnancy planning, to manage PKU pregnancies, and to follow up the offspring.

Child Behavior↗
PubMed2003

Children with autistic spectrum disorders. I: comparison of placebo and single dose of human synthetic secretin.

AIMS: To examine the effect of a single dose of human synthetic secretin (HSS) on behaviour and communication in children with autism spectrum disorder (ASD) using an objective measure of communication and social reciprocity and standardised rating scales. METHODS: Randomised, crossover, double blind, and placebo controlled trial of a single intravenous dose of human synthetic secretin (HSS) 2 CU/kg. The 62 subjects (3-8 years) were assigned to group 1 (saline placebo/HSS) or group 2 (HSS/saline placebo). Diagnosis was confirmed by ADI-R (Autism Diagnostic Interview-Revised) algorithm. Severity of symptoms was rated using the CARS (Childhood Autism Rating Scale). Outcome measures included Communication and Symbolic Behavior Scale (CSBS), Ritvo Real-life Rating Scale, weekly Global Rating Scale (GBRS) by parents and teachers, and daily log of gastrointestinal symptoms. The communication subscale of the CSBS, specifying communication function, reciprocity, and social-affective signalling was videotaped and scored by a blinded, trained observer. RESULTS: Sixty one children completed the study. After randomisation, there were no significant differences in gender, race, age, and parent and teacher GBRS and Ritvo Scale between the two groups. Compared with placebo, secretin treatment was not associated with significant improvement of CSBS standard scores from baseline to 2 or 4 weeks post-infusion. Five children showed clinical improvement in standard scores: two after HSS and three after placebo. There were no significant changes in gastrointestinal symptoms after HSS or saline placebo. CONCLUSIONS: A single dose of intravenous human secretin is not effective in changing behaviour and communication in children with ASD when compared to placebo.

Autistic Disorder↗
PubMed2003

Children with autistic spectrum disorders. II: parents are unable to distinguish secretin from placebo under double-blind conditions.

BACKGROUND: Standardised measures of behaviour have failed to detect short term improvement in children with autism following treatment with secretin. However, it is possible that standardised measures are insensitive to dimensions of child behaviour that are nonetheless detectable by parents. AIM: To determine the ability of parents of children with autism to guess, under double blind conditions, whether their child had received secretin or placebo. METHODS: 2x2 crossover randomised blinded study, comparing the effect of synthetic human secretin 2 U/kg to placebo (saline). Sixty two children with autism (aged 43-103 months) were randomly allocated to two groups: group 1 received placebo, followed six weeks later by secretin, and group 2 received secretin followed by placebo. At the conclusion of the study, parents were asked to guess their child's group assignment. RESULTS: Twenty seven families guessed their child's group assignment correctly and 27 guessed incorrectly. In 48 instances, parents based their guess on perceived improvement; in six cases, parents based their guess on perceived deterioration. Six families saw no difference after either infusion, and offered no guess. One family dropped out after the first infusion, and one family was lost to follow up after the second infusion. CONCLUSION: In a controlled setting, parents of young children with autism are unable to distinguish the short term behavioural effects of secretin from placebo.

Autistic Disorder↗
PubMed2001

A fluorescence temperature-jump study of conformational transitions in myosin subfragment 1.

The tryptophan fluorescence of unmodified myosin subfragment 1 (S1) from rabbit and chicken skeletal muscle with various nucleotides and phosphate analogues bound was measured after rapid temperature jumps. The fluorescence decreased during the temperature rise. Under some conditions, this decrease was followed by an increase, reflecting structural transitions within the protein. With adenosine 5'-[beta,gamma-imido]triphosphate (p[NH]ppA) or with ADP and BeF(x) bound, this rise was very rapid (reciprocal time constant approx. 2000 s(-1)) and varied only slightly with starting temperature, suggesting that, with these ligands, two different protein conformations were present in rapid equilibrium over a large temperature range. In the presence of ATP, the transient included several relaxation processes. Overall, the results suggest that complexes of S1 with ATP or with a number of other ligands exist as a mixture of two forms in temperature-dependent equilibrium. The results throw light on the finding of different forms of S1 in recent crystallographic studies and indicate a surprising lack of strong coupling between myosin's structural state and the nature of the nucleotide bound.

Adenosine Diphosphate↗
PubMed2001

Returning to school after heart or heart-lung transplantation: how well do children adjust?

BACKGROUND: Up to 40% of children and adolescents with chronic illness experience school-related problems, including learning difficulties and problems in social adjustment and peer relationships. Despite the life-threatening nature of heart and heart-lung transplantation and the severity of illness, which results in the necessity for surgery, there is little information on the school performance of children after transplantation. METHODS: Eighty-one children and adolescents were assessed with regard to their academic attainments and behavior at school at regular intervals after heart (n=47) or heart-lung (n=34) transplantation and comparisons made with a group of healthy children. RESULTS: Cognitive ability and performance on academic attainments were within the normal range and did not change significantly as a function of time since transplant. However, performance was at a significantly lower level than that of the healthy children. Although the prevalence of behavior problems was only 8% at 6 months posttransplant, at 3 years, it had increased to 29% and, at 5 years, it was still 27%. Children with an initial diagnosis of congenital heart disease had more academic and behavioral difficulties than those with either cardiomyopathy or cystic fibrosis. CONCLUSIONS: A significant number of children who had undergone successful transplantation experienced difficulties at school. Contrary to expectations, educational problems were more prevalent in the medium term, rather than short term, after transplant. Initial diagnosis was a salient factor in posttransplant psychological functioning at school. Early intervention and close liaison with schools is indicated to reduce psychological morbidity and enhance adaptation within the school environment.

Absenteeism↗
PubMed2001

Behaviour modification and gentle teaching workshops: management of children with learning disabilities exhibiting challenging behaviour and implications for learning disability nursing.

BACKGROUND: Challenging behaviours (behaviour difficulties) represent a problem of considerable clinical significance for learning disability nurses, and a source of much human distress. Gentle teaching is a relatively new approach to dealing with behavioural difficulties, and has been received with enthusiasm by clinicians, but has so far received little empirical support. The current study attempted to compare gentle teaching with a well-established alternative (behaviour modification) and a control group. OBJECTIVES: To examine the comparative effectiveness of gentle teaching, behaviour modification and control interventions for challenging behaviour amongst children with learning disabilities. DESIGN: Nonrandomized controlled trial. SETTING: Service users' homes in East Yorkshire. PARTICIPANTS: Seventy-seven children who presented with learning disabilities and challenging behaviour (behaviour difficulties) and their parents. PROCEDURE: One-day workshops in were offered by recognized authorities in either behaviour modification or gentle teaching that were not otherwise involved with the research project. Forty-one participants were recruited to the gentle teaching condition; 36 to behaviour modification; 26 to the control group. Random allocation was not possible, because of the slow uptake by interested parents. Measures was preintervention, and at assessment points up until 12 months following intervention. ANALYSIS: Quantitative analysis of pre-post differences between the groups, using t-test. RESULTS: In general, no significant differences were found between the treatment groups and controls. Significant improvements were found for both gentle teachingand behaviour modification children over controls on the AAMR ABS XVII (social engagement) subscale. Controls had more contact with medical practitioner (GP) services than behaviour modification children and less than gentle teaching children. CONCLUSION: Although very few differences were found between the three groups, those that did exist generally favoured behaviour modification. Implications for service provision and learning disability nursing practice are described.

Adolescent↗
PubMed2001

Congenital heart disease and cardiac surgery in childhood: effects on cognitive function and academic ability.

OBJECTIVE: To evaluate changes in cognitive and academic functioning following cardiac surgery in children with congenital heart disease. DESIGN: A prospective cross sectional study in which patients were assessed immediately before treatment and 12 months later. PATIENTS: Three groups of children aged 3.5-17 years: a group with congenital heart disease awaiting surgery, another awaiting bone marrow transplantation, and a healthy comparison group. MAIN OUTCOME MEASURES: Intelligence quotient and measures of academic attainment, evaluated with the British Ability Scales. RESULTS: Preoperatively, children with cyanotic lesions showed deficits in comparison with those with acyanotic lesions. Postoperatively, children with cyanotic lesions showed a deterioration in performance and achieved significantly lower scores than those with acyanotic lesions. While there were significant differences between the congenital heart disease and bone marrow transplantation groups preoperatively, these were no longer apparent at follow up. CONCLUSIONS: In contrast to previously published findings, the present results suggest that cardiac surgery does not result in early postoperative improvements in cognitive function for children with congenital heart disease. The nature of the cardiac lesion continues to affect cognitive and academic performance, even after surgery.

Adolescent↗
PubMed2000

Modeling LGN responses during free-viewing: a possible role of microscopic eye movements in the refinement of cortical orientation selectivity.

Neural activity appears to be essential for the normal development of the orientation-selective responses of cortical cells. It has been proposed that the correlated activity of LGN cells is a crucial component for shaping the receptive fields of cortical simple cells into adjacent, oriented subregions alternately receiving ON- and OFF-center excitatory geniculate inputs. After eye opening, the spatiotemporal structure of neural activity in the early stages of the visual pathway depends not only on the characteristics of the environment, but also on the way the environment is scanned. In this study, we use computational modeling to investigate how eye movements might affect the refinement of orientation tuning in the presence of a Hebbian scheme of synaptic plasticity. Visual input consisting of natural scenes scanned by varying types of eye movements was used to activate a spatiotemporal model of LGN cells. In the presence of different types of movement, significantly different patterns of activity were found in the LGN. Specific patterns of correlation required for the development of segregated cortical receptive field subregions were observed in the case of micromovements, but were not seen in the case of saccades or static presentation of natural visual input. These results suggest an important role for the eye movements occurring during fixation in the refinement of orientation selectivity.

Animals↗
PubMed2000

Management of women who are Rhesus D negative in Northern Ireland.

In 1998, all 15 maternity hospitals in Northern Ireland participated in a retrospective survey of the management of pregnant women who were Rhesus D (RhD) negative. This area of clinical practice was chosen for study in the knowledge that a policy of routine antenatal prophylaxis had been proposed at the RCP/RCOG Consensus Conference, and that a change in current clinical practice was likely to follow. It was therefore felt prudent to survey critically current practice in order to collect baseline data before the introduction of new guidelines. Data were analysed on 3274 RhD negative women. The findings from this study show that current management of women who are RhD negative is suboptimal. In particular, there is poor adherence to the guidelines relating to the management of potentially sensitising events in pregnancy. Testing to assess the size of a feto-maternal bleed was often omitted, and it was therefore impossible to ascertain whether an appropriate dose of anti-D immunoglobulin had been administered. Recent publications have also identified this as an area of concern. Trauma during pregnancy was often not managed appropriately, as a potentially sensitising event. Better compliance with the current guidelines was noted when management after delivery was considered. The reasons for this apparent inconsistency should be addressed. New guidelines, recommending the use of routine antenatal prophylaxis to all women who are RhD negative, have recently been published. Providers of antenatal care will need to address this issue within the context of their own practice environment. In doing so they should be mindful of current deficits in guideline compliance which may need to be remedied, to avoid building new practices upon fragile foundations.

Journal Article↗
PubMed1999

Controlled study of preschool development after surgery for congenital heart disease.

AIM: Research into intellectual impairment among children with congenital heart disease has focused mainly on older children. This study was designed to determine whether previous findings are applicable to preschool children. METHODS: Three groups of children under 31/2 years old were assessed immediately before treatment and 12 months later: a group with congenital heart disease awaiting surgery, another awaiting bone marrow transplantation, and a healthy comparison group. RESULTS: Although the means of the three groups were within the normal range, preoperatively the cardiac and transplant groups showed deficits compared with the healthy controls. Postoperatively, continuing developmental deficits were significant only in the children with cyanotic lesions. CONCLUSIONS: Conclusions about intellectual development in older children with congenital heart disease do not apply to preschool children. Before corrective surgery, chronic illness itself appears to be the predominant influence on development. Postoperatively, cyanotic and acyanotic lesions are associated with different short term outcomes.

Child Development↗