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Comparison of three generic questionnaires measuring quality of life in adolescents and adults with cystic fibrosis: the 36-item short form health survey, the quality of life profile for chronic diseases, and the questions on life satisfaction.

OBJECTIVE: To compare different generic instruments in measuring quality of life and to demonstrate dimensions of quality of life (QL) in patients with cystic fibrosis (CF). METHODS: The short-form-36 health survey (SF-36), the quality of life profile for chronic diseases (PLC), and the questions on life satisfaction (FLZ(M)) were simultaneously employed in a cross-sectional study with 70 adolescents and adults with CF. The different concepts of the measures were compared. Internal consistency (Cronbach's alpha), convergent and construct validity (correlation patterns, common factor analysis), and external validity (correlations with symptom and pulmonary function scores, with intensity of therapy; comparisons with healthy peers) of the three instruments were investigated. RESULTS: Similar reliability, but different validity of the questionnaires are demonstrated. Seventy-three percent of the total variance across the three measures could be explained with a seven-factor-solution: (1) physical functioning (19.3% of total variance), (2) mental health (19.3%), (3) social integration (7.5%), (4) role function/pain (7.5%), (5) economic/material living conditions (7.5%), (6) partnership/family (6.7%) and (7) anxiety (5.2%). DISCUSSION: The different validity of the instruments has to be considered in chosing a questionnaire appropriate to the purpose of measuring. Shortcomings of each instrument can be overcome by multimethod designs and by developing disease-specific scales.

Adolescent↗

Performance evaluation and patient outcomes monitored by nurse practitioners and certified nurse-midwives in Florida.

In this descriptive survey study, four elements of performance evaluation of advanced practice nurses (APNs) currently in clinical practice were examined: evaluation frequency, evaluation responsibility, evaluation parameters, and monitored patient outcomes. Questionnaires were mailed to 2,093 APNs living in Florida and licensed by the Florida State Board of Nursing. Eight hundred twenty-two usable surveys were returned, for a 39% response rate. Of these respondents, 654 were in clinical practice as APNs, and comprised the final sample. The majority (56.9%) received formal annual evaluations, and most (75.9%) were evaluated primarily by physicians; only 17.5% reported peer evaluation. The most frequent evaluation parameters were appropriateness of care, patient satisfaction, patient outcomes, and patient volume. Patient outcomes fell into four categories: clinical endpoints, complications, compliance, and functional status. A need to increase the regularity, formality, and peer review elements of APN performance evaluation was concluded.

Adult↗

Neuropsychology of early-treated phenylketonuria: specific executive function deficits.

This study explored the hypothesis that children with early-treated phenylketonuria (PKU) are selectively impaired on executive function measures, even when still on diet. The rationale for this hypothesis is that even mild elevations in phenylalanine (Phe) can lead to lower central levels of biogenic amines, including dopamine (DA). We hypothesize that this mild DA depletion causes subtle prefrontal dysfunction, which in turn affects executive functions such as set maintenance, planning, and organized search. 11 preschool early-treated PKU children (M age = 4.64) and a sample of age- and IQ-matched unaffected peers (n = 11) were evaluated on a battery of executive function (EF) measures. In addition, a "non-executive function" task, recognition memory, was administered to all subjects. Group comparisons demonstrated that PKU children were significantly impaired on an executive function composite score; there were no group differences, however, in recognition memory. These results supported the hypothesized specific deficit in executive function. Furthermore, within the PKU group the executive function composite score was significantly negatively correlated with concurrent phenylalanine levels, even after controlling for the correlation between IQ and executive function skills. This second finding provides support for the proposed biochemical mechanism underlying the specific cognitive deficits.

Child, Preschool↗

The impact of social support and self-esteem on adolescent substance abuse treatment outcome.

Although poor social resources and low self-esteem have been implicated in the development of teenage drug abuse, the role of these factors in the remission of adolescent addiction remains unclear. This study examines social support characteristics and self-esteem in relation to outcome following adolescent chemical dependency treatment. Adolescents and their parents completed self-report questionnaires and a research interview during treatment and at 6 and 12 months post-treatment. Two types of outcome were assessed at follow-up: (1) alcohol and drug use, and (2) functioning in major life domains (e.g., family, school/work, peers). Results indicate that the quality of social resources (i.e., drug-use patterns of supports) reported during treatment was related to alcohol- and drug-use status post-treatment, with abstainers reporting more nonusing supports than teens who returned to heavy drug use. Self-esteem and the degree of satisfaction with social support during treatment were negatively correlated with the number of major life problems during the 6 months following discharge. Altogether, inpatient measures of self-esteem, number of high-quality supports, and social support satisfaction accounted for 16% of the variance in 6-month substance use outcome and 25% of the variance in psychosocial functioning 6 months post-treatment. Six-month social support and self-esteem measures were similarly related to 1-year outcome. The implications of these findings for adolescent drug abuse treatment are discussed.

Adaptation, Psychological↗

Time costs of caring for children with severe disabilities compared with caring for children without disabilities.

To assess time costs of caring for children with severe disabilities in the community compared to caring for children without disabilities, a diary- and questionnaire-based study was carried out. Sixteen complete data sets were obtained from families with children who have disabilities (mean age 8.7 years) and 31 complete data sets from families with normally developing children (mean age 4.9 years). Diagnoses in the study group included cerebral palsy, autism, Sanfillipo syndrome, lissencephaly, and osteogenesis imperfecta. Items of personal care per waking hour were significantly greater in children with disabilities than non-disabled children (p<0.001). In the study group, there was no correlation (r=-0.12) between age and frequency of care whereas a significant correlation was observed between degree of disability as measured by the Functional Independence Measure for children (WeeFIM) and frequency of care items (r=0.89). Twelve of the 16 mothers in the study group were not in paid employment. Twelve had little or no extended family support. Benefits awarded did not correlate with the degree of disability as measured by the WeeFIM (r=-0.11). Care needs of children with severe disabilities are significantly greater than those of non-disabled children and do not decrease with advancing age. Mothers of children with disabilities are unable to work outside the home because of these care needs. This brings the family income, even when benefits are included, to a level that is less than peer families with non-disabled children. A Functional Disability Score may help to achieve more appropriate allocation of state resources.

Child↗

The ghost of selection past: rates of evolution and functional divergence of anciently duplicated genes.

The duplication of genes and even complete genomes may be a prerequisite for major evolutionary transitions and the origin of evolutionary novelties. However, the evolutionary mechanisms of gene evolution and the origin of novel gene functions after gene duplication have been a subject of many debates. Recently, we compiled 26 groups of orthologous genes, which included one gene from human, mouse, and chicken, one or two genes from the tetraploid Xenopus and two genes from zebrafish. Comparative analysis and mapping data showed that these pairs of zebrafish genes were probably produced during a fish-specific genome duplication that occurred between 300 and 450 Mya, before the teleost radiation (Taylor et al. 2001). As discussed here, many of these retained duplicated genes code for DNA binding proteins. Different models have been developed to explain the retention of duplicated genes and in particular the subfunctionalization model of Force et al. (1999) could explain why so many developmental control genes have been retained. Other models are harder to reconcile with this particular set of duplicated genes. Most genes seem to have been subjected to strong purifying selection, keeping properties such as charge and polarity the same in both duplicates, although some evidence was found for positive Darwinian selection, in particular for Hox genes. However, since only the cumulative pattern of nucleotide substitutions can be studied, clear indications of positive Darwinian selection or neutrality may be hard to find for such anciently duplicated genes. Nevertheless, an increase in evolutionary rate in about half of the duplicated genes seems to suggest that either positive Darwinian selection has occurred or that functional constraints have been relaxed at one point in time during functional divergence.

Amino Acid Sequence↗

Color and pulsed Doppler ultrasound findings in normally functioning transjugular intrahepatic portosystemic shunts.

OBJECTIVE: To evaluate blood flow changes inside normally functioning transjugular intrahepatic portosystemic shunts (TIPS), using color Doppler ultrasound (CDUS) and pulsed Doppler ultrasound (PDUS). METHODS: A total of 72 patients (mean age 54, range 36-78 years) underwent TIPS placement, portal angiography, CDUS and PDUS examinations. Measurements inside the stent were taken at the portal side, at the central part and at the venous end of the TIPS. RESULTS: In well functioning TIPS the mean peak velocity (PV) on the portal side was 37 m/s (range 22-65 cm/s), in the area of the incoming intrahepatic portal branch the mean PV was 59 cm/s (range 40-95 cm/s) and at the side of the incoming hepatic vein the mean PV was 135 cm/s (range 88-220 cm/s). In the punctured hepatic and portal veins the mean PV was 25 cm/s (range 15-30 cm/s) and 18 cm/s (10-22 cm/s), respectively. The flow increase from the portal to the mid part (P less than or = 0.001) and to the venous side was statistically significant (P less than or = 0.001). CONCLUSION: A velocity gradient between the portal and the venous side of a TIPS is a normal finding caused by branches of the portal and hepatic vein joining the TIPS from the side and it is characteristic of a normally functioning TIPS.

Adult↗

[Results of doppler sonography in normally functioning transjugular portosystemic shunts].

PURPOSE: The objective of this study was to evaluate blood flow changes inside normally functioning transjugular intrahepatic portosystemic shunts (TIPS), using Colour Doppler ultrasound (CDUS) and pulsed Doppler ultrasound (PDUS). MATERIAL AND METHODS: In a prospective study 72 patients (mean age 54, range 36-78 years) underwent TIPS placement, portal angiography, CDUS and PDUS examinations. Along the TIPS Doppler measurements were taken at the portal in the central part and at the venous end of the TIPS. RESULTS: In well functioning TIPS the mean peak velocity (PV) on the portal side was 39 cm/s (range 22-72 cm/s), in the area of the incoming right portal end, branch 62 cm/s (range 40-109 cm/s) and at the site of the incoming hepatic vein 139 cm/s (range 88-220 cm/s). In the punctured portal and hepatic vein the mean PV was 25 cm/s (range 15-30 cm/s) and 18 cm/s (15-22 cm/s) respectively. CONCLUSION: A velocity gradient between the portal and the venous side of the TIPS is a normal finding, caused by the branches of the portal and hepatic vein, joining the TIPS from the side and is characteristic of a normally functioning TIPS.

Adult↗

The reactivity of the antistriational antibodies associated with thymoma and myasthenia gravis.

Using immunofluorescence forty-nine sera giving striational staining of skeletal and cardiac muscle have been tested on glycerinated myofibrils. By comparing immunofluorescence and phase contrast it has been found that these antibodies are rather heterogeneous but at least three different staining patterns can be identified. Some patterns may be associated with the presence of a thymoma or may be induced by penicillamine treatment of patients with rheumatoid arthritis. Routine screening for antistriational antibodies may lead to the recognition of undiagnosed myasthenia gravis and thymoma. Antistriational autoantibodies may be useful in the further study of the structure and function of the sarcomere.

Antibodies↗

Preschool peer perceptions of the behavior of hyperactive and aggressive children.

To assess if preschool children can successfully identify externalizing symptomatic behaviors in their male classmates, and if these perceptions are associated with peer-rated popularity and rejection, 154 preschool boys and girls were interviewed using a peer nomination procedure. Behavioral data on the same preschool boys (N = 86) were also provided by their respective teachers. Preschool children were capable of providing stable nominations of popularity, rejection, and aggression, boys and girls significantly agreed in their nominations, and these nominations were not a function of the age of the rated child, although they differed somewhat as a function of the age of the rater. Teachers and peers reflected significant convergence in ratings of hyperactivity and aggression and teacher ratings of peer problems significantly agreed with actual peer nominations of popularity and rejection. Boys nominated as aggressive were more rejected by their classmates, whereas boys nominated as hyperactive were either more popular and/or more rejected. Limited evidence for differential patterns of relationships among hyperactivity, aggression, and peer status was obtained for both the peer and teacher data.

Aggression↗

Young children's play qualities in same-, other-, and mixed-sex peer groups.

This research examined how children's play varied as a function of the sex of the child, the sex of the play partner, and whether they played in dyads or groups. Children (mean age = 52 months) were observed during their free play. Data revealed that boys' same-sex play was more active-forceful, further from adults, and more stereotyped than girls' play, and that this pattern was generally exaggerated in groups versus dyads. Children rarely played only with children of the other sex (other-sex peers), but about one fourth of their interactions involved children of both sexes (mixed-sex peers). Compared with same- or dyadic other-sex play, mixed-sex play was more likely to occur near adults, and was intermediate in active-forceful and stereotyped activity choices. Findings confirmed that children's experiences in peer groups vary depending on the sex of the child and sex of play partners.

Age Factors↗

Associations among family relationships, antisocial peers, and adolescents' externalizing behaviors: gender and family type differences.

This study investigated the relations among parenting, sibling relationship, peer group, and adolescent externalizing behaviors. With data obtained from a sample of 341 male and 313 female adolescents (M age = 14.4 years) and their parents and siblings from nonstepfamilies and stepfather families, cross-sectional analyses supported the hypothesis that the contributions of parental negativity, parental monitoring, and sibling negativity to adolescents' externalizing behaviors would operate directly and also indirectly through deviant peer associations. The findings of multigroup comparison analyses suggested that the relationships between family and peer correlates and adolescent externalizing behaviors vary as a function of family type and adolescent gender.

Adolescent↗

Teacher- versus peer-mediated instruction: an ecobehavioral analysis of achievement outcomes.

In three experiments, we compared the effects of instructional arrangements that varied in: teacher versus peer mediators, methods used, levels of student academic responding generated, and content taught and tested. Instructional arrangements (i.e., tasks, structure, teacher position, teacher behavior) and students' levels of academic responding were measured by an observation system which served as an index of the independent variables. Students' accuracy on weekly spelling, arithmetic, and vocabulary tests and pre- and post-standardized achievement tests (Experiments 2 and 3 only) were the dependent variables. Results indicated that the classwide peer tutoring, compared to the teacher's procedure, produced more student academic responding and higher weekly test scores, regardless of treatment order or subject matter content (Experiment 1). The four lowest performing students in each class, in particular, benefited from peer tutoring, often performing as well as the other students. These findings were replicated in Experiments 2 and 3 wherein content taught/tested was also manipulated. Standardized test score gains were higher in those areas in which peer tutoring was used longest. Issues related to the functional analysis of instruction and achievement gain are discussed.

Achievement↗

Bowel function, mental health, and psychosocial function in adolescents with Hirschsprung's disease.

Congenital intestinal malformations are uncommon and may pose lasting somatic difficulties. Patients with anorectal anomalies have a high frequency of persistent faecal dysfunction and psychosocial problems. This study examined whether adolescents with Hirschsprung's disease have more psychosocial problems than their healthy peers. Nineteen adolescents (mean age 15.7 years) with Hirschsprung's disease were assessed for bowel function, anorectal physiology, mental health, and psychosocial functioning by physical examinations, semistructured interview, and standardised questionnaires. The adolescents were compared with controls. The parents of 13 adolescents with Hirschsprung's disease were interviewed and completed questionnaires. Thirty two per cent of the adolescents with Hirschsprung's disease had significant impairment of continence, but no more psychopathology (16%) nor psychosocial dysfunction as a group than their healthy peers. Faecal incontinence was associated with poorer psychosocial functioning and parental criticism. The fact that a significant number of patients with Hirschsprung's disease have incontinence into adulthood indicates the need for parental counselling, encouraging realistic expectations about continence.

Adolescent↗

Individual risk and social risk as interacting determinants of victimization in the peer group.

This study evaluated the hypothesis that the behavior problems that place children at risk for victimization by peers are associated with victimization primarily when children are also at social risk for victimization. Social risk was defined as lacking supportive friends or as being rejected by the peer group. Participants were 229 boys and girls in the 3rd through 7th grades (M age = 11 years 2 months). As predicted, behavior problems (internalizing problems, externalizing problems, and physical weakness) were more strongly related to victimization when children had few friends, had friends who were incapable of fulfilling a protective function (e.g., were physically weak), or were rejected by peers than when children had more friends, had friends capable of defending them, or were better liked by peers. Results illustrate the principle that individual risk variables depend on social context for expression.

Child↗

Creating and being created: the changing panorama of paediatric rehabilitation.

Paediatric rehabilitation as a discipline is rapidly changing, especially during the last decades. In the past, paediatric rehabilitation was characterized by merely adult intervention strategies in a miniaturized form, delivered by a merely adult patients-oriented profession. Theories on childhood development, however, changed, as did the focus of interventions: from impairments to function, from the child itself to family, community and peers. The call for outcome-oriented and evidence-based medicine lastly, changed paediatric rehabilitation into a mature paediatric profession with it's own scientific framework. This is reflected among other things in the increasing number of paediatric measures and instruments specifically geared to the paediatric rehabilitation profession, for example the Gross Motor Function Measure, Paediatric Evaluation of Disability Inventory and Movement ABC. More recently, paediatric exercise physiologists are pointing to the benefits of an active lifestyle and training for patients with chronic diseases and disabilities. Several studies have evaluated the effects of such training programmes and came up with positive results. It shows that paediatric rehabilitation continues to develop as a dynamic profession, having growth, childhood development and childhood activities as it's core business.

Child↗

Relationship between social perception and peer status in children with learning disabilities.

This study was undertaken to examine the social perceptual skill deficit theory in explaining the low peer acceptance of children with learning disabilities. The quality of tests measuring social perception was also examined. Thirty 9- to 12-year-old children with learning disabilities and a matched control group were given two measures of social perception: a laboratory task and a behavior rating scale. The behavior rating scale was completed by the children's teachers. In addition, the Peer Acceptance Scale (Bruininks, Rynders, & Gross, 1974) was administered to assess peer status. Results showed that the children with learning disabilities differed significantly from their nondisabled peers on each of the three measures-the children with learning disabilities obtained lower social perception and peer acceptance scores. However, the relationships between sociometric status and social perception varied as a function of task. A small but significant correlation wa found between the behavior rating scale and peer status. The laboratory task was not correlated with either the behavior rating scale or peer status. Results are discussed in terms of the psychometric properties of laboratory versus naturalistic measures of social perception and the importance of establishing the external validity of social skill measures by correlating them with outcome measures such as peer status.

Child↗

Peer relationship problems in children with Tourette's disorder or diabetes mellitus.

Peer relationships, social skills, self-esteem, parental psychopathology, and family functioning of children with Tourette's disorder and a chronic disease control group of children with diabetes mellitus were compared. Children with Tourette's disorder had poorer peer relationships than their classmates and were more likely to have extreme scores reflecting increased risk for peer relationship problems than children with diabetes mellitus, but did not report self-esteem problems or social skills deficits. Measures of peer relationships were not related to severity or duration of tics. Children with Tourette's disorder and Attention Deficit Hyperactivity Disorder were at increased risk for poor peer relationships. The psychosocial problems of children with Tourette's disorder do not appear to be the generic result of having a chronic disease.

Adolescent↗