Biomedical subjects

D L Fehlings

Publications and source records attributed to D L Fehlings.

4 recordsLinked to original sources

PubMed2001

Deflazacort treatment of Duchenne muscular dystrophy.

OBJECTIVE: We report the long-term effects on muscle strength and side effects with deflazacort in Duchenne muscular dystrophy (DMD). STUDY DESIGN: Boys with DMD between the ages of 7 and 15 years were reviewed retrospectively; 30 had been treated with deflazacort, and 24 had not. Muscle function, pulmonary function, and side effects were compared. RESULTS: The boys not treated with deflazacort stopped walking at 9.8 +/-1.8 years. Seven of 30 treated boys had stopped walking at 12.3+/-2.7 years (P<.05), and of the 23 boys who were still walking, 21 were older than 10 years. Pulmonary function (percent predicted functional vital capacity) was significantly greater in treated boys at 15 years (88% +/- 18%) than in boys not treated (39%+/-20%) (P<.001). Between 9 and 15 years, treated boys were shorter. Between 9 and 13 years, treated boys weighed less. After 13 years the treated boys maintained their weight, whereas boys not treated lost weight. Asymptomatic cataracts developed in 10 of 30 boys who received deflazacort. Other potential side effects of deflazacort such as hypertension, glucosuria, acne, infection, or bruising were not more common. CONCLUSIONS: We conclude that deflazacort can preserve gross motor and pulmonary function in boys with DMD with limited side effects.

Activities of Daily Living↗

PubMed2000

Quality of life in spina bifida: importance of parental hope.

BACKGROUND AND AIMS: Prognosis in spina bifida (SB) is often based only on neurological deficits present at birth. We hypothesised that both parental hope and the neurophysical examination predict quality of life in children and adolescents with SB. METHODS: A previously validated disease and age specific health related quality of life (HRQL) instrument was posted to families of children (aged 5-12 years) and adolescents (aged 13-20 years) with SB. We measured parental hope, determined the child's current physical function, and obtained retrospective data on the neonatal neurophysical examination (NPE). Regression analysis modelled HRQL firstly as a dependent variable on parental hope and NPE ("birth status"); and secondly on parental hope and current physical function ("current function"). RESULTS: Response rates were 71% (137 of 194) for families of children, and 54% (74 of 138) for families of adolescents. NPE data were available for 121 children and 60 adolescents. In children, the birth status model predicted 26% of the variability (R(2) hope 21%) compared with 23% of the variability (R(2) hope 23%)in the adolescents. The current function model explained 47% of the variability (R(2) hope 19%) in children compared with 31% of the variability (R(2) hope 24%) in the adolescents. CONCLUSIONS: In both age groups, parental hope was more strongly associated with the HRQL than neonatal or current physical deficits. A prospective study is required to determine whether a causal relation exists between parental hope and HRQL of children and adolescents with SB.

Adolescent↗

PubMed1997

Development of a health-related quality of life instrument for use in children with spina bifida.

The objective of this study was to develop a spina bifida health-related quality of life (HRQOL) instrument. Items were generated through semistructured interviews, and reduced by frequency-importance product ranking. Validity was assessed by correlating the HRQOL score with a global question concerning the child's well-being using the Spearman's rank coefficient, and the Piers-Harris Children's Self-Concept Scale (P-H) using the Pearson correlation coefficient. Reproducibility was assessed at 2-week intervals using the intra-class correlation coefficient (ICC). Field testing was undertaken in a larger sample to evaluate item-total correlation, internal consistency and construct validity. Patients taking part in the study were 329 children and adolescents with spina bifida attending two treatment centres. Over 600 items were generated. These were reduced to 47 questions and 50 questions, for children and adolescents respectively. The correlation between the HRQOL score and the global question was r = 0.57, and with the P-H was 0.26 (children). These values for adolescents were 0.63, and 0.89, respectively. Reproducibility was ICC = 0.78 (children) and 0.96 (adolescents). Following field testing, the questionnaire was further reduced to 44 questions (children) and 47 questions (adolescents) by eliminating questions with an item- total correlation less than 0.20. Cronbach's alphas for the final instrument were 0.93 (children) and 0.94 (adolescents), and construct validity correlations were 0.63 (children) and 0.37 (adolescents). The spina bifida HRQOL instrument has good measurement properties and may be used as a discriminative instrument. Assessment of responsiveness is necessary before using it to evaluate therapy in clinical trials.

Adolescent↗

PubMed1991

Attention deficit hyperactivity disorder: does cognitive behavioral therapy improve home behavior?

This study evaluates the effectiveness of cognitive behavioral therapy (CBT) in improving the home behavior of children with attention deficit hyperactivity disorder (ADHD). Twenty-five boys (age 7 to 13) with a diagnosis of ADHD were randomized to a CBT or supportive therapy control group. Outcome measures included parent and teacher ratings of the child on the Behavior Problem Checklist-Attention Problem Subscale (BPC-AP), and the Self-Control Rating Scale (SCRS), parent ratings on the Modified Werry Weiss Activity Scale, and child ratings on the Piers Harris Self-Concept Scale and Matching Familiar Figures Task. Data were analyzed using a two-way analysis of variance for main effects. A significant improvement favoring CBT was found on the Werry Weiss Scale, which measures the parent's perception of the child's hyperactivity in the home, and the child's rating of his/her self-esteem on the Piers Harris Self-Concept Scale. Other outcome measures did not demonstrate statistical differences. This research provides support for the use of CBT in children with ADHD. CBT was found to improve the parent's perception of the child's hyperactivity in the home as well as the child's self-esteem.

Adolescent↗