Validation of the Childhood Health Assessment Questionnaire in the juvenile idiopathic myopathies. Juvenile Dermatomyositis Disease Activity Collaborative Study Group.

OBJECTIVE: To examine the validity of the Childhood Health Assessment Questionnaire (CHAQ) in patients with juvenile idiopathic inflammatory myopathy (IIM). METHODS: One hundred fifteen patients were enrolled in a multicenter collaborative study, during which subjects were assessed twice, 7-9 months apart. Physical function was measured using the CHAQ. Internal reliability was assessed using adjusted item-total correlations and item endorsement rates. Construct validity was assessed by comparing predicted and actual correlations of the CHAQ with other measures of physical function and disease activity. Responsiveness was assessed by calculating effect size (ES) and standardized response mean (SRM) in a group of a priori defined "improvers." RESULTS: Item-total correlations were high (rs range = 0.35-0.81), suggesting all items were related to overall physical function. Manual muscle testing and the Childhood Myositis Assessment Scale correlated moderate to strongly with the CHAQ (r = -0.64 and -0.75, both p < 0.001). Moderate correlations were also seen with the physician global assessment of disease activity (rs = 0.58, p < 0.001), parent global assessment of overall health (rs = -0.65, p < 0.001), Steinbrocker function class (rs = 0.69, p < 0.001), and global skin activity (rs = 0.40, p < 0.001), while global disease damage and skin damage had low correlations (rs = 0.13 and 0.07, p > or =0.17). Responsiveness of the CHAQ was high, with ES = 1.05 and SRM = 1.20. CONCLUSION: In this large cohort of patients with juvenile IIM, the CHAQ exhibited internal reliability, construct validity, and strong responsiveness. We conclude that the CHAQ is a valid measure of physical function in juvenile IIM, appropriate for use in therapeutic trials, and potentially in the clinical care of these patients.

Transition to adulthood.

Over the past 20 years, survival of youth with special health care needs, including youth with rheumatic diseases, has markedly improved. Over 90% will survive past their twentieth birthday and health care providers are recognizing a need for transition services to assist youth with disabilities to become successful adults. The barriers to, and the principles of, transition services are outlined. A discussion of available models and assessment tools for transition readiness and lessons learned on how to provide successful transition services is included.

Stigmatized sources and persuasion: prejudice as a determinant of argument scrutiny.

Two experiments examined the viability of several explanations for why majority group individuals process persuasive messages from stigmatized sources more than those from nonstigmatized sources. In each study, majority group participants who either were high or low in prejudice or were high or low in ambivalence toward a stigmatized source's group were exposed to a persuasive communication attributed to a stigmatized (Black, Experiment 1; homosexual, Experiment 2) or nonstigmatized (White, Experiment 1; heterosexual, Experiment 2) source. In both studies, source stigmatization increased message scrutiny only among those who were low in prejudice toward the stigmatized group. This finding is most consistent with the view that people scrutinize messages from stigmatized sources in order to guard against possibly unfair reactions by themselves or others.

Juvenile idiopathic inflammatory myopathy: exercise-induced changes in muscle at short inversion time inversion-recovery MR imaging.

PURPOSE: To study the effect of exercise on short inversion time inversion-recovery (STIR) magnetic resonance (MR) images of thigh muscles in children with juvenile idiopathic inflammatory myopathy. MATERIALS AND METHODS: Thirty-two MR studies were performed in 19 patients with juvenile idiopathic inflammatory myopathy who performed stair-stepping exercise for up to 10 minutes (mean, 5.7 minutes). Baseline T1-weighted (n = 32) and STIR (n = 32) images and STIR images immediately (n = 32) and at 30 (n = 24) and 60 (n = 29) minutes after exercise were obtained at 0.5 T. Four radiologists graded STIR signal intensity changes, in observer performance experiments in which they were blinded to the order of image acquisition in relation to exercise. RESULTS: Changes in muscle signal intensity were observed on STIR images obtained immediately after exercise in 20 of 32 (63%) studies. The mean signal intensity score immediately after exercise (1.7 +/- 1.0 [SD]) increased compared with the mean baseline score (1.4 +/- 1.1) (P = .0005) and resolved by 30 minutes after exercise. The magnitude of exercise-induced changes correlated with the amount of work performed (r = 0.51, P = .003) but not with disease activity or baseline signal intensity when the changes were corrected for work (r < 0.17, P > .35). Radiologists demonstrated moderate to substantial agreement in the grading of signal intensity changes after exercise (kappa = 0.60-0.84). CONCLUSION: In patients with juvenile idiopathic inflammatory myopathy, stair-stepping exercise induces signal intensity changes on STIR MR studies of muscle for approximately 30 minutes after exercise, in a distribution that may mimic active muscle inflammation.

Psychosocial aspects of rheumatic disease in childhood and adolescence.

The increasing number of adolescents with disabilities such as systemic lupus erythematosus who survive into adulthood has prompted the medical community to study the psychological and physical functioning of adolescents after many years of chronic illness. The author uses a generic chronic illness model to augment observations from studies about young people with rheumatic disease.

Success on the road to adulthood. Issues and hurdles for adolescents with disabilities.

Outcome data from children with connective tissue disease or other disabilities and how they function in adulthood continue to underscore the need for transition planning. This article addresses the components of transition and the barriers to its accomplishment along with the special issues adolescents with disabilities face in attaining the developmental tasks along the road to adulthood. Health care professionals can be catalyst in the transition process in their role as a consultant to young persons with disabilities and their families. It can be rewarding and challenging to be a catalyst for a successful transition process that results in a happy, meaningful life as an adult with a disability.

Race of source effects in the elaboration likelihood model.

In a series of experiments, we investigated the effect of race of source on persuasive communications in the Elaboration Likelihood Model (R.E. Petty & J.T. Cacioppo, 1981, 1986). In Experiment 1, we found no evidence that White participants responded to a Black source as a simple negative cue. Experiment 2 suggested the possibility that exposure to a Black source led to low-involvement message processing. In Experiments 3 and 4, a distraction paradigm was used to test this possibility, and it was found that participants under low involvement were highly motivated to process a message presented by a Black source. In Experiment 5, we found that attitudes toward the source's ethnic group, rather than violations of expectancies, accounted for this processing effect. Taken together, the results of these experiments are consistent with S.L. Gaertner and J.F. Dovidio's (1986) theory of aversive racism, which suggests that Whites, because of a combination of egalitarian values and underlying negative racial attitudes, are very concerned about not appearing unfavorable toward Blacks, leading them to be highly motivated to process messages presented by a source from this group.

2,3,7,8-Tetrachlorodibenzo-p-dioxin reduces the number, size, and organelle content of Leydig cells in adult rat testes.

Exposure to 2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD) alters testicular steroidogenesis and reduces total Leydig cell volume in the testis. However, its effect on Leydig cell number, size, and organelle content had not been determined in adult rats. Adult male rats received a single intraperitoneal injection of TCDD at a dose of 0, 12.5, 25.0, or 50.0 micrograms/kg body weight. Testicular tissues were obtained from rats 4 weeks after treatment. Testes were vascularly perfused with glutaraldehyde, embedded in Epon 812, sectioned at 0.5 micron, stained with toluidine blue, and evaluated by stereology for number and size of Leydig cells. Specimens from control and high dose groups were prepared for electron microscopy to quantify Leydig cell organelle content. TCDD treatment reduced (P < 0.01) body weight in a dose-dependent fashion. Testicular weight was not significantly reduced by TCDD treatment. The volume of Leydig cell cytoplasm per testis was reduced (P < 0.01) four weeks after treatment. Reduction in total Leydig cell volume resulted from a reduced (P < 0.05) number of Leydig cells and a reduced (P < 0.01) size of individual Leydig cells. However, the volume density (percentage) of Leydig cells occupied by specific organelles was not influenced by TCDD treatment. As a result of reduced total Leydig cell volume with no change in volume density of organelles in Leydig cells, the volumes per testis of smooth endoplasmic reticulum and mitochondria were reduced (P < 0.01) by TCDD treatment. In conclusion, the TCDD-induced reduction in Leydig cell volume per testis is explained by reduced number and size of individual Leydig cells and resulted in a significant reduction in total volume of both Leydig cell smooth endoplasmic reticulum and mitochondria per testis. Reduction in content of organelles that are responsible for various key steps in steroidogenesis, could explain TCDD-reduced production of testosterone in rats.

Pediatric systemic lupus erythematosus and neonatal lupus.

The clinical manifestations of pediatric systemic lupus erythematosus (SLE) are similar to those seen in adults with SLE with increased frequency of the following features: hepatosplenomegaly, chorea, nephritis, and avascular necrosis. Similarly, pediatric SLE patients are now showing the same improvement in survival as adult SLE patients, and it is no longer felt that the course of childhood-onset SLE is more severe than that seen in adult-onset SLE. Children of mothers with SLE can develop both transient and persistent features of SLE in the neonatal period. Transient features include photosensitive discoid rash, cytopenia, hepatosplenomegaly, myocarditis, and pericarditis; the permanent features include congenital complete heart block, endomyocardial fibroelastosis, and other structural cardiac defects.

Transition/job readiness for adolescents with juvenile arthritis and other chronic illness.

A successful transition from school to the world of work is crucial for all adolescents but can be particularly difficult for adolescents with chronic illnesses and disabilities (CI/D). The need for prevocational services for this population is especially urgent as more children with CI/D are surviving, and in the USA, to obtain their needed health care coverage, employment is essential. Yet 50-75% of young disabled adults are jobless in the USA and only 21% of the more than 300,000 handicapped students leaving special education become employed. We outline the current barriers to prevocational readiness for the adolescents with CI/D and describe how 2 programs are working with solutions.

Use of a radioreceptor assay in the assessment of cushingoid features in patients with juvenile rheumatic diseases.

A marked variation has been observed in severity of cushingoid appearance in patients with rheumatic diseases (RD) following steroid administration. We studied ten children with RD to determine if a relationship exists between cushingoid features and an individual's steroid activity as measured by prednisolone equivalents using a radioreceptor assay. Cushingoid features were clinically assessed by a "cushing score" according to the method of Bergrem. Patients were assigned to either the cushingoid (C) or noncushingoid (NC) group at study entry according to their cushing score. Blood was drawn prior to prednisone ingestion and then at 30, 60, 90, 120, 240, and 360 minutes and each sample was assessed for prednisolone equivalents and also for free and total cortisol. Group comparisons of dose-adjusted area under curve (AUC) and peak response are reported. Cushingoid patients had higher plasma prednisolone equivalents (PE) than noncushingoid patients as measured by peak PE and AUC. The PE.6 h/L average AUC for C patients was 248 micrograms PE.6 h/L versus 134 micrograms PE.6 h/L for NC patients. This nearly twofold difference was also noted between mean peak values (C 82 micrograms/L vs. NC 44 micrograms/L). Spearman correlations of Cushing scores with these two parameters indicated significant (p less than 0.05) relationships. A patient's Cushing score correlated best with peak response (rs = 0.78) and also with AUC (rs = 0.72). Measurement of plasma peak PE or AUC could be valuable for individualizing steroid dosing in children with RD.

Ethnic group and the British census: the search for a question.

"The 1991 census contained, for the first time, a question on the ethnic group of each member of the population of Great Britain. This paper reports on how a question was developed which has a sufficiently high degree of acceptance from all the main ethnic groups, and which is answered sufficiently accurately, to justify inclusion in the census."

Parents of children with chronic health impairments: a new approach to advocacy training.

Consumer advisory coalitions recognize the effectiveness of parents as advocates in obtaining services for their children with special health care needs. As a result, advocacy training programs for parents of children with developmental disabilities have become popular. Parents of children with chronic health conditions, who are not traditionally served through special education, find that they have concerns and needs not addressed by these advocacy training programs. This paper describes a unique program that targets these parents. A distinctive feature of this program is its focus on helping parents develop competency in utilizing communications skills to deal effectively with education and medical systems.

Juvenile rheumatoid arthritis. Aquatic exercise and lower-extremity function.

This pilot study investigates the effects of aquatic therapeutic exercise on lower-extremity range of motion, gait, balance, and functional mobility in children with juvenile arthritis. Eleven patients, aged 4-13, with lower-extremity joint involvement, diagnosed as functional class I-III, completed a 6-week program of aquatic exercise aimed at increasing lower-extremity range of motion and strength. Despite the small sample size and short duration of the study program, significant improvement was noted in external and internal hip rotation, bilaterally (p < 0.05). Improvement was noted in the median scores for most other parameters; however, these did not reach statistical significance. Aquatic exercises performed in a group setting can serve as an enjoyable and beneficial part of therapy for children with arthritis. Further investigation is recommended to determine fully the effects of aquatic therapeutic exercise on mobility and fitness in children with juvenile arthritis.