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Biomedical subjects

R M Hodapp

Publications and source records attributed to R M Hodapp.

At least 19 recordsLinked to original sources
PubMed2005

Children with Prader-Willi syndrome vs. Williams syndrome: indirect effects on parents during a jigsaw puzzle task.

BACKGROUND: Genetic disorders predispose individuals to exhibit characteristic behaviours, which in turn elicit particular behaviours from others. In response to the strength of Prader-Willi syndrome (PWS) and weakness of Williams syndrome (WS) in visual-spatial tasks such as jigsaw puzzles, parents' behaviours can be affected by their child's level of puzzle ability or syndrome diagnosis. METHODS: Children were asked to complete two jigsaw puzzles (one with the experimenter and the other with the parent) for 5 min each. Frequencies of parental helping and reinforcement behaviours, along with ratings of parental directiveness, were examined as parents interacted with their children on a jigsaw puzzle task. Within each aetiological group, correlates of parental behaviours with child characteristics were also examined. RESULTS: Compared to parents of children with PWS, parents of children with WS engaged in a more directive style of interaction, and provided more help and reinforcement. Relative to parents of children with higher puzzle abilities (from both aetiologies), parents of children with lower abilities also showed the same pattern. Both the child's aetiology and puzzle abilities were important in predicting parents' directiveness and helping and reinforcement behaviours. Within the PWS group, parents' behaviours correlated negatively with the child's puzzle abilities and general cognitive functioning; no such relations occurred in the WS group. CONCLUSIONS: Parents' behaviours were affected by both the child's diagnosis and actual puzzle abilities, suggesting important implications for understanding and intervening with parents and children with different genetic syndromes.

Adolescent↗
PubMed2001

Strengthening behavioral research on genetic mental retardation syndromes.

In this article we examine the status of behavioral research on genetic mental retardation syndromes. Although surveys suggest increased interest in such research, the field continues to struggle with three methodological issues: (a) how to think about control or contrast groups, (b) the interplay of behavioral phenotypes with development and other factors relating to within-group variation, and (c) the efficacy of etiology-based interventions. For each issue, we discuss salient concerns and make suggestions for future work.

Humans↗
PubMed2001

Research in mental retardation: toward an etiologic approach.

Over the past two decades, mental retardation research converges on three general themes: co-occurring mental retardation and psychopathology; families of offspring with mental retardation; and the developmental approach regarding behavioral sequences and profiles. Intertwined with each theme is a mounting body of research on genetic mental retardation syndromes. We first review recent progress in each of the three domains--psychopathology, families, and development--based on studies of groups with heterogenous or nonspecific mental retardation. We then show how new findings from specific genetic syndromes take this knowledge even further, as well as aid in the search for genetic, physiological, and environmental mechanisms associated with certain behaviors. We end the review by briefly summarizing our reasons for promoting an etiological approach to future mental retardation research, as well as by discussing methodological and other challenges.

Adolescent↗
PubMed1999

No relationship between the size of the deletion and the level of developmental delay in cri-du-chat syndrome.

Molecular cytogenetic and developmental assessment was performed on 50 individuals with cri-du-chat syndrome. Fluorescent in situ hybridization analysis was used to confirm a terminal deletion karyotype and map more precisely the location of the deletion breakpoint. We identified terminal deletion breakpoints mapping from 5p15.2 to 5p13. Developmental assessment was performed using the Vineland Adaptive Behavior Scales test. Composite Vineland Scores ranged from 20-75. In general, the communication score was higher than the composite score. Comparison of the size of the deletion with the composite Vineland score, as well as the Vineland Communication score, demonstrated that there was no correlation between the size of the deletion and the level of developmental delay. These results demonstrate that patients with cri-du-chat syndrome show high variability in the level of developmental achievement.

Chromosome Deletion↗
PubMed1999

Craniofacial maturity and perceived personality in children with Down syndrome.

In this pair of studies, we examined whether the common perception of a positive Down syndrome personality is associated with a youthful craniofacial appearance, similar to Zebrowitz's (1997) "babyface." In Study 1, 43 observers rated photographs of age-matched children with Down syndrome, another mental retardation syndrome (5p- syndrome), and typically developing children. Those with Down syndrome were perceived as being more physically babyfaced and more likely to behave in an immature manner. We controlled for the effect of familiarity with Down syndrome in Study 2 by employing a within-etiology design in which 128 observers rated 12 pictures of 10-year-old children with Down syndrome. Results showed that more physically babyfaced children with Down syndrome are more subject to the overgeneralization.

Adult↗
PubMed1998

Stress and coping in families of children with Smith-Magenis syndrome.

To describe stress and coping in families of children with Smith-Magenis syndrome, the present authors interviewed and received questionnaires from families of 36 children with this disorder. For measures of total stress, and of parent and family problems, the best predictors were the family's number of friends and the child's degree of impairment on the Vineland socialization domain; the single best predictor of parental pessimism was the child's degree of maladaptive behaviour. Although the stress levels of the families of children with Smith-Magenis syndrome are comparable to the levels shown by the families of children with Prader-Willi and 5p- syndromes, these levels are much higher than the stress levels reported by families of children with mixed or non-specific developmental disabilities. Stress levels may be similar across aetiologies involving high levels of maladaptive behaviour, but the correlates of family stress--particularly the moderating role of family friends--seem specific to Smith-Magenis syndrome.

Adaptation, Psychological↗
PubMed1997

Families of children with Prader-Willi syndrome: stress-support and relations to child characteristics.

This study examined stress-support in 42 families of 3 to 18-year-old children with Prader-Willi syndrome. Parents were asked about themselves and their families, their child with Prader-Willi syndrome, family supports, and family stress. Compared to reported stress levels in families of children with mixed etiologies of retardation, parents of children with Prader-Willi syndrome showed higher levels of parent and family problems, and comparable levels of pessimism. Parents of children with Prader-Willi syndrome listed other family members and friends as their main supporters; often such supporters lived outside of the respondent's town or city. Although the child's age, IQ, and degree of obesity were not related to familial stress, families experienced greater stress when the child showed more behavior problems overall, more externalizing and internalizing problems, and more problems on five of the nine narrow-band domains of Achenbach's Child Behavior Checklist.

Adaptation, Psychological↗
PubMed1997

Families of children with 5p- (cri du chat) syndrome: familial stress and sibling reactions.

This research examined family stress and sibling reactions in families of children with 5p- (cri du chat) syndrome aged 1 to 18 years who were living at home. In Study 1, 99 parents reported on themselves and their child with 5p-, as well as on family demographics, social supports, and stress. The best predictor of familial stress was the child's amount of maladaptive behavior, accounting for 12 to 38% of the variance across different stress measures. In Study 2, sibling concerns were examined in 44 unaffected siblings. The major finding was that parents and siblings disagreed on the extent of the siblings' interpersonal concerns. Parents reported that siblings felt ignored and misunderstood, whereas siblings themselves rated these concerns at much lower levels.

Adolescent↗
PubMed1997

Direct and indirect behavioral effects of different genetic disorders of mental retardation.

Direct and indirect behavioral effects of genetic disorders of mental retardation were identified. Three models of direct effects were examined: the no-specific effect model (all genetic disorders lead to identical behavioral outcomes), the totally specific model (each genetic disorder leads to unique outcomes), and the partially specific model (two or more genetic disorders lead to outcomes not shared by others with mental retardation). Although several cases of totally specific outcomes have been identified, partially specific effects most often occur. Persons in the surrounding environment are indirectly affected by behavioral propensities of different genetic disorders. Direct and indirect effects are also related to biological and developmental theory.

Behavior↗
PubMed1995

Mental and chronological age as predictors of age-appropriate leisure activity in children with mental retardation.

Effects of MA and CA on everyday leisure-time behaviors of children with mild and moderate mental retardation were examined by comparing 40 children with mental retardation to groups of 40 MA- and 40 CA-matched children without mental retardation on an adult-reported scale. Child's overall score, highest single behavior performed by the child, and highest behavior not performed because it was considered below the child's level were measured. Children with retardation performed most similarly to their MA-matches and well-below levels of CA-matches. Older children received higher scores in all groups. Late adolescents with retardation performed between MA- and CA-levels on the highest behavior considered too immature. Although MA best predicted overall functioning on leisure-time behavior of children with retardation, CA best predicted both single highest behavior performed and highest behavior considered too immature. Implications for teaching leisure-time behaviors to individuals with mental retardation were discussed.

Activities of Daily Living↗
PubMed1994

Mental retardation's two cultures of behavioral research.

People with mental retardation are generally classified by either level of retardation (usually by psychologists and special educators) or etiology (usually by geneticists and psychiatric researchers). We have surveyed nine journals from 1985-1990 to identify use of these two distinct research approaches, or cultures. After providing brief vignettes to delineate the separateness of the two cultures, we documented the varied effects that genetic etiology may have on behavior. The problems and issues inherent in reconciling these two research cultures were also emphasized, including variability within etiological groups, etiologic specificity versus nonspecificity, and service delivery implications. Several points of reconciliation between the two research cultures were offered, with the goal being better integrated approaches to the study of behavior in mental retardation.

Activities of Daily Living↗
PubMed1994

Profiles and development of adaptive behavior in children with Down syndrome.

The profiles and developmental trajectories of adaptive behavior were cross-sectionally examined in 80 children with Down syndrome ages 1 to 11.5 years using the Vineland Adaptive Behavior Scales. Profile findings indicated a significant weakness in communication relative to daily living and socialization skills. Within communication itself, expressive language was significantly weaker than receptive skills, especially when children's overall communicative levels were above 24 months. One to 6-year-old children showed significant age-related gains in adaptive functioning, but older subjects showed no relation between age and adaptive behavior. There was, however, increased variability within this older group, implying that not all children plateau in adaptive development during the middle childhood years. Implications for development in Down syndrome and intervention programs were discussed.

Activities of Daily Living↗
PubMed1993

Trajectory of adaptive behavior in males with fragile X syndrome.

Adaptive behavior in males with fragile X syndrome was longitudinally examined in 17 subjects, ages 1 to 17. Subjects received adaptive behavior evaluations on two occasions within one of three age periods. All domains of the Vineland Adaptive Behavior Scales increased from youngest to oldest age groups, yet older subjects (ages 10 to 17) showed significant declines in their adaptive behavior scores from first to second testing. A relative strength in Daily Living Skills and weakness in Socialization emerged only among older subjects. There was a significant relationship between adaptive behavior and mental age scores in all subjects. Discussion emphasized the parallels between declines in IQ and adaptive behavior as well as the need for further research on adaptive skills in young adults with fragile X syndrome.

Activities of Daily Living↗
PubMed1992

Profiles, correlates, and trajectories of intelligence in Prader-Willi syndrome.

Three aspects of intellectual functioning in persons with Prader-Willi syndrome were examined in two, related studies. In study 1, 21 subjects were evaluated with a psychometric instrument that assesses neuropsychological styles of cognitive processing, the Kaufman Assessment Battery for Children. Prader-Willi subjects showed deficits in sequential processing, and strengths in academic achievement tasks such as reading and vocabulary. In contrast to previous reports on the syndrome, no relationship was found between weight and degree of intellectual impairment. Study 2 included a cross-sectional examination of the trajectory of IQ in 21 subjects aged 13 to 46 years, as well as a longitudinal analysis of 31 subjects aged 5 to 30 years who were tested twice with the same IQ test. No evidence of the previously described decline in IQ over time was noted in either the cross-sectional or longitudinal analyses. The implications of these findings for interventions are discussed.

Achievement↗
PubMed1992

Adaptive and maladaptive behavior in Prader-Willi syndrome.

The development and profiles of adaptive and maladaptive behavior of 21 adolescents and adults with Prader-Willi syndrome were cross-sectionally examined with the Vineland Adaptive Behavior Scales and Achenbach's Child Behavior Checklist (CBCL). Adaptive strengths emerged for the group as a whole in daily living skills, and this strength became more pronounced with increasing age. A relative weakness was found in socialization, most notably in coping skills. CBCL findings indicated that externalizing behaviors were particularly heightened in adolescence and that many behaviors previously described as either emerging or worsening in adolescence also persist into the adult years (e.g., temper tantrums, arguing, irritability, stubbornness, lying, skin picking, obsessions, defiance). Certain elevated CBCL behaviors were unique to young versus old age groups, and aging in this syndrome may be associated with heightened confusion, withdrawal, and fatigue. The need to study adaptive and maladaptive features in a wider age range of subjects with Prader-Willi syndrome was emphasized.

Activities of Daily Living↗