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Biomedical subjects

K Sarimski

Publications and source records attributed to K Sarimski.

At least 19 recordsLinked to original sources
PubMed2004

Communicative competence and behavioural phenotype in children with Smith-Magenis syndrome.

Smith-Magenis syndrome is characterized by a range of minor physical and facial abnormalities and is caused by a de novo deletion on chromosome 17. Most children function in the moderate to severe ranges of mental retardation. Results of a survey on adaptive skills, communicative competence and behavioural abnormalities in 20 children are reported. The findings suggest a strong desire to get in social contact and maintain conversations in spite of their limited cognitive processing. As a group, children with SMS are presenting with severe behavioural abnormalities, e.g. self-injury, extreme irritability, ritualistic behaviour. Behaviour problems are more severe than in other genetic syndrome groups as a comparison with Prader-Willi- and Fragile-X-syndrome children reveals. However, functional analysis suggests that it is not independent from situational variables. There is a strong need for behavioural intervention planning as part of family services.

Adaptation, Psychological↗
PubMed2003

Early play behaviour in children with 5p- (Cri-du-Chat) syndrome.

BACKGROUND: 5p- (Cri-du-Chat) syndrome (5p-S) is a well defined chromosomal condition. While the physical symptoms have frequently been documented, the developmental and behavioural aspects of the syndrome have not been explored adequately, especially in young children. METHOD: Mental level, and complexity and style of play were analysed in 10 children who were homogeneously selected with respect to their chronological age (range = 2-7 years). RESULTS: A high rate of distractability and a low level of object-directed behaviours were observed in the play sessions. The findings were compared to two comparison groups (subjects with Down's syndrome or Cornelia-de-Lange syndrome) matched for mental age in order to analyse the specificity of these behavioural features for young children with 5p-S. CONCLUSIONS: A low level of object-directed behaviours may be an early precursor of hyperactivity, distractability and stereotypy, which have been reported to be the characteristic features of the behavioural phenotype of older individuals with 5p-S.

Child↗
PubMed2001

Social adjustment of children with a severe craniofacial anomaly (Apert syndrome).

OBJECTIVE: Children with a severe craniofacial anomaly are at risk for emotional and behavioural problems. Do children with Apert syndrome present with a special psychological profile? METHOD: Parent reports (Child Behaviour Checklist) were obtained from 25 children with Apert syndrome. RESULTS: Fourteen children were characterized by clinically significant social problems, 10 by attentional problems, nine by social withdrawal. Total CBCL-scores were in the clinical range for eight children. CONCLUSION: Information on psychosocial state should be included in regular check-ups of children with a severe craniofacial anomaly in order to identify children who are in need of psychological interventions.

Acrocephalosyndactylia↗
PubMed2000

Developmental and behavioural phenotype in Noonan syndrome?

Developmental and behavioural phenotype in Noonan syndrome? Noonan syndrome is characteristic by facial dysmorphology, congenital heart defects, short stature, developmental retardation and severe early feeding disorders in many cases. Data from a postal survey on physical development, feeding difficulties, developmental problems and behavioural aspects in 26 children are reported. The findings suggest developmental and behavioural difficulties in 46 per cent, but do not support the notion of a behavioural phenotype specific to Noonan syndrome. Having to cope with early surgery, feeding, developmental and behavioural problems establishes a need for psychological counseling for families receiving a diagnosis of Noonan syndrome.

Child↗
PubMed1999

[Observations of play and verbal behavior of boys with fragile X syndrome in early childhood].

Reports on development and behaviour in boys with fragile-X syndrome support the idea of a characteristic behavioural phenotype in this special population. Preliminary results are presented for 10 boys with fragile-X syndrome in early childhood. Severe mental handicaps and communicative abnormalities are observed less frequently than was expected on the basis of results reported for school-age children or adults. Boys with fragile-X syndrome show goal-directed and cooperative play behaviours in a Montessori play session, but less persistence and organisation than children with normal development or a mental handicap of heterogeneous origin. Results confirmed these behavioural differences as characteristic aspects of a "behavioural phenotype" in children who already in early childhood have fragile-X syndrome.

Adult↗
PubMed1999

Early development of children with Williams syndrome.

Developmental observations in ten young children with Williams syndrome (1-6 years old) are presented from developmental tests, symbolic play sessions and play sessions with a special educator following the non-directive Montessori approach. There is a considerable individual variability in performance. Overall, the children are engaged in goal-directed activities for more than 35% of the time during play sessions. Overactivity and distractability seem to be more age-dependent and situation-specific than thought before. Developmental interventions may include play sessions following the Montessori approach.

Age Factors↗
PubMed1998

Children with Apert syndrome: behavioural problems and family stress.

Apert syndrome is characterized by severe craniosynostosis, midface hypoplasia, and symmetric syndactyly of the hands and sometimes of the feet. In this study, postal questionnaires were completed by parents of 41 children with Apert syndrome. Data from this preliminary survey revealed that the children functioned fairly well socially despite their disabilities and potential for psychological difficulties. Parents suffered from stress caused by problems over acceptance of the child's appearance, the child's behavioural problems, and parental low self-esteem. Ten mothers and eight fathers scored in the deviant range for parental stress, suggesting a need for psychological counselling.

Acrocephalosyndactylia↗
PubMed1998

[Contribution of the clinical psychologists to rehabilitation of children with craniofacial abnormalities].

This paper describes the role of the clinical psychologist collaborating in an interdisciplinary rehabilitation team for children with severe congenital craniofacial disorders. This includes: 1. Psychological counseling during the initial period of responding to the traumatic experience of the child's birth; 2. Preparing the child and parents for surgery and hospitalisation; 3. Developmental assessment and counseling in parenting and educational issues; 4. Prevention of social-emotional disorders and psychological support for the long-term adjustment to facial difference.

Adaptation, Psychological↗
PubMed1997

Behavioural phenotypes and family stress in three mental retardation syndromes.

The behavioural phenotype of 30 fragile-X, 35 Prader-Willi and 35 Williams-Beuren syndrome children was explored using a psychometric approach. Results confirmed some distinct behaviours as syndrome-specific, but revealed a high degree of within-syndrome variability and overlap between syndromes as well. Parental stress was high in each of the groups, but was mediated by maternal dissatisfaction with family relationships. A multimethod approach with detailed syndrome-specific observations is recommended for further research.

Child↗
PubMed1997

Communication, social-emotional development and parenting stress in Cornelia-de-Lange syndrome.

Cornelia-de-Lange syndrome is a dysmorphogenic disorder characterized by multiple congenital abnormalities, and in most cases, severe cognitive limitations. Parents of 27 children with Cornelia-de-Lange syndrome completed psychological questionnaires concerning communicative and social-emotional behaviours, and reported on their parenting stress. Only four children were able to use speech, but most of the older children expressed needs through non-verbal means. Feeding problems, over-activity, irritability and stereotyped behaviours were characteristic for the majority of the group. Self-injurious behaviours occur in more than 40%. Parenting stress is very high, especially in parents of older children.

Abnormalities, Multiple↗
PubMed1997

[Parent stress in complex craniofacial abnormalities].

The birth of a child with craniofacial disorders creates tremendous stress on the parents' coping resources. In a postal survey 41 mothers and 32 fathers of children with Apert syndrome report stress concerning acceptance problems and low self-confidence. Both, mothers and fathers express a comparable degree of emotional stress. The quality of family relationships reveals as a buffering factor in coping.

Acrocephalosyndactylia↗
PubMed1997

Cognitive functioning of young children with Apert's syndrome.

Apert's syndrome is characterized by severe craniosynostosis, midface hypoplasia, symmetric syndactyly of the hands and sometimes feet. Cognitive functioning was evaluated in 11 children between 2.5 and 12.3 years. Four children had a normal IQ, four children had an intellectual ability in the borderline range and three children were mentally retarded. There was a consistent relative deficit in short-term memory and arithmetics. Some recommendations for psychological monitoring are discussed.

Acrocephalosyndactylia↗
PubMed1996

Specific eating and sleeping problems in Prader-Willi and Williams-Beuren syndrome.

Eating and sleeping problems have a high prevalence in mental retardation in general, but are also discussed as characteristic in some genetically determined disorders. A comparative analysis of eating and sleeping behaviours in 28 Prader-Willi- and 32 Williams-Beuren syndrome children by psychometric instruments confirms excessive food-seeking behaviours in PWS and selective food refusal in WBS as specific problems. In both syndromes, however, there is considerable individual variability in these symptoms.

Behavior Therapy↗
PubMed1995

[Psychological aspects of Prader-Willi syndrome. Results of a parent survey].

Forty-six parents of children and adolescents with Prader-Willi syndrome reported on the social and emotional behavior of their children and on parenting stress. Standardized questionnaires were used, e.g. a German behavioral questionnaire for preschool children and the Child Behavior Checklist. The results showed a high frequency of problem behaviors, including stubbornness, temper tantrums, obsessions and underactivity. In comparison to age norms, 40% of the preschool children were socially immature and 20% had social adaptation problems. Among the school-age children 70% had social adaptation problems. More than 70% of the mothers reported a high level of parenting stress and are in need of psychological counseling.

Adaptation, Psychological↗
PubMed1993

[Focused counseling of mothers of former premature infants].

Pre-term birth of a baby means a period of considerable emotional stress for parents. Even after discharge they remain anxious about caring and future developmental problems. A concept for psychological counseling in the first weeks after home transition is presented which relates parental fantasies and anxiousness with early interactional problems. This approach aims at an enhancement of the intuitive parental behaviors for compensation of early risk.

Adaptation, Psychological↗
PubMed1993

[Overprotectiveness as a coping reaction in intensive physical therapy].

25 mothers of children with cerebral palsy who were doing daily intensive physical therapy (Vojta) completed questionnaires concerning their child's temperament, parental attitudes and how they coped with everyday problems. Their responses were compared to those given by mothers who were not under the stress of conducting therapy. The results revealed compensatory coping processes: Mothers doing therapy try to compensate for their child's stress by being overprotective in ambiguous everyday situations.

Adaptation, Psychological↗