BIOMAT Database Logo BIOMAT Database Logo

Family contexts, parental behaviour, and personality profiles of children and adolescents with Prader-Willi, fragile-X, or Williams syndrome. 1998

C F van Lieshout, and R E De Meyer, and L M Curfs, and J P Fryns
University of Nijmegen, The Netherlands. VANLIESHOUT@PSYCH.KUN.NL

The personality profiles for youths with Prader-Willi, fragile-X, or Williams syndrome were compared to three matched groups attending regular schools. Using the California Child Q-Set (CCQ), both of the parents of the 39 children with Prader-Willi syndrome, 32 boys with fragile-X syndrome, 28 children with Williams syndrome, and children in the comparison groups provided independent personality descriptions in terms of the Big Five personality factors of Extraversion, Agreeableness, Conscientiousness, Emotional Stability, and Openness, along with Motor Activity and Irritability. Specific personality phenotypes for each of the three syndrome groups were found to be differentially related to parental behaviours (i.e. control and anger) and family contexts (i.e. experienced family stress, marital conflict, and parental consistency).

UI MeSH Term Description Entries
D008297 Male Males
D010554 Personality Disorders A major deviation from normal patterns of behavior. Avoidant Personality Disorder,Impulse-Ridden Personality,Inadequate Personality,Avoidant Personality Disorders,Impulse Ridden Personality,Personality Disorder,Personality Disorder, Avoidant,Personality Disorders, Avoidant,Personality, Impulse-Ridden,Personality, Inadequate
D010555 Personality Inventory Check list, usually to be filled out by a person about himself, consisting of many statements about personal characteristics which the subject checks. Edwards Personal Preference Schedule,Myers-Briggs Type Indicator,Indicator, Myers-Briggs Type,Inventories, Personality,Inventory, Personality,Myers Briggs Type Indicator,Personality Inventories
D010641 Phenotype The outward appearance of the individual. It is the product of interactions between genes, and between the GENOTYPE and the environment. Phenotypes
D011218 Prader-Willi Syndrome An autosomal dominant disorder caused by deletion of the proximal long arm of the paternal chromosome 15 (15q11-q13) or by inheritance of both of the pair of chromosomes 15 from the mother (UNIPARENTAL DISOMY) which are imprinted (GENETIC IMPRINTING) and hence silenced. Clinical manifestations include MENTAL RETARDATION; MUSCULAR HYPOTONIA; HYPERPHAGIA; OBESITY; short stature; HYPOGONADISM; STRABISMUS; and HYPERSOMNOLENCE. (Menkes, Textbook of Child Neurology, 5th ed, p229) Labhart-Willi Syndrome,Royer Syndrome,Labhart-Willi-Prader-Fanconi Syndrome,Prader Labhart Willi Syndrome,Prader-Labhart-Willi Syndrome,Royer's Syndrome,Willi-Prader Syndrome,Labhart Willi Prader Fanconi Syndrome,Labhart Willi Syndrome,Prader Willi Syndrome,Royers Syndrome,Syndrome, Labhart-Willi,Syndrome, Labhart-Willi-Prader-Fanconi,Syndrome, Prader-Labhart-Willi,Syndrome, Prader-Willi,Syndrome, Royer,Syndrome, Royer's,Syndrome, Willi-Prader,Willi Prader Syndrome
D011779 Q-Sort A personality assessment technique in which the subject or observer indicates the degree to which a standardized set of descriptive statements actually describes the subject. The term reflects "sorting" procedures occasionally used with this technique. Q Sort,Q-Sorts
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002653 Child Behavior Disorders Disturbances considered to be pathological based on age and stage appropriateness, e.g., conduct disturbances and anaclitic depression. This concept does not include psychoneuroses, psychoses, or personality disorders with fixed patterns. Childhood Behavior Disorders,Behavior Disorder, Child,Behavior Disorder, Childhood,Child Behavior Disorder,Childhood Behavior Disorder,Disorder, Childhood Behavior
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D005195 Family Relations Behavioral, psychological, and social relations among various members of the nuclear family and the extended family. Family Relationship,Family Dynamics,Family Relationships,Dynamic, Family,Dynamics, Family,Family Dynamic,Family Relation,Relation, Family,Relations, Family,Relationship, Family,Relationships, Family

Related Publications

C F van Lieshout, and R E De Meyer, and L M Curfs, and J P Fryns
The Profiles and Correlates of Psychopathology in Adolescents and Adults with Williams, Fragile X and Prader-Willi Syndromes.
March 2020, Journal of autism and developmental disorders,
C F van Lieshout, and R E De Meyer, and L M Curfs, and J P Fryns
The Prader-Willi phenotype of fragile X syndrome.
April 2007, Journal of developmental and behavioral pediatrics : JDBP,
C F van Lieshout, and R E De Meyer, and L M Curfs, and J P Fryns
Brief Report: Repetitive Behaviour Profiles in Williams syndrome: Cross Syndrome Comparisons with Prader-Willi and Down syndromes.
January 2018, Journal of autism and developmental disorders,
C F van Lieshout, and R E De Meyer, and L M Curfs, and J P Fryns
The developmental trajectory of disruptive behavior in Down syndrome, fragile X syndrome, Prader-Willi syndrome and Williams syndrome.
June 2015, American journal of medical genetics. Part C, Seminars in medical genetics,
C F van Lieshout, and R E De Meyer, and L M Curfs, and J P Fryns
Prader-Willi-like phenotype in fragile X syndrome.
April 1994, Clinical genetics,
C F van Lieshout, and R E De Meyer, and L M Curfs, and J P Fryns
Stress, locus of control, and family cohesion and adaptability in parents of children with Down, Williams, Fragile X, and Prader-Willi syndromes.
May 2012, American journal on intellectual and developmental disabilities,
C F van Lieshout, and R E De Meyer, and L M Curfs, and J P Fryns
Pragmatic Profiles of Adults with Fragile X Syndrome and Williams Syndrome.
March 2022, Brain sciences,
C F van Lieshout, and R E De Meyer, and L M Curfs, and J P Fryns
The neurobiology of the Prader-Willi phenotype of fragile X syndrome.
November 2016, Intractable & rare diseases research,
C F van Lieshout, and R E De Meyer, and L M Curfs, and J P Fryns
French database of children and adolescents with Prader-Willi syndrome.
October 2008, BMC medical genetics,
C F van Lieshout, and R E De Meyer, and L M Curfs, and J P Fryns
FMR1 premutation with Prader-Willi phenotype and fragile X-associated tremor/ataxia syndrome.
May 2017, Clinical case reports,
Copied contents to your clipboard!