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Turner syndrome (TS) is a rare chromosomal disorder characterized by partial or complete loss of one of the X chromosomes that affects about one in every 2000 female babies born. These young patients described difficulties making friends, understanding others' emotions and intentions, and controlling their own emotions. Difficulties in these domains could led to social withdrawal, to reduced social skills and could have a significant impact on self esteem and mental health as well as on long-term academic and social functioning in affected individuals. The purpose of this project is to identify functional and dysfunctional cognitive and socio-cognitive abilities in these young patients which could account social difficulties described by some of them and their family. To this end, 35 girls with TS and 35 girls with isolated growth hormone deficiency and normal cerebral MRI will be recruited. Subjects will be 7 to 16 years and 11 months of age. Socio-cognitive and cognitive functions will be assessed with neuropsychological and experimental tasks. Questionnaires completed by patient, parents or teacher, will evaluate social and behavioral functioning.
Cross-sectional study (2 groups : Turner syndrome, isolated growth hormone deficiency), not randomized, controlled, associating several specialized centers in endocrine diseases.
The pediatric endocrinologist of the participating centers will contact parents of each patient or voluntary child/adolescent in order to inform them of the study.
The visit of inclusion will take place in the participant center during a routine monitoring visit.
The first visit (V1) will take place immediately after the visit of inclusion in order to avoid a specific displacement for the needs for the study. The second visit (V2) will take place in participant's home. Each visit (V1 and V2) will last half-day. The period between V1 and V2 will be three months maximum.
The visits V1 and V2 will be carried out by neuropsychologists of the Laboratoire de Psychologie des Pays de la Loire (LPPL - EA 4638).
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Turner Syndrome |
|
| |
| isolated GHD |
|
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| psychological evaluation | Behavioral |
|
| Measure | Description | Time Frame |
|---|---|---|
| Wechsler Intelligence Scale for Children (WISC-V) | intelligence scale. | 3 months |
| Cognitive theory of mind first order | First order theory of mind tasks (Baron-Cohen et al., 1985) | 3 months |
| Cognitive theory of mind second order | second-order theory of mind tasks Perner & Wimmer, 1983) | 3 months |
| Cognitive theory of mind advanced | advanced theory of mind tasks (Happé, 1994) | 3 months |
| Cognitive theory of mind : humor | Humor understanding task | 3 months |
| Affective theory of mind : faux pas | Faux pas recognition test (Baron Cohen et al., 1999) | 3 months |
| Affective theory of mind : first order | First-order affective theory of mind tasks | 3 months |
| Affective theory of mind : second order | Second-order affective theory of mind tasks | 3 months |
| Affective theory of mind : emotional inference |
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INCLUSION CRITERIA
Turner syndrome group :
Isolated GHD group :
All participants :
EXCLUSION CRITERIA :
Turner syndrome group :
Isolated GHD group :
All participants :
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| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Régis Coutant, MD PhD | Contact | 33(02)41-35-56-55 | ReCoutant@chu-angers.fr | |
| Emmanuel Quemener, CRA | Contact | 33(02)41-35-45-81 | EmQuemener@chu-angers.fr |
| Name | Affiliation | Role |
|---|---|---|
| Régis Coutant, MD PhD | UH Angers | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| CHU Rennes | Recruiting | Rennes | Ile Et Vilaine | 35056 | France |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 25610415 | Background | LoBue V, Thrasher C. The Child Affective Facial Expression (CAFE) set: validity and reliability from untrained adults. Front Psychol. 2015 Jan 6;5:1532. doi: 10.3389/fpsyg.2014.01532. eCollection 2014. | |
| 25601846 | Background | Olszanowski M, Pochwatko G, Kuklinski K, Scibor-Rylski M, Lewinski P, Ohme RK. Warsaw set of emotional facial expression pictures: a validation study of facial display photographs. Front Psychol. 2015 Jan 5;5:1516. doi: 10.3389/fpsyg.2014.01516. eCollection 2014. |
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| ID | Term |
|---|---|
| D014424 | Turner Syndrome |
| D004393 | Dwarfism, Pituitary |
| ID | Term |
|---|---|
| D006059 | Gonadal Dysgenesis |
| D012734 | Disorders of Sex Development |
| D014564 | Urogenital Abnormalities |
| D052776 | Female Urogenital Diseases |
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Emotional inference for context test |
| 3 months |
| Emotional valence recognition : valence | Emotional valence rating from visual scenes | 3 months |
| Facial emotion recogniton | Facial emotion recognition task | 3 months |
| Vocal emotion recognition | Emotional prosody recognition task | 3 months |
| Emotional regulation | Emotion Regulation Questionnaire for Children and Adolescents (ERQ-CA) | 3 months |
| Alexithymia | Questionnaire d'alexithymie chez l'enfant (QAE) | 3 months |
| Empathy | Interpersonal Reactivity Index (IRI) | 3 months |
| Oral Language comprehension abilities 1 | Comprehension of Instructions (NEPSY-II, Korkman et al., 2012) | 3 months |
| Oral Language comprehension abilities 2 | Verbal comprehension index (from WISC-V) | 3 months |
| Visual and visual-spatial abilities:1 | Arrows subtest (NEPSY-II - Korkman et al., 2012), | 3 months |
| Visual and visual-spatial abilities:2 | Picture Puzzles subtest (NEPSY-II - Korkman et al., 2012) | 3 months |
| Visual and visual-spatial abilities:3 | Benton Facial Recognition Test (Benton et al., 1983) | 3 months |
| Executive abilities-Verbal Working memory 1 | Verbal information updating task | 3 months |
| Executive abilities-Visuo-Spatial Working memory 2 | Visuo-spatial information updating task | 3 months |
| Executive abilities-Inhibition 1 | Stroop test. | 3 months |
| Executive abilities-Inhibition 2 | Tapping test | 3 months |
| Executive abilities-Inhibition 3 | non-verbal cancellation task | 3 months |
| Executive abilities-Cognitive Flexibility 1 | Verbal fluency task (NEPSY-II Korkman et al., 2012) | 3 months |
| Executive abilities-Cognitive Flexibility 2 | Modified Wisconsin Card Sorting Test for children | 3 months |
| Executive abilities-Cognitive Flexibility 3 | Trail Making Test | 3 months |
| Executive abilities-Higher-order executive functions 1 | script generation task | 3 months |
| Executive abilities-Higher-order executive functions 2 | Rey Complex Figure Test : Copy | 3 months |
| Executive abilities in daily life | Behavioral Rating Inventory of Executive Functions | 3 months |
| Self Esteem | Rosenberg self-esteem scale (1965). | 3 months |
| Depression inventory | Depression: Children's Depression Inventory (Kovacks & Beck, 1977). | 3 months |
| Anxiety inventory | Revised Children's Manifest Anxiety Scale (Reynolds & Richmond, 1999). | 3 months |
| Social desirability | Children's Social Desirability Scale (Crandall et al., 1965) | 3 months |
| social adjustment 1 | Conners' Parent Rating Scale-Revised: Short Form (Conners, 1997) | 3 months |
| social adjustment 2 | Conners' Teacher Rating Scale-Revised: Short Form (Conners, 1997) | 3 months |
| behavioral adjustment | Child Behavior Checklist (Achenbach, 1991). | 3 months |
| CHU Nantes | Recruiting | Nantes | Loire Atlantique | 44300 | France |
|
| CHU Angers | Recruiting | Angers | Maine Et Loire | 49933 | France |
|
| 8040158 | Background | Happe FG. An advanced test of theory of mind: understanding of story characters' thoughts and feelings by able autistic, mentally handicapped, and normal children and adults. J Autism Dev Disord. 1994 Apr;24(2):129-54. doi: 10.1007/BF02172093. |
| 2934210 | Background | Baron-Cohen S, Leslie AM, Frith U. Does the autistic child have a "theory of mind"? Cognition. 1985 Oct;21(1):37-46. doi: 10.1016/0010-0277(85)90022-8. No abstract available. |
| 6681741 | Background | Wimmer H, Perner J. Beliefs about beliefs: representation and constraining function of wrong beliefs in young children's understanding of deception. Cognition. 1983 Jan;13(1):103-28. doi: 10.1016/0010-0277(83)90004-5. No abstract available. |
| 22023559 | Background | Gullone E, Taffe J. The Emotion Regulation Questionnaire for Children and Adolescents (ERQ-CA): a psychometric evaluation. Psychol Assess. 2012 Jun;24(2):409-17. doi: 10.1037/a0025777. Epub 2011 Oct 24. |
| D005261 | Female Urogenital Diseases and Pregnancy Complications |
| D000091642 | Urogenital Diseases |
| D058533 | Sex Chromosome Disorders of Sex Development |
| D052801 | Male Urogenital Diseases |
| D006330 | Heart Defects, Congenital |
| D018376 | Cardiovascular Abnormalities |
| D002318 | Cardiovascular Diseases |
| D006331 | Heart Diseases |
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D025064 | Sex Chromosome Disorders |
| D025063 | Chromosome Disorders |
| D030342 | Genetic Diseases, Inborn |
| D006058 | Gonadal Disorders |
| D004700 | Endocrine System Diseases |
| D004392 | Dwarfism |
| D001848 | Bone Diseases, Developmental |
| D001847 | Bone Diseases |
| D009140 | Musculoskeletal Diseases |
| D001849 | Bone Diseases, Endocrine |
| D007018 | Hypopituitarism |
| D010900 | Pituitary Diseases |
| D007027 | Hypothalamic Diseases |
| D001927 | Brain Diseases |
| D002493 | Central Nervous System Diseases |
| D009422 | Nervous System Diseases |