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A Description of Adaptive and Maladaptive Behaviour in Children and Adolescents with Cri-du-Chat Syndrome

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Title: A Description of Adaptive and Maladaptive Behaviour in Children and Adolescents with Cri-du-Chat Syndrome
Language: English
Authors: Teixeira, M. C. T. V., Emerich, D. R., Orsati, F. T.
Source: Journal of Intellectual Disability Research. Feb 2011 55(2):132-137.
Availability: Wiley-Blackwell. 350 Main Street, Malden, MA 02148. Tel: 800-835-6770; Tel: 781-388-8598; Fax: 781-388-8232; e-mail: cs-journals@wiley.com; Web site: http://www.wiley.com/WileyCDA/
Peer Reviewed: Y
Physical Description: PDF
Page Count: 6
Publication Date: 2011
Document Type: Journal Articles
Reports - Research
Descriptors: Severe Mental Retardation, Adolescents, Genetic Disorders, Behavior Problems, Adjustment (to Environment), Coping, Observation, Intervention
DOI: 10.1111/j.1365-2788.2010.01377.x
ISSN: 0964-2633
Abstract: Background: Psychological tests can be useful to record adaptive and maladaptive behaviours of children with intellectual disability. The objective of this study was to describe the adaptive and maladaptive behaviour of children and adolescents with Cri-du-chat syndrome. Methods: The sample consisted of 10 children and adolescents with Cri-du-chat syndrome (mean chronological age = 11.3 years, mean mental age = 18 months). The developmental quotient was calculated through the Psychoeducational Profile-Revised. An observational protocol was used to record adaptive and maladaptive behaviours. Results: The number of maladaptive behaviours observed was different among participants. However, all of them had high rates of adaptive behaviours, such as rule-following. Conclusions: These results, though preliminary, justify that we continue to think about the need for psychoeducational interventions aimed at stimulating the repertoire of adaptive behaviours, in people with Cri-du-chat syndrome.
Abstractor: As Provided
Number of References: 22
Entry Date: 2011
Accession Number: EJ927738
Database: ERIC
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  Value: <anid>AN0057367136;eul01feb.11;2019Jun04.10:45;v2.2.500</anid> <title id="AN0057367136-1">A description of adaptive and maladaptive behaviour in children and adolescents with Cri-du-chat syndrome. </title> <p>Background  Psychological tests can be useful to record adaptive and maladaptive behaviours of children with intellectual disability. The objective of this study was to describe the adaptive and maladaptive behaviour of children and adolescents with Cri‐du‐chat syndrome. Methods  The sample consisted of 10 children and adolescents with Cri‐du‐chat syndrome (mean chronological age = 11.3 years, mean mental age = 18 months). The developmental quotient was calculated through the Psychoeducational Profile – Revised. An observational protocol was used to record adaptive and maladaptive behaviours. Results  The number of maladaptive behaviours observed was different among participants. However, all of them had high rates of adaptive behaviours, such as rule‐following. Conclusions  These results, though preliminary, justify that we continue to think about the need for psychoeducational interventions aimed at stimulating the repertoire of adaptive behaviours, in people with Cri‐du‐chat syndrome.</p> <p>Keywords: behaviour; child; Cri‐du‐chat syndrome; development; adolescent</p> <p>The Cri‐du‐chat syndrome (CCS) is a chromosomal disorder resulting from the deletion of the short arm of chromosome 5 ([<reflink idref="bib22" id="ref1">22</reflink>]). The incidence of this syndrome is of 1:15 000 to 1:50 000 live‐born infants ([<reflink idref="bib15" id="ref2">15</reflink>]; [<reflink idref="bib10" id="ref3">10</reflink>]).</p> <p>The main maladaptive behaviours presented by CCS patients are: self‐injurious behaviour, repetitive movements, obsessive attachment to objects, head banging, stereotyped movements, destructive and aggressive behaviour, tantrums, hypersensitivity to sensory stimuli, sleep problems, stubbornness and clumsiness ([<reflink idref="bib8" id="ref4">8</reflink>]; [<reflink idref="bib5" id="ref5">5</reflink>]; [<reflink idref="bib4" id="ref6">4</reflink>]).</p> <p>To assess behavioural problems in children with atypical development, two procedures are traditionally used: observational studies in natural or artificial environments ([<reflink idref="bib2" id="ref7">2</reflink>]) and the use of standardised tools with parents and carers ([<reflink idref="bib9" id="ref8">9</reflink>]; [<reflink idref="bib12" id="ref9">12</reflink>]). As for the assessment of global indicators of development, researchers have resorted preferably to normalised and standardised tests ([<reflink idref="bib3" id="ref10">3</reflink>]).</p> <p>To make any decision that involves clinical, social, educational or familiar criteria, it is imperative to assess development indicators of children with intellectual disability (ID, [<reflink idref="bib18" id="ref11">18</reflink>]; [<reflink idref="bib20" id="ref12">20</reflink>]). Development tests usually offer sufficiently varied stimuli for the verification of different classes of behaviour. The objective of the study was to describe the adaptive and maladaptive behaviour of children and adolescents with CCS. An observational protocol was used to record adaptive and maladaptive behaviours.</p> <hd id="AN0057367136-2">Methods</hd> <p></p> <hd id="AN0057367136-3">Participants</hd> <p>The sample consisted of a group of five boys and five girls (age range: 4–16 years, mean age = 11.3 years, mean mental age = 40.3 months, compatible with 18‐month‐old children) with clinical and cytogenetic‐molecular diagnosis of CCS. The sizes of deletions oscillated between 21 megabases and 40 megabases; the mean of genetic deletion covered a distance of 27.6 megabases. Participants were recruited from the records of the Center of Counseling and Research on Cri‐du‐chat in São Paulo (Núcleo de Aconselhamento e Pesquisa de Cri du Chat). They received medical care at the Genetic Unit of Child Institute in Clinics Hospital of University of São Paulo (Unidade de Genética do Instituto da Criança do Hospital das Clínicas da Universidade de São Paulo). The families of two children declined to participate. The project was approved by the Ethics Committee for Researches with Human Beings of Mackenzie Presbyterian University. All participants lived with their parents, had independent mobility and did not use hearing aid or had hearing impairments. Only one child goes to a special school.</p> <hd id="AN0057367136-4">Instruments</hd> <p></p> <hd id="AN0057367136-5">Psychoeducational Profile – Revised</hd> <p>The Psychoeducational Profile – Revised (PEP‐R, [<reflink idref="bib19" id="ref13">19</reflink>]) consists of a series of toys, games, objects and pictures, which are offered to the child during structured play sessions. The adopted version included a scale of development with 131 items distributed into seven developmental areas: (<reflink idref="bib1" id="ref14">1</reflink>) imitation (16 items); (<reflink idref="bib2" id="ref15">2</reflink>) perception (13 items); (<reflink idref="bib3" id="ref16">3</reflink>), (<reflink idref="bib4" id="ref17">4</reflink>) fine and gross motor activity (16 and 18 items respectively); (<reflink idref="bib5" id="ref18">5</reflink>) eye–hand coordination (15 items); (<reflink idref="bib6" id="ref19">6</reflink>) cognitive performance (26 items); and (<reflink idref="bib7" id="ref20">7</reflink>) cognitive verbal operations (27 items). An overall developmental score for each of the development areas can be calculated by the sum of all correct items, which estimates age‐equivalent scores. Dividing the child's overall age‐equivalent score by his/her chronological age, an overall developmental quotient can be calculated.</p> <hd id="AN0057367136-6">Observation of adaptive and maladaptive behaviour</hd> <p>Table 1 shows adaptive and maladaptive behaviours observed. Maladaptive behaviours were defined according to the phenotype of CCS: repetitive movements, obsessive attachment to objects, repetitive sounds, hypersensitivity to sensory stimuli, aggressiveness, self‐injurious behaviour and disruptive behaviour ([<reflink idref="bib5" id="ref21">5</reflink>]; [<reflink idref="bib21" id="ref22">21</reflink>]; [<reflink idref="bib4" id="ref23">4</reflink>]). Adaptive behaviours were defined through those items of PEP‐R that required at least imitation and rule‐following.</p> <p>1 Operational definition of behavioural events observed</p> <p> <ephtml> <table><thead valign="bottom"><tr><th><bold>Behavioural pattern</bold></th><th><bold>Behaviour</bold></th><th><bold>Operational definition</bold></th><th><bold>Illustrative example</bold></th></tr></thead><tbody valign="top"><tr><td>Repetitive movements</td><td>Stereotyped behaviour with objects</td><td>Making repetitive movements with objects without a purpose</td><td>To beat objects, to twist objects</td></tr><tr><td>Body stereotyped behaviour (except self‐injury)</td><td>Repetitive movements with the whole body or a part of it without apparent purpose</td><td>To rotate the body, to shake the body, postural rigidity, to shake the head</td></tr><tr><td>Stereotyped gestures with hands (except self‐injury)</td><td>Repetitive movements with hands without apparent purpose</td><td>To shake the fingers, to crack the fingers, rigidity of hands, to polish or rub objects or parts of the body, to remove small particles from surfaces</td></tr><tr><td>Obsessive attachment to objects</td><td>Fixation with a particular object</td><td>Need to have a particular object around all the time</td><td>To carry a particular object (a game, a toy or any other object)</td></tr><tr><td>Repetitive sounds</td><td>Repetition of sounds</td><td>Repetition of particular phrases or sounds unrelated to the situation</td><td>Repeating words (telephone, bus) or sounds (trim‐trim, vrum‐vrum)</td></tr><tr><td>Hypersensitivity to sensory stimuli</td><td>Hypersensitivity to sensory stimuli</td><td>Behaviours that denote sensitivity to sounds coming from the environment</td><td>To cover the ears, to become agitated after hearing a sound not present before</td></tr><tr><td>Imitation</td><td>Imitation</td><td>To intentionally imitate common actions performed by another person</td><td>When someone acts in a certain way and the person imitates that action</td></tr><tr><td>Aggressiveness</td><td>Aggressiveness addressed to other people</td><td>Offensive action or deliberate attack addressed to another person</td><td>To beat other people, to bite, scratch or pinch others</td></tr><tr><td>Aggressiveness addressed to objects</td><td>Offensive action or deliberate attack addressed to objects</td><td>To throw objects, to beat objects</td></tr><tr><td>Self‐injury behaviour</td><td>Self‐injury behaviour</td><td>Behaviour causing demonstrable damages to one's own body</td><td>To beat the head with the hand or other part of the body, to bite oneself, to beat the head with or against objects, to pull one's own hair, to pinch the skin, to prod wounds</td></tr><tr><td>Disruptive behaviour</td><td>'Off‐task' behaviour</td><td>To abandon a task during its execution. The person seems not to listen to others when they talk to him/her and is easily distracted by foreign‐to‐task stimuli</td><td>To abandon task, to look at other places and not focus on the task, to refuse to accomplish the task, to be distracted by other stimuli (noise or objects)</td></tr><tr><td>Rule‐following</td><td>Rule‐following</td><td>Follow rules/obey instructions and/or rules</td><td>To obey and perform an action according to the given instruction: 'make a play‐dough ball'</td></tr></tbody></table> </ephtml> </p> <p>For this study, the PEP‐R was chosen because it is a global instrument to assess development, with most items not dependant upon language skills. The items are not time‐limited, and the materials are concrete and appealing to children with severe IDs ([<reflink idref="bib19" id="ref24">19</reflink>]).</p> <hd id="AN0057367136-7">Procedure</hd> <p>Each PEP‐R session took approximately 90 min and was recorded on tape in order to register behavioural events that were analysed afterwards. A camcorder (Sony DCR SR85) was used to video‐record the sessions. Every tape was watched three consecutive times for the identification of the time interval in which the items of the test allowed the registration of a significant number of adaptive and maladaptive behaviours. The duration of responses were defined in 10 s monitored with the use of a Timex chronometer (TI5G811N). The software EthoLog 2.2 ([<reflink idref="bib16" id="ref25">16</reflink>]) was used to record the frequency of each behaviour. The observations were performed by two trained observers. The percentage of concordance between observers was of 83% and it was calculated through the formula: percentage of concordance =  number of concordances/number of concordances + number of discordances × 100.</p> <hd id="AN0057367136-8">Results</hd> <p>There is a clear discrepancy between mean developmental age and mean chronological age in months (difference = 117.6 months). More severe disabilities were identified in the fields of: imitation (development compatible with 18.6‐month‐old children), fine motor activity (compatible with 17.8‐month‐old children), eye–hand coordination (compatible with 19.1‐month‐old children) and cognitive verbal operations (compatible with 17.6‐month‐old children). A better level of development was found in the areas of: perception (development compatible with 20.3‐month‐old children) and gross motor activity (compatible with 23.7‐month‐old children).</p> <p>As shown above, the mean development of the sample in the scales of imitation, perception and fine motor activity was very low, except for three participants (<reflink idref="bib2" id="ref26">2</reflink>, 5 and 6, see Table 2) that were successful in more than half of the items comprised in these scales. Some examples are the tasks of: manipulating a kaleidoscope and a puppet, ringing a bell, imitating animal sounds, touching the nose, waving goodbye – imitation; following and reaching objects visually, organising geometric objects according to size, orienting through sounds, responding to gestures – perception; unscrewing a lid, holding a stick, using the hands cooperatively, switching the light – fine motor activity.</p> <p>2 Mean and standard deviations of responses for each observed behaviour class (n = 10)</p> <p> <ephtml> <table><thead valign="bottom"><tr><th><bold>Behavioural pattern</bold></th><th><bold>Participant</bold></th><th><bold>Mean</bold></th><th><bold>Standard deviation</bold></th></tr><tr><th><bold>1</bold></th><th><bold>2</bold></th><th><bold>3</bold></th><th><bold>4</bold></th><th><bold>5</bold></th><th><bold>6</bold></th><th><bold>7</bold></th><th><bold>8</bold></th><th><bold>9</bold></th><th><bold>10</bold></th></tr></thead><tbody valign="top"><tr><td>Repetitive movements</td><td>0</td><td>0</td><td>0</td><td>42</td><td>0</td><td>0</td><td>8</td><td>121</td><td>37</td><td>26</td><td>23.40</td><td>38.06</td></tr><tr><td>Obsessive attachment to objects</td><td>0</td><td>0</td><td>0</td><td>19</td><td>0</td><td>0</td><td>0</td><td>12</td><td>0</td><td>0</td><td>3.10</td><td>6.74</td></tr><tr><td>Repetitive sounds</td><td>0</td><td>0</td><td>0</td><td>10</td><td>13</td><td>5</td><td>8</td><td>2</td><td>3</td><td>1</td><td>4.20</td><td>4.66</td></tr><tr><td>Hypersensitivity to sensory stimuli</td><td>0</td><td>0</td><td>0</td><td>0</td><td>0</td><td>0</td><td>0</td><td>0</td><td>0</td><td>0</td><td>0.00</td><td>0.00</td></tr><tr><td>Imitation</td><td>2</td><td>5</td><td>0</td><td>0</td><td>7</td><td>9</td><td>0</td><td>0</td><td>0</td><td>2</td><td>2.50</td><td>3.34</td></tr><tr><td>Aggressiveness</td><td>0</td><td>0</td><td>0</td><td>0</td><td>0</td><td>0</td><td>21</td><td>34</td><td>18</td><td>3</td><td>7.60</td><td>12.26</td></tr><tr><td>Self‐injurious behaviour</td><td>0</td><td>0</td><td>0</td><td>0</td><td>0</td><td>0</td><td>1</td><td>6</td><td>20</td><td>3</td><td>3.00</td><td>6.29</td></tr><tr><td>Disruptive behaviour</td><td>5</td><td>2</td><td>4</td><td>5</td><td>0</td><td>1</td><td>5</td><td>17</td><td>6</td><td>4</td><td>4.90</td><td>4.68</td></tr><tr><td>Rule‐following</td><td>27</td><td>27</td><td>29</td><td>18</td><td>37</td><td>33</td><td>12</td><td>18</td><td>18</td><td>21</td><td>24.00</td><td>7.85</td></tr></tbody></table> </ephtml> </p> <p>As shown in Table 2, the number of maladaptive behaviours observed was different among participants. Participants 8, 9 and 10 displayed the highest frequencies of repetitive movements, aggressiveness, self‐injurious behaviour and disruptive behaviour. However, all of them had high rates of adaptive behaviours, such as rule‐following (mean frequency = 24.0, compared to the 20‐min record). Participants had 12 to 37 rule‐following responses out of 120 possible responses, according to the demands of PEP‐R with duration of 10 s (1200 s of observation).</p> <hd id="AN0057367136-9">Discussion</hd> <p>The developmental retardation observed in this group was compatible with prior studies ([<reflink idref="bib6" id="ref27">6</reflink>]; [<reflink idref="bib18" id="ref28">18</reflink>]; [<reflink idref="bib11" id="ref29">11</reflink>]). Although the present study has focused on only 20 min of observation, the frequency of maladaptive responses, such as obsessive attachment to objects, self‐injurious behaviour and hypersensitivity to sensory stimuli, was low when compared to research to date ([<reflink idref="bib5" id="ref30">5</reflink>]; [<reflink idref="bib7" id="ref31">7</reflink>]; [<reflink idref="bib4" id="ref32">4</reflink>]; [<reflink idref="bib14" id="ref33">14</reflink>]). One possible limitation of this study was the small size of the sample. Typical problems of CCS patients, such as aggressive responses and disruptive behaviours, were displayed fundamentally by the oldest participants (Subjects 7, 8 and 9, who were 16, 13 and 16 years old respectively, mean age = 13 years old), as it can be seen on Table 2. This result does not match the results of [<reflink idref="bib4" id="ref34">4</reflink>]), who found statistically significant negative correlations between age and number of aggressive behaviours.</p> <p>There is a significant number of studies that focus on maladaptive behaviours in patients with severe ID, such as CCS or other genetic syndrome ([<reflink idref="bib4" id="ref35">4</reflink>]; [<reflink idref="bib17" id="ref36">17</reflink>]; [<reflink idref="bib1" id="ref37">1</reflink>]; [<reflink idref="bib13" id="ref38">13</reflink>]). The present study has a methodological limitation because of the fact that the age range (4–16 years old) comprised children and adolescents and also that the observation was performed during the undertaking of a test. On the other hand, the methodological design adopted has been proved to be a feasible alternative for the identification of adaptive behaviours which are usually difficult to register ([<reflink idref="bib18" id="ref39">18</reflink>]). The demands of the test must have contributed to the arousal of rule‐following responses. These results, though preliminary, justify that we continue to think about the need for psychoeducational interventions and parent training aimed at stimulating the repertoire of adaptive behaviours, even in people with severe ID. Rule‐following behaviour might be one of the target responses of such interventions.</p> <ref id="AN0057367136-10"> <title> Footnotes </title> <blist> <bibl id="bib1" idref="ref14" type="bt">1</bibl> <bibtext> Conflict of interest: No conflict of interest has been declared.</bibtext> </blist> <blist> <bibl id="bib2" idref="ref7" type="bt">2</bibl> <bibtext> Source of funding: CNPq, Capes and Mackpesquisa.</bibtext> </blist> </ref> <ref id="AN0057367136-11"> <title> References </title> <blist> <bibtext> Allen D. (2008) The relationship between challenging behaviour and mental ill‐health in people with intellectual disabilities: a review of current theories and evidence. Journal of Intellectual Disabilities 12, 267 – 94.</bibtext> </blist> <blist> <bibtext> Batista C. G. (2010) Observação do comportamento. In: Instrumentação psicológica. Fundamentos e Práticas (ed. L. Pasquali), pp. 273 – 307. Artmed, Porto Alegre.</bibtext> </blist> <blist> <bibl id="bib3" idref="ref10" type="bt">3</bibl> <bibtext> Borkowski J. G., Carothers S. S., Howard K., Schatz J. & Farris J. R. (2007) Intellectual assessment and intellectual disability. In: Handbook of Intellectual and Developmental Disabilities (eds J. W. Jacobson, J. A. Mulick & J. Rojahn), pp. 261 – 77. Springer Science, New York.</bibtext> </blist> <blist> <bibl id="bib4" idref="ref6" type="bt">4</bibl> <bibtext> Collins M. S. & Cornish K. (2002) A survey of the prevalence of stereotypy, self‐injury and aggression in children and young adults with Cri du Chat syndrome. Journal of Intellectual Disability Research 46, 133 – 40.</bibtext> </blist> <blist> <bibl id="bib5" idref="ref5" type="bt">5</bibl> <bibtext> Cornish K. M. & Pigram J. (1996) Developmental and behavioural characteristics of Cri du Chat syndrome. Archives of Disease in Childhood 75, 448 – 50.</bibtext> </blist> <blist> <bibl id="bib6" idref="ref19" type="bt">6</bibl> <bibtext> Cornish K. M., Bramble D., Munit F. & Pigram J. (1999) Cognitive functioning in children with typical Cri du Chat (5p‐) syndrome. Developmental Medicine and Child Neurology 41, 263 – 6.</bibtext> </blist> <blist> <bibl id="bib7" idref="ref20" type="bt">7</bibl> <bibtext> Dykens E. M. & Clarke D. J. (1997) Correlates of maladaptive behaviour in individuals with 5p‐ (Cri du Chat) syndrome. Developmental Medicine and Child Neurology 39, 752 – 6.</bibtext> </blist> <blist> <bibl id="bib8" idref="ref4" type="bt">8</bibl> <bibtext> Frodi A. & Senchak M. (1990) Verbal and behavioral responsiveness to the cries of atypical infants. Child Development 61, 76 – 84.</bibtext> </blist> <blist> <bibl id="bib9" idref="ref8" type="bt">9</bibl> <bibtext> Glascoe F. P. (2007) Developmental and behavioral screening. In: Handbook of Intellectual and Developmental Disabilities (eds J. W. Jacobson, J. A. Mulick & J. Rojahn), pp. 353 – 71. Springer Science, New York.</bibtext> </blist> <blist> <bibtext> Higurashi M., Oda M., Lijima K., Takeshita T., Watanabe N. & Yoneyama K. (1990) Livebirth prevalence and follow‐up of malformation syndromes in 27,472 newborns. Brain & Development 12, 770 – 73.</bibtext> </blist> <blist> <bibtext> Mainardi P. C. (2006) Cri du Chat syndrome. Orphanet Journal of Rare Diseases 33, 1 – 9.</bibtext> </blist> <blist> <bibtext> Martin P. & Pear J. (2009) Modificação do comportamento. O que fazer e como fazer. Roca, São Paulo.</bibtext> </blist> <blist> <bibtext> Mercadante M. T., Evans‐Lacko S. & Paula C. S. (2009) Perspectives of intellectual disability in Latin American countries: epidemiology, policy, and services for children and adults. Current Opinion in Psychiatry 22, 469 – 74.</bibtext> </blist> <blist> <bibtext> Moss J., Oliver C., Arron K., Burbidge C. & Berg K. (2009) The prevalence and phenomenology of repetitive behavior in genetic syndromes. Journal of Autism and Developmental Disorders 39, 572 – 88.</bibtext> </blist> <blist> <bibtext> Niebuhr E. (1978) The Cri du Chat syndrome: epidemiology, cytogenetics, and clinical features. Human Genetics 44, 227 – 75.</bibtext> </blist> <blist> <bibtext> Ottoni E. B. (2000) EthoLog 2.2: a tool for the transcription and timing of behavior observation sessions. Behavior Research Methods, Instruments, & Computers 32, 446 – 9.</bibtext> </blist> <blist> <bibtext> Ruggieri V. L. & Arberas C. L. (2003) Behavioural phenotypes. Biologically determined neuropsychological patterns. Revista de Neurologia 37, 239 – 53.</bibtext> </blist> <blist> <bibtext> Sarimski K. (2003) Early play behavior in children with 5p‐ (Cri‐du‐chat) syndrome. Journal of Intellectual Disability Research 47, 113 – 20.</bibtext> </blist> <blist> <bibtext> Schopler E., Reichler R. J., Rashlord A. & Marcos L. M. (1990) Perfil Psicoeducacional Revisado (PEP‐R). Pro‐ed, Austin, TX.</bibtext> </blist> <blist> <bibtext> Snell M. E., Brady N., McLean L., Ogletree B. T., Siegel E., Sylvester L. et al. (2010) Twenty years of communication intervention research with individuals who have severe intellectual and developmental disabilities. American Journal on Intellectual and Developmental Disabilities 115, 364 – 80.</bibtext> </blist> <blist> <bibtext> Van Buggenhout G. J., Pijkels E., Holvoet M., Schaap C., Hamel B. C. & Fryns J. P. (2000) Cri du chat syndrome: changing phenotype in older patients. American Journal of Medical Genetics 90, 203 – 15.</bibtext> </blist> <blist> <bibtext> Wu Q., Niebuhr E., Yang H. & Hansen L. (2005) Determination of the 'critical region' for cat‐like cry of Cri‐du‐chat syndrome and analysis of candidate genes by quantitative PCR. European Journal of Human Genetics 13, 475 – 85.</bibtext> </blist> </ref> <aug> <p>By M. C. T. V. Teixeira; D. R. Emerich; F. T. Orsati; R. C. Rimério; K. R. Gatto; I. O. Chappaz and C. A. Kim</p> <p>Reported by Author; Author; Author; Author; Author; Author; Author</p> </aug> <nolink nlid="nl1" bibid="bib22" firstref="ref1"></nolink> <nolink nlid="nl2" bibid="bib15" firstref="ref2"></nolink> <nolink nlid="nl3" bibid="bib10" firstref="ref3"></nolink> <nolink nlid="nl4" bibid="bib12" firstref="ref9"></nolink> <nolink nlid="nl5" bibid="bib18" firstref="ref11"></nolink> <nolink nlid="nl6" bibid="bib20" firstref="ref12"></nolink> <nolink nlid="nl7" bibid="bib19" firstref="ref13"></nolink> <nolink nlid="nl8" bibid="bib21" firstref="ref22"></nolink> <nolink nlid="nl9" bibid="bib16" firstref="ref25"></nolink> <nolink nlid="nl10" bibid="bib11" firstref="ref29"></nolink> <nolink nlid="nl11" bibid="bib14" firstref="ref33"></nolink> <nolink nlid="nl12" bibid="bib17" firstref="ref36"></nolink> <nolink nlid="nl13" bibid="bib13" firstref="ref38"></nolink>
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  Data: A Description of Adaptive and Maladaptive Behaviour in Children and Adolescents with Cri-du-Chat Syndrome
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  Data: <searchLink fieldCode="AR" term="%22Teixeira%2C+M%2E+C%2E+T%2E+V%2E%22">Teixeira, M. C. T. V.</searchLink><br /><searchLink fieldCode="AR" term="%22Emerich%2C+D%2E+R%2E%22">Emerich, D. R.</searchLink><br /><searchLink fieldCode="AR" term="%22Orsati%2C+F%2E+T%2E%22">Orsati, F. T.</searchLink>
– Name: TitleSource
  Label: Source
  Group: Src
  Data: <searchLink fieldCode="SO" term="%22Journal+of+Intellectual+Disability+Research%22"><i>Journal of Intellectual Disability Research</i></searchLink>. Feb 2011 55(2):132-137.
– Name: Avail
  Label: Availability
  Group: Avail
  Data: Wiley-Blackwell. 350 Main Street, Malden, MA 02148. Tel: 800-835-6770; Tel: 781-388-8598; Fax: 781-388-8232; e-mail: cs-journals@wiley.com; Web site: http://www.wiley.com/WileyCDA/
– Name: PeerReviewed
  Label: Peer Reviewed
  Group: SrcInfo
  Data: Y
– Name: PhysDesc
  Label: Physical Description
  Group: PhysDesc
  Data: PDF
– Name: Pages
  Label: Page Count
  Group: Src
  Data: 6
– Name: DatePubCY
  Label: Publication Date
  Group: Date
  Data: 2011
– Name: TypeDocument
  Label: Document Type
  Group: TypDoc
  Data: Journal Articles<br />Reports - Research
– Name: Subject
  Label: Descriptors
  Group: Su
  Data: <searchLink fieldCode="DE" term="%22Severe+Mental+Retardation%22">Severe Mental Retardation</searchLink><br /><searchLink fieldCode="DE" term="%22Adolescents%22">Adolescents</searchLink><br /><searchLink fieldCode="DE" term="%22Genetic+Disorders%22">Genetic Disorders</searchLink><br /><searchLink fieldCode="DE" term="%22Behavior+Problems%22">Behavior Problems</searchLink><br /><searchLink fieldCode="DE" term="%22Adjustment+%28to+Environment%29%22">Adjustment (to Environment)</searchLink><br /><searchLink fieldCode="DE" term="%22Coping%22">Coping</searchLink><br /><searchLink fieldCode="DE" term="%22Observation%22">Observation</searchLink><br /><searchLink fieldCode="DE" term="%22Intervention%22">Intervention</searchLink>
– Name: DOI
  Label: DOI
  Group: ID
  Data: 10.1111/j.1365-2788.2010.01377.x
– Name: ISSN
  Label: ISSN
  Group: ISSN
  Data: 0964-2633
– Name: Abstract
  Label: Abstract
  Group: Ab
  Data: Background: Psychological tests can be useful to record adaptive and maladaptive behaviours of children with intellectual disability. The objective of this study was to describe the adaptive and maladaptive behaviour of children and adolescents with Cri-du-chat syndrome. Methods: The sample consisted of 10 children and adolescents with Cri-du-chat syndrome (mean chronological age = 11.3 years, mean mental age = 18 months). The developmental quotient was calculated through the Psychoeducational Profile-Revised. An observational protocol was used to record adaptive and maladaptive behaviours. Results: The number of maladaptive behaviours observed was different among participants. However, all of them had high rates of adaptive behaviours, such as rule-following. Conclusions: These results, though preliminary, justify that we continue to think about the need for psychoeducational interventions aimed at stimulating the repertoire of adaptive behaviours, in people with Cri-du-chat syndrome.
– Name: AbstractInfo
  Label: Abstractor
  Group: Ab
  Data: As Provided
– Name: Ref
  Label: Number of References
  Group: RefInfo
  Data: 22
– Name: DateEntry
  Label: Entry Date
  Group: Date
  Data: 2011
– Name: AN
  Label: Accession Number
  Group: ID
  Data: EJ927738
PLink https://search.ebscohost.com/login.aspx?direct=true&site=eds-live&db=eric&AN=EJ927738
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      – Type: doi
        Value: 10.1111/j.1365-2788.2010.01377.x
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      – Text: English
    PhysicalDescription:
      Pagination:
        PageCount: 6
        StartPage: 132
    Subjects:
      – SubjectFull: Severe Mental Retardation
        Type: general
      – SubjectFull: Adolescents
        Type: general
      – SubjectFull: Genetic Disorders
        Type: general
      – SubjectFull: Behavior Problems
        Type: general
      – SubjectFull: Adjustment (to Environment)
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      – SubjectFull: Coping
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      – SubjectFull: Observation
        Type: general
      – SubjectFull: Intervention
        Type: general
    Titles:
      – TitleFull: A Description of Adaptive and Maladaptive Behaviour in Children and Adolescents with Cri-du-Chat Syndrome
        Type: main
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            NameFull: Teixeira, M. C. T. V.
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            NameFull: Emerich, D. R.
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            NameFull: Orsati, F. T.
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              Y: 2011
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            – TitleFull: Journal of Intellectual Disability Research
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