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130 resources found.

  • Verbal and non-verbal requests in Spanish speaking children with Down syndrome
    Donna Jackson-Maldonado, Bertha Badillo and Nancy Aguilar
    Children with Down syndrome have been described to have language, social-communicative and intentional skills that lag behind age-matched peers, with frequent gesture use that is not accompanied by vocalisations or words. Early intentional communication, particularly requests, has been shown to be an important step in future language development. This is a preliminary study that explores the use of verbal and non-verbal requests in 4 Spanish-speaking children with Down syndrome from very low income families. Children were observed in an elicited request task and language level was evaluated using a parental report. Results showed that all participants were very delayed in their language production. Children used mostly combinations of non-verbal requests. Data support previous studies in that Spanish-speaking children also use non-verbal requests for a prolonged period of time as a strategy to compensate for their linguistic deficits. Findings differ from previous studies in that most combinations of gestures were complementary and not equivalent. Their language development was much more depressed than most data published to date. This lag could be explained because children were from very low income environments and they may lack stimulation because of family situations. It is suggested that data from a larger sample be collected and compared to typically developing children in order to make a stronger proposal about the use of non-verbal forms as a bridge to verbal productions.
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    Down Syndrome Research and Practice. 2010. doi:10.3104/reports.2102
  • Electropalatography in the assessment and treatment of speech difficulties in children with Down syndrome
    Sara Wood
    Many children with Down syndrome experience significant speech difficulties which in turn affects their speech intelligibility. This paper describes how electropalatography, a computer-based technique which uses visual feedback to alter speech production, is being used at Queen Margaret University, to assess and treat speech difficulties in a group of children and young people with Down syndrome. Encouraging results from a single case are reported.
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    Down Syndrome Research and Practice. 2010. doi:10.3104/reports.2093
  • Literacy environments for children with Down syndrome: What’s happening at home?
    Anne van Bysterveldt, Gail Gillon and Susan Foster-Cohen
    This descriptive study investigated the home literacy environment of New Zealand children with Down syndrome. Participants were 85 children with Down syndrome enrolled in predominantly mainstream school programmes in years 1-8, who were aged between 5;4 (y; m) and 14;11 (M = 8;11, SD = 2;6), comprising an estimated 15% of children with Down syndrome in New Zealand primary education[1]. Survey data via questionnaire (modelled on Boudreau[2]), was gathered from participant's parents and targeted three broad themes including parents’ priorities regarding literacy for their child with Down syndrome, ways in which the HLE of children with Down syndrome supports literacy development and the ways children with Down syndrome participate in literacy interactions. Results were analysed for all participants and by age group which are presented when group differences were apparent. Results indicated the majority of parents are involved in regular literacy interactions with their child, although more with reading than with writing. Many children played an active role in joint reading activities, interacting with both pictures and text, although more with pictures than with text. Children were reported to use a wide range of writing materials. Parents also reported other ways in which they facilitated literacy development including active teaching, language games and library visits. Clinical implications for parents and professionals working with children with Down syndrome are discussed with reference to relationships between HLE variables and positive literacy outcomes and provide support for the development of targeted interventions specifically aimed at facilitating literacy with this population.
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    Down Syndrome Research and Practice. 2010. doi:10.3104/reports.2111
  • A preliminary study of intervention addressing early developing requesting behaviours in young infants with Down syndrome
    Emily Jones, Kathleen Feeley and Catherine Blackburn
    Infants with Down syndrome display characteristic deficits in early communicative behaviours, including requesting. This deficit significantly and negatively impacts later communication and cognitive development. In this study, we explored intervention to address requesting in young infants with Down syndrome. Two infants with Down syndrome were taught increasingly sophisticated forms of early requesting skills (i.e., gaze shifting and gaze shifting paired with vocalisation). One of the infants was also taught a verbal approximation of the word "more". The application of interventions to address and prevent impairments characteristic of the behavioural phenotype demonstrated by infants and children with Down syndrome is discussed.
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    Down Syndrome Research and Practice. 2010. doi:10.3104/reports.2059
  • Low consumption of fruit and vegetables, and markers of oxidative stress in children with Down syndrome
    Seyyed Nachvak, Soltan Mahboob, Tirang Neyestani, Seyyed Keshawarz and John Speakman
    Background – Epidemiological, in vitro and in vivo evidence suggests that oxidative stress is elevated in children with Down syndrome. Other studies have indicated that consumption of diets with a high content of fruit and vegetables resulted in a significant reduction in markers of oxidative cellular damage to DNA and lipids in individuals without Down syndrome. Aim – We investigated the frequency of consumption of fruit and vegetables by children with Down syndrome and the influence of variations in the level of this consumption on biomarkers of oxidative stress. Methods – Frequency of consumption of fruit and vegetables by Iranian children with Down syndrome were recorded over 7 days by the children’s parents. Serum malondialdehyde (MDA) and urinary 8-Hydroxydeoxyguanosine (8-OHdG) were also measured. Results – Fruit consumed by children with Down syndrome was only 26% of the recommended daily servings (RDS) and vegetable consumption was only 4% of the RDS. Fruit and vegetable consumption was not related to socio-economic status. No significant relationships were found between consumption of fruit and vegetables with markers of oxidative damage. Conclusion – Consumption of fruit and vegetables as an essential part of healthy diet was very low in children with Down syndrome. This seemed unrelated to the cost and availability of these food items, but could be related to the difficulties patients with Down syndrome report chewing. Parents of children with Down syndrome should be encouraged to give more fruit and vegetables to their children and choose soft fruits and preparation methods that make them easier to consume.
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    Down Syndrome Research and Practice. 2010. doi:10.3104/reports.2146
  • Maternal teaching behaviour and pre-verbal development of children with Down syndrome and typically developing children
    Pnina Klein, Esther Adi-Japha and Varda Rosenthal
    The objective of the current study was to identify and compare maternal teaching behaviours in interactions with infants with Down syndrome and typically developing infants, in relation to their pre-verbal development. Teaching behaviours defined based on a mediational theory perspective, were videotaped and examined in interactions with 38 infants with Down syndrome and 38 typically developing infants, in relation to their Pre-verbal Communication development from 7-9 to 20-22 months. The gap in the development of Pre-verbal Communication between the two groups in favour of typically developing children became clearly apparent at 14-16 months and coincided with a gap in frequency of maternal teaching behaviours provided to each group. Mothers of children with Down syndrome used more ‘isolated’ Focusing which was negatively related to measures of Pre-verbal Communication and less Affecting and Expanding which were positively related to Pre-verbal Communication as compared with mothers of typically developing children. For children with Down syndrome, Regulation of behaviour and Affecting observed at 14-16 months were the best predictors of pre-verbal development at 20-22 months. For children with Down syndrome and typically developing children, sequences of parental behaviour including Focusing accompanied by Affecting or Expanding, observed at 14-16 months, predicted pre-verbal development and Bayley scores, at 20-22 months, whereas ‘isolated’ Focusing behaviour was a negative predictor of the above. These findings suggest the need to direct teaching behaviour in meaningful sequences rather than in isolation.
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    Down Syndrome Research and Practice (Online). 2010. doi:10.3104/reports.2105
  • Autoimmune hepatitis in Down syndrome
    Kim-Doan Nguyen, Scott Duong, Farrah Lazare, Rajeev Nagpal, J Decker Butzner, Maria Triantafyllopoulou, William Treem and Ian Leibowitz
    We sought to determine the clinical features of autoimmune hepatitis in children with Down syndrome. After an inquiry on the PEDS GI Board, a questionnaire was sent to interested colleagues. Seven patients with autoimmune hepatitis and Down syndrome were reported. The median age at diagnosis was 10 yrs, range 3-15 yrs. All seven were ANA+ and SMA+; none were anti-LKM1+. Initially, three were treated with corticosteroids alone, three with corticosteroids and azathioprine (AZA), and one with cyclosporine alone. Three are currently on AZA alone; two remain on corticosteroids. No patients underwent liver transplant. There were two deaths. We concluded that autoimmune hepatitis is another autoimmune disease to consider in the evaluation of children with Down syndrome.
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    Down Syndrome Research and Practice (Online). 2009. doi:10.3104/reports.2098
  • Production and perception of word stress in children and adolescents with Down syndrome
    Michèle Pettinato and Jo Verhoeven
    This article reviews the importance of word stress for typical language acquisition and presents evidence that in certain developmental language disorders, processing of word stress is disrupted. Two novel experiments were carried out testing the production and perception of word stress in a group of 16 children and adolescents with Down syndrome (ages 11-20) matched on receptive vocabulary level to 12 typically developing children (ages 4.06-7). The results indicated processing difficulties in both the production and perception of more difficult and later acquired stress patterns as well as weak initial syllables at the beginnings of words. The impact of these difficulties on language acquisition processes is discussed and future avenues for research are sketched.
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    Down Syndrome Research and Practice (Online). 2009. doi:10.3104/reports.2036
  • Phonetic intelligibility testing in adults with Down syndrome
    Kate Bunton, Mark Leddy and Jon Miller
    The purpose of the study was to document speech intelligibility deficits for a group of five adult males with Down syndrome, and use listener based error profiles to identify phonetic dimensions underlying reduced intelligibility. Phonetic error profiles were constructed for each speaker using the Kent, Weismer, Kent, and Rosenbek word intelligibility test[1]. The test was designed to allow for identification of reasons for the intelligibility deficit, quantitative analyses at varied levels, and sensitivity to potential speech deficits across populations. Listener generated profiles were calculated based on a multiple-choice task and a transcription task. The most disrupted phonetic features, across listening task, involved simplification of clusters in both the word initial and word final position, and contrasts involving tongue-posture, control, and timing (e.g., high-low vowel, front-back vowel, and place of articulation for stops and fricatives). Differences between speakers in the ranking of these phonetic features was found, however, the mean error proportion for the six most severely affected features correlated highly with the overall intelligibility score (0.88 based on multiple-choice task, 0.94 for the transcription task). The phonetic feature analyses are an index that may help clarify the suspected motor speech basis for the speech intelligibility deficits seen in adults with Down syndrome and may lead to improved speech management in these individuals.
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    Down Syndrome Research and Practice (Online). 2008. doi:10.3104/reports.2027
  • Parent/carer ratings of self-talk behaviour in children and adults with Down syndrome in Canada and the United Kingdom
    Paul Patti, Nancy Andiloro and Maureen Gavin
    Parents of children and adults with Down syndrome have observed many of them engage in self-talk or ‘private speech’. They may use self-talk to work out situations, express inner feelings, and entertain themselves. Some reports have stressed that this behaviour is not a delusion or hallucination. The Self-Talk Survey was developed to investigate the quality, style, and content of self-talk. Surveys from Canada and the United Kingdom were obtained on 97 males and 80 females with Down syndrome aged 5–60 years. Analysis revealed that self-talk was present in 91% of the survey sample and occurred less frequently when the person was alone. A person’s age and level of disability did not influence their ability to engage in self talk. Observed self-talk was generally understandable and displayed in a normal tone. A person’s self-talk was typically directed to themselves, but could also involve a real or imaginary person or a favourite toy/object. The content of self-talk focused on an event that recently occurred or was expected to occur, a television programme or movie, family or friends, completion of an activity, or various complaints. Self-talk also served as an outlet for planning or rehearsing an activity, working out a problem, or as a self-dialogue about something interesting or important.
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    Down Syndrome Research and Practice (Online). 2008. doi:10.3104/reports.2063
  • Cytogenetic profile of Down syndrome cases seen by a general genetics outpatient service in Brazil
    Joice Biselli, Eny Goloni-Bertollo, Mariângela Ruiz and Érika Pavarino-Bertelli
    Down syndrome or trisomy 21 can be caused by three types of chromosomal abnormalities: free trisomy 21, translocation or mosaicism. The cytogenetic diagnosis, made through karyotypic examination, is important mainly to determine recurrence risks to assist genetic counselling. The object of this work was to carry out a cytogenetic profile of confirmed cases of Down syndrome seen in the General Genetics Outpatient Service in a teaching hospital – Hospital de Base in São José do Rio Preto – from the implementation of the service in 1973 to November 2005, with the purpose of establishing the nature of the cytogenetic abnormalities of these patients. A retrospective study was performed, in which the karyotypes of patients with Down syndrome consulted at the General Genetics Outpatient Service of HB-FAMERP from 1973 to November 2005 were investigated. The results of cytogenetic analysis were obtained from the consultation register and patients’ hospital records. The results show 387 Down syndrome cases confirmed by karyotypic examinations. Of these, 357 (92.2%) patients had free trisomy of chromosome 21, 24 (6.2%) had translocation involving chromosome 21 and 6 (1.5%) had mosaicism. Nondisjunction was the main cause of Down syndrome, as the majority of the patients have free trisomy of chromosome 21. The cytogenetic pattern of Down syndrome is variable among different studies.
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    Down Syndrome Research and Practice (Online). 2008. doi:10.3104/reports.2010
  • Medical conditions and medication use in adults with Down syndrome: A descriptive analysis
    Gerard Kerins, Kimberly Petrovic, Mary Bruder and Cynthia Gruman
    Background: We examined the presence of medical conditions and medication use within a sample of adults with Down syndrome. Methods: Retrospective chart review using a sample of 141 adults with Down syndrome and age range of 30 to 65 years. Results: We identify 23 categories of commonly occurring medical conditions and 24 categories of medications used by adults with Down syndrome. Conclusion: Approximately 75 of older adults with Down syndrome in our sample experience memory loss and dementia. Hypothyroidism, seizures, and skin problems also occur commonly. The prevalence of cancer (i.e., solid tumours) and hypertension is extremely low. Older adults with Down syndrome use anticonvulsant more often than younger adults with Down syndrome. The use of multivitamins and medications such as pain relievers, prophylactic antibiotics, and topical ointments is common.
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    Down Syndrome Research and Practice (Online). 2007. doi:10.3104/reports.2009
  • Medical issues among children and teenagers with Down syndrome in Hong Kong
    Winnie Yam, Philomena Tse, Chak Yu, Chun Chow, Wai But, Kit Li, Lai Lee, Eva Fung, Pauline Mak and Joseph Lau
    We examined the prevalence of medical problems in children and teenagers with Down syndrome in Hong Kong. Methods: Children with Down syndrome receiving care from seven regional hospitals were included and their hospital records were reviewed. A total of 407 patients, aged between 0.06 and 17.16 years were included. Cardiovascular problems were observed in 216 (53%), endocrine problems in 111 (27%), gastrointestinal problems in 46 (11%), haematological problems in 18 (4%), neurological problems in 27 (7%), sleep problems in 36 (9%), skeletal problems in 56 (14%), visual problems in 195 (48%) and auditory problems in 137 (34%). Conclusions: The prevalence of medical problems was high in children and teenagers with Down syndrome in Hong Kong and similar to previous findings elsewhere. Future studies on the local prevalence of medical problems in the adult population with Down syndrome would help to define their medical needs.
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    Down Syndrome Research and Practice (Online). 2007. doi:10.3104/reports.2005
  • Prevalence of MTHFR C677T polymorphism in north Indian mothers having babies with Trisomy 21 Down syndrome
    Utkarsh Kohli, Sadhna Arora, Madhulika Kabra, Lakshmy Ramakrishnan, Sheffali Gulati and Ravindra Pandey
    Recent studies have evaluated possible links between polymorphisms in maternal folate metabolism genes and Down syndrome. Some of these studies show a significantly increased prevalence of the C677T polymorphism of the 5,10-methylene tetrahydrofolate reductase (NADPH) gene (MTHFR) among mothers who have had babies with Down syndrome. This study examined the prevalence of the MTHFR C677T polymorphism among 104 north Indian mothers of babies with Down syndrome and 109 control mothers. The prevalence of MTHFR C677T polymorphism observed among mothers of babies with Down syndrome was 28% compared to 35% in controls (C677T/T677T). There was no significant difference between the two groups (p = 0.294). Mean homocysteine level in mothers of children with Down syndrome was lower than the level in the controls. Our data suggests that the MTHFR C677T polymorphism is not associated with an increased risk of Down syndrome in the north Indian population. Homocysteine levels in our study were higher when compared to other studies. Methylcobolamin and folate deficiency or use of random samples for homocysteine determination could possibly account for this observation.
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    Down Syndrome Research and Practice (Online). 2007. doi:10.3104/reports.2004
  • Oral health condition and treatment needs of a group of Nigerian individuals with Down syndrome
    Folakemi Oredugba
    Objective: This study was carried out to determine the oral health condition and treatment needs of a group of individuals with Down syndrome in Nigeria. Method: Participants were examined for oral hygiene status, dental caries, malocclusion, hypoplasia, missing teeth, crowding and treatment needs. Findings were compared with controls across age group, sex and educational background of parents. Result: Participants with Down syndrome had poorer oral hygiene than controls, with no significant sex difference. Oral hygiene was similar in the lower age groups but deteriorated with age in the Down syndrome group. Conclusion: Individuals with Down syndrome in Nigeria have poorer oral health and more treatment needs than controls. They would benefit from frequent oral health assessment.
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    Down Syndrome Research and Practice. 2007. doi:10.3104/reports.2022
  • Down syndrome in the neurology clinic: Too much? Too little? Too late?
    Andrew Larner
    This paper presents a review of all patients with Down syndrome seen over a 5-year period by one consultant neurologist in general outpatient and specialist cognitive function clinics. It revealed only 7 cases in > 4500 general referrals (= 0.2%), all referred with suspected seizure disorders. The diagnosis of epilepsy was confirmed in 6 patients. Only one new, comorbid, diagnosis was made. Neurologists have little exposure to, and hence little chance to develop expertise in, the neurological complications of Down syndrome. It is suggested that closer liaison between neurologists and psychiatrists with an interest in learning disability might improve the management of neurological problems in patients with Down syndrome.
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    Down Syndrome Research and Practice. 2007. doi:10.3104/reports.2011
  • Prevalence of bruxism among Mexican children with Down syndrome
    Rubén López-Pérez, Patricia López-Morales, Borges-Yáñez, Gerardo Maupomé and Gustavo Parés-Vidrio
    This study sought to determine the prevalence of bruxism in a Mexican community of children with Down syndrome, and to evaluate bruxism’s relationship with age, sex, intellectual disability level, and type of chromosomal abnormality of trisomy 21. Using a cross-sectional design, 57 boys and girls (3 to 14 years old) were examined. Three approaches to establish presence or absence of bruxism were employed: parental questionnaire, clinical examination, and dental study casts. Data were analysed using bivariate analyses and conditional logistic regression. We found that the overall prevalence of bruxism was 42%. No statistically significant associations between bruxism and age, sex, or intellectual disability level were found. There was, however, a significant association between bruxism and type of chromosomal abnormality, with mosaicism being more frequently associated with bruxism.
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    Down Syndrome Research and Practice (Online). 2007. doi:10.3104/reports.1995
  • Parents’ perceptions of health and physical activity needs of children with Down syndrome
    Kristi Menear
    Individuals with Down syndrome typically have low fitness levels and obesity despite data that indicate physiological gains from physical activity and exercise interventions. Low fitness levels and obesity in individuals with Down syndrome may be related to sedentary lifestyles, social and recreational opportunities, or low motivation to be physically active. These causal influences on the overall health of individuals with Down syndrome may be related to parental or caregiver support. Through this study, parents of children with Down syndrome from preschool to adolescent ages were interviewed about their perceptions of the health and physical activity needs of their children. Data from four focus groups indicated the following most salient themes: (1) all parents believed participation in physical activity has immediate and long-term positive health impacts on their child with Down syndrome, and most of the parents thought their child would benefit from being more physically active, (2) most parents observed that their child participated in physical activities primarily for social reasons, most notably to be with their peers with or without Down syndrome or to be with their sibling(s), and that without such motivation their child would choose sedentary activities, (3) parents of teenagers identified a need for their child to learn an individual sport to have sporting opportunities that do not require ability-matched teammates and opponents, and (4) parents recognised their need for help from physical activity specialists through either parent education regarding home-based physical activity programmes or an increase in appropriate community-based physical activity programmes for their child with Down syndrome. The interview data suggest future research should evaluate the outcomes of long-term individualised home-based physical activity interventions for children with Down syndrome. Additionally, educators, recreation specialists, and therapists should assist children and youth with their acquisition of skills used in individual and dual sports.
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    Down Syndrome Research and Practice (Online). 2007. doi:10.3104/reports.1996
  • The impact of periodontal disease on the quality of life of individuals with Down syndrome
    Ana Loureiro, Fernando Costa and José da Costa
    Objective: This study aimed to determine the prevalence of periodontal disease among children and adolescents with Down syndrome and the possible repercussions of such pathology in the quality of life of the group in question. Method: The sample consists of 93 individuals with Down syndrome 6 - 20 years old, living in Brazil (Minas Gerais). Periodontal probing was carried out on every site of each tooth. The Plaque Index and periodontal clinical parameters were recorded. A broad interview was carried out with the mothers, consisted of an adaptation of the Oral Health Impact File OHIP-14 that was used to measure the negative repercussions of periodontal disease in the daily lives of these individuals. Results: The prevalence of gingivitis was 91%, whereas periodontitis was found in 33% of the individuals. When the impact of periodontal disease on the quality of life was correlated with the clinical periodontal parameters, it was observed that there are significant statistical differences among them: bleeding on probing, probing depth and attachment loss. These same results, correlated with all the different groups that are categorised according to the diagnosis of periodontal disease, also show significant differences. Conclusions: Periodontal disease can be considered as a condition with high prevalence within the group in question, which has negative effects on the quality of life of the subjects. These effects are aggravated by the seriousness of the disease.
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    Down Syndrome Research and Practice (Online). 2007. doi:10.3104/reports.1998
  • Metric analysis of the hard palate in children with Down syndrome - a comparative study
    Gopalan Bhagyalakshmi, Annappa Renukarya and Sayee Rajangam
    The hard palate is viewed as playing an important role in the passive articulation of speech. Its probable role in the defective articulation of speech in individuals with Down syndrome has been examined in the present study. In individuals with Down syndrome, the hard palate is highly arched, constricted, and narrow and stair type with malformed misaligned teeth and a large and fissured tongue. As a result good palato-lingual contact is not achieved, with resulting defective articulation. Using orthodontic and prosthodontic principles could modify this situation, i.e. the anatomy of the hard palate. The altered palatal contour may give better placing to the tongue, leading to improved palato-lingual contact and articulation. The dimensional parameters measured were: average linear width (AVL), average curvilinear width (AVCL), average height (AVH) at different planes; average antero-posterior length (AAP), average volume (V), palatal arch length (PAL), and palatal index (PI). The findings were compared with those of controls of the same age and sex. The AVL, AVCL, AAP, PAL, V and PI values of patients with Down syndrome were found to be less than the corresponding values of controls and the average height values of patients with Down syndrome were greater than the corresponding values of controls. Statistical significance was observed in all measurements between the controls and the patients with Down syndrome, especially in those concerning the height and the volume of the oral cavity. Observations from this study have suggested that prostheses might be designed to modify the palatal anatomy and produce better articulation in people with Down syndrome.
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    Down Syndrome Research and Practice (Online). 2007. doi:10.3104/reports.1999
  • Temperament and behaviour problems in young children with Down syndrome at 12, 30, and 45 months
    Deborah Fidler, David Most, Cathryn Booth-LaForce and Jean Kelly
    Though cross-sectional studies have yielded important information regarding the trajectory of psychopathology in middle childhood and adolescence in Down syndrome, there has been little exploration of maladaptive behaviour in the earliest years of development. In this study, we explore the emergence of maladaptive behaviour in young children with Down syndrome (n = 24) and a mental age-matched comparison group (n = 33) of young children with developmental disabilities of mixed etiologies. Behavioural data (Bayley Scales of Infant Development, Infant Temperament Questionnaire, Achenbach's Child Behaviour Checklist) were collected for children in each group at 12 months, 30 months, and 45 months. Findings from this study suggest that the onset of internalising behaviour difficulties in young children with Down syndrome may emerge later in early development than in children with developmental disabilities of mixed/nonspecific etiologies. In addition, temperament at Time 1 appeared to be a much stronger predictor of maladaptive behaviour outcomes at Time 3 in the mixed comparison group than in the Down syndrome group. Implications of this trajectory of the early development of maladaptive behaviour in Down syndrome for intervention for young children with Down syndrome are discussed.
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    Down Syndrome Research and Practice. 2006. doi:10.3104/reports.302
  • Changes in maternal age in England and Wales - implications for Down syndrome
    Elizabeth Crane and Joan Morris
    The risk of having a pregnancy with Down syndrome increases with maternal age. The percentage of all births in England and Wales to mothers aged 35 and over increased from 9% in 1989 to 19% in 2003. A 51% increase in the numbers of pregnancies with Down syndrome has been observed over the same time period (from 954 to 1440). Due to improvements in antenatal screening for Down syndrome and the subsequent termination of affected pregnancies, the total number of births with Down syndrome decreased from 770 in 1989 to 609 in 2003. However the number of births with Down syndrome to women aged 35 and over increased from 186 in 1989 to 310 in 2003 because of the increasing number of pregnancies amongst these women.
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    Down Syndrome Research and Practice. 2006. doi:10.3104/reports.304
  • Speech intelligibility and childhood verbal apraxia in children with Down syndrome
    Libby Kumin
    Many children with Down syndrome have difficulty with speech intelligibility. The present study used a parent survey to learn more about a specific factor that affects speech intelligibility, i.e. childhood verbal apraxia. One of the factors that affects speech intelligibility for children with Down syndrome is difficulty with voluntarily programming, combining, organising, and sequencing the movements necessary for speech. Historically, this difficulty, childhood verbal apraxia, has not been identified or treated in children with Down syndrome but recent research has documented that symptoms of childhood verbal apraxia can be found in children with Down syndrome. The survey examined whether and to what extent childhood verbal apraxia is currently being identified and treated in children with Down syndrome. The survey then asked parents to identify certain speech characteristics that occur always, frequently, sometimes or never in their child's everyday speech. There were 1620 surveys received. Survey results indicated that approximately 15% of the parents responding to the survey had been told that their child has childhood verbal apraxia. Examination of the everyday speech characteristics identified by the parents indicated that many more children are showing clinical symptoms of childhood verbal apraxia although they have not been given that diagnosis. The most common characteristics displayed by the subjects included decreased intelligibility with increased length of utterance, inconsistency of speech errors, difficulty sequencing oral movements and sounds, and a pattern of receptive language superior to expressive language. The survey also examined the impact of childhood verbal apraxia on speech intelligibility. Results indicated that children with Down syndrome who have clinical symptoms of childhood verbal apraxia have more difficulty with speech intelligibility, i.e. there was a significant correlation between childhood verbal apraxia and parental intelligibility ratings. Children with apraxia often do not begin to speak until after age 5. There was a significant correlation between speech intelligibility and age at which the child began to speak, i.e. children who began to speak after age 5 had lower parental intelligibility ratings. A diagnosis of difficulty with oral motor skills is more frequently given than a diagnosis of apraxia; 60.2% of parents had been given this diagnosis. According to survey results, it is rare (2%) for a diagnosis of childhood verbal apraxia to be made without a diagnosis of difficulty with oral motor skills.
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    Down Syndrome Research and Practice. 2006. doi:10.3104/reports.301
  • A comparison of mainstream and special education for teenagers with Down syndrome: Implications for parents and teachers
    Sue Buckley, Gillian Bird, Ben Sacks and Tamsin Archer
    This article presents data from a research study designed to compare the achievements of teenagers with Down syndrome educated in mainstream classrooms or in special education classrooms throughout their full-time education. Progress is reported for speech and language, literacy, socialisation, daily living skills and behaviour. For all the teenagers, there is evidence of progress with age on all the measures except for communication. Communication continued to improve through teenage years for the included children but not for those in special education classrooms. There were no significant differences in overall outcomes for daily living skills or socialisation. However, there were large significant gains in expressive language and literacy skills for those educated in mainstream classrooms. Teenagers educated in mainstream classrooms showed fewer behavioural difficulties. Further, comparison with data published by these authors in an earlier study, showed no improvements in school achievements in special education over a 13 year period in the UK (1986-1999).
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    Down Syndrome Research and Practice. 2006. doi:10.3104/reports.295
  • Early learning and adaptive behaviour in toddlers with Down syndrome: Evidence for an emerging behavioural phenotype?
    Deborah Fidler, Susan Hepburn and Sally Rogers
    Background: Though the Down syndrome behavioural phenotype has been described as involving relative strengths in visuo-spatial processing and sociability, and relative weaknesses in verbal skills and motor planning, the early emergence of this phenotypic pattern of strengths and weaknesses has not yet been fully explored. Method: In this study, we compared the performance of eighteen 2 to 3-year-olds with Down syndrome to an MA-matched comparison group of nineteen 2 to 3-year-olds with mixed developmental disabilities, and an MA-matched comparison group of 24 children with typical development on two developmental measures: the Mullen Scales of Early Learning and the Vineland Adaptive Behaviour Scales. Results: While the specificity of the Down syndrome profile was (for the most part) not yet evident, results showed that toddlers with Down syndrome in this study did show emerging areas of relative strength and weakness similar to that which has been described in older children and young adults with Down syndrome. This pattern included relatively stronger social skills, weaker expressive language, and poor motor coordination. When this pattern of strengths and weaknesses was compared to the developmental profiles of the two comparison groups, socialisation strengths differentiated the Down syndrome group from the mixed developmental disabilities group.
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    Down Syndrome Research and Practice. 2006. doi:10.3104/reports.297
  • Language learning in Down syndrome: The speech and language profile compared to adolescents with cognitive impairment of unknown origin
    Robin Chapman
    Children and adolescents with Down syndrome show an emerging profile of speech and language characteristics that is typical of the syndrome (Chapman & Hesketh, 2000; Chapman, 2003; Abbeduto & Chapman, 2005) and different from typically developing children matched for nonverbal mental age, including expressive language deficits relative to comprehension that are most severe for syntax, and, in adolescence, strengths in comprehension vocabulary, improvements in expressive syntax, but losses in comprehension of syntax (Chapman, Hesketh & Kistler, 2002). Here we compare 20 adolescents with Down syndrome to16 individuals with cognitive impairment of unknown origin, statistically matched for age and nonverbal mental age, to show that the age-related strengths in vocabulary comprehension are not limited to the Down syndrome phenotype, but are limited to a certain type of vocabulary test: for both groups, performance on the Peabody Picture Vocabulary Test-3 is significantly greater than performance on the vocabulary subtest of the Test of Auditory Comprehension of Language-3, which does not differ from the syntax comprehension subtests. Vocabulary size, but not conceptual level, is a strength for adolescents with cognitive impairment. In contrast, deficits in auditory-verbal working memory, syntax and vocabulary comprehension, and narration of picture-books without an opportunity to preview them are all specific to the adolescent group with Down syndrome. The expressive language deficit disappears when a preview opportunity and picture support is given.
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    Down Syndrome Research and Practice. 2006. doi:10.3104/reports.306
  • Gross motor skill acquisition in adolescents with Down syndrome
    Sarah Meegan, Brian Maraj, Daniel Weeks and Romeo Chua
    The purpose of this study was to assess whether verbal-motor performances deficits exhibited by individuals with Down syndrome limited their ability to acquire gross motor skills when given visual and verbal instruction together and then transferred to either a visual or verbal instructional mode to reproduce the movement. Nine individuals with Down syndrome (6 males, 3 females) performed 3 gross motor skills. Both visual and verbal instructional guidance was given to the participants over a 4-day period. Twenty-four hours later, the participants were video recorded as they produced the movements (used as baseline measures). On Day 6, they were randomly assigned into verbal and visual groups and required to reproduce the skills while the experimenter provided either visual demonstration or verbal instructions depending on the group. Based on skill performance scores, participants in the verbal-motor performance group demonstrated a lower level of proficiency and an increased number of performance errors when compared to participants in the visual-motor performance group. Moreover, while the visual group demonstrated an increase in performance levels compared to baseline measures, the opposite effect was seen for the verbal group.
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    Down Syndrome Research and Practice. 2006. doi:10.3104/reports.298
  • Perceptual-motor deficits in children with Down syndrome: Implications for intervention
    Naznin Virji-Babul, Kimberly Kerns, Eric Zhou, Asha Kapur and Maggie Shiffrar
    Early intervention approaches for facilitating motor development in infants and children with Down syndrome have traditionally emphasised the acquisition of motor milestones. As increasing evidence suggests that motor milestones have limited predictive power for long-term motor outcomes, researchers have shifted their focus to understanding the underlying perceptual-motor competencies that influence motor behaviour in Down syndrome. This paper outlines a series of studies designed to evaluate the nature and extent of perceptual-motor impairments present in children with Down syndrome. 12 children with Down syndrome between the ages of 8-15 years with adaptive ages between 3-7 years (mean age = 5.6 years +/- 1.45 years) and a group of 12 typically developing children between the ages of 4-8 years (mean age = 5.4 +/- 1.31 years) were tested on their ability to make increasingly complex perceptual discriminations of motor behaviours. The results indicate that children with Down syndrome are able to make basic perceptual discriminations but show impairments in the perception of complex visual motion cues. The implications of these results for early intervention are discussed.
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    Down Syndrome Research and Practice. 2006. doi:10.3104/reports.308
  • Home and community literacy experiences of individuals with Down syndrome
    Brian Trenholm and Pat Mirenda
    This exploratory survey was conducted to gain a detailed understanding of the home and community literacy experiences of children, adolescents and adults with Down syndrome. The data were collected from 224 parents/guardians across Canada who were asked to indicate literacy goals and priorities for their children with Down syndrome, the literacy resources they and their children utilised at home and in the community, perceived barriers to their children's literacy attainment, and solutions for alleviating the barriers. The results were analysed according to age when appropriate, in order to better understand the course of literacy development. Overall, the number of respondents who indicated their children with Down syndrome could read and write appeared to be consistent with previously published estimates, including the number reporting advanced reading levels. The wide range of reading and writing materials observed in use at home appeared to be greater than the range of materials actually used by children with Down syndrome. Relatively few of the parents who read storybooks to their children reported asking higher-level questions, suggesting that some parents might benefit from support in this activity. Many respondents reported using the library, and many expressed concerns about the quality and scarcity of literacy programs. The results are discussed with regard to their implications for how parents, caregivers, teachers, and program providers can encourage literacy development in persons with Down syndrome, and suggestions for future research.
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    Down Syndrome Research and Practice. 2006. doi:10.3104/reports.303
  • Specific instructions are important for continuous bimanual drumming in adults with Down syndrome
    Shannon (Robertson) Ringenbach, Heather Allen, Susan Chung and Michelle Jung
    The present study examined continuous and discrete bimanual drumming in response to different instructions in 10 adults with Down syndrome, 10 mental age-matched and 10 chronological age-matched groups. For continuous drumming, participants hit two drums with both hands at the same time following verbal (e.g., "up" and "down"), visual (e.g., video of both drumsticks moving up and down together) and auditory (e.g., sound of both drums being hit, then symbol being hit) instructions for 10 s. For discrete drumming participants hit two drums with both hands at the same time once in response to the instructions described above. In general, for all groups spatial measures showed a performance advantage when using the visual metronome in continuous tasks but no advantage with any instructions for discrete tasks.
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    Down Syndrome Research and Practice. 2006. doi:10.3104/reports.312
  • Sensory impairments and health concerns related to the degree of intellectual disability in people with Down syndrome
    Tuomo Määttä, Markus Kaski, Anja Taanila, Sirkka Keinänen-Kiukaanniemi and Matti Iivanainen
    The relationship between poor health and cognitive impairment is not fully understood yet. People with Down syndrome are prone to a number of health problems, including congenital heart defect, visual impairment, hearing loss, autoimmune diseases, epilepsy, early-onset Alzheimer's disease and intellectual disability. Our aim was to assess the impact of impaired health on cognitive performance in people with Down syndrome. A series of people with Down syndrome (n=129) were studied for their intellectual disability, sensory impairments and health concerns. The medical and psychological records of all persons with Down syndrome in the Intellectual Disability Service Register of Kainuu from 1970 to 2004 were analysed. The detected health issues were related to the individuals' cognitive levels. Visual impairment, poor dental health and acute neurological disease were associated with the severity of intellectual disability and ageing.
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    Down Syndrome Research and Practice. 2006. doi:10.3104/reports.317
  • Glucose-6-phosphate-dehydrogenase is also increased in erythrocytes from adolescents with Down syndrome
    Francisco Ordonez, Manuel Rosety-Plaza and Manuel Rosety-Rodriguez
    For some time it has been claimed that trisomic cells are more sensitive to oxidative stress since there is an imbalance in hydrogen peroxide metabolism due to an increase in superoxide dismutase (SOD) catalytic activity. We designed the present study to assess activity levels of antioxidant enzymes [superoxide dismutase (SOD), glutathione peroxidase (GPx), catalase (CAT) and glucose-6-phosphate-dehydrogenase (G6PDH)] in erythrocytes in 31 male adolescents with Down syndrome (mean age 16.3 ± 1.1). An increase of 35.2%, 15.3% and 14.9% in the catalytic activity of SOD, GPx and G6PDH respectively was observed in male adolescents with Down syndrome compared to age-matched controls. For CAT, a slight increase of 6.0% was also found. It is concluded that our data are consistent with previous evidence of the existence of oxidative stress in individuals with Down syndrome as revealed by significantly enhanced activities of SOD and GPx. The most striking feature was that G6PDH, in contrast to CAT, presented a similar behaviour. Further studies are required to identify other antioxidant enzymes in red blood cells as well as in white blood cells in order to increase the range of potential bioindicators of oxidative stress.
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    Down Syndrome Research and Practice. 2006. doi:10.3104/reports.318
  • Parental strategies for assisting children to wait
    Monica Cuskelly, Anne Jobling, Linda Gilmore and Sheila Glenn
    This study examined parents' behaviours as they waited with their child. Children were presented with an attractively wrapped gift and then asked not to touch it until the experimenter returned from finishing some work in another room. Three parent groups and their children participated in the study - parents of children with Down syndrome, parents of children with intellectual disability from another cause, and parents of children who were developing typically. There were no significant differences between children in how long they were able to wait before touching the gift. The data from the first two groups were combined for all analyses after it was established that there were no significant differences between them. There were few significant differences between parents of a child with intellectual disability and comparison parents. The former group were more likely to be classified as Authoritarian than were comparison parents, however with one exception, parenting style was unrelated to the strategies parents used in the waiting situation. Very few parents in either group used the opportunity to teach or explicitly praise effective waiting strategies in their children.
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    Down Syndrome Research and Practice. 2006. doi:10.3104/reports.315
  • The development of oral motor control and language
    Katie Alcock
    Motor control has long been associated with language skill, in deficits, both acquired and developmental, and in typical development. Most evidence comes from limb praxis however; the link between oral motor control and speech and language has been neglected, despite the fact that most language users talk with their mouths. Oral motor control is affected in a variety of developmental disorders, including Down syndrome. However, its development is poorly understood. We investigated oral motor control in three groups: adults with acquired aphasia, individuals with developmental dysphasia, and typically developing children. In individuals with speech and language difficulties, oral motor control was impaired. More complex movements and sets of movements were even harder for individuals with language impairments. In typically developing children (21-24 months), oral motor control was found to be related to language skills. In both studies, a closer relationship was found between language and complex oral movements than simple oral movements. This relationship remained when the effect of overall cognitive ability was removed. Children who were poor at oral movements were not good at language, although children who were good at oral movements could fall anywhere on the distribution of language abilities. Oral motor skills may be a necessary precursor for language skills.
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    Down Syndrome Research and Practice. 2006. doi:10.3104/reports.310
  • Mental health, behaviour and intellectual abilities of people with Down syndrome
    Tuomo Määttä, Tuula Tervo-Määttä, Anja Taanila, Markus Kaski and Matti Iivanainen
    The mental health, adaptive behaviour and intellectual abilities of people with Down syndrome (n=129) were evaluated in a population-based survey of social and health care records. Females had better cognitive abilities and speech production compared with males. Males had more behavioural problems than females. Behaviour suggestive of attention deficit hyperactivity disorder was often seen in childhood. Depression was diagnosed mainly in adults with mild to moderate intellectual disability. Autistic behaviour was most common in individuals with profound intellectual disability. Elderly people often showed decline of adaptive behaviour associated with Alzheimer's disease. Case descriptions are presented to illustrate the multitude of mental health and behavioural issues seen from childhood to old age in this population.
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    Down Syndrome Research and Practice. 2006. doi:10.3104/reports.313
  • Simultaneous treatment of grammatical and speech-comprehensibility deficits in children with Down syndrome
    Stephen Camarata, Paul Yoder and Mary Camarata
    Children with Down syndrome often display speech-comprehensibility and grammatical deficits beyond what would be predicted based upon general mental age. Historically, speech-comprehensibility has often been treated using traditional articulation therapy and oral-motor training so there may be little or no coordination of grammatical and speech-comprehensibility treatment. The purpose of this paper is to provide the rationale for and preliminary evidence in support of integrating speech and grammatical intervention using a type of recast treatment in six children with Down syndrome. Speech-comprehensibility and MLU growth in generalisation sessions occurred in 4/6 and 5/6 participants, respectively. Using multiple baseline design logic, two of these participants showed evidence of treatment effects on speech-comprehensibility and two in MLU in generalisation sessions, respectively. The study constitutes a conservative test of the intervention effects for reasons that are discussed. The theoretical and applied significance of these findings are discussed.
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    Down Syndrome Research and Practice. 2006. doi:10.3104/reports.314
  • Hair zinc level in Down syndrome
    Ayse Yenigun, Ferda Ozkinay, Ozgur Cogulu, Canan Coker, Nurten Cetiner, Gonca Ozden, Oguz Aksu and Cihangir Ozkinay
    Immunological, endocrinological, and haematological abnormalities are relatively common in people with Down syndrome (Cuadrado & Barrena, 1996; Decoq & Vincker, 1995; Hestnes et al., 1991; Sustrova & Strbak, 1994; Nespoli, Burgio, Ugazio & Maccario, 1993; Kempski, Chessells & Reeves, 1997; Kivivuori, Rajantie, & Siimes, 1996; David et al., 1996; Gjertson, Sturm & Berger, 1999). Zinc is one of the elements that act in the maintenance of normal function of these systems. This study was designed to investigate zinc levels in children with Down syndrome. Zinc levels were measured in hair using atomic absorption spectrophotometry. The hair zinc level of 19 children with Down syndrome was compared with the zinc level of 11 typically developing children. Hair zinc levels were found to be significantly lower (p < .05) in those with Down syndrome (average 95.18 ± 56.10 ppm) than in the typically developing children (average 208.88 ± 152.37 ppm). Some of the problems experienced by children with Down syndrome may be due to these low zinc levels, but further research is required to confirm these results, and to establish any correlation with these problems.
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    Down Syndrome Research and Practice. 2004. doi:10.3104/reports.292
  • What's it all about? Investigating reading comprehension strategies in young adults with Down syndrome
    Michelle Morgan, Karen Moni and Anne Jobling
    The purpose of reading is for the reader to construct meaning from the text. For many young adults with Down syndrome, knowing what the text is all about is difficult, and so for them the activity of reading becomes simply the practice of word calling. It is suggested in the literature that for those individuals with Down syndrome, learning can continue into adolescence and that this may be the optimal time for learning to occur. However, a review of the literature revealed limited empirical research specifically relating to the reading comprehension of young adults with Down syndrome. Recent findings from Latch-OnTM(Literacy And Technology Hands On), a research-based literacy and technology program for young adults with Down syndrome at the University of Queensland, revealed that comprehension remained the significant area of difficulty and showed least improvement (Moni & Jobling, 2001). It was suggested by Moni and Jobling (2001) that explicit instruction in comprehension using a variety of strategies and meaningful, relevant texts was required to improve the ability of young adults with Down syndrome to construct meaning from written texts. This paper is based on an action research project that was developed within the Latch-OnTM program. The project utilised a modification of Elliot's (1991) action research model and was conducted to investigate specific teaching and learning strategies that would enhance the reading comprehension of young adults with Down syndrome. The participants were 6 young adults with Down syndrome ranging in age from 18 to 25 years. As the data from this project are still being analysed, preliminary findings of one participant are presented as a case study. The preliminary findings appear to indicate that the program of specific teaching and learning reading comprehension strategies used in this project was beneficial in the participant's reading comprehension.
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    Down Syndrome Research and Practice. 2004. doi:10.3104/reports.290
  • Parental experiences of feeding problems in their infants with Down syndrome
    Eilene Lewis and Alta Kritzinger
    Although feeding difficulties in infants with Down syndrome are described in the literature, the experiences of parents regarding the feeding problems of their infants are largely omitted. In order to promote closer collaboration with families and speech-language therapists in early communication intervention, the study investigated some experiences of a group of parents concerning the feeding problems of their infants with Down syndrome. A descriptive survey approach was implemented to collect quantitative data. The results revealed the type of feeding problems and associated conditions occurring in the infants of the twenty participants; the type of feeding methods used; the assistance received during the feeding problems; the emotions experienced in relation to the feeding problems in their infants as well as suggestions made by the participants regarding the management of early feeding problems. Numerous feeding problems occurred in the infants and the variety of emotions experienced by the participants indicated that feeding therapy and counseling were required. As a result of the different conditions underlying the feeding problems of the participants' infants, a comprehensive approach, which included therapeutic and medical intervention, was necessary. In order to provide effective early communication intervention services from birth, the results indicated that the knowledge, skills and sensitivity of speech language therapists to identify caregiver needs, to provide appropriate family-focused intervention and to make recommendations regarding the management of feeding problems in infants with Down syndrome is of great importance
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    Down Syndrome Research and Practice. 2004. doi:10.3104/reports.291
  • Early phonological awareness and reading skills in children with Down syndrome
    Esther Kennedy and Mark Flynn
    Increasingly, children with Down syndrome receive literacy instruction with the expectation of acquiring functional reading skills. Unfortunately, little is known about the processes underlying literacy skills in this special population. Phonological awareness contributes to literacy development in typically developing children, however, there is inconclusive evidence about these skills in younger children with Down syndrome. 9 children with Down syndrome (5;6 - 8;10 years) participated in this investigation. Due to the paucity of standardised phonological awareness measures for children with special needs, in particular children with Down syndrome, a variety of tasks were adapted from the literature. The assessment battery examined the skills of phonological awareness, literacy, speech production, expressive language, hearing acuity, speech perception, and auditory visual memory. The results suggest that children with Down syndrome are at risk for reading acquisition difficulties due to reduced phonological awareness skills. These deficits are in addition to delays caused by reduced cognitive skills. Only one of the participants was able to demonstrate rhyme awareness, which may have been due to task effects. Written word recognition ability was correlated with tests of phonemic awareness, and error analysis of the spelling and nonword reading tasks suggested grapheme phoneme connections deficits. Further research is needed to determine the best methods of assessment and intervention for phonological awareness in children with Down syndrome.
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    Down Syndrome Research and Practice. 2003. doi:10.3104/reports.136
  • Performing movement sequences with knowledge of results under different visual conditions in adults with Down syndrome
    Naznin Virji-Babul, Jennifer Lloyd and Geraldine Van Gyn
    The purpose of this study was to examine the learning of movement sequences in individuals with Down syndrome under different visual information conditions. 10 adults with Down syndrome and 14 neurologically typical adults performed a sequence of movements under two different visual information conditions: full visual feedback of the limb and environment and no visual feedback of the limb. Participants were given knowledge of results of their total movement time after each trial. The entire task was presented as a game and movement time information was given as a "score" after each trial. Participants were also given verbal encouragement throughout the task. As expected, individuals with Down syndrome had significantly slower reaction and movement times than neurologically typical participants. Interestingly, however, mean reaction and movement time was not affected by the visual condition, in either group. Participants with Down syndrome improved their performance over the presented trials, in both visual information conditions. These findings indicate that providing knowledge of results of movement performance can facilitate the performance and coordination of movement sequences even under conditions where visual information of the moving limb is restricted.
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    Down Syndrome Research and Practice. 2003. doi:10.3104/reports.137
  • Sleep related breathing disorders in adults with Down syndrome
    Onofrio Resta, Maria Foschino Barbaro, Tiziana Giliberti, Gennaro Caratozzolo, Maria Cagnazzo, Franco Scarpelli and Maria Nocerino
    Background. While the prevalence of obstructive sleep apnoea syndrome among children with Down syndrome is reported to vary from 30 to 50%, the nocturnal respiratory patterns of adults with Down syndrome is not well known. Objectives. The aim of this study is to evaluate sleep related breathing disorders in a sample of adults with Down syndrome. Methods. We studied the nocturnal respiratory patterns of 6 adults with Down syndrome, aged 28 to 53 years. All participants were monitored for 8 hours using a 12 channel polysomnograph. Respiratory events (apnoeic and hypopnoeic) were classified as obstructive or central, in relation to the presence or the absence of paradoxical breathing. Results. All participants had respiratory pauses during sleep. 5 of them had an apnoea hypopnoea index >10, justifying the diagnosis of sleep apnoea syndrome. About 85% of the respiratory events were apnoeic, the others being hypopnoeic. Among all the respiratory events 89.2% were obstructive, whereas only 10.8% were central. The central events were almost always organised in very low and regular sequences and respiration frequently showed a true periodic pattern, consisting of short periods of augmented breath followed by central events causing oxygen desaturation. Conclusions. According to the literature and in conjunction with the current study's results it could be hypothesised that the nocturnal respiratory pattern of adults with Down syndrome depends on several pathogenetic factors such as age, severity of upper airway abnormalities, body mass index (BMI), other pathological conditions and age related brainstem dysfunction.
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    Down Syndrome Research and Practice. 2003. doi:10.3104/reports.138
  • Is there a relationship between zinc and the peculiar comorbidities of Down syndrome?
    Corrado Romano, Rosa Pettinato, Letizia Ragusa, Concetta Barone, Antonino Alberti and Pinella Failla
    Zinc plays a central role in the immune system and has been found to be significantly reduced in people with Down syndrome. The effectiveness of zinc supplementation in people with Down syndrome has been reported with discordant results. A comparison was made between a range of clinical and biochemical variables and zinc levels in 120 individuals with Down syndrome. Two groups of participants, one with normal zinc levels and the second with low zinc levels, were compared on the following measures: growth hormone secretion, IgA and IgG antigliadin antibodies, presence of coeliac disease, T3, T4, fT3, fT4, TSH, hypothyroidism, hyperthyroidism, CD4/CD8 ratio, total immunoglobulins G and subclasses. No significant difference was found between the two groups, except for IgG4 which was, unexpectedly, significantly decreased in the group with normal zinc levels. In conclusion, an impairment of zinc blood level in individuals with Down syndrome does not necessarily impact on the organs and systems evaluated here.
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    Down Syndrome Research and Practice. 2002. doi:10.3104/reports.126
  • Oesophageal atresia and Down syndrome
    Sebastiano Bianca, Marco Bianca and Giuseppe Ettore
    Infants with Down syndrome are known to have a high frequency of associated birth defects and some authors have suggested an association between Down syndrome and oesophageal atresia. We evaluated data from the Sicilian Registry of Congenital Malformations. Our finding of an incidence of 0.9% of oesophageal atresia in children with Down syndrome is more than 30 times higher than expected and more than reported in other studies where the association was present in 0.5% of cases. Our results confirm that the relationship between Down syndrome and oesophageal atresia is a non random association and the observation of this association in several populations with different genetic backgrounds allows us to conclude that a causal relationship may exist between Down syndrome and oesophageal atresia.
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    Down Syndrome Research and Practice. 2002. doi:10.3104/reports.127
  • Phonological awareness in children with Down syndrome
    Helen Fletcher and Sue Buckley
    Research in the area of phonological awareness has mainly focused on the nature of the relationship between reading ability and awareness of phonemes. However, a recent study of phonological awareness in children with Down syndrome questioned the existence of any necessary relationship (Cossu, Rossini & Marshall, 1993). This paper describes a study of phonological awareness in children with Down syndrome with varying levels of reading ability. The sample consisted of 10 male and 7 female children with Down syndrome (aged 9 years 2 months to 14 years 5 months). All children received a battery of tests which consisted of assessments of: 1) phonological awareness, 2) reading and spelling competence, 3) non-word reading and spelling ability, and 4) non-verbal measures. Children with Down syndrome demonstrated measurable levels of phonological awareness. Significant positive correlations were found between phonological awareness and: reading and spelling competence, ability to spell non-words and non-verbal measures.
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    Down Syndrome Research and Practice. 2002. doi:10.3104/reports.123
  • Language in ageing persons with Down syndrome
    Jean Rondal and Annick Comblain
    Several cross-sectional studies and one longitudinal study were conducted on the language abilities of various cohorts of persons with Down syndrome aged between 14 and 50 years. No significant difference was observed on any of the receptive and productive morphosyntactic and lexical measures used, suggesting no marked change in the language of these persons from adolescence onto late adulthood. Repeated measures of cerebral metabolic rate (CMR) for fluorodeoxyglucose using a Positron Emission Tomography were made over a 4-year interval with 7 participants with Down syndrome aged between 37 and 49 years. A gradual decrease in global CMR for both cerebral hemispheres and for each participant was documented. It was particularly marked for 3 participants. However, no language deterioration could be associated with their marked lowering in CMR.
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    Down Syndrome Research and Practice. 2002. doi:10.3104/reports.122
  • Improving the classroom listening skills of children with Down syndrome by using sound-field amplification
    Lee Bennetts and Mark Flynn
    Many children with Down syndrome have fluctuating conductive hearing losses further reducing their speech, language and academic development. It is within the school environment where access to auditory information is crucial that many children with Down syndrome are especially disadvantaged. Conductive hearing impairment which is often fluctuating and undetected reduces the child's ability to extract the important information from the auditory signal. Unfortunately, the design and acoustics of the classroom leads to problems in extracting the speech signal through reduced speech intensity due to the increased distance of the student from the teacher in addition to masking from excessive background noise. One potential solution is the use of sound-field amplification which provides a uniform amplification to the teacher's voice through the use of a microphone and loudspeakers. This investigation examined the efficacy of sound-field amplification for 4 children with Down syndrome. Measures of speech perception were taken with and without the sound-field system and found that the children perceived significantly more speech in all conditions where the sound-field system was used (p < .0001). Importantly, listening performance with the sound-field system was not affected by reducing the signal-to-noise ratio through increasing the level of background noise. In summary, sound-field amplification provides improved access to the speech signal for children with Down syndrome and as a consequence leads to improved classroom success.
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    Down Syndrome Research and Practice. 2002. doi:10.3104/reports.124
  • Gender differences in coping strategies of parents of children with Down syndrome
    Anthony Sullivan
    Little research has been conducted on the reactions of parents, and fathers in particular, following the birth of a child with Down syndrome. Previous studies suggest that gender differences exist in coping strategies and a number of theories have supported this. The current study is informed by Pleck's (1981) Gender Role Strain model which attempts to explain the different socialisation processes males encounter which influence their development in our society. Questionnaires from Carver, Scheier and Weintraub's COPE inventory (1989) were given to parents (n = 150) to measure coping strategies and a number of gender differences were found. Females scored significantly higher than males in seeking instrumental and emotional support; in focusing on and venting emotions; and suppression of competing activities. An additional analysis carried out on parents of young children (n = 74) yielded similar results. The overall findings from the study provides mixed implications for Pleck's theory. Gender differences were found but no value can be ascribed to these different coping strategies.
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    Down Syndrome Research and Practice. 2002. doi:10.3104/reports.132
  • Oxidative stress in Portuguese children with Down syndrome
    Monica Pinto, Joaquim Neves, Miguel Palha and Manuel Bicho
    Background - Individuals with Down syndrome have an accelerated process of ageing which is thought to be associated with oxidative stress. Aim - Since Zn/Cu superoxide dismutase is increased by about 50% in children with Down syndrome, glutathione and other less known antioxidant mechanisms were studied to determine whether there were changes in reactive oxygen species. Methods - Plasma reduced and oxidised glutathione and red blood cells enzymes including acid phosphatase, methemoglobin reductase and transmembrane reductase were evaluated in Portuguese children with Down syndrome and their siblings, who were used as a control group. Results - No significant differences were found between the study and control groups. A negative correlation was noted between total glutathione and acid phosphatase in the siblings without Down syndrome, but not in the children with Down syndrome. Conclusion - Although it is claimed that the production of hydrogen peroxide is enhanced in children with Down syndrome, their antioxidant mechanisms do not seem to be significantly different compared with their siblings. This may result in an excess of reactive oxygen species that could help to explain accelerated ageing in children with Down syndrome. Further studies will be needed to shed light on these mechanisms.
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    Down Syndrome Research and Practice. 2002. doi:10.3104/reports.134
  • Work stress and people with Down syndrome and dementia
    Stephen Donaldson
    This study aimed to assess how staff ratings of challenging behaviour for people with Down syndrome and dementia affected the self-reported well-being of care staff. Data were collected from 60 care staff in 5 day centres in a large city in England. The data were collected by use of a questionnaire. There was no significant difference between those who cared for individuals with Down syndrome and dementia and those caring for service users with other non-specified learning disabilities without dementia, regarding their self-reported well-being. Self-reported well-being did correlate with staff rating of challenging behaviour in both those who cared for people with Down syndrome and dementia and those who did not care for such service users, with well-being declining as perceived challenging behaviour increased. The findings indicate that challenging behaviour prevention and reduction may be of benefit to both service users and care staff well-being.
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    Down Syndrome Research and Practice. 2002. doi:10.3104/reports.133
  • Cancer incidence in persons with Down syndrome in Israel
    Lital Boker and Joav Merrick
    The purpose of this study was to assess the incidence rates of leukaemia and other malignancies in persons with Down syndrome in Israel. The target population consisted of all persons with Down syndrome in the period of 1948-1995 and the study population was divided into two subgroups: (1) Persons born in Israel between 1979-95 (registry group) and (2) Persons currently or past-institutionalised, born before 1979 (institution group). The study population was linked to the Cancer Registry and cases that had been diagnosed through December 1995 were subsequently identified. The observed incidence rates were compared to expected rates in the general population. Standardised Incidence Ratios (SIR) and 95% confidence intervals were computed for each disease category. Analyses of results were performed separately for each subgroup of our study population. In the registry group seven cancer cases were observed as compared to 1.5 expected (SIR=4.67 95% CI 1.9-9.6), all of which were leukaemia cases. For the institution group a total of 17 cancer cases were observed compared to 12.8 expected. These included four cases of leukaemia (SIR=6.90 95% CI 1.9-17.7). An excess of gastric cancer in males, based on two cases (SIR=11.9 95% CI 1.3-42.9) was also observed. The significant excess of leukaemia in the Down syndrome population in Israel is in accordance with other international studies. The excess of gastric cancer in males with Down syndrome, which has not been reported before, should be further explored.
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    Down Syndrome Research and Practice. 2002. doi:10.3104/reports.128
  • Sharing laughter: The humour of pre-school children with Down syndrome
    Vasudevi Reddy, Emma Williams and Amy Vaughan
    Humour and laughter have often been portrayed as fundamentally cultural and social phenomena. They can be used to tell us about children's ability to engage socially and to understand others, but have rarely been explored for this purpose. The present paper summarises the results of a study of simple forms of humour in children with Down syndrome and with autism, two groups which are reported to differ in their sociality and _ interpersonal understanding. Sixteen children with Down syndrome and 19 children with autism, matched on non-verbal mental age, participated in a cross-sectional study. Parental reports and video-tapes of naturalistic interaction between parents and children were analysed to show that although there were no overall differences in the presence or frequency of child or parent laughter between the two groups, there were differences in what sorts of events were more likely to prompt child laughter, the extent to which child laughter was shared, and how the children responded to others' laughter. The children with Down syndrome were more likely than the children with autism to laugh at funny faces and socially inappropriate acts and less likely to laugh in strange or inexplicable situations, and more likely to laugh at shared events. They also responded to others' laughter with attention or smiles more, and tried to re-elicit it through acts of clowning. Children with Down syndrome are thus active participants in humour and laughter, sharing it at both an emotional and a cultural level.
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    Down Syndrome Research and Practice. 2001. doi:10.3104/reports.121
  • Implicit versus explicit memory function in children with Down and Williams syndrome
    Stefano Vicari
    The present study was aimed at evaluating implicit memory processes in participants with Williams syndrome and comparing them to children with Down syndrome and to mental-age matched typically developing children. For this purpose, tests of verbal and visuo-perceptual explicit memory, verbal and visual repetition priming as well as procedural learning tasks were administered to 12 participants with Williams syndrome, 14 with Down syndrome and 32 typically developing children. Participants with Williams syndrome showed a level of repetition priming similar to that of mental-age typically developing controls. In contrast, children with Williams syndrome showed a reduced learning rate in the two procedural tasks. As regards children with Down syndrome, we document comparable implicit memory abilities. In contrast, regarding explicit memory, typically developing children performed better than individuals with Down syndrome. This finding is relevant for our knowledge about the qualitative aspects of the anomalous cognitive development in individuals with intellectual disabilities and the neurobiological substrate underlying this development.
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    Down Syndrome Research and Practice. 2001. doi:10.3104/reports.112
  • Motivation and learning styles in young children with Down syndrome
    Jennifer Wishart
    There are both psychological and biological reasons to expect that certain areas of learning will present young children with Down syndrome with significant problems. Knowledge of the neurological underpinnings of these specific difficulties can often allow compensatory teaching strategies to be put in place, however, and some of these have proved highly effective. The impact of the psychological environment on the progress of development in children with Down syndrome is less well understood. Experience of how others respond to their attempts at understanding the physical and social world and the balance of successes and failures they experience in their early learning are both likely to influence the approach to learning adopted when faced with mastering new skills. Findings from inter-linking studies of cognitive and socio-cognitive development which have explored learning behaviours at different ages and at different developmental stages illustrate how a learning style can sometimes evolve over time in which less than efficient use is made of current levels of cognitive ability. Social ploys are sometimes used to avoid engagement in learning, with the net effect that opportunities to learn new skills are not fully exploited and old skills are sometimes inadequately consolidated. Findings of a misuse of social skills in cognitive contexts do not necessarily provide support for the widely-held view that social understanding is an area of strength in children with Down syndrome and less vulnerable to disruption than cognitive development. Data from a recent study of face-processing abilities suggest that there may in fact be a specific weakness in a fundamental skill normally underpinning the development of social understanding: the ability to recognise differences in emotional expressions. The children with Down syndrome in this study had few problems in correctly identifying individual faces but evidenced difficulties in reliably interpreting the emotional expressions portrayed in these faces. These findings are consistent with the emerging picture of neurological disruption in Down syndrome and with what is known of the neurology underlying this key component in social cognition. As most learning takes place in a social context, the findings have implications for adult-child and child-child learning partnerships and would seem to merit further investigation.
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    Down Syndrome Research and Practice. 2001. doi:10.3104/reports.113
  • Memory training for children with Down syndrome
    Frances Conners, Celia Rosenquist and Lori Taylor
    One well-established fact concerning cognitive and language development in individuals with Down syndrome is that working memory is particularly poor, with auditory working memory worse than visual working memory. Working memory serves the functions of control, regulation, and active maintenance of information and is critical in daily complex cognitive activities. Thus, there is a strong need to find effective and practical interventions targeted at improving working memory in individuals with Down syndrome. The present paper reviews research on rehearsal training and concludes that it can be used successfully to increase working memory in individuals with Down syndrome. However, there are still questions about whether auditory working memory can be improved reliably, whether improvement can be maintained over the long term, and whether improvement exists beyond any effect of increased attention. We describe our in-progress study which addresses these concerns
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    Down Syndrome Research and Practice. 2001. doi:10.3104/reports.111
  • The linguistic and cognitive profile of Down syndrome: Evidence from a comparison with fragile X syndrome
    Leonard Abbeduto, Melissa Pavetto, Erica Kesin, Michelle Weissman, Selma Karadottir, Anne O’Brien and Stephanie Cawthon
    In this paper, we report on the results of our research, which is designed to address two broad questions about the cognitive and linguistic profile of Down syndrome: (1) Which domains of functioning are especially impaired in individuals with Down syndrome? and (2) Which aspects of the language and cognitive profile of Down syndrome are syndrome specific? To address these questions, we focused on three dimensions of the Down syndrome profile - receptive language, expressive language, and theory of mind ? and made comparisons to individuals with fragile X syndrome, which is an X-linked form of intellectual disability. We identified Down syndrome impairments on all three dimensions that were substantially greater than those seen in nonverbal cognition and that were not shared by individuals with fragile X syndrome. Clinical implications of these findings are considered.
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    Down Syndrome Research and Practice. 2001. doi:10.3104/reports.109
  • Characterisation of the somatic evolution of Portuguese children with Trisomy 21 - Preliminary results
    Armando Fernandes, Ana Mourato, Mª Xavier, David Andrade, Cláudio Fernandes and Miguel Palha
    We present preliminary results of a cross-sectional study which had the following objectives: 1- to develop percentile curves of weight, height and head circumference of Portuguese children with Trisomy 21 from 0 to 48 months of age; 2- a comparison of the growth of children with Trisomy 21 with a control population of their siblings, and 3- a comparison between the growth of Portuguese and American children with Trisomy 21 (based on the data of Cronk et al). We conclude that: 1- there is growth delay (weight, height, head circumference) in the Portuguese children with Trisomy 21, in all of the parameters evaluated and in all age groups; 2- Portuguese children with Trisomy 21 present values similar to those obtained by Cronk et al until 24 months of age; 3- from the age of 30 months onward Portuguese children with Trisomy 21 were heavier and taller than American children with Trisomy 21. This supports the usefulness of percentile curves specifically for Portuguese children with Trisomy 21.
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    Down Syndrome Research and Practice. 2001. doi:10.3104/reports.106
  • Mastery motivation in children with Down syndrome
    Sheila Glenn, Beverley Dayus, Cliff Cunningham and Maureen Horgan
    Mastery motivation refers to the intrinsic motivation children have to interact with their environments in order to learn about them. It appears early in life, and has been regarded by many researchers as a key motivator for development. It has also been suggested that young school age children with Down syndrome show lowered motivation to perform tasks. It is important to know if this low motivation is present from the start, or develops as a result of environmental experiences; studies of mastery motivation have been one way of investigating this issue. However definitions of mastery motivation, and hence empirical studies, have varied. Thus this paper starts by revisiting the issues surrounding definition and measurement. There is general agreement on some issues: that mastery motivation is intrinsic, that it is manifest in different behaviours as the child develops, that there are individual differences in mastery behaviour, and that these are affected by environmental factors. There is also current agreement that it is essential to remove the confound of differing levels of developmental competence by using individualised measurement. However there is disagreement about which behaviours best index mastery motivation. Some empirical work with infants with Down syndrome is reviewed, and results from a recent longitudinal study on the development of mastery motivation are presented. The results concurred with most others in the recent literature, suggesting that low mastery motivation is not inevitable in infancy in Down syndrome. Infants with Down syndrome showed similar patterns of development as typically developing children, with slight delays. It is argued that longitudinal studies are needed to demonstrate such patterns of development. As the children developed from 6 to 24 months mental age there was no evidence for decreasing levels of mastery motivation. Thus there was no support for the view that more failure experiences impact on levels of mastery motivation. In contrast caregivers did see their young children with Down syndrome as less object mastery oriented than did caregivers of typically developing children. The caregivers of children with Down syndrome were also significantly more directive in their interactions with their children, and there was some suggestion that individual differences in mastery behaviours were related to levels of mastery behaviours in their children. The final section speculates on reasons for these results, and makes suggestions for future work.
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    Down Syndrome Research and Practice. 2001. doi:10.3104/reports.114
  • Counting and cardinal understanding in children with Down syndrome and typically developing children
    Joanna Nye, Michael Fluck and Sue Buckley
    This study compares the procedural counting ability (independently and with parental support) and conceptual understanding of cardinality of a group of children with Down syndrome and a group of typically developing children, matched for non-verbal mental age. Participants were 23 children with Down syndrome (chronological age range: 3.5 - 7 years; mental age range: 2.5 - 4 years) and 20 typically developing children (chronological age range: 2 - 4 years; mental age range: 2.5 - 4 years), and their main caregiver. The children were asked to count sets of toys (assessing procedural counting skills) and to give sets of toys (assessing understanding of cardinality), with set sizes between 2 and 18 items. The counting task was performed in two conditions, with and without parental support. The children were also asked to say the count word sequence aloud, to assess sequence production independent from object counting. The typically developing children produced significantly more number words altogether, longer standard number sequences and could count larger sets than the children with Down syndrome. Support from an adult improved performance on the count task significantly for both groups of children, and there was no significant difference between the groups in the degree of improvement, i.e. the zone of proximal development. No significant differences were found between the frequency of children (approximately one third) in each group who used counting to solve the give task, indicating an understanding of cardinality.
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    Down Syndrome Research and Practice. 2001. doi:10.3104/reports.116
  • Online data collection with special populations over the World Wide Web
    Michael Marcell and Amanda Falls
    The quick ascendance of the World Wide Web as the dominant vehicle for Internet communication has recently made experimentation in a multimedia environment feasible on the Internet. Although web sites containing online psychology demonstrations and experiments for non-handicapped individuals have appeared in recent years (especially in the areas of cognitive and social psychology), there appear to have been few attempts to conduct online experimentation with special populations. We recently completed two online pilot studies of families with Down syndrome or Williams syndrome members: a) A survey that asks (via Likert rating scales, adjective checklists, multiple-choice style questions, and text-entry boxes) about family background, computer use, and temperament of the special needs family member; and b) An experiment (completed by an individual with special needs) that includes auditory and visual digit span tasks and a memory-for-orientation task in which responses are entered via mouse clicks. Recruiting began with e-mail announcements to representative Down syndrome and Williams syndrome discussion groups, listserves, and bulletin boards, and submission of the project's URL (http://www.cofc.edu/~marcellm/testaw.htm) and key indexing terms to selected search engines. This paper reviews technical aspects of developing the online programmes as well as the strengths and weaknesses of online vs. traditional laboratory-based research in relation to issues such as experimental control, delivery of instructions, experimenter bias, participant recruitment, sample heterogeneity, generalization, attrition, privacy, financial costs, data integrity, and ethics. We conclude by offering our thoughts on two ways of implementing online experimentation with special populations: a) Using a remote parent 'helper' as a proxy to work with the target individual; and b) Collaborating with professional colleagues in Web-based projects conducted in traditional laboratory settings.
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    Down Syndrome Research and Practice. 2001. doi:10.3104/reports.120
  • Language and number in Down syndrome: The complex developmental trajectory from infancy to adulthood
    Sarah Paterson
    This paper examines language and number understanding in infants with Down syndrome and Williams syndrome and compares infant performance to that of adults. The cross-syndrome/ cross-domain studies demonstrate that the pattern of performance of infants with Down syndrome and Williams syndrome on two tasks assessing language and number cannot be derived from the pattern of proficiencies and impairments in the adult phenotypic outcome. Single word comprehension was assessed using a visual preference paradigm. All groups (Williams syndrome, Down syndrome, chronological age and mental age-matched controls) looked longer at the stimuli which matched the verbal label but the infants with Down syndrome and Williams syndrome were equally delayed (equivalent to their mental age controls). The similarity between the infants with Down syndrome and those with Williams syndrome did not parallel the difference present in the adult phenotypes, where vocabulary skill in Down syndrome is significantly lower than that in Williams syndrome. Number was assessed using a novelty preference paradigm, in which infants were familiarised with displays of 2 objects and then presented with 2 versus 3 objects. Infants with Williams syndrome discriminated between the familiar and novel numerosities. Infants with Down syndrome did not. Again, the difference between the Down syndrome and Williams syndrome infant groups did not parallel the pattern seen in the adult phenotypes, where individuals with Down syndrome performed better than those with Williams syndrome. It is therefore crucial to characterise the infant state, in order to understand fully the developmental trajectories of atypical groups.
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    Down Syndrome Research and Practice. 2001. doi:10.3104/reports.117
  • Delay of gratification in young adults with Down syndrome
    Monica Cuskelly, Maria Einam and Anne Jobling
    Thirty-one young adults (17-23 years of age) with Down syndrome participated in two self-imposed delay of gratification trials. Thirty-six and forty-eight percent waited for the experimenter to return (15 minutes) on Trials 1 and 2 respectively, and thirty-six percent waited for the experimenter on both occasions. Expressive language differentiated those who waited from those who did not. A discriminant analysis which included measures of expressive language, temperament characteristics and parental attitudes to childrearing gave very good separation of the two groups. Directions for future researched are discussed.
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    Down Syndrome Research and Practice. 2001. doi:10.3104/reports.115
  • Refractive errors and visual anomalies in Down syndrome
    Joav Merrick and Kenneth Koslowe
    A comparatively high incidence of ocular and orbital abnormalities has been reported in persons with Down syndrome. Eighty six children (50% male, 50% female) with Down syndrome in several institutions for individuals with learning difficulties (age range 5-18 years, mean 12.5) were examined for visual impairment in order to relate the ocular impairment to the level of learning difficulty. 6% had mild, 7% moderate, 45% severe and 42% profound learning difficulty. 9% of the children had no refractive errors. A significant (P<0.01) positive correlation was found between progressive amounts of strabismus and ocular pathology with increasing amount of learning difficulty. On the other hand no correlation was found between refractive errors and the level of learning difficulty. Due to the significant number of ocular disorders found it is recommended that all children with Down syndrome should have an eye examination during the first six months of life and annually thereafter.
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    Down Syndrome Research and Practice. 2001. doi:10.3104/reports.105
  • The relation between the psychological functioning of children with Down syndrome and their urine peptide levels and levels of serum antibodies to food proteins
    Egil Nygaard, Karl Ludvig Reichelt and Joseph Fagan
    Aims: To investigate the relation between psychological functioning of subjects with Down syndrome, and their levels of urine peptide and serum antibodies to food proteins. Methods: 55 children with Down syndrome in a cross-sectional study. Psychological functioning was measured by the Stanford-Binet Intelligence Scale: Fourth Edition, McCarthy Scales of Children's Abilities and Fagan's computer based test of novelty preference. Results: The participants, and their siblings, were found to have significantly increased total urine peptide levels. There were no significant correlations between peptide levels and psychological functioning. Significantly increased levels of IgG activity to gliadin and gluten, and IgA activity to gliadin, gluten and casein were found. There were significant negative correlations (Spearman r=-0.13 to -0.51) between psychological functioning, and IgG and IgA activity to gliadin and gluten. Conclusions: A significant relation between antibodies to gluten and psychological functioning was documented. The mechanism and potential causal link are still unknown.
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    Down Syndrome Research and Practice. 2001. doi:10.3104/reports.107
  • Down syndrome in Israel
    Joav Merrick
    The incidence of Down syndrome was studied in Jerusalem for the years 1964-1970 showing an overall incidence rate of 2.43 per 1,000 live births. A National Down Syndrome Register was established in 1978 and data on annual incidence and mortality rates from 1979-1997 is presented. The incidence in 1997 was 1.0 per 1,000 live births, but 2.32 per 1,000, when live births and terminated pregnancies are summed. Infant mortality has generally decreased in the past 20 years in Israel, and a decrease in infant mortality in Down syndrome has also been noted. This is due to better medical treatment and increased parental involvement in the care for infants with Down syndrome.
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    Down Syndrome Research and Practice. 2001. doi:10.3104/reports.104
  • Debunking the pathological model - The functions of an Internet discussion group
    Robert Jones and Heledd Lewis
    In recent years there has been a profusion of resources about Down syndrome available on the World Wide Web and for increasing numbers of people the internet is the resource first contacted when new information is required on a topic. The present paper examined the function of a discussion group which is used primarily by parents of people with Down syndrome. Qualitative research methodology (content analysis) was used to analyse the daily discussions over a 5 month period in 1998 (February to July) and over a 2-week follow up period six months later (January 1999). The analysis revealed themes of celebration, seeing the child before seeing the handicap, hope and optimism, a sense of purpose in life and of being like a family within this group.
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    Down Syndrome Research and Practice. 2001. doi:10.3104/reports.103
  • The relevance of a nonword repetition task to assess phonological short-term memory in individuals with Down syndrome
    Annick Comblain
    Phonological short-term memory capacity is generally measured with a word span task or a digit span task. Another way to measure it is to use a nonword repetition task. Gathercole and Adams (1993) claimed that this procedure can be used with children as young as two-years old. It seems that in normally developing children the quality of nonword repetition is influenced both by the length of nonwords and by the degree of wordlikeness. Can the phonological short-term memory of individuals with Down syndrome be assessed with a nonword repetition task? In order to answer this question, we decided to replicate Gathercole and collaborators' experiments (1991,1993) but with individuals with Down syndrome. The quality of nonword repetition in individuals with Down syndrome is, as in normally developing children, influenced both by the length of nonwords and by their degree of wordlikeness. Furthermore, our results seem to confirm the hypothesis which states that nonwords are temporarily stored in the phonological short-term memory system. As this system has a limited capacity, both normally developing children and people with Down syndrome recall more short nonwords than long nonwords. In conclusion, nonword repetition is a reliable task with which to assess phonological short-term memory in individuals with Down syndrome as well as in normally developing children.
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    Down Syndrome Research and Practice. 1999. doi:10.3104/reports.98
  • Observational Learning in Children with Down Syndrome and Developmental Delays: The Effect of Presentation Speed in Videotaped Modelling
    Gerald Biederman, Stephen Stepaniuk, Valerie Davey, Kim Raven and Darlene Ahn
    Children with severe developmental delays (three with Down syndrome and three with autism as the primary diagnosis) observed a videotaped model performing two basic dressing skills without prompting, verbal or otherwise, or explanation by an instructor. In a within-subjects design, dressing skills that were presented at a relatively slow presentation speed through videotaped modelling were eventually performed better than those presented at a relatively fast speed. These data in combination with evidence from this laboratory that passive modelling of basic skills is more effective than interactive modelling (e.g., Biederman, Fairhall, Raven, Davey, 1998; Biederman, Davey, Ryder, Franchi, 1994; Biederman, Ryder, Davey, Gibson, l991) suggest that standard instructional techniques warrant reexamination both from the basis of instructional effectiveness and the efficient use of the allotment of teacher time.
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    Down Syndrome Research and Practice. 1999. doi:10.3104/reports.93
  • Learning to Count: A Difficult Task?
    Jill Porter
    This article is concerned with the acquisition of counting skills in pupils with Down syndrome. Data from a larger survey of pupils with severe learning difficulties is explored to investigate the types of errors children make at the earliest stages of learning to count. The pattern of responding was consistent with the view that children with Down syndrome have particular difficulties in tasks utilising auditory sequential memory, in this case learning the number string. The practical implications of these findings are discussed.
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    Down Syndrome Research and Practice. 1999. doi:10.3104/reports.99
  • Hardiness in families with and without a child with Down syndrome
    Anna Bower, David Chant and Sara Chatwin
    The paper reports on mothers' perceptions, ideas and beliefs about the hardiness and resilience of their families, in families with and without a child with Down syndrome. Mothers in Australia and New Zealand responded to The Family Hardiness Index (FHI), a standardised psychometric instrument, suitable for the purpose of this investigation. Furthermore the Australian cohort responded to an in-depth interview schedule, in which family hardiness was the main feature. Results suggest that there are minimal differences between families with and without a child with disability. Furthermore, mean differences between the two groups were not statistically significant. These findings point to increasing evidence that the previously identified pathology and dysfunction in families with a child with disability cannot be easily generalised to all families with disability, and in fact there exists emerging evidence that the presence of disability can have the opposite effect and provide families with a sense of hardiness or resilience. The close agreement between the means obtained in this study and the means of the published comparative data of the index suggests that the FHI is a suitable instrument to complement full family hardiness assessment.
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    Down Syndrome Research and Practice. 1998. doi:10.3104/reports.79
  • Reference and representation in children with Down syndrome
    Lois Moore, John Clibbens and Ian Dennis
    To maintain coherent discourse linguistic and non-linguistic information must be integrated using a mental representation of current discourse. The ability of children with Down syndrome to use mental representations in discourse was assessed in this study by investigating the use of referential forms using a narrative task. The narratives produced by children with Down syndrome were compared with those of typically developing children. The influence of certain contextual features on the ability of children with Down syndrome to use pronouns and other referential forms was also investigated. These features included: the relative status of the characters, the number of characters in the story, the method of presenting the story, and the position of a listener while narrating the story. Findings from this study indicate that typically developing children use a particular referential strategy when narrating a story. Children with Down syndrome do not use the same referential strategy. At a local level children with Down syndrome seem able to integrate sources of information in a mental representation of the discourse, but difficulty arises at a more general level.
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    Down Syndrome Research and Practice. 1998. doi:10.3104/reports.78
  • Sleep Neurophysiopathology in Down syndrome
    Raffaele Ferri, Lilia Curzi-Dascalova, Stefano Del Gracco, Maurizio Elia, Salvatore Pettinato and Sebastiano Musumeci
    In this paper, research on sleep characteristics of subjects with Down syndrome is briefly reviewed and some new data on autonomic function during sleep are reported. Subjects with Down syndrome show a significant reduction in percentage of REM sleep (probably more evident in the most severely retarded subjects), a marked delay in first REM latency and a statistically significant decrease in high-frequency rapid eye movements during REM sleep. The percentage of REM sleep in humans can be considered as an index of brain "plasticity" and the high-frequency REMs can represent an index of the brain ability to organize information; thus, these studies have provided a neurophysiological basis to a psychopedagogical approach for the treatment of learning and memory disabilities in Down syndrome. Moreover, children with Down syndrome show a clearly decreased peak amplitude of growth hormone during sleep; this causes the poor physical development in these subjects and might be related to the occurrence of sleep apnea. Obstructive sleep apnea has also been repeatedly reported in these children; however, if obvious risk factors are absent, children with Down syndrome tend to show the presence of central sleep apnea which is caused by a probable dysfunction of autonomic control at a brainstem level.
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    Down Syndrome Research and Practice. 1998. doi:10.3104/reports.83
  • Using augmentative communication with infants and young children with Down syndrome
    Phil Foreman and Geoff Crews
    This paper reports the use of two forms of augmentative and alternative communication (AAC) with young children with Down syndrome: a program using signing (Makaton), and the COMPIC system of computerised pictographs. Children with Down syndrome are frequently reported to have difficulties in the area of language and communication, with relative strengths in visual and perceptual areas. This suggests possible benefits from the use of AAC systems to enhance language development. The paper discusses the use of AAC systems to assist young children with Down syndrome, and reports an experimental study of the use of such systems with an object naming task.
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    Down Syndrome Research and Practice. 1998. doi:10.3104/reports.71
  • Teaching basic skills to children with Down Syndrome and developmental delays: The relative efficacy of interactive modeling with social rewards for benchmark achievements and passive observation
    Gerald Biederman, Fairhall, Raven and Valerie Davey
    In interventions attempting to remediate deficiencies in the skills repertoire of developmentally delayed children, no less than in medical interventions, it may be fairly said that less is more. That is, the instructor should intervene as little as possible both from the perspective of efficient instructional practice and from time allotment concerns which modern classrooms face. Evidence from this laboratory has indicated that in skills training for children with severe developmental delays the passive observation of a model demonstrating the target skill is more effective than interactive modeling involving hand-over-hand instruction with verbal prompting. We have considered the role of verbal prompting in interactive modeling and have found that prompts intended to provide typical social reinforcers are counterproductive (e.g. Biederman, Davey, Ryder, & Franchi, 1994). The present study examines the efficacy of hand-over-hand modeling with response-contingent verbal prompts. In such instruction, tasks are divided into identifiable sequential components, and the achievement of each component is marked by the delivery of some form of verbal prompt. In a within-subjects design, children were trained in one skill with response-contingent verbal prompts and in a second skill with simple passive observation. A separate group of children were trained with less rigorous verbal prompting in one skill and with passive observation in a second. Consistent with previous research, we found that passive modeling was overall significantly more effective than hand-over-hand modeling and moreover that passive modeling was significantly more effective than hand-over-hand modeling with response-contingent prompting. Our evidence therefore indicates that current classroom practice in training basic skills to children with severe developmental delays may require reassessment in that simple observation of modeled skills appears to be more effective than more labor-intensive instruction.
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    Down Syndrome Research and Practice. 1998. doi:10.3104/reports.72
  • Incidence of Helicobacter Pylori Antibodies in subjects with Down Syndrome
    Pinella Failla, Rosa Pettinato, Concetta Barone and Corrado Romano
    The Helicobacter pylorus (HP) is a Gram-negative bacterium that colonizes the antral portion of the stomach. It is the major aetiological agent responsible for chronic gastritis, gastric and duodenal ulcer disorders. This bacterium is widespread within the adult population (36.9%-53%), and the incidence of infection is positively correlated with age (6-20% in paediatric populations of Western Europe). The higher frequency among subjects with immunodeficiency and poor hygienic conditions has been pointed out. People with Down syndrome show higher susceptibility to this infection. The present study deals with the serological assay of IgG antibodies to HP in subjects with Down syndrome. We evaluated 77 subjects with Down syndrome, sex ratio M:F, 1:3, aged 1 to 40.7 years (mean 13.5). All were assayed for IgG antibodies to HP by the ELISA method. Subjects were separated into two groups according to age. The first group was made up of 46 subjects aged up to 14 years, and the second group contained 31 subjects over 14 years of age. Nine subjects (19.5%) in the first group and 16 subjects (51.6%) in the second group showed an antibody titre compatible with HP infection. The incidence in the whole sample of 77 subjects with Down syndrome was 32.4%. Therefore, people with Down syndrome do not seem to show a higher susceptibility to HP infection.
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    Down Syndrome Research and Practice. 1998. doi:10.3104/reports.85
  • Prelinguistic communication and Down syndrome
    Christine Jenkins and Brickchand Ramruttun
    The goal of this study was to examine the prelinguistic communication skills of children with Down syndrome. Ten children with Down syndrome, ten non-delayed children and five children with learning disabilities but not Down syndrome were matched for one word comprehension level on the Derbyshire Language Scheme. Data was gathered by completing the "Pragmatics Profile of Early Communication Skills" and by making ten minute samples of video recordings of mother and child engaged in low structured free play situations. Results and preliminary analyses showed that there was no significant difference in the extent to which children with Down syndrome use most non-verbal communicative behaviours compared to non-delayed infants when matched for comprehension. However, they were significantly delayed at using referential eye contact and words. These results and the issue of whether they are specific to children with Down syndrome are discussed in the light of contemporary findings.
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    Down Syndrome Research and Practice. 1998. doi:10.3104/reports.76
  • IGF-1 Levels in Down Syndrome
    Letizia Ragusa, Proto, Alberti, Corrado Romano, Rossodivita and Colabucci
    The authors examined IgF-1 plasma levels in 113 patients with Down Syndrome (DS) (68 males and 45 females, aged 1-23.66 years), all without severe congenital malformations, malnutrition, coeliac disease, alterations in thyroid or adrenal function, or taking any medication which might affect the study. The authors used a radioimmunoassay (RIA) method with plasma previously treated by acid-ethanol extraction and correlated the IgF-1 plasma levels with age and BMI (Body Mass Index) using multiple regression analysis. The BMI was higher in Down syndrome compared with normal subjects (NS), both in males (p=0.000006) and females (p= 0.034829). The two groups showed a positive correlation of IgF-1 levels with age. However, no correlation was found between IgF-1 levels and BMI.
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    Down Syndrome Research and Practice. 1998. doi:10.3104/reports.87
  • Development of a scale for the evaluation of listening behaviour of children with Down syndrome
    Rene Hugo, Brenda Louw and Alta Kritzinger
    Practical experience indicates a lack of clinical evaluation procedures for the evaluation of the listening behaviour of children with Down syndrome. This is especially important because these children are at risk for developmental communication delays concomitant to the high prevalence of recurrent otitis media and resultant auditory processing disorders. The aim of this study was to develop and then apply an evaluation procedure for listening behaviour to 10 children with Down syndrome. This scale was then evaluated in terms of its usefulness, by applying it to 56 children (younger than three years) attending an early intervention programme. Subsequently it was clinically used with 32 children with Down syndrome. It was found to be a useful and practical instrument for the assessment of listening behaviour.
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    Down Syndrome Research and Practice. 1998. doi:10.3104/reports.90
  • Down Syndrome in Kuwait: Recurrent Familial Trisomy 21 in Siblings
    Al Awadi, Naguib, Bastaki, Gouda, Mohammed, Abulhasan, Al-Ateeqi and Krishna Murthy
    Three young unrelated multiplex Kuwaiti families each confirmed to have 3 sibs with recurrent regular Trisomy 21 Down syndrome are reported. Chromosomal study of the parents showed normal karyotype for their mothers and two of their fathers (family II and III). These findings of multiplexes of regular Trisomy 21 patients resulted from the possibility of genetic predisposition for nondisjunction. Increased occurrence of Trisomy 21 in offspring of consanguineous marriages suggests that Trisomy 21 is etiologically heterogeneous and in a sub group of Trisomy 21 families nondisjunction may be genetically determined. In spite of extensive studies with regard to the origin of trisomy, the prime mechanism leading to recurrent Trisomy 21 or aneuploidy is not well understood. Genetic counselling of families with (maternal age <35 years), is a difficult task. Parental mosaicism, as well as "Genetic predisposition" should be considered in counselling families with sibships of regular trisomy-21.
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    Down Syndrome Research and Practice. 1998. doi:10.3104/reports.89
  • IGG Antibodies to Beta-Lactoglobulin and Cow's Milk Protein Intolerance in Down Syndrome
    Pinella Failla, Concetta Barone, Rosa Pettinato and Corrado Romano
    Cow's milk protein intolerance (CMPI) is the most frequent food intolerance in infancy. Its incidence ranges from 0.3 to 7.5%, according to different diagnostic criteria and clinical features. The diagnosis of CMPI is generally based on the level of IgG antibodies to beta lactoglobulin. An association of insulin-dependent diabetes mellitus (IDDM) with CMPI has been reported. CMPI in subjects with Down syndrome (DS) has been investigated by Nespoli et al. (1993) and Reichelt et al. (1994). We assayed IgG antibodies to beta-lactoglobulin, by the ELISA-method, in 78 subjects with Down syndrome and 97 mentally retarded and home-reared control subjects. Gastrointestinal disorders and IDDM was ruled out in both groups. Eleven (14.1%) and 5 (5.1%) subjects among Down syndrome group and control group respectively, were put on a CMP-free diet, and showed relief of symptoms and normalization of IgG antibodies after 6-21 months. In conclusion, CMPI appears to be more frequent in Down syndrome versus the control group and the specificity of IgG antibodies approaches 100% in our study.
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    Down Syndrome Research and Practice. 1998. doi:10.3104/reports.86
  • Why it is necessary to study the role of mitochondrial genome in Trisomy 21 Pathogenesis
    Svetlana Arbuzova
    An analysis of 190 pedigrees with Trisomy 21 suggests a cytoplasmic predisposition for the inheritance of Down syndrome and an association with other aneuploidies, non-chromosomal syndromes, autoimmune, neurodegenerative and oncological diseases.The extra chromosome seems to be responsible for the increase in free-radical intensity and the decrease of SOD-1 activity with age. The disturbance of the oxidant-antioxidant system could be the direct cause of this chromosomal nondisjunction. These data as well as the predominant maternal origin of the extra chromosome and the age-dependent incidence was the basis for the mtDNA sequencing in a donor of extra chromosome 21. Three new point, non-polymorphic mutations, not described before, were found in the following genes:- ATP-ase, G8764A and ND-5 G13243A with the same amino-acid substitutions Ala/Thr and in ND-1 G3337A-Val/Met. The mtDNA mutations detected in cases of Alzheimer's disease, insulin-independent diabetes, some cancers, and in somatically healthy people of 40 years and older could explain the connection of Down syndrome with these diseases and aging. Moreover, mtDNA analysis in patients with trisomy 21 might help to elucidate the nature of free-radical damage. This phenomenon is directly related to the manifestation of the syndrome and cannot be explained solely by the secondary SOD-1 gene dosage effect.
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    Down Syndrome Research and Practice. 1998. doi:10.3104/reports.88
  • An action research study on the provision of training for special needs support assistants by a speech and language therapy department
    Susan Dobson
    This action research project describes an investigation into the effectiveness of the provision of five courses by speech and language therapists. These courses attempted to clarify and establish the role of the speech and language therapists in one locality's schools and the role of the support assistants in relation to communication disabled children. The sixty support assistants attending the courses all worked with children who had statements of special educational needs which indicated that speech and language therapy was required. The project used one small questionnaire from twenty-seven special support assistants (SSA's) to identify the support assistants training needs. Interviews with two education officers established the courses relationship to existing education training policies. The views, feelings and perceptions of the seven therapists providing the courses and the twenty-four support assistants attending the courses were investigated by the qualitative research technique of semi-structured interviews. The investigation suggested course attendance seemed to have little impact on the SSA's role perception of speech and language therapy. The courses had however been popular, well received and had been successful in providing an appropriate style of presentation and information level. The investigation resulted in some suggestions for the planning of future training courses for support assistants by the speech and language therapy service and proposals for possible future changes to foster role definitions.
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    Down Syndrome Research and Practice. 1996. doi:10.3104/reports.61
  • Self-concept in children with Down syndrome
    Monica Cuskelly and Inge de Jong
    There are very few studies which have examined self-concept in individuals with an intellectual disability and none which have focussed on those with Down syndrome. The study found that children with Down syndrome with a developmental age of 4 to 6 years 11 months have a self-concept which is similar to that of normally developing children of a similar developmental age. The subscale means for both groups were positively skewed, and the subscale scores for both groups of children were found to be reliable over a one week period. Mothers' views of the self-concept of their child with Down syndrome were not correlated with children's own reports.
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    Down Syndrome Research and Practice. 1996. doi:10.3104/reports.63
  • Raising a child with Down syndrome: Parents' evaluations of health information and support
    Linda Eaves, Helena Ho, Barbara Laird and Sheryl Dickson
    This study surveyed over 200 caregivers of persons with Down syndrome about availability of health information and resources. Only a third had excellent resources with clear and consistent information to support the development of their child. Half knew about the Preventive Medicine Guidelines; 36% reported health concerns in their children. Most felt they were required to seek information on their own. Pediatricians, Infant Development Program workers and family physicians were all found to be important and helpful to parents. There was a clear need expressed for professionals and public to be better informed regarding Down syndrome and for improved access to information for themselves.
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    Down Syndrome Research and Practice. 1996. doi:10.3104/reports.64
  • The integration of children with Down syndrome in mainstream schools: Teachers' knowledge, needs, attitudes and expectations
    Heather Petty and Jane Sadler
    Nine mainstream primary teachers from one LEA in the North East of England (six with past and three with present experience of pupils with Down syndrome) took part in a study which aimed to identify those factors which may influence the outcome of full integration. Teacher knowledge, attitudes and expectations, levels of support and perceived needs were examined by means of semi-structured interviews and a questionnaire. A pilot information pack was developed on the basis of their perceived needs. Findings showed an increase in the number of children with Down syndrome integrated into mainstream primary schools, more pre-placement information regarding the child and higher levels of additional classroom staff than in the past. The main source of teacher knowledge was from background reading, the majority of teachers having received little or no input on SEN or Down syndrome during initial training or in-service apart from that offered by the Down Syndrome Association in two cases. While parental involvement was seen as an additional source of information by a few teachers, it was not generally either very frequent or highly valued. Teacher attitudes to integration and their expectations regarding the social and academic abilities of their pupils with Down syndrome varied considerably. This variation appears to be related not only to personal factors such as the perceived ability to meet the children's needs and degree of specialist knowledge, but also to external factors such as the degree of classroom support, information/resources and professional guidance available. All teachers who took part in the study were unanimous in the need to improve integration.
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    Down Syndrome Research and Practice. 1996. doi:10.3104/reports.59
  • Classroom behaviour, language competence, and the acceptance of children with Down syndrome by their mainstream peers
    Glynis Laws, Maura Taylor, Susan Bennie and Sue Buckley
    This study investigated the popularity of children with Down syndrome with their peers in mainstream classrooms using established sociometric techniques. The classroom behaviour of 16 children with Down syndrome aged 8 to 11 years were assessed and the relationship between these behaviours and acceptance investigated. For comparison, the relationship between classroom behaviour and acceptance of 122 typically developing children from the same classes was examined. The majority of the children with Down syndrome were found to enjoy average levels of acceptance in the class. Although behaviour problems were significantly worse in the children with Down syndrome, poor behaviour did not influence the other children to reject them. There was a different picture for typically developing children where there was a strong relationship between behaviour and peer acceptance. Language skills were also assessed for the children with Down syndrome. The children's language skills were not related to their popularity with the other children. Neither problem behaviours nor language difficulties influenced friendships in or out of the classroom for the children with Down syndrome.
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    Down Syndrome Research and Practice. 1996. doi:10.3104/reports.68
  • The effects of a short training in the use of a rehearsal strategy on memory for words and pictures in children with Down syndrome
    Glynis Laws, John MacDonald and Sue Buckley
    This study describes the effects of using a memory training programme with children with Down syndrome at schools for children with severe learning difficulties. The results suggest a small but significant improvement in memory spans for children at schools where they were trained by teachers or teaching assistants. There were no significant differences between auditory or visual stimulus presentation or between manual or verbal responses, but a three-way interaction between assessment point, stimulus presentation and response mode showed that memory and verbal recall of longer words particularly improved. Responses demonstrated a classic word length effect. One further finding was a significant correlation between reading and memory scores after the training.
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    Down Syndrome Research and Practice. 1996. doi:10.3104/reports.65
  • Working memory in children with Down syndrome
    Irene Broadley, John MacDonald and Sue Buckley
    A group of 4 to 18 year old children with Down syndrome (N=62) was presented with a set of working memory tasks, including auditory and visual serial recall of words; standardised digit span tasks and a rhyme judgement task. The serial recall tasks involved pictures of common objects or the spoken names of these objects and the children had to recall lists which varied on a number of parameters, including word length and the acoustic similarity of the object names. It was found that contrary to expectation the children's performance showed significant effects of word length and acoustic similarity, which are normally taken to indicate phonological storage and speech based rehearsal. These effects were found in both the auditory and visual presentation conditions and for the youngest age group. In addition to this evidence for speech based storage in short-term memory there was also evidence of the children utilising visual information in the serial recall tasks. The results are discussed in terms of working memory operation and the implications for memory remediation strategies in children with Down syndrome.
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    Down Syndrome Research and Practice. 1995. doi:10.3104/reports.44
  • Parents' reported responses to the disclosure of Down syndrome
    Elaine Herbert
    Few studies have centred on the reactions of fathers to the births of children with special needs, the majority have reported mothers' responses and their perceptions of the fathers' reactions and needs. Literature on the subject is sparse. This paper reports two studies which focused on the parents of ten pre-school children with Down syndrome. These studies explored mothers' and fathers' responses to (i) the disclosure of diagnosis and (ii) the events during the six months following disclosure. Study 1 involves the mothers of these children and study 2 was carried out with the fathers of seven of these children. The studies were carried out by semi-structured personal interviews. Some of the key issues raised by the fathers were the ways in which the diagnosis was disclosed, the initial and ongoing access and availability of professionals, the focus of services on mothers and children, their inability to discuss their feelings with their partners and their role as supporter.
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    Down Syndrome Research and Practice. 1995. doi:10.3104/reports.49
  • An exploratory study to investigate different methods for teaching sight vocabulary to people with learning disabilities of different aetiologies
    Lynda Hill
    Two contrasting theories of learning were compared to ascertain the most effective method for teaching sight vocabulary to children and adults with learning disabilities of differing aetiologies (Down syndrome and non-specific learning disabilities). A control group of children, matched for mental age, was included for comparison. Samuels' (1967) focal attention theory suggests that, for beginning readers, when a new word to be learned is accompanied by other stimuli, such as a related picture, less efficient learning occurs. Goodman's (1965) theory, however, would refute this. His theory suggests that the presence of a related picture improves learning. Results of the present study reinforced Samuels' theory. Children of normal ability learned best when the target words were presented on their own (p<0.02). All other individuals (children and adults) followed this trend, however, results did not reach statistical significance when learning conditions were compared. The study suggests that sight vocabulary is learnt most efficiently by all participants using a similar strategy, that is, when the target word is presented alone. However, before firm conclusions can be drawn, it is recommended that further study is carried out relating to both children and adults with learning disabilities. Findings will have an impact upon teaching practice.
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    Down Syndrome Research and Practice. 1995. doi:10.3104/reports.48
  • The influence of reading instruction on language and memory development in children with Down syndrome
    Glynis Laws, Sue Buckley, John MacDonald and Irene Broadley
    This paper reports evidence for the importance of reading instruction for memory and language development in children with Down syndrome. Language and memory measures for 14 children were obtained over nearly four years as part of our research investigating the effect of teaching memory strategies. Half of the children were readers or became readers in the course of the study. At the start of the study, there were no significant differences between readers and non-readers in vocabulary and grammar understanding, or in auditory and visual memory performance. By the end of the study, a significant advantage for the readers was noted for all language and memory measures. Possible reasons for these findings are discussed, as well as the implications for educational intervention.
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    Down Syndrome Research and Practice. 1995. doi:10.3104/reports.52
  • How do speech and language therapists work with support assistants attached to communication disabled children?
    Susan Dobson
    This paper analyses the role and training needs of classroom support assistants, as perceived by ten speech and language therapists. All identified the value of the assistants' detailed knowledge of the child, availability to the child to mediate communication in the classroom and natural 'maternal' style of interaction. However, the limitations of 'natural' responsiveness and the need for specific training is also recognised, as is the need to balance the benefit of an individual support for the child against the disadvantage of too much dependence on one person in the classroom.
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    Down Syndrome Research and Practice. 1995. doi:10.3104/reports.47
  • Comparison of physical and psychiatric status in individuals with translocation and trisomy 21 Down syndrome
    Vee Prasher
    No study to date has investigated clinical differences between adults with translocated Down syndrome and those with trisomy 21. Nine translocated Down syndrome individuals were matched to 9 trisomy 21 controls and assessed for medical differences. Significant findings included the translocated group having less severe learning disability according to ICD 10 criteria, less obesity and increased frequency of psychiatric disorders (in particular dementia and depression). However, on the Adaptive Behaviour Scale, the translocated group have significantly poorer independent functioning skills and more maladaptive behaviour, possibly as a consequence of the higher incidence of dementia and depression. Further studies investigating differences between the differing cytogenetic forms of Down syndrome is recommended.
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    Down Syndrome Research and Practice. 1995. doi:10.3104/reports.45
  • Differential diagnosis between Alzheimer's disease and hypothyroidism in adults with Down syndrome
    Vee Prasher
    The differential diagnosis between Alzheimer's disease and hypothyroidism in adults with Down syndrome who begin to show clinical deterioration needs to be emphasised. This study investigated clinical features which could be used to differentiate between the two conditions. Memory loss, mood and personality change, speech and gait deterioration, and slowing down were significantly associated with dementia but not with hypothyroidism. It is recommended that specific questions should be asked to elicit the presence of these features particularly in those individuals in whom assessment of biochemical thyroid status is not possible.
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    Down Syndrome Research and Practice. 1995. doi:10.3104/reports.46
  • Relationships between hearing and auditory cognition in Down syndrome youth
    Michael Marcell
    Twenty-six adolescents and young adults with Down syndrome and 26 IQ- and CA-matched youth with other causes of intellectual impairment (comparison group) repeated a battery of audiological and auditory-cognitive tests on three annual assessments. Audiological tests revealed the following differences between the group with Down syndrome and the comparison group: a) Poorer acuity and longitudinal declines at each frequency from 250-8000 Hz for the group with Down syndrome, particularly at the highest frequencies; b) A tendency for the middle ear problems of Down syndrome individuals to be bilateral, chronic, and to reflect no mobility, retraction, or reduced mobility of the tympanic membrane; and c) Poorer reception and discrimination of speech in the group with Down syndrome. Correlational analyses revealed the following reliable relationships between performance on audiological and auditory-cognitive tests: a) individuals with Down syndrome who had lower speech discrimination scores, poorer acoustic reflexes, or bilaterally impaired tympanograms repeated spoken sentences less accurately; b) individuals with Down syndrome who had lower speech discrimination scores performed more poorly on a language comprehension task; and c) individuals with Down syndrome with impaired hearing (regardless of how it was measured) identified fewer spoken words when the words were rapidly followed by a masking noise or made discriminable by brief consonant sounds. It was suggested that poorer performance by hearing-impaired subjects with Down syndrome on auditory-cognitive tasks may have been due to an interaction of lower auditory acuity and slower processing speed. Also, because relationships between hearing and cognitive variables were not present in the comparison group, it was tentatively suggested that hearing loss may be more detrimental to cognitive abilities in intellectually impaired individuals with Down syndrome.
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    Down Syndrome Research and Practice. 1995. doi:10.3104/reports.54
  • Investigating the literacy, language and memory skills of children with Down syndrome
    Angela Byrne, Sue Buckley, John MacDonald and Gillian Bird
    This paper presents the first phase of a longitudinal study following 24 children with Down syndrome who are receiving their education in mainstream primary schools. The literacy, numeracy, language and memory skills of the children with Down syndrome were compared to 2 groups of children selected from their classmates. The comparison groups were a group of typically developing children who were average readers in the classes and a group of children who were matched to the children with Down syndrome for reading age. The baseline data revealed that the children with Down syndrome had uneven cognitive profiles with relatively advanced reading skills compared to their other cognitive skills. The group of ordinary children who were matched to the children with Down syndrome on reading ability, attained significantly higher scores than the children with Down syndrome on all assessments other than reading. However, the reading matched group who were generally of below average reading ability for their age, were also significantly delayed relative to the average readers on measures of language, number and memory. As a group the average readers were average or above average on all measures. A cross-sectional analysis which divided the children with Down syndrome into 3 groups according to age and school year group indicates steady progress in all skills as the children move through primary school.
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    Down Syndrome Research and Practice. 1995. doi:10.3104/reports.51
  • Numerical ability, general ability and language in children with Down syndrome
    Joanna Nye, John Clibbens and Gillian Bird
    The aims of this study were to investigate the relationship between numerical and general ability and the contribution that receptive language makes to numerical ability in children with Down syndrome. Sixteen children with Down syndrome were tested on the following measures: two nonstandardised tests of numerical ability, two standardised numerical tests, two measures of receptive language and an IQ scale. Only one of the sixteen children attained a score on the IQ measure so the relationship between general and numerical ability in this population could not be assessed. All four numerical measures significantly correlated (positively) with each other, and receptive grammar (but not vocabulary) was found to significantly correlate (positively) to numerical skills. Details of the children's performance on the two main numerical measures under investigation are presented.
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    Down Syndrome Research and Practice. 1995. doi:10.3104/reports.55
  • Improving the expressive language skills of teenagers with Down syndrome
    Sue Buckley
    This article reports on the effectiveness of an intervention programme designed to improve the morpho-syntax used by teenagers with Down syndrome in their speech. The intervention used reading to teach the language. All but one of the teenagers were using longer, more complex sentences, in their conversation at the end of the year's training. The extent of individual progress was significantly related to speech production ability and comprehension of grammar at the start of the year. Future language intervention programmes should focus on both phonology and grammar.
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    Down Syndrome Research and Practice. 1995. doi:10.3104/reports.57
  • Long-term maintenance of memory skills taught to children with Down syndrome
    Glynis Laws, John MacDonald, Sue Buckley and Irene Broadley
    Children with Down syndrome who had followed a memory training programme were reassessed three years later. The programme, which involved training rehearsal and organisation strategies to improve short term memory, had resulted in significant gains on tests of auditory and visual memory skills. These gains were maintained for at least eight months after the end of the training period. However, after three years, memory capacity was found to have declined, although word spans were still significantly greater than those found before the training programme began. By comparing the performance of the children in the follow-up study with an untrained group matched for age, vocabulary and grammar understanding, it was concluded that this increase could be attributed to developmental progress and not to any residual effects of training. None of the children had continued to practice the memory training routines resulting in the loss of the trained memory skills over time.
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    Down Syndrome Research and Practice. 1995. doi:10.3104/reports.56
  • Screening of medical problems in adults with Down syndrome
    Vee Prasher
    Two-hundred and one adults with Down syndrome, living in the hospital and community, were assessed to ascertain prevalence of medical problems. Eighty-one percent of individuals examined were found to be overweight or obese (BMI >24), significant impaired vision was present in 18% and significant impaired hearing in 12%. A further 12% had a cardiac murmur and 15% of males had an undescended testis. Flat feet were found in the majority of subjects (70%) with xerosis in over a third of subjects (39%). A low heart rate and low blood pressure recordings were typical findings. A history of epilepsy was found in 8% of subjects. Particular concern needs to be given to obesity, sensory impairment and thyroid dysfunction and regular screening of all adults with Down syndrome is recommended.
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    Down Syndrome Research and Practice. 1994. doi:10.3104/reports.31
  • The language development of children with Down syndrome: First words to two-word phrases
    Bonamy Oliver and Sue Buckley
    A longitudinal study was conducted to investigate the early language development of children with Down syndrome. Using parental records of their children's speech, the study investigated very early language development, from first words to the consolidation of two-word phrases. Seventeen children were studied, all with Down syndrome and the age range of these subjects when the study began was one year to four years. The mean age for reaching the ten word stage was 27.3 months, a delay of around 12 months. The results showed that the children with Down syndrome studied here learned very similar words to those of typically developing children. The results also indicated that, as with typically developing children, some children with Down syndrome experienced a vocabulary explosion. The mean age for this language explosion was 30 months, with a mean vocabulary of 24.4 words. However, it was clear that there was a wide range of individual differences with some children showing no explosion. The mean age for two-word phrase consolidation was 36.9 months, suggesting a delay of around 18 months. The results indicated that, having reached the ten word stage, children with Down syndrome proceed in their language development at a slower rate to a two-word stage than typically developing children. Detailed individual profiles are included to illustrate the wide range of individual differences observed in the rate of language acquisition in this group.
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    Down Syndrome Research and Practice. 1994. doi:10.3104/reports.33
  • The acquisition of grammatical morphemes in children with Down syndrome
    Turid Rutter and Sue Buckley
    The aim of this study was to investigate the acquisition of grammar in children _ with Down syndrome, in particular to look at the production of morphological rules (stated by Brown 1973), and onset ages at which they are acquired in their language. In a study of three children in the United States, Brown found that between the ages of 2 and 4 years, typically developing children gradually incorporated a variety of different morphemes in their speech. Although there was little correspondence between the inclusion of separate morphemes and chronological age, there was a strong similarity in relation to the sequence in which the different morphemes appeared in their speech. In this longitudinal study the children with Down syndrome produce their first 10 words on average 12 months later than typically developing children, then the majority of the early morphological rules were in fact acquired by the children with Down syndrome, at a similar rate to typically developing children. This therefore would suggest that once the children with Down syndrome get going with the production of language, they in fact show a similar pattern as typically developing children in the early acquisition of grammar.
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    Down Syndrome Research and Practice. 1994. doi:10.3104/reports.34
  • The stability and transferability of errorless learning in children with Down syndrome
    Louise Duffy and Jennifer Wishart
    An errorless learning procedure was used to teach discrimination to two groups of children with learning disabilities, one composed of children with Down syndrome, the other of children without Down syndrome. Both groups responded positively to errorless teaching, with the children with Down syndrome requiring fewer training trials and showing better retention of the target discrimination than the children without Down syndrome. The errorless technique proved to have little value in teaching children with Down syndrome discrimination skills per se, however, since they showed little evidence of any transfer of learning to a second discrimination task. Errorless teaching strategies may nevertheless have an important role to play in increasing motivation to learn in children with Down syndrome, being most effective when used in conjunction with conventional trial-and-error methods.
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    Down Syndrome Research and Practice. 1994. doi:10.3104/reports.30
  • Down syndrome and quality of life: some challenges for future practice
    Roy Brown
    This article examines the perceptions and performance of a group of people with Down syndrome. This quality of life research is based on a six year study and compares the individuals' performance with others with developmental disabilities. Twenty-seven people with Down syndrome ranging in age from 18-43 took part in the project and received individually chosen intervention for the last three years of the study. Over the six years, their Verbal IQ increased significantly. They also made significant gains in applying reading, writing and numerical skills in social contexts. Surprisingly, social behaviour and performance in a work setting declined after community training according to the rating of service providers, suggesting that training leads to gains in behaviour which are useful in the community, but less valued in traditional agency services. Individuals tended to be more dissatisfied after intervention as they became more aware of their needs and therefore more critical of their environment.
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    Down Syndrome Research and Practice. 1994. doi:10.3104/reports.26
  • An investigation into the experiences of parents and head teachers involved in the integration of primary aged children with Down syndrome into mainstream school
    Kate Petley
    This study details the results of an investigation into the experiences of parents and head teachers involved in the integration of primary aged children with Down syndrome into mainstream schools. Ten families were involved in the study, each with a child who attended a mainstream primary school within Hampshire. Structured interviews were held with parents (mothers) of each of the ten children and nine of the head teachers in order to gain information about the process of integration and the experiences of those involved. The information elicited by means of these interviews was used to draw up a theoretical `model of good practice' which might facilitate the integration of a pupil with special educational needs into a mainstream school
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    Down Syndrome Research and Practice. 1994. doi:10.3104/reports.36
  • Early communication and cognition in children with Down syndrome
    David Messer and Patricia Hassan
    The relationship between cognitive abilities and communication continues to be a topic of interest and argument. These theoretical debates in psychology and linguistics have a relevance to the way we plan intervention. A modular view of language suggests that intervention should be focussed on language itself. Cognitive views suggest that intervention should be more broadly based. In our research we have examined predictions from cognitive theories which suppose that there are particular relations between communications and cognition. The research concerned a small group of children with Down's syndrome who were visited every four weeks over a period of six months. Our findings provide some limited support for one of the cognitive theories; this implies a linking of communication and cognition during certain periods of development. Another notable feature of our findings was the delay in language production, relative to other abilities in children with Down's syndrome.
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    Down Syndrome Research and Practice. 1994. doi:10.3104/reports.24
  • A comparison of the development of independence in two cohorts of young people with Down syndrome
    Billie Shepperdson
    Studies in infancy, teens and the mid-twenties were made of an original cohort of children with Down syndrome, born in the 60s and brought up at home in South Wales. The young people continued to improve in their self-care and daily living skills into adulthood, although no one had reached full independence. Studies were made in infancy and teens of a second comparative cohort of children with Down syndrome, born in the 70s and brought up at home in South Wales. In the teens, significantly more of the comparative cohort were independent in their self-care skills and daily living skills compared with the original cohort when they were in their teens, nine years earlier. It was not that there were individuals in the comparative cohort who were superior to individuals in the original cohort, rather there were far fewer in the comparative cohort with extremely limited achievements who were not reaching their potential.
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    Down Syndrome Research and Practice. 1994. doi:10.3104/reports.25
  • Short-term memory deficits and Down syndrome: A comparative study
    Anna Bower and Alan Hayes
    This study provides an evaluation of the short-term memory performance of children with Down syndrome (DS) and children with intellectual disability of other etiologies (ID/OE) on the Stanford-Binet 4th Edition (SB4). Results revealed a significant difference between the two groups for short term memory scores on the SB4, indicating that on short-term memory tasks children with Down syndrome function at a significantly lower level, than a group of intellectually disabled peers with other etiologies. Differences between visual and auditory short-term memory sub-scores for the two groups also were identified, with significantly lower scores for auditory short-term memory for the group with Down syndrome. Finally it was established that while the SB4 appears to be a suitable instrument for the identification of intellectual disability, the test is limited in its range of short-term memory subtests for young children with Down syndrome.
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    Down Syndrome Research and Practice. 1994. doi:10.3104/reports.29
  • Attainments in reading and number of teenagers and adults with Down syndrome
    Billie Shepperdson
    Studies were made of the reading and number abilities of two cohorts of people with Down syndrome. One cohort consisted of people who were born in the sixties, and the other of people who were born in the seventies. Both cohorts were seen in their teens, and the sixties cohort were also seen in their mid-twenties. The studies confirmed that some people with Down syndrome are able to master `academic' skills, and that some people not only retain skills, but continue to improve into the adult years. Comparing the two cohorts at teenage, more of the seventies cohort possessed `academic' skills, compared with the sixties cohort at the same chronological age, but the skills of the seventies cohort were not of a substantially higher order. Looking specifically at reading, in both cohorts teenage language scores were significantly related to reading scores but, for the sixties cohort, there was no relationship between language and reading scores by the mid-twenties. In the sixties cohort more girls than boys could read. In the teens this difference was not significant but it became so in adulthood. This difference between girls and boys was not found in the seventies cohort
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    Down Syndrome Research and Practice. 1994. doi:10.3104/reports.37
  • Prevelance of thyroid dysfunction and autoimmunity in adults with Down syndrome
    Vee Prasher
    This study investigated thyroid dysfunction in 160 adults with Down syndrome; mean age 43.4 years, 57.5% over the age of 40 years. Thirty-five percent had evidence of thyroid dysfunction with subclinical hypothyroidism (11.9%) and definite hypothyroidism (8.1%) being the commonest abnormalities. An association between thyroid dysfunction and thyroid autoimmunity was found (P= 0.01). Monitoring of thyroid status in subjects on thyroxine replacement was found to be poor. Recommendations are discussed.
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    Down Syndrome Research and Practice. 1994. doi:10.3104/reports.32
  • The voice of people with Down syndrome: An EMG biofeedback study
    Mary Pryce
    The laryngeal muscle tension of a group of thirty people with Down syndrome was compared with that of three other groups of people: those with learning disabilities, those with functional dysphonia (that is a voice disorder caused by misuse of the vocal mechanism) and a normal control group. The scores obtained were analysed by SPSS to determine whether there were significant differences in the way in which voice is produced by the four groups. The chief finding is that the energy level needed to activate the vocal mechanism from its at rest level to its voicing level is almost twice as great for the group with Down syndrome as for the control group. Implications for therapeutic interventions are considered. It is felt that new strategies to aid voicing need to be developed. The importance of keeping up fluid levels is highlighted.
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    Down Syndrome Research and Practice. 1994. doi:10.3104/reports.39
  • Actions speak louder than words: signing and speech intelligibility in adults with Down syndrome
    Gaye Powell and John Clibbens
    Previous studies into the use of key-word signing with people with a learning disability have concentrated on its contribution to the development of speech and language. The few studies focusing on its relationship to actual speech production and intelligibility were based on taught target words or phrases. This study, which was of quasi-experimental design, looked at whether reported improvements in intelligibility were supported in spontaneous speech production. Communication samples were collected by video recording `good' and `poor' speakers under `high' and `low' signing conditions. `Skilled' and `naive' raters assigned intelligibility ratings under `seen' and `unseen' conditions. It was predicted that speech from the `high' signing condition would be rated more highly than that from the `low' signing condition. This was supported. The iconicity of signs was shown to have a positive effect for `naive' raters when rating the `poor' speakers, which may counter previous arguments that the general public would be unable to understand communication attempts by people using key-word signing.
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    Down Syndrome Research and Practice. 1994. doi:10.3104/reports.43
  • Working memory in Down syndrome: Training the rehearsal strategy
    Annick Comblain
    Verbal short term memory skills of individuals with Down syndrome are very poor (Hulme and MacKenzie, 1992; Bower and Hayes, 1994). This study reports on the verbal short term memory skills of individuals with Down syndrome and on the possibility of increasing memory span durably by using a rehearsal training strategy. Three tasks (letter span, digit span and word span) were presented to two groups of 12 individuals with Down syndrome as a pre-test. A global span measure was established for each individual. Each group contained four children, four teenagers and four young adults. The groups had similar memory span and mental age at the beginning of the study. None of these individuals seemed to clearly rehearse. One group of 12 was exposed to an intensive rehearsal training during eight weeks (half an hour a week). The methodology was inspired from that used by Hulme and MacKenzie (1992), and partially from that used by Broadley and MacDonald (1993). The other group of 12 received no training. After the training, the three initial memory tasks were presented again to the two groups as a post-test. The trained participants significantly improved their memory span, whereas the non-trained participants did not improve at all. Only the trained individuals showed, at this time, clear signs of systematic rehearsal. Two other post-tests were presented to them, one six weeks and the other six months after the first post-test. The trained participants did not seem, at these times, to rehearse systematically any more. Their memory performances fell significantly lower than after the first post-test but remained significantly higher than at the beginning of the study.
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    Down Syndrome Research and Practice. 1994. doi:10.3104/reports.42
  • Phonological awareness in children with Down syndrome
    Ryka Evans
    Opinions vary as to whether phonological awareness is a necessary prerequisite for beginning reading, or a product of learning to read. This paper describes a study of phonological skills in six children with Down syndrome who were participating in a larger study of literacy development. The results indicated that some logographic reading ability had developed, while alphabetic and phonological skills were largely absent.
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    Down Syndrome Research and Practice. 1994. doi:10.3104/reports.38
  • Are children with Down syndrome able to maintain skills learned from a short-term memory training program?
    Irene Broadley, John MacDonald and Sue Buckley
    The ability of children with Down syndrome to maintain a set of trained short-term memory skills was assessed by follow up of a group who had previously undergone training in using rehearsal and organisation based memory strategies. That first study (Broadley and MacDonald, 1993) found that training in rehearsal and organisation skills led to an improvement in short-term memory ability in children with Down syndrome. That study also found that the effects applied across a wide age range; that the training could be conducted effectively by different people and that the type of training (rehearsal or organisation) acts independently, affecting only the targeted memory measures. The study reported here assesses the trained children's short-term memory abilities, 2 months and 8 months after the training had ended. Comparison with their own baseline performance and with a group of untrained children allowed assessment of the long and short term gains in memory performance. It was found that the trained children maintained the level of performance attained at the end of the training study. Training by keyworkers showed advantages for maintenance of some of the gains.
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    Down Syndrome Research and Practice. 1994. doi:10.3104/reports.41
  • Sleep problems in children with Down syndrome: A summary report
    Rebecca Stores
    This article presents a summary of the findings of the first part of a research programme being carried out at The Sarah Duffen Centre in Portsmouth. The findings will be published in full in the future. It follows an article published in the last edition of the journal entitled A preliminary study of sleep disorders and daytime behaviour problems in children with Down syndrome. The first stage of the current research programme has been an extensive survey of sleep problems in a group of children with Down syndrome compared with other children with learning disabilities and non-disabled children.The daytime behaviour of all these children and their mothers' stress levels have also been investigated. This information, as well as associations between sleep disorders, daytime behaviour problems and maternal stress will be reported in future publications.
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    Down Syndrome Research and Practice. 1993. doi:10.3104/reports.13
  • Attitudes and the experience of integration
    Anastasia Vlachou
    This paper is an attempt to raise awareness to attitudinal research regarding integration by highlighting some of the less visible factors that may influence teachers' attitudes towards the integration of students with Down syndrome. I make no claims for the general application or representativeness of the statements, but I will claim that the following analysis will raise some challenging issues for further thinking.
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    Down Syndrome Research and Practice. 1993. doi:10.3104/reports.14
  • Developing the speech and language skills of teenagers with Down syndrome
    Sue Buckley
    This paper reports on the outcomes of a project designed to evaluate the effectiveness of language teaching for teenagers. The aim was to improve the structure of the language used by teenagers with Down syndrome. Twelve teenagers took part in the study, which involved them in a variety of experimental and practical teaching activities over the period of a year. All the teenagers benefited from the intervention, though there were large individual differences in how much and why they benefited. Teaching which capitalised on the visual perceptual and visual memory strengths of these teenagers, by making use of reading, was the most effective. A key reason for delay in language development appears to be limited auditory short-term memory span
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    Down Syndrome Research and Practice. 1993. doi:10.3104/reports.12
  • Teaching short term memory skills to children with Down syndrome
    Irene Broadley and John MacDonald
    This study investigates a range of short term memory skills and the effectiveness of memory training procedures in improving these skills. The initial sample was 63 children with Down syndrome, aged 4-18 years, from two geographical areas in the UK. Phase 1 of the study assessed each child on a battery of tests including short term memory skills in different modalities, language skills, speech rate, word identification and a number of general IQ measures. Two groups were formed, one from one geographical area identified as the experimental group (n=25). A control group was formed from a subset of the remainder of children (n=26). Analysis verified that the two groups were similar and matched in terms of age and abilities. Phase 2 of the research consisted of a longitudinal training study of two memory strategies (rehearsal and organisation) which lasted for six weeks. For the experimental group (n=25), a cross-over design was employed to assess the effect of each strategy independently. Half the group received the rehearsal training first and the other half, the organisation-based training. Fifteen children from the group were taught by the first author and the rest by 'keyworkers'. In Phase 3 the initial assessment battery was repeated. The results demonstrated that each training programme was effective and enhanced only those specific memory skills addressed.
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    Down Syndrome Research and Practice. 1993. doi:10.3104/reports.11
  • A preliminary study of sleep disorders and daytime behaviour problems in children with Down syndrome
    Rebecca Stores
    The recent occurrence and severity of a range of sleep disorders were determined in a group of children with Down syndrome, and compared with those in a group of non-disabled children with the same mean age and similar Social Economic Status distribution. Associations were explored between the sleep disorders in children with Down syndrome and measures of their daytime behaviour. Frequently occurring sleep problems were found to be significantly more common in the group with Down syndrome compared with the non-disabled group. The most common sleep problems showed a different pattern in the two groups. In the children with Down syndrome, various significant associations were seen between the number of frequent sleep problems and specific types of disturbed daytime behaviour. Within the Down syndrome group, boys had significantly more frequent sleep problems than girls. The findings show that sleep problems are common in children with Down syndrome and that they are linked with disturbed behaviour during the day. The requirements for possible further research are discussed.
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    Down Syndrome Research and Practice. 1993. doi:10.3104/reports.8
  • Investigation of the articulatory patterns of young adults with Down syndrome using electropalatography
    Christine Hamilton
    Tongue movements for speech in three adults with Down syndrome and one normal speaking control were investigatedusing electropalatography (EPG) and diadochokinetic rate (DDK)techniques. The subjects with Down syndrome all showedsubstantially larger areas of tongue-palate contact comparedwith the control subject for alveolar sounds /t, d, n, l/, anddifferences in fricative articulation. Undershoot (incompleteclosure) was a feature of velar articulations /k, g/ in the subjectswith Down syndrome. Longer closure duration, longer consonanttransition times within clusters and consistently slower DDKrates were also noted. Asymmetry and variabilitywere evident in two of the subjects with Down Syndrome.The results support the hypothesis that people with Downsyndrome have difficulties with coordinating the rapid tonguemovements necessary for clear speech with impaired tonguemotility and motor programming as well as phonological delay.Implications for treatment are discussed.
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    Down Syndrome Research and Practice. 1993. doi:10.3104/reports.7
  • Integration does make a difference
    Anastasia Vlachou
    This paper is an attempt to present some of the mechanisms which contribute in promoting integration as a regular educational activity, and not as a distinctive special task which has to be transplanted in ordinary educational programming. Continuity, participation and an extended notion of integration are integral parts of such mechanisms
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    Down Syndrome Research and Practice. 1993. doi:10.3104/reports.19
  • The significance of maternal speech styles for children with Down syndrome
    Angela Byrne and Sue Buckley
    The aim of this study was to investigate whether mothers of children with Down syndrome are finding it difficult to provide their children with communicative experiences that might help promote linguistic progress beyond simple grammar, as a consequence of the children's own speech production difficulties. Nine children with Down syndrome were matched with nine typically developing children on level of receptive grammar. Video recordings of mothers engaging their children in ten minute conversations,in their own homes, were analysed. Mothers of children with Down syndrome made significantly more repetitions and expansions of their children's utterances than mothers of typically developing children did. Significant differences were not found in the amount of wh-questions, yes/no questions, tag questions, or two choice questions asked by mothers. Analysis of children's speech showed that children with Down syndrome had significantly shorter MLUs than the typically developing children. A greater percentage of children's longer utterances occurred when the preceding maternal utterance was a wh-question compared to a closed question. Almost three-quarters of closed questions received no verbal response or a single word answer from the child. Alternative strategies for managing conversations are discussed with the aim of extending the linguistic ability of children with Down syndrome.
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    Down Syndrome Research and Practice. 1993. doi:10.3104/reports.21
  • Group differences in response to charity images of children with Down syndrome
    Beth Miller, Robert Jones and Nick Ellis
    The advertisements produced by charities for people with learning disabilities have recently been the subject of much research. The present study investigated the responses of five different groups - school-children, university students, the `general public', care-staff and parents of children with Down syndrome - to two charity posters. Significant group differences were found across all measures, with university students and school-children being most likely to say they would donate money on seeing the poster. School-children were also most likely to predict a positive change of feelings on their next meeting, and to assign positive attributes to the person they saw in the poster, whilst care-staff and parents were significantly more positive than the other groups about the capabilities of people with Down syndrome. One initially depressing finding showed that the general public would be more likely to donate on seeing the more traditional, `guilt-evoking' poster. However, a closer analysis revealed that this group was actually the least likely of all the groups to donate money: those groups who were most likely to donate showed a slight preference in favour of the less stereotyped poster. Thus it is concluded that charities who are looking for donations do not need to rely on feelings of pity and guilt; and in fact, for reasons of both fund-raising and consciousness-raising, would do better to use images which are positive and non-stigmatising.
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    Down Syndrome Research and Practice. 1993. doi:10.3104/reports.22
  • Ocular disorders in children with Down syndrome
    Siegfried Pueschel and Stefan Gieswein
    Seventy-three patients with Down syndrome between the ages of 5 to 18 years were initially enrolled in this study and there were 68 patients in the final sample. Information was obtained from previous ophthalmologic examinations and parents completed a questionnaire pertaining to ocular disorders. Subsequently, the patients' visual acuity was assessed using Snellen or Kindergarten Test Charts for far vision testing and the Rosenbaum Pocket Vision Screener or the Child's Recognition and Near Point Test for near vision screening. A select group of children underwent a detailed ophthalmologic examination. The results of the parent questionnaire and data from the initial ophthalmologic screening are presented in Table 1 and 2, respectively. Results obtained from screening as well as from ophthalmologic evaluations indicate that 12 of 68 patients had bilateral poor vision (20/50 or below) and 15 patients were found to be amblyopic. Five of the 15 children with amblyopia had associated strabismus, another five had anisometropic amblyopia, two had both strabismus and anisometropia and three patients had no associate findings. This study suggests that children with Down syndrome may be at a greater risk for visual impairment than previously reported and that many of them may have amblyopia. Therefore, it is important that these children be examined ophthalmologically at regular intervals and treated appropriately if a visual disorder has been identified.
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    Down Syndrome Research and Practice. 1993. doi:10.3104/reports.23
  • Expressive language delay in children with Down syndrome
    Christine Jenkins
    The productive language skills of a group of children with Down syndrome were compared with those of normally developing children, and children with learning difficulties but not Down syndrome. The three groups of children were matched for their level of verbal comprehension skills. The children with learning difficulties were the same age as those with Down syndrome, the normally developing children younger. The South Tyneside Assessment of Syntactic Structures (STASS) was used to elicit language samples from the children, which were analysed using the Language Assessment Remediation Screening Procedure (LARSP) and percentage scores obtained. Although the children with Down syndrome produced as many utterances as the other two groups, their language contained significantly fewer Stage III and IV structures. They also showed a deficit in pronouns and auxiliary verbs used in comparison with the other two groups. Implications for language teaching are discussed in the light of these findings.
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    Down Syndrome Research and Practice. 1993. doi:10.3104/reports.6
  • A comparison between older persons with Down syndrome and a control group: Clinical characteristics, functional status and sensori-motor function
    Eli Carmeli, Shlomo Kessel, Joav Merrick and Shmuel Bar-Chad
    The increase in life expectancy within the general population has resulted in an increasing number of elderly adults with intellectual disability, and this is reflected in the increased life expectancy in persons with Down syndrome, currently about 56 years. The aim of this study was to study the clinical characteristics, the functional status and sensori-motor function of 10 older persons with Down syndrome (mean age 59 years), 13 younger persons with Down syndrome (mean age 44 years) and compare them with 38 adults with intellectual disability without Down syndrome and a control group of people without intellectual disability. All the persons with Down syndrome and intellectual disability resided in two residential living centres in Israel, while the 31 older persons without intellectual disability (mean age 75 years), who served as the control group, lived in an independent living facility. The study considered demographic data, medical backgrounds, physical and functional tests. The results showed that the older persons with Down syndrome in the study were more obese, shorter and had more medical problems than both the older persons with intellectual disability and the control group. The functional performance of the older adults with Down syndrome was more impaired in comparison with both other groups. It is postulated that their slower responses may be explained by a less physically active lifestyle, that may accelerate the onset of disease, resulting in symptoms associated with aging that are detrimental to health.
    doi:10.3104/reports.282
  • Developing mental imagery using a digital camera: A study of adult vocational training
    Ken Ryba, Linda Selby and Roy Brown
    This study was undertaken to explore the use of a digital camera for mental imagery training of a vocational task with two young adult men with Down syndrome. The results indicate that these particular men benefited from the use of a collaborative training process that involved mental imagery for learning a series of photocopying operations. An outline of a mental imagery assessment method is provided along with a description of the training procedures that were used in the study. Mental imagery was taught and rehearsed by means of a PowerpointTM computer presentation. Trainers and participants worked collaboratively to complete a task analysis and to take photos of each person performing the operations correctly. Through the use of self-modelling and feed forward, participants were able to learn from observing their own actions and, in particular, to learn from ‘successes’ that they have not yet had. On the basis of this pilot study, it is proposed that mental imagery training is an important new approach for collaborative training, especially for individuals whose language systems are not well developed. There is a need, however, for further investigation into the role of mental imagery as this relates to memory, self-regulation and metacognition.
    doi:10.3104/reports.279
  • Mental rotation abilities in individuals with Down syndrome - a pilot study
    Claire Hinnell and Naznin Virji-Babul
    This pilot study was designed to examine mental rotation ability in individuals with Down syndrome. 7 individuals with Down syndrome (mean mental age = 8.18 +/- 2.73 years; mean chronological age = 29.8 +/- 5.4 years) and a group of 9 typically developing children, matched for mental age, (mean mental age = 8.40 +/- 1.73 years; mean chronological age = 7.2 +/- 1.2 years) were given a version of Cooper and Shepherd's (1973) mental rotation paradigm. On each trial, participants viewed a symbol representing an upper case 'F' or a mirror image of an 'F'. The symbol was presented at one of eight different orientations. The participant's task was to determine whether the letter was reversed or non-reversed. Interestingly, both groups showed similar trends in increased reaction times with increasing angular disparity, suggesting that both groups were performing mental rotations. There was no significant difference in reaction time between the typically developing and Down syndrome groups, however, the Down syndrome group made significantly more errors than the typically developing group. Participants with Down syndrome were able to carry out the mental rotations at well above chance level and mental rotation ability was shown to correlate with mental age.
    doi:10.3104/reports.281
  • Changes in maternal age in England and Wales – implications for Down syndrome
    The risk of having a pregnancy with Down syndrome increases with maternal age. The percentage of all births in England and Wales to mothers aged 35 and over increased from 9% in 1989 to 19% in 2003. A 51% increase in the numbers of pregnancies with Down syndrome has been observed over the same time period (from 954 to 1440). Due to improvements in antenatal screening for Down syndrome and the subsequent termination of affected pregnancies, the total number of births with Down syndrome decreased from 770 in 1989 to 609 in 2003. However the number of births with Down syndrome to women aged 35 and over increased from 186 in 1989 to 310 in 2003 because of the increasing number of pregnancies amongst these women.
    doi:10.3104/reports.284

New from DSE: See and Learn Numbers

See and Learn Numbers is designed to help parents and educators teach children with Down syndrome basic number skills and concepts.

See and Learn Numbers is designed to teach young children to count, to link numbers to quantity, to understand important concepts about the number system and to calculate with numbers up to 10.

Now available as teaching kits and apps. Find out more...