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Adolescent injury risk behavior.

Health-risk behaviors that contribute to the leading causes of morbidity and mortality among adolescents and young adults are often established during adolescence and extended into adulthood. Unintentional motor vehicle injury is the leading cause of mortality in childhood and adolescence in developed countries. This review presents some of the risk factors found in research on unintentional injury and death in adolescence, including risk factors for siblings and adolescents with intellectual disability. These findings should be connected with the findings of a recent study that showed that about one third of all unintentional childhood injury deaths in the United States were preventable. For injury prevention to take place and being effective a multidisciplinary approach is needed to identify host, agent and environmental factors using epidemiology research and biomechanics. In the population of adolescents with intellectual disability there has been little research on injury epidemiology or injury prevention and the service provider will need to focus and educate staff on this issue in order to prevent injury that can result in further disability.

Accident Prevention↗
PubMed2006

Parental strategies for assisting children to wait.

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.

Adult↗
PubMed2003

Epilepsy and behaviour.

PURPOSE OF REVIEW: Whilst behaviour disorder is so commonly a challenge to physicians in the field of epilepsy, comparatively little is known of its exact association with the epilepsies. This review highlights advances in knowledge, focussing on a broad definition of behaviour to include psychiatric pathology. RECENT FINDINGS: Differential rates of disturbance appear to be associated with concurrent intellectual disability. Advances in imaging, in particular, suggest that structural brain changes may underlie the increased susceptibility in some epilepsy patients. SUMMARY: Clinical practice should recognize that, with the possible exception of seizure freedom in postictal behavioural disturbance, epilepsy treatment alone is unlikely to treat the associated psychological and behavioural disturbance. Research continues to focus on assessing the correlates of epilepsy and psychopathology whilst the association between epilepsy and behaviour disorder in people with an intellectual disability remains largely unknown.

Anxiety↗
PubMed2025

A novel frameshift variant leads to familial osteopetrosis with variable phenotypes in a Chinese Han consanguineous family.

Osteopetrosis, a group of highly heterogeneous genetic bone disorders, is characterized by deafness, increased bone density, hepatosplenomegaly, pancytopenia and intellectual disability. Osteopetrosis can be divided into three subtypes: autosomal recessive osteopetrosis (ARO), intermediate autosomal recessive osteopetrosis (IARO), and autosomal dominant osteopetrosis (ADO). CLCN7 has been reported to be the most common gene responsible for the ADO-II subtype. In this study, a novel variant, c.175dupA (p.Met59Asnfs*8), of CLCN7 was identified in a Chinese Han consanguineous family with suspected ADO-II. The proband was homozygous for the p.Met59Asnfs*8 variant and exhibited multiple severe phenotypes, including deafness, short stature, brittle bones, optic atrophy, hepatosplenomegaly, intellectual disability, cleft palate and recurrent infection. However, except for the mother of the proband, who presented a series of clinical phenotypes caused by bone marrow failure, all the other family members who were heterozygous had no obvious abnormal phenotypes. Our study suggested that the novel variant p.Met59Asnfs*8 in CLCN7 was very likely pathogenic factor in our suspected ADO-II family. The phenotypes of heterozygous carriers may be affected by incomplete penetrance. Loss of function of CLCN7 caused by nonsense-mediated mRNA decay (NMD) due to the frameshift variant was likely the underlying pathogenic mechanism. This study broadened the mutation spectrum of CLCN7, provided a foundation for timely and effective clinical intervention for related diseases, and demonstrates the importance of genetic counselling.

Adult↗
PubMed2001

Life expectancy among people with cerebral palsy in Western Australia.

This report describes trends, predictors, and causes of mortality in persons with cerebral palsy (CP) using individuals identified by the Western Australian Cerebral Palsy Register and born between 1958 and 1994. Two thousand and fourteen people were identified (1154 males, 860 females), of whom 225 had died by 1 June 1997. Using date-of-death data, crude and standardized mortality rates were estimated and predictors of mortality sought using survival analysis stratified by decade of birth, description of impairments, and demographic and perinatal variables. For those born after 1967, the cause of death profile was examined over time. Mortality exceeded 1% per annum in the first 5 years and declined to age 15 years after which it remained steady at about 0.35% for the next 20 years. The strongest single predictor was intellectual disability, but all forms of disability contributed to decreased life expectancy. Half of those with IQ/DQ score <20 survived to adulthood, increasing to 76% with IQ/DQ score 20-34, and exceeding 92% for higher scores. Severe motor impairment primarily increased the risk of early mortality. Despite there being 72 persons aged from 25 to 41 years with severe motor impairment in our data set, none had died after the age of 25 years. Infants born after more than 32 weeks' gestation were at significantly higher risk of mortality than very preterm infants, accounted for by their higher rates of intellectual disability. No improvements in survival of persons with CP were seen over the study period despite advances in medical care, improved community awareness, and the increasing proportion of very preterm births among people with CP. This may be the result of improved neonatal care enabling the survival of infants with increasingly severe disabilities.

Adolescent↗
PubMed2006

Aspects of digestive tract tumors in Down syndrome: a literature review.

The purpose of this study was to describe the digestive neoplasms found in persons with Down syndrome. Due to intellectual disability, persons with Down syndrome do not convey their symptoms and pain, leading to delayed diagnosis and potentially worse outcome. It is thus important to know which organs are at risk for tumors and possible tumor risk factors. In a review of the literature, we found 13 benign tumors and 127 cancers in 1 fetus, 8 children, and 131 adults with Down syndrome. The review suggests a decreased incidence of digestive cancer, however, with a possible increased incidence of neoplasms of the pancreas and gallbladder. The distribution of cancers is distinct from that in the general population and that in persons with other intellectual disabilities who share the same life conditions, suggesting that constitutional protective factors exist. This review may allow a more specific, adapted medical follow-up for persons with Down syndrome and could help to elucidate the oncogenesis of digestive neoplasms.

Colorectal Neoplasms↗
PubMed1998

Health registers as a feasible means of measuring health status in childhood--a 7-year follow-up of the 1987 Finnish birth cohort.

Follow-up studies on health have usually been based on ad hoc cohort studies in which detailed information is collected specifically for research purposes on a certain group of people. The increasing collection of routine health data provides an alternative method of gathering follow-up data. In this study, the feasibility of using routinely collected health-register data and data linkages to follow up children's health was investigated. Five nationwide registers, 18 regional registers of intellectually disabled children and school administration data in one county were found to be of use for our follow-up and were combined with the 1987 Finnish Medical Birth Register (n = 60,254 births). In the follow-up, 62 children were untraced (0.1%), 327 were stillborn (0.5%), 440 died after birth (0.7%) and 287 emigrated (0.5%) before the age of 7 years. The cumulative incidences for all diseases (8.9% of all children living in Finland at the age of 7 years), for diabetes (3.0/1000), for epilepsy (6.8/1000) and for asthma (34.2/1000) correspond to the estimates of other studies, but our estimate for intellectual disabilities (18.0/1000, of whom 18% were reported to have an IQ of 70 or less) seems to be an underestimate. Our data collection did not provide reliable information on institutionalised children or children taken into care. Data collection conducted by using health registers is a feasible method, and it saves both time and financial resources compared with cohort studies. Potential problems with data linkage studies are variation in the content of data and in data quality of different registers and data protection issues.

Adolescent↗
PubMed2006

Successful experience of people with Down syndrome.

The aim of this study was to investigate the successful experiences of people with Down syndrome to provide insights into the potential of people with intellectual disabilities. A qualitative approach was adopted, and 10 recipients of the Stevie Award for Outstanding Persons with Down syndrome were interviewed. Interviews with the participants' parents and other significant persons were also conducted. Content analysis and constant comparative method were adopted for data analysis. All 10 participants achieved in life when they demonstrated the motivation and perseverance to try new things and active involvement in extra-curricular activities and volunteer service. Role modeling and support from parents and teachers helped participants to develop talents in sports and arts. Effective family support and ongoing collaboration among family, school and community facilities are vital in enabling people with intellectual disabilities to develop their potential for a life of quality.

Achievement↗
PubMed1992

Assessment and training of functional conversation behaviour.

Measures for the assessment of initial, dyadic conversations are critically reviewed, and an alternative approach is described. The Verbal Interaction Analysis System is illustrated by two case studies featuring mildly to moderately intellectually disabled adults. These studies show how the VIAS provides an index of competence and assists in the development of specific treatment strategies. Social validity is examined in each case, and suggestions are made for improvements to the VIAS.

Adult↗
PubMed1996

A preliminary analysis of the effects of prior classroom conditions on performance under analogue analysis conditions.

We systematically manipulated two prior classroom conditions to explore their effects on performance under analogue assessments for a 7-year-old girl with severe intellectual disabilities who exhibited aggressive behavior. Results demonstrated consistently higher levels of aggression during demand analogue conditions when they were preceded by one of the classroom conditions.

Aggression↗
PubMed2006

Verbal short-term memory in individuals with congenital articulatory disorders: new empirical data and review of the literature.

BACKGROUND: To investigate the nature of the articulatory rehearsal mechanism of the Articulatory Loop in Baddeley's Working Memory model, it seems particularly important to study individuals who developed a deficit (dysarthria) or total abolition (anarthria) of the ability to articulate language following a cerebral lesion. METHOD: In this study, a forced-choice recognition procedure for word sequences of increasing length was used to evaluate verbal short-term memory in nine individuals with severe congenital motor and verbal disabilities (seven anarthric and two severely dysarthric) and associated intellectual disability (ID) and 30 normal children of comparable mental age. RESULTS: The normal children exhibited classical phonological similarity effects (better performance on acoustically dissimilar than on similar word lists), word length (greater accuracy on two-syllable than on four-syllable word lists) and frequency of occurrence (an advantage of high-frequency over low-frequency words). Instead, all of these effects were lacking in the experimental group. CONCLUSIONS: These data suggest that persons with congenital anarthria/dysarthria and ID present defective maturation at many levels of the Articulatory Loop and reduced contribution of semantic-lexical processing in the temporary retention of phonological sequences. It is likely that in these individuals both communicative deficits and ID play a role in the impaired development of verbal short-term memory abilities.

Adolescent↗
PubMed2006

Spectrum of general surgical problems in the developmentally disabled adults.

OBJECTIVE: This study highlights the spectrum of general surgical problems necessitating admission on intellectually disabled adult patients. Problems encountered in the management and the ways to overcome various difficulties are highlighted. METHODS: Prospective collection of data on 63 consecutive developmentally disabled adult patients admitted to the Department of General Surgery, Riyadh Medical Complex (RMC), Riyadh, Kingdom of Saudi Arabia for various indications from January 2000 through December 2004. Demographic details, clinical presentation, diagnostic modalities, associated physical and neurological disabilities, coexisting medical condition, treatment options, morbidity and mortality were analyzed. Various difficulties encountered during the management and mean to overcome these problems are addressed. RESULTS: Sixty-three patients accounted for 71 admissions. Mean age was 26.7 years with a male preponderance (4.25:1). Fifty-four patients were admitted for various emergency conditions. History of pica could be obtained in 33% of the cases. Twenty-seven patients were admitted for acute abdomen. Volvulus of the colon (22.2%) and pseudo-obstruction (18.5%) were the most common causes of acute abdomen. Twenty-one patients were admitted with upper gastrointestinal bleeding. Reflux esophagitis was the most common cause of bleeding (62%). Overall morbidity was 41% for emergency admissions and 22% for elective surgery. Hospital mortality was 21.4% for emergency surgery. There was no death in elective cases. CONCLUSION: Developmentally disabled patients comprise a special class of patients with peculiar management problems. The treating clinician should be aware of various unexpected conditions not found as frequently in the normal patient population. Apparent lack of pain does not exclude an acute emergency. Possible surgical condition should be suspected if there is vomiting, abdominal distension, fever, increased irritability of recent onset. Male gender and history of pica are added risk factors.

Adolescent↗
PubMed1996

Increasing on-task behaviour through interruption-prompting.

This study was designed to assess the effects of response-contingent interruption-prompting of stereotypic behaviour on on-task behaviour, inactivity and inappropriate behaviour. Also, the relationship between these behaviours was investigated. Five individuals with severe intellectual disability and autism participated. Data were collected within a reversal design. The results showed a statistically significant increase of on-task behaviour when interruption-prompting of stereotypic behaviour was in effect. Inactivity was statistically related to inappropriate behaviour. In terms of the continuous effort to identify procedures that are least intrusive for the client to attain behaviour improvement, it is suggested that interruption-prompting of stereotypic behaviour may be a reasonable choice for practicians.

Adolescent↗
PubMed1998

Two women with multiple disabilities sharing an acoustic orientation system and traveling together to indoor destinations.

This study assessed whether two women with total blindness and profound intellectual disability could share an acoustic orientation system and travel together simultaneously to common indoor destinations to perform occupational and vocational activities. The orientation system provided acoustic cues which indicated the direction to the destinations. Analysis of data indicated that the women were successful in sharing the system and could reach the destinations independently.

Acoustic Stimulation↗
PubMed2000

Implicit learning differences: a question of developmental level?

A. S. Reber's (1992) proposition that the implicit learning system should demonstrate invariance of intellectual level (IQ) was examined by comparing 20 children with intellectual disability (mean mental age [MA] = approximately 5.8 years) with 20 intellectually gifted children (mean MA = approximately 12.4 years) of similar chronological age (CA; approximately 9.5 years). Implicit learning was assessed using a task involving covariation of 2 incidental cues. Explicit learning was assessed using a task of similar logical structure. Contrary to the IQ-invariance proposition, implicit learning as well as explicit learning varied with intellectual level. A secondary aim was to distinguish the contributions of CA, IQ, and MA to implicit learning. This was done by combining the samples of children in the present study with 2 samples of younger and older children of average ability from a study by M. Maybery, M. Taylor, and A. O'Brien-Malone (1995). Analyses showed that MA is critical to implicit learning.

Association Learning↗
PubMed1993

Chromosome testing in children with developmental delay in whom the aetiology is not evident clinically.

A review was carried out to establish the value of chromosome testing in children with significant developmental delay, where the aetiology was not evident clinically. During 1990, 315 children had been assessed at a child development clinic and found to be significantly delayed in one or more areas of development; in 256, the aetiology was not evident clinically. Chromosome testing of these children revealed an abnormality in 10 (3.9%). Thirty children had dysmorphic features; six (20%) of these had an abnormal karyotype. Four (2%) of the 226 who had no dysmorphic features had a chromosome abnormality. One hundred and fifty-five children had intellectual disability; eight (5%) of these had an abnormal karyotype. Two (2%) of 101 who had a specific delay in their development had a chromosome abnormality. The advantages of chromosome testing in children with developmental delay in whom the aetiology is not evident clinically are discussed.

Child↗
PubMed2004

Sexual and nonsexual offenders with intellectual and learning disabilities: a comparison of characteristics, referral patterns, and outcome.

This article reports an evaluation of a community intellectual disability offender service over the period from 1990 to 2001. Men who committed sex offenses or sexually abusive incidents (n = 106) and men who committed other types of offenses and serious incidents (n = 78) are compared on personal characteristics, referral sources, forensic details, and outcome up to 7 years after referral. The cohorts are older than one would expect from the criminology literature, and, at about 33%, the incidence of mental illness is consistent with some previous studies. A greater proportion of sex offenders had criminal justice involvement and a formal disposal from court. Fire raising was not overly represented as an offense. There was a higher rate of reoffending in the nonsexual cohort, which persisted up to 7 years. Investigating only reoffenders, there was a considerable amount of harm reduction recorded up to 7 years, statistically significant up to 5 years following initial referral.

Adult↗