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The effects of low-dose fentanyl on emergence agitation and quality of life in patients with moderate developmental disabilities.

STUDY OBJECTIVE: To analyze whether emergence agitation could be reduced by using a low dosage of fentanyl without causing an increase in postoperative adverse effects and/or affecting the patient's quality of life after discharge. DESIGN: Randomized, controlled crossover trial. SETTING: University medical center. PATIENTS: One hundred ten ASA physical status I and II ambulatory patients with moderate developmental disabilities who received anesthesia for dental care. INTERVENTIONS: During their first visit, patients were randomly treated either with a placebo or a 1- to 1.5-microg/kg dose of fentanyl before inserting a reinforced laryngeal mask airway and treated with the remaining method during their second visit 6 to 12 months later. MEASUREMENTS: Measured variables included anesthetic and emergence period, the occurrence of emergence agitation, and postanesthetic adverse effects such as nausea and vomiting. After discharge, the patient's quality of life was evaluated based on disturbances in daily activity, eating habit, and sleeping behavior. Results were analyzed by Student t test and McNemar test (P < 0.05). MAIN RESULTS: Between the two treatments, there was a significant difference in the frequency of emergence agitation (P = 0.04) but no postoperative adverse effects such as postoperative nausea and vomiting. We also found no significant disturbances in the patient's quality of life according to daily activity, eating habits, or sleeping behavior. CONCLUSION: Emergence agitation may be reduced by using 1 to 1.5 microg/kg of fentanyl during induction while maintaining anesthesia by sevoflurane in patients with moderate developmental disabilities undergoing dental care, without causing an increase in postoperative adverse effects and affecting the patient's life quality after discharge.

Adolescent↗

Psychotropic medication use in people with developmental disabilities.

Psychotropic medications are frequently used to treat undesirable behaviors in persons with developmental disabilities. Successful use of these drugs is dependent on accurate assessment of the psychiatric disorder or behavioral problem. Treatment of aggression and self-injurious behavior and the use of antipsychotics, antidepressants, mood stabilizers, anxiolytics, beta-blocking agents, and naltrexone will be discussed.

Aggression↗

Mechanism for testing operant conditioning and body rocking in developmental disabilities.

A mechanism is described for testing operant conditioning and body rocking in developmentally disabled persons. The system is made up of a reinforcement dispenser, a lever-pulling mechanism, and flexible chair back for testing body rocking. Experience with the system has shown it to be sturdy and reliable, and the reinforcement dispenser to be adaptable to a wide range of food preferences.

Conditioning, Operant↗

Psychiatric disorders in adolescents with developmental disabilities: longitudinal data on diagnostic disagreement in 150 clients.

The current paper describes the prevalence of psychiatric diagnoses in a large sample (n = 150) of adolescents with developmental disabilities who were hospitalized for inpatient psychiatric treatment. Differential diagnoses made during their inpatient stay in a specialty unit for the assessment and treatment of dually diagnosed adolescents are presented and contrasted with longitudinal/historical data on these same patients' diagnoses prior to admission. Results indicate that these individuals received a wide spectrum of diagnoses during their adolescent years. The paper offers indirect support that correctly diagnosing psychiatric conditions is often challenging in adolescents with developmental disabilities. Factors related to diagnostic complexity and misdiagnoses (false positives and false negatives) are discussed. Longitudinal data on psychotropic medication usage for these individuals are also presented.

Adolescent↗

The Behavior Problems Inventory: an instrument for the assessment of self-injury, stereotyped behavior, and aggression/destruction in individuals with developmental disabilities.

The Behavior Problems Inventory (BPI-01) is a 52-item respondent-based behavior rating instrument for self-injurious, stereotypic, and aggressive/destructive behavior in mental retardation and other developmental disabilities. Items are rated on a frequency scale and a severity scale. The BPI-01 was administered by interviewing direct care staff of 432 randomly selected residents from a developmental center between the ages of 14 to 91 years. For 73% of those selected, at least one problem was endorsed on the BPI-01. A total of 43% showed self-injury, 54% stereotyped behavior, and 38% aggressive/destructive behavior. Confirmatory factor analysis and item-total correlations supported the three a priori factors. Analyses of variance (ANOVA) showed that of the variables age, sex, and level of mental retardation, only the latter had a significant effect on the BPI-01 total score, the SIB subscale score, and the Stereotyped Behavior subscale score. Aggression/destruction was not significantly related to any of the three variables. Individuals with a diagnosis of pervasive developmental disorder had higher scores on all three subscales than those without, whereas residents with a diagnosis of stereotyped movement disorder had higher Stereotyped Behavior scale scores than those without. The BPI-01 was found to be a reliable (retest reliability, internal consistency, and between-interviewer-agreement) and valid (factor and criterion validity) behavior rating instrument for problem behaviors in mental retardation and developmental disabilities with a variety of potentially useful applications. Strengths and limitations of the instrument are discussed.

Adolescent↗

Job satisfaction of childcare workers for children with developmental disabilities in Hong Kong.

The importance of early intervention for young children with developmental disabilities is recognized in different parts of the world. In Hong Kong, the special childcare worker (SCCW) is one of the key professionals responsible for intervention programs for these children. This study explores the level of job satisfaction of SCCWs with a view to identifying factors and issues relevant for policy-makers. Contrary to common belief, the 105 respondents surveyed were satisfied with their work, although not to a great extent. The main sources of job satisfaction were the progress made by the children and the appreciation shown by the parents; job dissatisfaction stemmed from insufficient support and training. Developments on the personal, agency and professional levels were discussed in relation to the enhancement of job satisfaction.

Caregivers↗

Partner sensitivity to communication behavior of young children with developmental disabilities.

Aspects of partner sensitivity to communication behaviors of 24 presymbolic children with developmental disabilities were examined. The children were grouped according to their movement abilities (normal vs. abnormal patterns) and communication status (intentional vs. preintentional). Participating communication partners were those with whom the children interacted on a regular basis and included their mothers, early childhood special educators, and speech-language pathologists. Procedures were developed whereby the partners served as informants in order to provide information regarding (a) recognition of the children's communicative cues and (b) consistency of cue recognition and descriptions across partners. Results indicated wide individual variability in the partners' basic recognition abilities as well as their consistency with each other. Further, the observed variations could not be attributed to the children's movement and communication abilities. It was concluded that sensitivity, as measured in the present investigation, was highly partner-child specific, with some children likely to be exposed to more optimal interactions than others.

Child, Preschool↗

Problem behavior and psychiatric impairment within a developmentally disabled population. III: Psychotropic medication.

This report is the third in a series on problem behavior and psychiatric impairment in a population of 35,000 individuals receiving developmental disabilities services. Young and middle-aged adults were found to receive psychotropic medication at higher rates than children, adolescents, or elderly persons. Psychotropic receipt rates were found to increase with increasing severity of mental retardation, but most evidently with increasing restrictiveness of residential setting, increasing rated severity of problem behaviors, and presence of a psychiatric impairment. Rates of medication receipt also varied as a function of psychiatric diagnostic category. Discussion remarks emphasize the need to include information relative to clinical and social aspects of program settings and the roles and decision-making performance of physicians and psychologists in research on settings serving persons with developmental disabilities.

Adolescent↗

Attenuating growth in children with profound developmental disability: a new approach to an old dilemma.

Caring for children with profound developmental disabilities can be difficult and demanding. For nonambulatory children with severe, combined neurologic and cognitive impairment, all the necessities of life must be provided by caregivers, usually parents, and these tasks become more difficult as the child grows to adolescence and adulthood. Many parents would like to continue caring for their child with special needs at home but find it difficult to do so as the child increases in size. If growth could be permanently arrested while the child was still small, both child and parent would likely benefit because this would facilitate the option of continued care in the home. Treatment of the child with high-dose estrogen, initiated at an early age, could provide this option. High-dose estrogen both inhibits growth and rapidly advances maturation of the epiphyseal growth plates, bringing about permanent attenuation in size after a relatively short period of treatment. We present a case report and discuss the medical and ethical considerations of such an intervention strategy. We suggest that after proper screening and informed consent, growth-attenuation therapy should be a therapeutic option available to these children should their parents request it.

Administration, Cutaneous↗

Relaxation training as a treatment for chronic headaches in an individual having severe developmental disabilities.

We have assessed effects of a simplified relaxation training on the frequency of headaches and consumption of analgesic headache medication in an adult male with severe developmental disabilities as well as chronic mixed headaches. The subject received Behavioral Relaxation Training (BRT) after a baseline period during which frequency of headache complaint, analgesic medication consumption, and independent relaxation behaviors were monitored. BRT consists of the utilization of modeling, prompting, feedback, and positive reinforcement in order to establish and maintain the subject's participation in 10 overt relaxed postures. The behaviors were learned to at least an 80% proficiency during a 10-minute alternating self-regulatory (1 min)/corrective feedback (1 min) relaxation phase across several sessions. Headache complaints were reduced by 48% and analgesic medication consumption by 51% as assessed during a 2-month posttreatment evaluation. These results should be considered not only as support of BRT as a viable method of relaxation training but also as a suggestion that BRT and other self-regulatory treatment should be considered for use with individuals having moderate to severe developmental disabilities.

Adult↗

Role of professional nurses in the field of developmental disabilities.

The definition and clarification of the role of professional nurses on the interdisciplinary team was the focus of this paper. Professional services that other team members, administrators, individuals with developmental disabilities, and their families may expect from registered nurses were outlined. The AAMR Nursing Division Position Statement was presented in order to inform the public and provide a framework of practice for professional nurse specialists in the field of developmental disabilities.

Education of Persons with Intellectual Disabilitie↗

Who will treat psychiatrically disturbed developmentally disabled patients? A health care nightmare.

The authors assess the impact of a policy adopted briefly in New York State to facilitate short-term hospitalization and long-term aftercare of psychiatrically disturbed, developmentally disabled patients. Denied admission to New York State facilities that provide long-term care for the psychiatrically disturbed or the developmentally disabled, these dually diagnosed patients have often languished in the emergency room of municipal hospitals or been inappropriately hospitalized in the acute-care psychiatric unit, sometimes for several months. The authors conclude that the policy expedited hospitalization but failed to facilitate patients' discharge because it did not address the underlying need for suitable aftercare facilities. Overlapping clinical and administrative issues and political exigencies that complicated the policymaking process are discussed.

Adult↗

Health care financing for severe developmental disabilities.

The 1985-86 data from 308 children and young adults under age 25 with autism and from 326 with severe or profound mental retardation can be compared to national data from the 1980 MNCUES and the 1987 NMES because the methods are similar. These data provide detailed answers to the questions, what health care services are used? what are the expenses? Who pays them? Until now, the absence of comprehensive national data had hindered the development of new approaches to financing the care of children with serious, lifelong conditions. These data permit policymakers to take into account the needs and expenditures for severely developmentally disabled children when reforming the health care financing system. None of the children or young adults had expenditures in excess of $50,000, and very few reached the upper $20,000s. For children with autism the average annual health care expenditure was about $1,000 and about $1,700 for young adults, compared to the $414 average for all American children. They received an average of four physician visits annually, slightly above the U.S. average for children. Their hospitalization rate was twice the average for children. Hospitalization accounted for one-third the health care expenditures among children with autism, but for two-thirds among young adults. For children and young adults with severe retardation the average expenditure on health care was about $4,000, due to the physical impairments in two thirds of the children. They averaged about 12 physician visits annually, falling to 8 among young adults. Children were hospitalized about eight times the national rate, and young adults about twice. Among severely retarded children and young adults living at home, hospitalization accounted for over half the health care expenses, but for only one third for those in residential placement. Unfortunately, preventive and habilitative services were but a tiny fraction of health care expenditures and were demonstrably underutilized. Only 60% of these children had routine dental examinations within the last 12 months, a worse record than the average child. For the individuals whose primary physicians judged that they would benefit from physical or speech therapy, less than one quarter were receiving them. Care for seriously, chronically disabled children places great burdens on immediate family members. Only 20% of the severely retarded youngsters from age 10 to 24 could be left alone at home, even for a few minutes, and only 30% of the autistic ones. These developmental disabilities create needs for personal care and family support that traditionally have not been considered health services.(ABSTRACT TRUNCATED AT 400 WORDS)

Autistic Disorder↗

Quality of life for persons with developmental disabilities.

A uniform and consensus definition for quality of life is not currently available. Although the topic of quality of life is pertinent for individuals with neurodevelopmental and related disabilities, the most appropriate means for assessing it as a basis for developing or evaluating programs need to be identified. A global viewpoint of one's quality of life when emotional, physical, or cognitive limitations are manifested may be too narrow for capturing a realistic perspective for planning programs. A more holistic approach that includes both individual and parental or caregiver perceptions may better address the conceptualization of quality of life for persons with developmental disabilities. Models of quality of life for this population reflect lifespan challenges for achieving personal satisfaction in the following areas: (1) physical well-being or functional status, (2) social and emotional well-being, (3) material well-being, and, (4) developmental abilities. This paper addresses current models of quality of life and methodological considerations for investigating this concept with persons who have developmental disabilities. Multidimensional methods of measurement, possibly including proxies, are necessary for a comprehensive approach to studying such an elusive construct, particularly when cognitive function is limited.

Activities of Daily Living↗

Macrocephaly in children with developmental disabilities.

In a community-based study of 4,309 children with neurodevelopmental disabilities who were referred to the Institute for Child Development, Tel Aviv, Israel, 62 (1.4%) had macrocephaly (head circumference above the ninety-eighth percentile for age), of whom 42 (1%) had macrocephaly not associated with hydrocephalus. With the exception of neonatal respiratory distress the incidence of perinatal complications was not different from that in other children referred to the Institute for Child Development. In children with developmental disabilities, macrocephaly was a significant risk factor for febrile seizures (odds ratio = 3.1, P < 0.001) and epilepsy (odds ratio = 7.7, P < 0.001), but not for mental retardation (odds ratio = 1.1, P = 0.78) or cerebral palsy (odds ratio = 1.3, P = 0.67). Children with macrocephaly had a high rate of comorbid diagnosis. We conclude that in children with developmental disabilities the presence of macrocephaly even when not associated with hydrocephalus is associated with an increased risk of seizures.

Cephalometry↗

Altered diurnal pattern of salivary substance P in adults with developmental disabilities and chronic self-injury.

Morning and afternoon salivary substance P and cortisol levels were measured in 26 adults with chronic self-injurious behavior (SIB) and severe developmental disabilities and compared with matched controls without SIB. Chronic SIB was associated with an altered diurnal pattern of salivary substance P relative to matched controls, characterized primarily by lower levels of morning substance P, which were significantly correlated with overall severity of SIB. There was a trend for SIB subjects to exhibit higher levels of cortisol, which was significantly correlated with overall severity of SIB. These results support a model of altered nociception and possible stress-induced analgesia among individuals with developmental disability and chronic SIB.

Adult↗

RORA-neurodevelopmental disorder: A unique triad of developmental disabilities, cerebellar anomalies, and myoclonic seizures.

PURPOSE: RORA encodes the RAR-related orphan receptor-&#x3b1;, playing a pivotal role in cerebellar maturation and function. Here, we report the largest series of individuals with RORA-related-neurodevelopmental disorder. METHODS: Forty individuals (30 unrelated; 10 siblings from 4 families) carrying RORA pathogenic/likely pathogenic variants were collected through an international collaboration. RESULTS: The 33 variants (29 de novo, 4 inherited, and 1 shared), identified by genome/exome sequencing (n&#xa0;= 21), chromosomal microarray analysis (n&#xa0;= 7), or gene panels (n&#xa0;= 4), included frameshift (n&#xa0;= 18/33), missense (n&#xa0;= 9/33), and stop codon (n&#xa0;= 6/33). Developmental disability (n&#xa0;= 32/37), intellectual disability (n&#xa0;= 22/32), and cerebellar signs (n&#xa0;= 25/34) were the most striking clinical features. Cerebellar symptoms were divided into early-onset, late-onset, and progressive subgroups. Cerebellar hypoplasia, atrophy, or both (n&#xa0;= 16/25) were more frequent in individuals with missense variants in the DNA-binding domain. Epilepsy (n&#xa0;= 18/38), with prominent myoclonic seizure types (n&#xa0;= 11/18), was classified in (1) genetic generalized epilepsy (n&#xa0;= 10/18) with a syndromic diagnosis identifiable for 6: epilepsy with eyelid myoclonia (n&#xa0;= 5/6) and epilepsy with myoclonic absence (n&#xa0;= 1/6); (2) developmental and epileptic encephalopathy (n&#xa0;= 5/18); and (3) unclassified (n&#xa0;= 3/18). A participant with rapid deterioration of visual acuity and cone/rod dystrophy was reported. CONCLUSION: Missense variants in DNA-binding domain correlate to a more severe cerebellar phenotype. The RORA-related-neurodevelopmental disorder triad comprises developmental disability, cerebellar features, and a spectrum of myoclonic epilepsy.

Humans↗

Reinforcer rate effects and behavioral momentum in individuals with developmental disabilities.

Behavioral momentum theory states that behavioral resistance to change is positively related to reinforcer rate and independent of response rate under most circumstances. We examined behavioral momentum in humans with developmental disabilities. The experimental procedures were implemented as a computer game. Different rates of positive reinforcement were programmed in two alternating components signaled by distinctive cues. For 10 participants who successfully completed testing, resistance to disruption by an alternate source of reinforcement was greater in the component with the higher reinforcer rate, although the magnitude of the difference varied among individuals. These results confirm reinforcer rate effects consistent with behavioral momentum under laboratory conditions and with the largest number of human beings with developmental disabilities studied to date.

Adolescent↗