-
Intellectual and Developmental... Dec 2014The literature related to people with borderline intellectual functioning (BIF) was systematically reviewed in order to summarize the present knowledge. Database... (Review)
Review
The literature related to people with borderline intellectual functioning (BIF) was systematically reviewed in order to summarize the present knowledge. Database searches yielded 1,726 citations, and 49 studies were included in the review. People with BIF face a variety of hardships in life, including neurocognitive, social, and mental health problems. When adults with BIF were compared with the general population, they held lower-skilled jobs and earned less money. Although some risk factors (e.g., low birth weight) and preventive factors (e.g., education) were reported, they were not specific to BIF. The review finds that, despite the obvious everyday problems, BIF is almost invisible in the field of research. More research, societal discussion, and flexible support systems are needed.
Topics: Adult; Employment; Humans; Intellectual Disability; Intelligence; Learning Disabilities; Mental Health; Severity of Illness Index
PubMed: 25409130
DOI: 10.1352/1934-9556-52.6.419 -
Journal of Intellectual Disabilities :... Mar 2022Several conditions related to serious difficulty in initiating and maintaining breastfeeding in neonates with Down syndrome are described in the literature. This study...
Several conditions related to serious difficulty in initiating and maintaining breastfeeding in neonates with Down syndrome are described in the literature. This study aimed to investigate the frequency of breastfeeding in neonates with Down syndrome, as well as the reasons for not breastfeeding, through a systematic literature review by searching MEDLINE via PubMed, Cochrane Library, Scopus, Embase via Elsevier, and Cumulative Index to Nursing and Allied Health Literature (CINAHL) databases. Sixteen studies were included with a total sample size of 2022 children with Down syndrome. The frequency of exclusive breastfeeding was 31.6-55.4%, with five studies reporting breastfeeding for longer than 6 months. Breastfeeding from birth was present for 48-92.5% of the children with Down syndrome in six studies. Two studies reported that around 50% and 23.3% of the children with Down syndrome were never breastfed, and rates of breastfeeding in infants with Down syndrome were lower than those in controls in three studies. The reasons for not breastfeeding or cessation of breastfeeding were associated with Down syndrome-specific challenges, maternal reasons, and healthcare aspects.
Topics: Breast Feeding; Child; Down Syndrome; Female; Humans; Infant; Infant, Newborn; Intellectual Disability
PubMed: 33234015
DOI: 10.1177/1744629520970078 -
International Journal of Environmental... Jan 2021Rett Syndrome is an x linked developmental disorder which becomes apparent in females after 6 to 18 months of age. It leads to severe impairments including loss of... (Review)
Review
Rett Syndrome is an x linked developmental disorder which becomes apparent in females after 6 to 18 months of age. It leads to severe impairments including loss of speech, loss of hand movements/manual dexterity, characteristic hand movements such as hang wringing and intellectual disability/learning problems. This systematic review was carried out to identify the dental manifestation of Rett syndrome and to shed light on treatment options available for oral health problems associated with Rett syndrome. A systematic literature search was conducted on the PubMed, Scopus, Biomed, Web of Science, Embase, Google Scholars, Cochrane and CINAHL using the following entries: Rett syndrome ( = 3790), Oral health and Rett syndrome ( = 17), dental health of Rett syndrome patients ( = 13), and the MeSH terms listed below: Rett syndrome and Oral Health ( = 17), Rett syndrome and dentistry ( = 29). The final review included 22 search articles. The most common oral findings was bruxism. Masseteric hypertrophy was also reported. Anterior open bite and non-physiological tooth wear was observed. Other oral manifestations of Rett syndrome included mouth breathing, tongue thrusting, digit/thumb sucking, high arch palate. Increased awareness and dental education amongst dentists and assistants regarding the dental manifestations of Rett syndrome and similar neurodevelopmental disorders is required to improve the level of care and empathy they can provide to these differently able patients. Research on dental aspects of Rett is scarce and this remains a neglected topic.
Topics: Bruxism; Female; Hand; Humans; Rett Syndrome; Speech
PubMed: 33525609
DOI: 10.3390/ijerph18031162 -
Journal of Intellectual Disability... Jun 2016People with intellectual disabilities have very high rates of mental ill health. Standard psychosocial interventions designed for the general population may not be... (Review)
Review
BACKGROUND
People with intellectual disabilities have very high rates of mental ill health. Standard psychosocial interventions designed for the general population may not be accessible for people with mild intellectual disabilities, and drug usage tends to be modified - 'start low and go slow'. This systematic review aims to synthesise the evidence on psychological, pharmacological and electroconvulsive therapy (ECT) interventions for adults with mild intellectual disabilities and mental ill health.
METHOD
PRISMA guidelines were followed. Medline, Embase, PsycINFO and CINAHL were searched, as was grey literature and reference lists of selected papers. Papers were selected based on pre-defined inclusion and exclusion criteria. A proportion of papers were double reviewed. Data was extracted using a structured table.
STUDY REGISTRATION
PROSPERO 2015:CRD42015015218.
RESULTS
Initially, 18 949 records were identified. Sixteen studies were finally selected for inclusion; seven on psychological therapies, two on group exercise, five on antipsychotics and two on antidepressants. They do not provide definitive evidence for effectiveness of psychosocial interventions, nor address whether starting low and going slow is wise, or causes sub-optimum therapy.
CONCLUSIONS
There are few evidence-based interventions for people with mild intellectual disabilities and mental ill-health; existing literature is limited in quantity and quality. Group cognitive-behavioural therapies have some supporting evidence - however, further randomised control trials are required, with longer-term follow-up, and larger sample sizes.
Topics: Comorbidity; Humans; Intellectual Disability; Mental Disorders
PubMed: 27109143
DOI: 10.1111/jir.12285 -
Journal of the American Academy of... Sep 2020The behavioral phenotype of neurogenetic disorders associated with intellectual disability often includes psychiatric comorbidity. The objectives of this systematic... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
The behavioral phenotype of neurogenetic disorders associated with intellectual disability often includes psychiatric comorbidity. The objectives of this systematic review and meta-analysis were to systematically review the prevalence of psychiatric disorders and symptoms in children and adolescents with these disorders and compare phenotypic signatures between syndromes.
METHOD
MEDLINE and PsycINFO databases were searched for articles from study inception to December 2018. Eligible articles were peer reviewed, were published in English, and reported prevalence data for psychiatric disorders and symptoms in children and adolescents aged 4 to 21 years using a formal psychiatric assessment or a standardized assessment of mental health symptoms. Pooled prevalence was determined using a random-effects meta-analysis in studies with sufficient data. Prevalence estimates were compared with general population data using a test of binomial proportions.
RESULTS
Of 2,301 studies identified for review, 39 articles were included in the final pool, which provided data on 4,039 children and adolescents. Ten syndromes were represented, and five were predominant: Down syndrome, 22q11.2 deletion syndrome, fragile X syndrome, Williams syndrome, and Prader-Willi syndrome. The Child Behavior Checklist was the most commonly used assessment tool for psychiatric symptoms. The pooled prevalence with total scores above the clinical threshold was lowest for Down syndrome (32% [95% confidence interval, 19%-44%]) and highest for Prader-Willi syndrome (74% [95% CI, 65%-82%]) with each syndrome associated with significantly higher prevalence than in the general population. Parallel trends were observed for the internalizing and externalizing domains and social subscale scores.
CONCLUSION
Differential vulnerability for psychiatric phenotype expression across the disorders was observed. Syndromes with higher levels of social ability or competence appear to offer relative protection against developing psychopathology. This preliminary finding merits further exploration.
Topics: Adolescent; Adult; Child; Child, Preschool; Comorbidity; Humans; Intellectual Disability; Mental Disorders; Mental Health; Prader-Willi Syndrome; Prevalence; Young Adult
PubMed: 31945412
DOI: 10.1016/j.jaac.2020.01.006 -
Journal of Applied Research in... Jul 2021Social inclusion has been explored using a wide range of theories and methods. Belonging and reciprocity have been identified as key components of social inclusion. This... (Review)
Review
BACKGROUND
Social inclusion has been explored using a wide range of theories and methods. Belonging and reciprocity have been identified as key components of social inclusion. This systematic methodological review identifies and evaluates the theoretical frameworks and qualitative approaches adopted to explore belonging and reciprocity in the lives of adults with intellectual disabilities.
METHOD
A systematic search was conducted across ten databases. Screening and quality appraisal were carried out independently by two researchers, and data were extracted to provide detailed accounts of the theories and methods employed.
RESULTS
Seventeen papers met inclusion criteria. Clear conceptualisations of belonging and reciprocity were lacking, and these concepts were rarely the focus of the research. Theoretical and methodological shortcomings across this literature were identified and discussed.
CONCLUSIONS
More nuanced conceptualisations of belonging and reciprocity may be helpful in future research, to better capture the context and meaning of individual lives and relationships.
Topics: Adult; Humans; Intellectual Disability; Social Participation
PubMed: 33723903
DOI: 10.1111/jar.12881 -
Journal of Intellectual Disability... Mar 2021The assessment of depression in people with severe to profound intellectual disability (severe-profound ID) is challenging, primarily due to inability to report internal... (Review)
Review
The assessment of depression in people with severe to profound intellectual disability (severe-profound ID) is challenging, primarily due to inability to report internal states such as mood, feelings of worthlessness and suicidal ideation. This group also commonly presents with challenging behaviours (e.g. aggression and self-injury) with debate about whether these behaviours should be considered 'depressive equivalents' or are sensitive for, but not specific to, depression in severe-profound ID. We conducted a systematic review exploring behaviours associated with depression and low mood in individuals with severe-profound ID. The review was conducted in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (2009) guidelines. Three electronic databases were searched (Embase, PsycINFO and Ovid MEDLINE), and 13 studies were included and rated for quality. Few studies were rated as having high methodological quality. Behaviours captured by standard diagnostic schemes for depression (e.g. Diagnostic and Statistical Manual of Mental Disorders and International Classification of Diseases) showed a relationship with depression in severe-profound ID, including the two core symptoms (depressed affect and anhedonia), as well as irritability, sleep disturbance, psychomotor agitation, reduced appetite and fatigue. Challenging behaviours such as aggression, self-injury, temper tantrums, screaming and disruptive behaviour were associated with depression. Challenging behaviours show a robust relationship with depression. Whilst these behaviours may suggest an underlying depression, study limitations warrant caution in labelling them as 'depressive equivalents'. These limitations include not controlling for potential confounds (autism, other affective disorders and pain) and bias associated with comparing depressed/non-depressed groups on the same behavioural criteria used to initially diagnose and separate these groups. Future studies that use depressive measures designed for ID populations, which control for confounds and which explore low mood irrespective of psychiatric diagnosis, are warranted to better delineate the behaviours associated with depression in this population (PROSPERO 2018: CRD42018103244).
Topics: Aggression; Depression; Humans; Intellectual Disability; Irritable Mood; Self-Injurious Behavior
PubMed: 33426741
DOI: 10.1111/jir.12807 -
American Journal of Medical Genetics.... Jul 2021Hyperprolinemia Type I and II are genetic metabolic disorders caused by disrupted proline degradation. It has been suggested that hyperprolinemia is associated with... (Review)
Review
Hyperprolinemia Type I and II are genetic metabolic disorders caused by disrupted proline degradation. It has been suggested that hyperprolinemia is associated with increased risk of developmental and mental disorders but detailed information on the psychiatric phenotype in hyperprolinemic patients is limited. Following PRISMA guidelines, we carried out a systematic review to clarify psychiatric phenotypes in patients with hyperprolinemia. We screened 1753 studies and included 35 for analysis, including 20 case reports and 15 case-control and cohort studies. From these studies, a common psychiatric phenotype is observed with a high prevalence of developmental delay, intellectual disability, autism spectrum disorders, and psychosis spectrum disorders. In most cases, a genetic cause of hyperprolinemia was known, these included mutations in the PRODH and ALDH4A1 genes and deletions of chromosome 22q11.2. No evidence for a biochemical phenotype-clinical phenotype correlation was found; that is, no association between higher proline levels and specific psychiatric phenotypes was observed. This suggests that genomic and environmental factors are likely to contribute to clinical outcomes. More studies are needed to clarify whether hyperprolinemia is a primary causal factor underlying the increased risk of developing psychiatric disorders seen in patients with hyperprolinemia, or whether hyperprolinemia and psychiatric disorders are both consequences of a shared underlying mechanism.
Topics: Case-Control Studies; Humans; Intellectual Disability; Phenotype; Proline; Proline Oxidase
PubMed: 34302426
DOI: 10.1002/ajmg.b.32869 -
American Journal of Medical Genetics.... Sep 2022Rubinstein-Taybi syndrome (RTS) is a rare genetic syndrome associated with growth delay, phenotypic facial characteristics, microcephaly, developmental delay, broad... (Review)
Review
Rubinstein-Taybi syndrome (RTS) is a rare genetic syndrome associated with growth delay, phenotypic facial characteristics, microcephaly, developmental delay, broad thumbs, and big toes. Most research on RTS has focused on the genotype and physical phenotype; however, several studies have described behavioral, cognitive, social, and emotional characteristics, elucidating the behavioral phenotype of RTS. The reporting of this review was informed by PRISMA guidelines. A systematic search of CINAHL, Medline, and PsychINFO was carried out in March 2021 to identify group studies describing behavioral, cognitive, emotional, psychiatric, and social characteristics in RTS. The studies were quality appraised. Characteristics reported include repetitive behavior, behaviors that challenge, intellectual disability, mental health difficulties, autism characteristics, and heightened sociability. Findings were largely consistent across studies, indicating that many characteristics are likely to form part of the behavioral phenotype of RTS. However, methodological limitations, such as a lack of appropriate comparison groups and inconsistency in measurement weaken these conclusions. There is a need for multi-disciplinary studies, combining genetic and psychological measurement expertise within single research studies. Recommendations are made for future research studies in RTS.
Topics: Genotype; Humans; Intellectual Disability; Phenotype; Rubinstein-Taybi Syndrome
PubMed: 35730128
DOI: 10.1002/ajmg.a.62867 -
Pediatrics Jun 2017The purpose of this systematic literature review is to describe what is known about fragile X syndrome (FXS) and to identify research gaps. The results can be used to... (Review)
Review
OBJECTIVES
The purpose of this systematic literature review is to describe what is known about fragile X syndrome (FXS) and to identify research gaps. The results can be used to help inform future public health research and provide pediatricians with up-to-date information about the implications of the condition for individuals and their families.
METHODS
An electronic literature search was conducted, guided by a variety of key words. The search focused on 4 areas of both clinical and public health importance: (1) the full mutation phenotype, (2) developmental trajectories across the life span, (3) available interventions and treatments, and (4) impact on the family. A total of 661 articles were examined and 203 were included in the review.
RESULTS
The information is presented in the following categories: developmental profile (cognition, language, functional skills, and transition to adulthood), social-emotional profile (cooccurring psychiatric conditions and behavior problems), medical profile (physical features, seizures, sleep, health problems, and physiologic features), treatment and interventions (educational/behavioral, allied health services, and pharmacologic), and impact on the family (family environment and financial impact). Research gaps also are presented.
CONCLUSIONS
The identification and treatment of FXS remains an important public health and clinical concern. The information presented in this article provides a more robust understanding of FXS and the impact of this complex condition for pediatricians. Despite a wealth of information about the condition, much work remains to fully support affected individuals and their families.
Topics: Adult; Caregivers; Child; Cross-Sectional Studies; DNA Mutational Analysis; Delivery of Health Care; Developmental Disabilities; Diagnosis, Differential; Female; Fragile X Mental Retardation Protein; Fragile X Syndrome; Genetic Testing; Humans; Language Development Disorders; Male; Parenting; Phenotype; Prognosis; Public Health; Social Adjustment; Trinucleotide Repeats
PubMed: 28814537
DOI: 10.1542/peds.2016-1159C