Adaptive Behavior Assessment System-II Research Articles

Intelligence profiles and adaptive behaviors of high-functioning autism spectrum disorder and developmental speech and language disorders.

The present study was aimed at investigating the intelligence profiles and adaptive behaviors of children with high-functioning autism spectrum disorder (HFASD) and developmental speech and language disorders (DSLDs). We compared the similarities and differences of cognitive capabilities and adaptive functions and explored their correlations in the HFASD and DSLDs groups. 128 patients with HFASD, 111 patients with DSLDs and 114 typically developing (TD) children were enrolled into our study. Wechsler Intelligence Scale for Children-IV (WISC-IV) and Adaptive Behavior Assessment System-II (ABAS-II) were respectively applied to evaluate intelligence profiles and adaptive behaviors. Intelligence quotient (IQ) scores and adaptive functioning scores among the HFASD, DSLDs and TD groups were compared through one-way ANOVA. Pearson correlation coefficient was applied to examine the relationships between WISC indices and ABAS domains. Outcomes showed significantly poorer intelligence profiles and adaptive behaviors in HFASD and DSLDs groups. Both children with HFASD and DSLDs demonstrated impairments in verbal comprehension and executive functions. Processing speed and working memory were the predominant defects of children with HFASD and DSLDs in the field of executive functions, respectively. Whereas perceptual reasoning was a relative strength for them. Children with DSLDs had balanced scores of all the domains in ABAS-II; nevertheless, HFASD individuals demonstrated striking impairments in Social domain. Correlation analysis showed IQs of children with HFASD were positively correlated with all the domains and General Adaptive Composite (GAC) of ABAS-II. Additionally, IQs were positively correlated with Conceptual domain and GAC for children with DSLDs. Compared with DSLDs group, intelligence displayed stronger correlations with adaptive behaviors in HFASD group. Our study expanded insights regarding intelligence profiles and adaptive behaviors of children with HFASD and DSLDs. Moreover, this study made breakthroughs in discovering positive correlations between IQs and adaptive functions in the two neurodevelopmental disorders.

Sensory processing and adaptive behavior in Phelan-McDermid syndrome: a cross-sectional study

Phelan-McDermid syndrome (PMS) is a genetic disorder caused by a mutation or deletion of the SHANK3 gene (chromosome 22q13.3), characterized by different sensory processing anomalies. The objective of this study is to expand and provide a detailed definition of the sensory profile of patients with PMS. The secondary objective was to examine the relationship between sensory patterns and adaptive behavior. A cross-sectional study was carried out among 51 Spanish patients with a confirmed genetic diagnosis of PMS. All the participants’ parents completed the Short Sensory Profile-Spanish (SSP-S) and the Adaptive Behavior Assessment System II (ABAS-II). Correlational, multiple regression and hierarchical cluster analyses were performed. An atypical sensory profile was identified in almost 75% of PMS patients. Definite differences were found among scores; nonetheless, sub-threshold values were observed in tactile sensitivity, underresponsive/seeks sensation, auditory filtering, and low energy/weak sensory categories. Conceptual, social, and practical domains, as well as the General Adaptive Composite (GAC) of the ABAS-II showed extremely low scores (i.e., <70). Significant correlations were found (p<0.005) between SSP-S scores and the conceptual, social, practical, and GAC index of the ABAS-II, whereby higher SSP-S scores were associated with better skills and higher adaptive performance. The cluster analysis indicated that the group with the largest mutation size (7.23 Mb) showed the greatest sensory processing difficulties and very low adaptive skills.Conclusions: Patients with PMS show an atypical sensory profile, which correlates with limitations in general adaptive behaviors.What is Known:• PMS sensory processing difficulties were associated with a pattern of underresponsive/seeks sensation, low energy/weak, and tactilehyporeactivity.• Sensory processing difficulties have been associated with limitations in the development of appropriate adaptive communication andinteraction behaviors.What is New:• Sensory definite differences associated with tactile hyperreactivity, as well as significant effects of underresponsiveness/seeks sensation and auditory filtering categories on the adaptive abilities were found in SHANK3deletionpatients.• Cluster analysis suggests that smaller mutation sizes were related to better sensory processing and higher adaptive skills, while patients with larger deletion sizes have greater adaptive difficulties and worse sensory processing skills.

Assessment of intellectual disability in Klinefelter’s Syndrome associated with other Prenatal and Perinatal risk factors. A case report

IntroductionKlinefelter’s syndrome (KS) is considered as a genetic risk factor for intellectual disability and specifically for impairment on verbal skills. Prenatal and perinatal morbidity concurrent with this diagnosis determine the entity and typology of neurodevelopmental deficits.ObjectivesTo describe the intellectual and adaptive functioning of a patient with KS and prenatal and perinatal difficulties, assessed in the Service of Psychiatry, Clinical Psychology and Mental Health at La Paz University Hospital (Madrid).MethodsA descriptive study was conducted of a 6 year and 7-month-old boy with diagnosis of KS (karyotype XXY), extremely low weight at birth (752 gr.), Arnold Chiari type I malformation with post-hemorrhagic hydrocephalus and periventricular leukomalacia, bronchopulmonary dysplasia, intra-atrial septal defect, left kidney agenesis with iatrogenic adrenal insufficiency, and acute ventriculitis due to E. Coli. Neuropsychological evaluation was carried out in October 2021. Leiter-3 scale was selected due to the absence of expressive language; and his parents completed the questionnaire Adaptive Behavior Assessment System-II.ResultsThe nonverbal intelligence quotient was observed in the very low range (full-scale IQ = 69). The result of general adaptive behavior (z = -2.83), showed very high functional disability both in the conceptual, social, and practical domains.ConclusionsThe high number of causes of disability in this patient is consistent with a high degree of functional disability. Efficient evaluation sessions of intellectual performance, adaptive functioning, and necessary supports, due to the absence of expressive language and limited receptive language, are required. A specific neuropsychological evaluation profile should be established for KS.DisclosureNo significant relationships.

Quality of Life in Patients with Chronic Psychotic Disorders: A Practical Model for Interventions in Romanian Mental Health Centers.

Background and Objectives: Current psychiatric care is increasingly focusing on patients’ quality of life (QoL). Research is still trying to determine the main factors which influence QoL. The present study aims to assess the QoL of patients with chronic psychotic-spectrum disorders, as well as its relation to symptomatology, functionality, adaptive behavior, and perceived level of recovery. Materials and Methods: The study included a sample of 78 patients with chronic psychosis. Symptomatology and illness severity were assessed with the Positive and Negative Syndrome Scale (PANSS) and the Clinical Global Impression Scale-Severity (CGI-S) scales, respectively. The Global Assessment of Functioning Scale (GAFS) and the Adaptive Behavior Assessment System II (ABAS-II)-Adult Form were used for the assessment of patients’ functionality, and the Quality-of-Life Inventory (QOLI) scale was applied for the evaluation of QoL. Results: According to the CGI-Severity scale, 20.5% of the patients were borderline mentally ill, and 24.4% were mildly ill. The highest number of patients (34.6%) were moderately ill, while 14.1% and 2.6% were markedly ill and severely ill, respectively. Among the moderately ill patients, more than half (63%) were patients with schizophrenia, 18.5% were patients with delusional disorder, and 18.5% were patients with schizoaffective disorder. Most of the patients (43.6%) presented moderate functioning deficiency, while 38.5% of the patients presented severe deficiency, according to the GAFS score. When assessed with the ABAS, we observed that almost half of the patients (44.9%) showed an average functioning across skill areas in the conceptual, social, and practical domains. A percent of 67.9% of the patients presented an average QoL, while 15.4% and 12.8% showed a very low and low QoL. QoL was not influenced by the patients’ symptomatology, gender, and education level. Having children, family support, better social and conceptual skills, and a higher perceived level of recovery was correlated with an increased QoL, hierarchical multiple regression R2 = 0.379, F(9, 68) = 2.616, and p = 0.012. Conclusions: Psychiatric interventions in psychosis should focus not only on symptoms’ control, but also on improving social and family support, as well as adaptive skills to increase the patients’ QoL.

Quickstart for toddlers with autism spectrum disorder: A preliminary report of an adapted community-based early intervention program

Early intervention (EI) for young children with autism spectrum disorder (ASD) must be resource-efficient while remaining effective; thus, clinicians are challenged to create and implement useful methods. Clinical evidence from community-based interventions that include reliable diagnoses, individual EI programs, along with comprehensive descriptions of participants, procedures, and participant outcomes can inform practice, translational research, and local policy. Parent-mediated EI for toddlers with ASD can promote positive developmental outcomes and lifelong well-being, but evidence of successful community uptake of research-based EIs is somewhat limited. The community-based, parent-mediated, evidence-informed QuickStart EI program aims to encourage toddlers' early social communication, social interactions, and relationship-building, in a community clinic setting.We aim to (1) describe our adaptations to the evidence-based Parent-Delivered Early Start Denver Model and (2) present promising findings for toddlers with or at risk for ASD and their families who received QuickStart. We also intend to motivate a similar study of EI in real-world situations to advance evidence-based practice and create relevant dialogue and questions for research. Complete data were identified and analyzed for up to 89 toddlers diagnosed with, or at risk of, ASD. Pre- and post-intervention parent- or self-report data were analyzed using descriptive statistics and paired-sample t-tests, as appropriate. Pre-intervention measures included demographic information (n = 89) and the Early Screening of Autism and Communication (ESAC; n = 89). Measures taken pre- and post-intervention included the Adaptive Behavior Assessment System-II (n = 60), MacArthur-Bates Communication Development Inventories (n = 58), and the parental sense of competence scale (n = 62). The Measure of Processes of Care (n = 60) was taken post-intervention. On enrollment, parents signed standard clinical agreements that included statements allowing their anonymous data to be analyzed for research. Using standardized parent/self-report measures, toddler gains were noted for social interaction, language, communication skills, and ASD symptoms, but not for parents' feelings of competence. Parents identified QuickStart procedures as family centered (Measure of Processes of Care). The QuickStart EI program, provided to toddlers and their families over 20 weeks in a community clinic, resulted in promising positive behavior and communication changes, as indicated on the parent-response measures, for a moderately large sample of toddlers. This study adds to the literature by describing a new EI program with clear procedures by which clinicians can create, provide, and evaluate a readily accessible, community-based EI for toddlers with or at risk of ASD. Methodological limitations inherent to our study design that precluded a control group and necessitated a reliance on available parent-report data are carefully critiqued and discussed.

Cognitive and Adaptive Characterization of Children and Adolescents with KBG Syndrome: An Explorative Study.

KBG syndrome (KBGS) is a rare Mendelian condition caused by heterozygous mutations in ANKRD11 or microdeletions in chromosome 16q24.3 encompassing the gene. KBGS is clinically variable, which makes its diagnosis difficult in a significant proportion of cases. The present study aims at delineating the cognitive profile and adaptive functioning of children and adolescents with KBGS. Twenty-four Italian KBGS with a confirmed diagnosis by molecular testing of the causative ANKRD11 gene were recruited to define both cognitive profile as measured by the Wechsler Intelligence Scale and adaptive functioning as measured by Vineland Adaptive Behavior Scales-II Edition or the Adaptive Behavior Assessment System-II Edition. Among children and adolescents, 17 showed intellectual disability, six presented borderline intellectual functioning and only one child did not show cognitive defects. Concerning cognitive profile, results revealed significant differences between the four indexes of Wechsler Intelligence Scale. Namely, the verbal comprehension index was significantly higher than the perceptual reasoning index, working memory index and the processing speed index. Concerning adaptive functioning, no difference between the domains was found. In conclusion, in our cohort, a heterogeneous profile has been documented in cognitive profiles, with a spike on verbal comprehension, while a flat-trend has emerged in adaptive functioning. Our cognitive and adaptive characterization drives professionals to set the best clinical supports, capturing the complexity and heterogeneity of this rare condition.

Outcomes of Preterm Infants With Congenital Heart Defects After Early Surgery: Defining Risk Factors at Different Time Points During Hospitalization.

Background: Compared with those born at term gestation, infants with complex congenital heart defects (CCHD) who were delivered before 37 weeks gestational age and received neonatal open-heart surgery (OHS) have poorer neurodevelopmental outcomes in early childhood. We aimed to describe the growth, disability, functional, and neurodevelopmental outcomes in early childhood of preterm infants with CCHD after neonatal OHS. Prediction models were evaluated at various timepoints during hospitalization which could be useful in the management of these infants.Study Design: We studied all preterm infants with CCHD who received OHS within 6 weeks of corrected age between 1996 and 2016. The Western Canadian Complex Pediatric Therapies Follow-up Program completed multidisciplinary comprehensive neurodevelopmental assessments at 2-year corrected age at the referral-site follow-up clinics. We collected demographic and acute-care clinical data, standardized age-appropriate outcome measures including physical growth with calculated z-scores; disabilities including cerebral palsy, visual impairment, permanent hearing loss; adaptive function (Adaptive Behavior Assessment System-II); and cognitive, language, and motor skills (Bayley Scales of Infant and Toddler Development-III). Multiple variable logistic or linear regressions determined predictors displayed as Odds Ratio (OR) or Effect Size (ES) with 95% confidence intervals.Results: Of 115 preterm infants (34 ± 2 weeks gestation, 2,339 ± 637 g, 64% males) with CCHD and OHS, there were 11(10%) deaths before first discharge and 21(18%) deaths by 2-years. Seven (6%) neonates had cerebral injuries, 7 had necrotizing enterocolitis; none had retinopathy of prematurity. Among 94 survivors, 9% had cerebral palsy and 6% had permanent hearing loss, with worse outcomes in those with syndromic diagnoses. Significant predictors of mortality included birth weight z-score [OR 0.28(0.11,0.72), P = 0.008], single-ventricle anatomy [OR 5.92(1.31,26.80), P = 0.021], post-operative ventilation days [OR 1.06(1.02,1.09), P = 0.007], and cardiopulmonary resuscitation [OR 11.58 (1.97,68.24), P = 0.007]; for adverse functional outcome in those without syndromic diagnoses, birth weight 2,000–2,499 g [ES −11.60(−18.67, −4.53), P = 0.002], post-conceptual age [ES −0.11(−0.22,0.00), P = 0.044], post-operative lowest pH [ES 6.75(1.25,12.25), P = 0.017], and sepsis [ES −9.70(−17.74, −1.66), P = 0.050].Conclusions: Our findings suggest preterm neonates with CCHD and early OHS had significant mortality and morbidity at 2-years and were at risk for cerebral palsy and adverse neurodevelopment. This information may be important for management, parental counseling and the decision-making process.

Concurrent Validity of the ABAS-II Questionnaire with the Vineland II Interview for Adaptive Behavior in a Pediatric ASD Sample: High Correspondence Despite Systematically Lower Scores.

We examined the correlation between interviewer-administered Vineland Adaptive Behavior Scale II (VABS-II) and the parent-rated Adaptive Behavior Assessment System II (ABAS-II) questionnaire in 352 participants (ages 1.5-20.8years) with autism spectrum disorder (ASD) to determine if ABAS could be used as a screen to reduce the number of VABS interviews. Corresponding domain scores between the two measures were highly correlated but scores were significantly lower on the ABAS-II. Screening with ABAS-II significantly reduced the number of VABS-II interviews required with little cost to overall accuracy. The ABAS-II provides a cost- and time-saving alternative to the VABS-II to rule out functional impairment; however, scores are not strictly comparable between the two measures.

Adaptive Behavioral, Social-Emotional, and Neurodevelopmental Outcomes at 2 Years of Age in Hungarian Preterm Infants Based on Bayley III

ABSTRACT Aim This study aimed to assess cognitive, language, and motor development as well as social-emotional and adaptive behavior skills of low birthweight (LBW) children. Methods Our cohort contained 305 two-year-old LBW children. Developmental functioning was assessed by the Bayley Scales of Infant and Toddler Development, 3rd Edition. Results Performance in language, particularly in expressive communication was the poorest. Extremely low birthweight (ELBW) children were found to be at increased risk of developmental delay: 12.73 times in motor (95%CI = 2.8–57.5), 9.81 times in cognitive (95%CI = 3.2–29.6) and 3.91 times in language (95%CI = 1.6–9.4), and social-emotional skills (95%CI = 1.6–9.5). Parents of children with lower birthweight reported poorer social and practical adaptive skills. Self-care domain seemed to be the least developed. Conclusion Additionally, the screening for developmental delays, monitoring the adaptive behavior skills may also be useful – especially among ELBW children – in order to identify the specific areas, situations, and functions that are most in need of interventions. Abbreviations AB: Adaptive Behavior; ABAS-II: Adaptive Behavior Assessment System-II; BPD: Bronchopulmonary Dysplasia; BSID-III: Bayley Scales of Infant Development 3rd Edition; BW: Birthweight; CI: Confidence Interval; CS: Composite Score; ELBW: Extremely Low Birthweight; GA: Gestational Age; IVF: In Vitro Fertilization; IVH: Intraventricular Hemorrhage; LBW: Low Birthweight; NEC: Necrotizing Enterocolitis; OR: Odds Ratio; ROP: Retinopathy of Prematurity; SE: Social-Emotional Development; VLBW: Very Low Birthweight

Pre-school neurocognitive and functional outcomes after liver transplant in children with early onset urea cycle disorders, maple syrup urine disease, and propionic acidemia: An inception cohort matched-comparison study.

BackgroundUrea cycle disorders (UCD) and organic acid disorders classically present in the neonatal period. In those who survive, developmental delay is common with continued risk of regression. Liver transplantation improves the biochemical abnormality and patient survival is good. We report the neurocognitive and functional outcomes post‐transplant for nine UCD, three maple syrup urine disease, and one propionic acidemia patient.MethodsThirteen inborn errors of metabolism (IEM) patients were individually one‐to‐two matched to 26 non‐IEM patients. All patients received liver transplant. Wilcoxon rank sum test was used to compare full‐scale intelligence‐quotient (FSIQ) and Adaptive Behavior Assessment System‐II General Adaptive Composite (GAC) at age 4.5 years. Dichotomous outcomes were reported as percentages.ResultsFSIQ and GAC median [IQR] was 75 [54, 82.5] and 62.0 [47.5, 83] in IEM compared with 94.5 [79.8, 103.5] and 88.0 [74.3, 97.5] in matched patients (P‐value <.001), respectively. Of IEM patients, 6 (46%) had intellectual disability (FSIQ and GAC <70), 5 (39%) had autism spectrum disorder, and 1/13 (8%) had cerebral palsy, compared to 1/26 (4%), 0, 0, and 0% of matched patients, respectively. In the subgroup of nine with UCDs, FSIQ (64[54, 79]), and GAC (56[45, 75]) were lower than matched patients (100.5 [98.5, 101] and 95 [86.5, 99.5]), P = .005 and .003, respectively.ConclusionThis study evaluated FSIQ and GAC at age 4.5 years through a case‐comparison between IEM and matched non‐IEM patients post‐liver transplantation. The neurocognitive and functional outcomes remained poor in IEM patients, particularly in UCD. This information should be included when counselling parents regarding post‐transplant outcome.

Developmental trajectories of adaptive functioning following early mild traumatic brain injury.

Adaptive behavior impairments have been reported in children with severe traumatic brain injury (TBI) but are not typically found following mild TBI. It is possible that mild TBI induces subtle changes in adaptive functioning that are not captured in conventional group comparisons. This study aimed to explore time course changes in adaptive functioning following early mild TBI. Parents of 63 children with mild TBI and 53 children with orthopedic injuries aged between 1.5 and 5years at the time of injury completed the Adaptive Behavior Assessment System-II at three time points: retrospectively to assess pre-injury functioning, then at 6 and 18months post-injury. Developmental trajectories of adaptive functioning domains (practical, conceptual, and social) reported by parents were modeled using linear mixed-model analyses. Findings suggest that mild TBI may disrupt the expected developmental progression of children's social adaptive behavior, but does not appear to alter practical and conceptual domains.

KINDERGARTEN-AGE NEUROCOGNITIVE AND FUNCTIONAL OUTCOMES AFTER LIVER TRANSPLANTATION DONE AT AGE &lt;6 YEARS

Abstract BACKGROUND Mortality after liver transplant has improved, making long-term outcomes increasingly important. OBJECTIVES To describe neurocognitive and functional outcomes after liver transplant done in young children, and determine potentially modifiable risk factors for adverse outcomes. DESIGN/METHODS Between 1999–2014, all &lt;6 years old liver transplant recipients at our center were enrolled in this ethics board approved, longitudinal inception-cohort. Demographic, pre-transplant, transplant, and post-transplant data were prospectively collected. Following informed consent, outcomes were determined by experienced paediatric psychologists using Wechsler Preschool and Primary Scale of Intelligence III, Beery-Buktenica Developmental Test of Visual-Motor Integration-V (VMI), and Adaptive Behavior Assessment System-II. Associations with outcomes (Full-Scale intelligence quotient [FSIQ], Performance IQ [PIQ], Verbal IQ [VIQ], VMI and General Adaptive Composite [GAC]) were determined using multiple linear regression. Population norms for each score are mean 100 (SD15). RESULTS 78 liver transplants were performed; 69 patients survived, and all completed follow-up. Outcomes for the 60 patients without metabolic disease are reported. FSIQ, PIQ, and VIQ were 94.5 (17.0), 95.4 (17.8), and 93.7 (17.6). VMI and GAC were 91.6 (16.2) and 89.7 (18.0). Outcomes were shifted to the left of population norms, with the proportion having IQ scores &gt;1 SD (score &lt;85, expected 15.9%) and &gt;2 SD (score &lt;70, expected 2.27%) below population norms being: 23.3% and 8.3%, 25% and 8.3%, and 21.7% and 11.7% respectively. For VMI and GAC these proportions were 25% and 8.3%, and 35% and 13.3%. There were few predictors of outcomes: for FSIQ, grade-IV encephalopathy [effect size -15, 95%CI -29,-1; p=0.03]; PIQ, grade-IV encephalopathy [effect size -16, 95%CI -31, -1; p=0.04], VIQ, grade-IV encephalopathy [effect size -17, 95%CI -31, -4; p=0.01], living-related donor [effect size 10, 95%CI 2, 18; p=0.02], and rejection in first 30d [effect size 9, 95%CI 0.5, 17; p=0.04]; and VMI, grade-IV encephalopathy [effect size -20, 95%CI -33, -7; p=0.002], and PELD at activation [effect size 0.5, 95%CI 0.2, 0.9; p=0.006]. Variables not associated with neurocognitive outcomes included: age at transplant, year of transplant, having any severe complication post-operatively, growth failure, and socioeconomic status. CONCLUSION Neurocognitive and functional outcomes after liver transplant at age &lt;6 years are shifted to the left of population norms. Severe encephalopathy at transplant predicted a poorer outcome. More research is needed to determine risk factors for the over 3X higher prevalence of scores &lt;70 compared to population norms.

Deterioration of functional abilities in children surviving the Fontan operation.

Functional abilities are needed for activities of daily living. In general, these skills expand with age. We hypothesised that, in contrast to what is normally expected, children surviving the Fontan may have deterioration of functional abilities, and that peri-Fontan stroke is associated with this deterioration. All children registered in the Western Canadian Complex Pediatric Therapies Follow-up Program who survived a Fontan operation in the period 1999-2016 were eligible for inclusion. At the age of 2 years (pre-Fontan) and 4.5 years (post-Fontan), the Adaptive Behavior Assessment System-II general adaptive composite score was determined (population mean: 100, standard deviation: 15). Deterioration of functional abilities was defined as ⩾1 standard deviation decrease in pre- to post-Fontan scores. Perioperative strokes were identified through chart review. Multivariable logistic regression analysis determined predictors of deterioration of functional abilities. Of 133 children, with a mean age at Fontan of 3.3 years (standard deviation 0.8) and 65% male, the mean (standard deviation) general adaptive composite score was 90.6 (17.5) at 2 years and 88.3 (19.1) at 4.5 years. After Fontan, deterioration of functional abilities occurred in 34 (26%) children, with a mean decline of 21.8 (7.1) points. Evidence of peri-Fontan stroke was found in 10 (29%) children who had deterioration of functional abilities. Peri-Fontan stroke (odds ratio 5.00 (95% CI 1.74, 14.36)) and older age at Fontan (odds ratio 1.67 (95% CI 1.02, 2.73)) predicted functional deterioration. The trajectory of functional abilities should be assessed in this population, as more than 25% experience deterioration. Efforts to prevent peri-Fontan stroke, and to complete the Fontan operation at an earlier age, may lead to reduction of this deterioration.

Conceptual skills in persons with visual impairment

Conceptual skills enable the development of abilities necessary for controlling certain aspects of life. They include communication skills, functional literacy, and self-direction skills. The aim of this paper was to determine the acquisition of conceptual skills in persons with visual impairment. The research was conducted on a sample of 127 persons with visual impairment, 19-60 years of age. Conceptual Skills Domain of Adaptive Behavior Assessment System II (ABAS-II) was used to obtain data on the acquisition of conceptual skills. It was determined that age (p=0.001) and the category of visual impairment (blindness and low vision) (p=0.000) were significant factors for the acquisition of conceptual skills in persons with visual impairment. On the other hand, time when vision loss occurred was not a significant factor for acquiring conceptual skills in persons with visual impairment (p=0.195).

Group Differences in Adaptive Behavior: Evidence from the USA

As a psychoeducational construct, adaptive behavior refers to an individual’s independent display of behaviors associated with meeting his/her daily personal and social needs, including behaviors expected in domestic and social environments. Since the 1960’s, adaptive behavior has been perceived as a viable alternative to standardized intelligence tests, especially in multicultural contexts. The present study compares the adaptive behavior of Whites and Blacks, using three large normative samples drawn from the standardization of the Adaptive Behavior Assessment System-II. The ABAS-II is an ideal subject for the study of race differences, as it provides a psychometrically solid measure of the spectrum of adaptive behaviors. Findings of multivariate analyses indicate that the adaptive behaviors of Blacks and Whites are largely similar among young children, from infancy to 5 years of age. After the age of 5 years, race differences in adaptive behavior emerge and become more pronounced with age. Appreciable race differences are noted for children, adolescents, and adults. Across the span of adaptive behaviors measured by the ABAS-II, most differences favor Whites. Possible mechanisms for these observed differences are offered, as well as suggestions for future investigation.

A Pilot Trial of Sodium Benzoate, a D-Amino Acid Oxidase Inhibitor, Added on Augmentative and Alternative Communication Intervention for Non-Communicative Children with Autism Spectrum Disorders

Background: This open-label clinical trial examined the efficacy and safety of a d-amino oxidase inhibitor, sodium benzoate, in the treatment of non-communicative children with autism spectrum disorder. We hypothesized that benzoate, through indirect glutamate stimulation, may enhance learning in communication. Method: Six children (five boys, one girl, 3-year-7-month to 9-year-6-month of age) completed the 12 weeks of study of receiving sodium benzoate in conjunction with communication training program. The outcome measures were Receptive and Expressive Vocabulary Test-Chinese, parent-reported Adaptive Behavior Assessment System-II, number of core vocabularies learned in the applied communication learning system, Chinese child developmental inventory and parenting stress index. Safety assessments included biweekly recorded vital signs, body weight, body height and adverse events. Results: We noted improvement of communication in half of the children on benzoate. An activation effect was reported by caregivers in three of the six children, and was corroborated by clinician’s observation. Conclusion: Though the data are too preliminary to draw any definite conclusions about efficacy, they do suggest this therapy to be safe, and worthy of a double-blind placebo-controlled study with more children participated for clarification of its efficacy.

Prevalence of autism and attention-deficit-hyperactivity disorder in Down syndrome: a population-based study.

To investigate the prevalence of autism spectrum disorder (ASD) and attention-deficit-hyperactivity disorder (ADHD) in a population-based group of children and adolescents with Down syndrome, and to relate the findings to level of intellectual disability and to medical conditions. From a population-based cohort of 60 children and adolescents with Down syndrome, 41 individuals (29 males, 12 females; mean age 11y, age range 5-17y) for whom parents gave consent for participation were clinically assessed with regard to ASD and ADHD. The main instruments used were the Autism Diagnostic Interview-Revised, Autism Diagnostic Observation Schedule, Swanson, Nolan, and Pelham-IV Rating Scale, and the Adaptive Behavior Assessment System-II. High rates of ASD and ADHD were found: 17 (42%) and 14 (34%) of the 41 children met DSM criteria for ASD and ADHD respectively. Children with Down syndrome and coexisting neurodevelopmental/neuropsychiatric disorders in addition to intellectual disability and medical disorders constitute a severely disabled group. Based on the results, we suggest that screening is implemented for both ASD and ADHD, at the age of 3 to 5 years and early school years respectively, to make adequate interventions possible.

Safety and efficacy of cognitive training plus epigallocatechin-3-gallate in young adults with Down's syndrome (TESDAD): a double-blind, randomised, placebo-controlled, phase 2 trial

Early cognitive intervention is the only routine therapeutic approach used for amelioration of intellectual deficits in individuals with Down's syndrome, but its effects are limited. We hypothesised that administration of a green tea extract containing epigallocatechin-3-gallate (EGCG) would improve the effects of non-pharmacological cognitive rehabilitation in young adults with Down's syndrome. We enrolled adults (aged 16-34 years) with Down's syndrome from outpatient settings in Catalonia, Spain, with any of the Down's syndrome genetic variations (trisomy 21, partial trisomy, mosaic, or translocation) in a double-blind, placebo-controlled, phase 2, single centre trial (TESDAD). Participants were randomly assigned at the IMIM-Hospital del Mar Medical Research Institute to receive EGCG (9 mg/kg per day) or placebo and cognitive training for 12 months. We followed up participants for 6 months after treatment discontinuation. We randomly assigned participants using random-number tables and balanced allocation by sex and intellectual quotient. Participants, families, and researchers assessing the participants were masked to treatment allocation. The primary endpoint was cognitive improvement assessed by neuropsychologists with a battery of cognitive tests for episodic memory, executive function, and functional measurements. Analysis was on an intention-to-treat basis. This trial is registered with ClinicalTrials.gov, number NCT01699711. The study was done between June 5, 2012, and June 6, 2014. 84 of 87 participants with Down's syndrome were included in the intention-to-treat analysis at 12 months (43 in the EGCG and cognitive training group and 41 in the placebo and cognitive training group). Differences between the groups were not significant on 13 of 15 tests in the TESDAD battery and eight of nine adaptive skills in the Adaptive Behavior Assessment System II (ABAS-II). At 12 months, participants treated with EGCG and cognitive training had significantly higher scores in visual recognition memory (Pattern Recognition Memory test immediate recall, adjusted mean difference: 6·23 percentage points [95% CI 0·31 to 12·14], p=0·039; d 0·4 [0·05 to 0·84]), inhibitory control (Cats and Dogs total score, adjusted mean difference: 0·48 [0·02 to 0·93], p=0·041; d 0·28 [0·19 to 0·74]; Cats and Dogs total response time, adjusted mean difference: -4·58 s [-8·54 to -0·62], p=0·024; d -0·27 [-0·72 to -0·20]), and adaptive behaviour (ABAS-II functional academics score, adjusted mean difference: 5·49 [2·13 to 8·86], p=0·002; d 0·39 [-0·06 to 0·84]). No differences were noted in adverse effects between the two treatment groups. EGCG and cognitive training for 12 months was significantly more effective than placebo and cognitive training at improving visual recognition memory, inhibitory control, and adaptive behaviour. Phase 3 trials with a larger population of individuals with Down's syndrome will be needed to assess and confirm the long-term efficacy of EGCG and cognitive training. Jérôme Lejeune Foundation, Instituto de Salud Carlos III FEDER, MINECO, Generalitat de Catalunya.

Clinical and Functional Developmental Outcomes in Neonates Undergoing Truncus Arteriosus Repair: A Cohort Study

Truncus arteriosus (TA) is an uncommon congenital cardiac lesion that portends an exceedingly poor prognosis if not repaired. The objective of this study was to assess the clinical and developmental outcomes in a prospective cohort of patients who underwent TA repair. All patients who underwent a TA repair between 1996 and 2012 were included. Follow-up clinical, neurologic, and developmental data were obtained from the Western Canadian Complex Pediatric Therapies Follow-up Program database. Functional developmental outcomes were assessed at 21.1 ± 2.5 months of age with the Adaptive Behavior Assessment System-II, General Adaptive Composite (GAC) score. Survival and outcomes were compared between those with and without chromosomal abnormalities (CA). Survival and freedom from reintervention were assessed by Kaplan-Meier analysis. The study comprised 36 infants (19 male). CA was identified in 13, with 22q11.2 deletion in 10 patients. Patients underwent TA repair at a median age of 10 days; 5patients underwent concomitant interrupted arch repair. There were 8 deaths, 2 of which occurred in the hospital. The 5-year survival was 79.4%. Survival was similar between those with and without CA. At 5 years, freedom from reoperation was 77.2%. The mean GAC was higher in the patients without CA (93.6 ± 12.8 vs 76.1 ± 13.1, p= 0.0016). Patients with surgically repaired TA continue to have significant postoperative mortality. Reoperation and cardiac catheterization are eventualities for a quarter of patients in the first 5 years of life. Functional developmental outcome in patients without CA is good, although it is significantly impaired in those with CA.

From the Editor

From the Editor