Vineland Adaptive Behavior Scales-II Research Articles

Fine Motor Skills, a Surrogate of Motor Planning Ability, at Age 2 Predict Social Skills at Age 6.

Motor planning is the cognitive process of planning necessary steps for achieving a purposeful movement and is specifically reflected through object manipulation. This study aimed to investigate whether fine motor skills, a surrogate of the motor planning ability of object manipulation, in early childhood are associated with later social skills, in a general-population birth cohort. A total of 913 children, participating in the Hamamatsu Birth Cohort for Mothers and Children, were enrolled. Social skills were measured using the Vineland Adaptive Behavior Scales-II, Socialization domain, at age 6 years. Fine motor skills were measured using the Mullen Scales of Early Learning at 14, 24, and 32 months. The associations between fine motor skills at ages 14, 24, and 32 months and social skills at age 6 years were tested separately through multivariable linear regression after adjusting for covariates, including gross motor and language skills at the contemporaneous age, autistic symptoms at age 6 years, and demographic factors. Fine motor skills at 24 and 32 months were significantly associated with social skills at age 6 years (at 24 months: nonstandardized regression coefficient = 1.38 [95% CI, 0.50-2.26], p = 0.002; at 32 months: 1.47 [0.56-2.38], p = 0.001). Fine motor skills in early childhood predicted social skills at age 6 years, indicating an association between the complex motor planning ability of object manipulation and later social skills. Children who demonstrate fine motor delay at as early an age as 2 years should be closely monitored by child professionals.

Effectiveness of a Training Program for Parents of Toddlers with or at Risk of Autism Spectrum Disorder

Purpose: This study aimed to evaluate a hospital-provided hybrid parental support program designed to help parents implement routine interventions for their toddlers under 34 months, with or at risk of autism spectrum disorder (ASD).Methods: An experimental group including 15 pairs of toddlers and their parents received eight weekly sessions, while a control group including nine pairs received no sessions. Toddlers’ joint attention, autism-related behaviors, and adaptive behaviors, as well as parenting stress and parenting efficacy, were compared between baseline and 8 weeks later. Results: The toddlers in the experimental group showed significant improvement in their joint attention scores on the Early Social Communication Scales (<i>P</i><0.01) and the Korean-Childhood Autism Rating Scale scores (<i>P</i><0.01). However, statistically significant differences were not found in the adaptive behavior composite scores of the Korean Vineland Adaptive Behavior Scales-II and total behavior problem scores of the Korean version of the Child Behavior Checklist. Parenting efficacy in the experimental group showed significant improvement (<i>P</i><0.05). Parenting stress increased in the experimental group, but the difference was not significant. Additionally, parents showed a statistically significant between-group difference in parenting efficacy (<i>P</i><0.05). Conclusion: A hospital-provided hybrid program affected the development of toddlers with ASD and parents’ implementation of interventions. It is significant that an online platform was used to provide individualized support for families to continue practicing the interventions and that the effectiveness of this program was demonstrated. This intervention can be considered as an efficient and sustainable alternative system to support toddlers with ASD and their families.

Specific cognitive dysfunctioning and vulnerability to specific psychopathology: A descriptive study on intellectual developmental disorder (intellectual disability)

IntroductionIntellectual Developmental Disorder (IDD) is diagnosed with cognitive and adaptive behaviour evaluations. There is increasing evidence of a high prevalence of psychiatric disorders comorbid with IDD. The relationship between specific cognitive dysfunctions and psychiatric vulnerability may provide the basis for a paradigm shift from “intellectually below average IQ” to “neuropsychological characterization”.Objectives1) reassessing an IDD sample in cognitive profile and psychiatric comorbidities 2) investigating the correlations between specific cognitive dysfunctions and specific psychiatric diagnoses in IDD.Methods120 individuals with IDD from 3 Italian facilities were consecutively evaluated, one group with mild IDD, using WAIS-IV or Leiter-3, TMT, Stroop and TOL tests, after which a professional caregiver did individual interviews (Vineland Adaptive Behavior Scale-II, SPAIDD-G, and STA-DI) to evaluate the patient adaptive behaviour, psychiatric comorbidities and presence of ASD. The second group (more severe IDD), was evaluated only with professional caregiver assessment tools.Results90 males and 30 females, mean age 57 years, institutionalized for a mean period of 36.44 years. 52% had no education, 19% a middle school diploma. IDD diagnoses: borderline 3%, mild 16%, moderate 11%, moderate-severe 4%, severe 59%, profound 0%.11% comorbid ASD diagnosis, 29% with ASD after diagnostic re-assessment (STA-DI). 89% physical comorbidities, 58% psychiatric comorbidities, 56% psychoses (Fig. 1). Psychiatric comorbidities re-assessment (SPAIDD-G) identified a significant number of disorders (Fig. 2), despite the medical records showed a low prevalence of psychiatric diagnoses. The consistent quantity of psychotropic drugs prescribed in the sample, possibly reflects the real prevalence of psychopathology. Pearson correlations (p<0.05). WAIS-IV and SPAIDD-G (N=29): Verbal Comprehension Index correlates with anxiety disorder and impulse control disorder; Perceptual Reasoning Index correlates with nutrition/feeding disorder; Processing Speed Index correlates with nutrition/feeding disorder and sexual disorder; IQ correlates with ASD, nutrition/feeding, anxiety, sexual disorders. Leiter-3 and SPAIDD-G (N=14): Form Completion and non-verbal IQ correlate with OCD negatively.Image:Image 2:ConclusionsIn conclusion, the SPAIDD-G evaluations revealed a greater prevalence of psychopathology than reported in the medical records. Using psychopathological screening tools can improve the diagnostic process in residential facilities for IDD cases. Pearson’s analyses revealed the need to further investigate the correlation between cognitive dysfunctions and psychopathological vulnerability, studying intelligence as a multi-component model and identifying specific behavioural and cognitive phenotypes in IDD cases.Disclosure of InterestNone Declared

Positive behavioral effect of sensory integration intervention in young children with autism spectrum disorder.

Sensory integration occupational therapy (SI-OT) might be useful for autism spectrum disorders (ASD) in younger age, according to Pusponegoro. Previous studies were still limited, especially in younger age. This study evaluated the effect of SI-OT in improving positive behaviors of ASD children aged 2-5 years. Non-randomized controlled trial of SI-OT evaluation, assessed with Vineland Adaptive Behavior Scale-II. A total of 72 subjects were studied. Following SI-OT, communication skills (expressive, receptive), socialization (coping skills), and daily living skills (personal, community) were improved significantly. SI-OT with Ayres theory in 60 min, twice a week for 12 weeks improved positive behaviors. To address the effectivity of sensory integration occupational therapy (SI-OT) in improving positive behaviors of autism spectrum disorders (ASD) children aged 2-5 years. The first study to evaluate evidence of SI-OT in ASD younger than 3 years. Non-randomized controlled trial of SI-OT evaluation of ASD in younger children aged 2-5 years with Vineland Adaptive Behavior Scale-II. SI-OT of Ayres theory in 60 min, twice a week for 12 weeks improves communication domain (expressive, receptive, written) aged 2-4 years, and socialization domain (interpersonal relationship, play, and leisure time) aged 2 years of ASD children.

Intellectual Disability Profiles, Quality of Life and Maladaptive Behavior in Deaf Adults: An Exploratory Study.

Individuals who are prelingually deaf and have intellectual disabilities experience great challenges in their language, cognitive and social development, leading to heterogeneous profiles of intellectual and adaptive functioning. The present study describes these profiles, paying particular attention to domain discrepancies, and explores their associations with quality of life and maladaptive behavior. Twenty-nine adults with prelingual deafness (31% female) and mild intellectual functioning deficits (mean IQ = 67.3, SD = 6.5) were administered the Vineland Adaptive Behavior Scales-II (VABS-II) and an adapted sign language version of a quality of life scale (EUROHIS-QOL 8). Intellectual disability domain discrepancies were characterized as at least one standard deviation difference between the social domain and IQ and the practical domain and IQ, and a significant difference, according to the VABS-II manual, between the social and practical domains. Domain discrepancies were found between intellectual functioning and both the practical (58.6%) and social domain (65.5%). A discrepancy between intellectual and social functioning was significantly associated with a higher level of internalizing maladaptive behavior (T = 1.89, p < 0.05). The heterogeneous profiles highlight the importance of comprehensive assessments for adequate service provision.

Cingulate-Prefrontal Connectivity During Dynamic Cognitive Control Mediates Association Between p Factor and Adaptive Functioning in a Transdiagnostic Pediatric Sample

BackgroundCovariation among psychiatric symptoms is being actively pursued for transdiagnostic dimensions of psychopathology with predictive utility. A superordinate dimension, the p factor, reflects overall psychopathology burden and has support from genetic and neuroimaging correlates. However, the neurocognitive correlates that link an elevated p factor to maladaptive outcomes are unknown. We tested the mediating potential of dynamic adjustments in cognitive control rooted in functional connections anchored by the dorsal anterior cingulate cortex (dACC) in a transdiagnostic pediatric sample. MethodsA multiple mediation model tested the association between the p factor (derived by principal component analysis of Child Behavior Checklist syndrome scales) and outcome measured with the Vineland Adaptive Behavior Scale-II in 89 children ages 8 to 13 years (23 female) with a variety of primary neurodevelopmental diagnoses who underwent functional magnetic resonance imaging during a socioaffective Stroop-like task with eye gaze as distractor. Mediators included functional connectivity of frontoparietal- and salience network–affiliated dACC seeds during conflict adaptation. ResultsHigher p factor scores were related to worse adaptive functioning. This effect was partially mediated by conflict adaptation–dependent functional connectivity between the frontoparietal network–affiliated dACC seed and the right dorsolateral prefrontal cortex. Post hoc follow-up indicated that the p factor was related to all Vineland Adaptive Behaviors Scale-II domains; the association was strongest for socialization followed by daily living skills and then communication. Mediation results remained significant for socialization only. ConclusionsHigher psychopathology burden was associated with worse adaptive functioning in early adolescence. This association was mediated by weaker dACC–dorsolateral prefrontal cortex functional connectivity underlying modulation of cognitive control in response to contextual contingencies. Our results contribute to the identification of transdiagnostic and developmentally relevant neurocognitive endophenotypes of psychopathology.

Characterization of Cognitive, Language and Adaptive Profiles of Children and Adolescents with Malan Syndrome

Malan Syndrome (MS) is an ultra-rare overgrowth genetic syndrome due to heterozygous variants or deletions in the Nuclear Factor I X (NFIX) gene. It is characterized by an unusual facial phenotype, generalized overgrowth, intellectual disability (ID) and behavioral problems. Even though limitations in cognitive and adaptive functioning have been previously described, systematic studies on MS cohorts are still lacking. Here, we aim to define the cognitive and adaptive behavior profile of MS children and adolescents, providing quantitative data from standardized evaluations. Subjects included in this study were evaluated from October 2020 to January 2022 and the study is based on a retrospective data archive: fifteen MS individuals were recruited and underwent evaluation with Wechsler Intelligence Scales, Leiter International Performance Scales and Griffith Mental Development Scales for cognitive profiles and with Vineland Adaptive Behavior Scales-II Edition (VABS-II) for adaptive functioning. Language skills and visuomotor integration abilities were assessed too. Comparisons and correlations between scales and subtests were performed. All the assessed MS individuals showed both low cognitive and adaptive functioning. One subject presented with mild ID, five had moderate ID and eight showed severe ID. One female toddler received a diagnosis of psychomotor delay. Linguistic skills were impaired in all individuals, with language comprehension relatively more preserved. Results revealed significant differences between VABS-II subdomains and a strong relationship between cognitive and adaptive functioning. All subjects exhibited mild to moderate ID and adaptive behavior lower than normal, with communication skills being the most affected. Regarding the daily living skills domain, personal and community subscale scores were dramatically lower than for the domestic subdomain, highlighting the importance of considering behavior within developmental and environmental contexts. Our cognitive and adaptive MS characterization provides a more accurate quantitative MS profiling, which is expected to help clinicians to better understand the complexity of this rare disorder.

The psychometric properties of the Norwegian version of the social responsiveness scale in a neuropediatric sample

BackgroundThe current study is an examination of the psychometric properties of the Norwegian Social Responsiveness Scale (SRS), a measure of deficits in social behavior, in a neuropediatric outpatient sample of children and adolescents with neurological and neurodevelopmental disorders. MethodThe internal consistency of the SRS, the convergent validity of the SRS with the Vineland Adaptive Behavior Scale-II (VABS-II), the Strengths and Difficulties Questionnaire (SDQ), and the Aberrant Behavior Checklist (ABC) were examined, in addition to four different factor models of the SRS (i.e., a one-factor, the original five-factor, a second-order five-factor model, and a 16-item one-factor model) using confirmatory factor analyses. ResultsThere was satisfactory internal consistency on all subscales, except for the Social Awareness subscale. The SRS showed a somewhat meaningful overlap with parts of the related scales on the VABS-II, the SDQ, and the ABC. Model fit indices were mixed for evaluating the four different factor models. Overall, however, the model fit was rather poor. ConclusionsThe original SRS subscales showed adequate internal consistency and satisfactory convergent validity on some of the subscales. The construct validity in terms of factor structure was not acceptable. Future research should examine the psychometric properties of an improved version of the SRS, especially in terms of improving the scale’s construct validity.

Neurobiological Correlates of Change in Adaptive Behavior in Autism

Autism spectrum disorder (ASD) is a lifelong neurodevelopmental condition that is associated with significant difficulties in adaptive behavior and variation in clinical outcomes across the life span. Some individuals with ASD improve, whereas others may not change significantly, or regress. Hence, the development of "personalized medicine" approaches is essential. However, this requires an understanding of the biological processes underpinning differences in clinical outcome, at both the individual and subgroup levels, across the lifespan. The authors conducted a longitudinal follow-up study of 483 individuals (204 with ASD and 279 neurotypical individuals, ages 6-30 years), with assessment time points separated by ∼12-24 months. Data collected included behavioral data (Vineland Adaptive Behavior Scale-II), neuroanatomical data (structural MRI), and genetic data (DNA). Individuals with ASD were grouped into clinically meaningful "increasers," "no-changers," and "decreasers" in adaptive behavior. First, the authors compared neuroanatomy between outcome groups. Next, they examined whether deviations from the neurotypical neuroanatomical profile were associated with outcome at the individual level. Finally, they explored the observed neuroanatomical differences' potential genetic underpinnings. Outcome groups differed in neuroanatomical features (cortical volume and thickness, surface area), including in "social brain" regions previously implicated in ASD. Also, deviations of neuroanatomical features from the neurotypical profile predicted outcome at the individual level. Moreover, neuroanatomical differences were associated with genetic processes relevant to neuroanatomical phenotypes (e.g., synaptic development). This study demonstrates, for the first time, that variation in clinical (adaptive) outcome is associated with both group- and individual-level variation in anatomy of brain regions enriched for genes relevant to ASD. This may facilitate the move toward better targeted/precision medicine approaches.

Characterization of phenotypic range in DYRK1A haploinsufficiency syndrome using standardized behavioral measures

DYRK1A haploinsufficiency syndrome is a well-established neurodevelopmental disorder, but detailed information on the range of cognitive and behavioral issues associated with the condition is limited. We studied 24 participants with likely pathogenic or pathogenic variants in DYRK1A through the Simons Searchlight study and systematically assessed their medical history and development using standardized instruments: Vineland Adaptive Behavior Scale II (VABS-II) and Child Behavior Checklists/1.5-5 and 6-18 (CBCL/1.5-5, CBCL/6-18). All of the individuals in the cohort had neurological manifestations including intellectual disability or developmental delay, microcephaly, autism spectrum disorder, and/or seizures. The severity of the neurodevelopmental disorder was variable with a few children scoring in the moderately low range on the adaptive behavior composite score on the VABS-II. This study confirms the association of DYRK1A haploinsufficiency with neurodevelopmental disabilities, microcephaly, autism spectrum disorder, and epilepsy and quantifies the range of adaptive behaviors.

A Mobile Game Platform for Improving Social Communication in Children with Autism: A Feasibility Study.

Background Many children with autism cannot receive timely in-person diagnosis and therapy, especially in situations where access is limited by geography, socioeconomics, or global health concerns such as the current COVD-19 pandemic. Mobile solutions that work outside of traditional clinical environments can safeguard against gaps in access to quality care. Objective The aim of the study is to examine the engagement level and therapeutic feasibility of a mobile game platform for children with autism. Methods We designed a mobile application, GuessWhat , which, in its current form, delivers game-based therapy to children aged 3 to 12 in home settings through a smartphone. The phone, held by a caregiver on their forehead, displays one of a range of appropriate and therapeutically relevant prompts (e.g., a surprised face) that the child must recognize and mimic sufficiently to allow the caregiver to guess what is being imitated and proceed to the next prompt. Each game runs for 90 seconds to create a robust social exchange between the child and the caregiver. Results We examined the therapeutic feasibility of GuessWhat in 72 children (75% male, average age 8 years 2 months) with autism who were asked to play the game for three 90-second sessions per day, 3 days per week, for a total of 4 weeks. The group showed significant improvements in Social Responsiveness Score-2 (SRS-2) total (3.97, p <0.001) and Vineland Adaptive Behavior Scales-II (VABS-II) socialization standard (5.27, p = 0.002) scores. Conclusion The results support that the GuessWhat mobile game is a viable approach for efficacious treatment of autism and further support the possibility that the game can be used in natural settings to increase access to treatment when barriers to care exist.

The adaptive functioning profile of Pitt-Hopkins syndrome

BackgroundThere are few cohort studies describing the adaptive functioning profile for Pitt-Hopkins syndrome (PTHS). In this study we examine the adaptive functioning profile for PTHS and compare it to Angelman syndrome (AS). MethodCaregivers of 14 individuals with PTHS, 33 with deletion AS and 23 with non-deletion AS, completed the Vineland Adaptive Behavior Scales-II. ResultsThe profile of adaptive functioning in PTHS was characterised by strengths in socialisation, followed by motor skills, communication then daily living skills. The PTHS group scored significantly lower than the non-deletion AS group on all domains except socialisation and significantly lower than the deletion AS group, for motor skills only. ConclusionsAn uneven adaptive behavior profile for individuals with PTHS mirrors that of AS, with implications for assessment and intervention.

Cyclohexanone Exposure in Children on Extracorporeal Membrane Oxygenation Support.

The aim of this study was to determine if plasma cyclohexanone and metabolites are associated with clinical outcomes of children on extracorporeal membrane oxygenation (ECMO) support. We performed a secondary analysis of a prospective observational study of children on ECMO support at two academic centers between July 2010 and June 2015. We measured plasma cyclohexanone and metabolites on the first and last days of ECMO support. Unfavorable outcome was defined as in-hospital death or discharge Pediatric Cerebral Performance Category score > 2 or decline ≥ 1 from baseline. Among 90 children included, 49 (54%) had unfavorable outcome at discharge. Cyclohexanediol, a cyclohexanone metabolite, was detected in all samples and at both time points; concentrations on the first ECMO day were significantly higher in those with unfavorable versus favorable outcome at hospital discharge (median, 5.7 ng/µl; interquartile range [IQR], 3.3-10.6 ng/µl vs. median, 4.2 ng/µl; IQR, 1.7-7.3 ng/µl; p = 0.04). Twofold higher cyclohexanediol concentrations on the first ECMO day were associated with increased risk of unfavorable outcome at hospital discharge (multivariable-adjusted hazard ratio [HR], 1.24 [95% CI, 1.05-1.48]). Higher cyclohexanediol concentrations on the first ECMO day were not significantly associated with new abnormal neuroimaging or 1-year Vineland Adaptive Behavior Scales-II score < 85 or death among survivors.

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.

Short caregiver interview and play observation for early screening of autism spectrum disorder: Behavior development screening for toddlers (BeDevel).

This study validates behavior development screening for toddlers (BeDevel), which utilizes a combination of short caregiver interviews (BeDevel-I) and semistructured play observations (BeDevel-P). The data of 431 toddlers (male 66.2%; mean age (SD) = 29.11 (8.59) months; ASD, n = 201; developmental delay, n = 46; typically developing, n = 184), aged 18 ~ 42 months, were included in the validation of BeDevel. The best clinical estimate diagnosis, screening rate, validity, sensitivity, and reliability of BeDevel were determined based on data cross-sectionally collected using BeDevel and existing diagnostic/screening instruments: autism diagnostic observation schedule (ADOS), autism diagnostic interview (ADI-R), Vineland adaptive behavior scales-II (VABS-II), social response scales (SRS), sequenced language scale for infants (SELSI), Korean childhood autism rating scale (K-CARS), and Korean social communication questionnaire (K-SCQ). The k values of BeDevel-I and BeDevel-P were 0.055 ~ 0.732 and 0.291 ~ 0.752, respectively. Items related to social referencing in BeDevel-P had a particularly high diagnostic validity (k = 0.483 ~ 0.684). Reliabilities of BeDevel-I and BeDevel-P were sufficient (Cronbach's alpha = 0.86 ~ 0.88 and 0.92 ~ 0.95, respectively). BeDevel-I and BeDevel-P showed high sensitivity (BeDevel-I: 85.00 ~ 89.29%; BeDevel-P: 85.00 ~ 91.75%), specificity (BeDevel-I: 77.55 ~ 89.55%; BeDevel-P: 85.09 ~ 97.01%), PPV (BeDevel-I: 70.83 ~ 88.54%; BeDevel-P: 81.52 ~ 94.68%), and NPV (BeDevel-I: 76.00 ~ 95.24%; BeDevel-P: 84.62 ~ 95.45%). The agreement between the composite BeDevel score and ADOS, ADI-R, K-CARS, and K-SCQ was >67.6% (range = 67.6 ~ 90.8%). Combining a short caregiver interview and direct play observation is a valid and reliable screening process. More studies on social referencing as an important early marker are needed. BeDevel can be utilized as a secondary screening instrument before diagnostic confirmation in clinical and community settings. LAY SUMMARY: BeDevel, which consists of a short caregiver interview and direct play observation, is a valid and reliable screening instrument for autism spectrum disorder (ASD). We suggest that BeDevel can be utilized as a secondary instrument before administering diagnostic assessments in clinical and community settings. More studies examining social referencing as a potential behavioral marker of ASD are needed.

Post-haemorrhagic hydrocephalus management: Delayed neonatal transport negatively affects outcome.

Post-haemorrhagic ventricular dilatation (PHVD) still represents an important cause of brain injury in premature infants. Intervention for PHVD is recommended once Ventricular Index (VI) crosses the 97th percentile + 4mm line according to Levene. We aimed to compare PHVD severity, timing of intervention, and outcome between outborn infants transferred to a level IV NICU in order to be treated for PHVD and a control population of inborn infants. Preterm infants with PHVD requiring treatment were divided into: outborn infants (transferred to our NICU in order to be treated for PHVD) and inborn infants (PHVD diagnosed at our NICU). Age at intervention, difference between VI and the 97th percentile according to postmenstrual age (VI-p97), permanent shunt rate, and developmental delay rate were compared between the two groups. Neurodevelopmental outcome was assessed using the Vineland Adaptive Behavior Scales II (VABS-II), a parental questionnaire investigating four domains of adaptive behaviour and overall adaptive functioning. Developmental delay was defined as a score <70 (-2 SD or less). Twelve outborn and 15 inborn infants were included. Age at intervention (31.6 vs 17.4 days) and VI-p97 (left 10.0 vs 5.1mm, right 7.7 vs 5.1mm) were significantly higher among outborn infants. A permanent shunt was inserted in 66.7% of outborn and in 40.0% of inborn infants (p=0.18). After excluding subjects with parenchymal lesions, a significantly higher rate of developmental delay was observed at 5 years in outborn patients compared to inborn patients (66.7% of outborn vs 18.2% of inborn patients with VABS-II composite score <70, p=0.04). Outborn infants reached a significantly more severe ventricular dilatation than inborn infants, largely exceeding the recommended cutoff for intervention. Our follow-up data showed a trend towards a higher rate of permanent shunt and developmental delay in outborn than in inborn patients. Infants requiring treatment should be timely transported to centres with adequate expertise in PHVD management.

Construct validity of the First-Year Inventory (FYI Version 2.0) in 12-month-olds at high-risk for Autism Spectrum Disorder

The First-Year Inventory 2.0 is a parent-report screening instrument designed to identify 12-month-old infants at risk for an eventual diagnosis of Autism Spectrum Disorder. This instrument focuses on Social-Communication and Sensory-Regulatory areas of infant behavior. Although the First-Year Inventory 2.0 screening performance has been previously studied, its validity has not been examined. Establishing validity of an instrument is important because it supports the effectiveness and the reliability of the instrument. In this study, we examined relationship between the First-Year Inventory 2.0 (Social-Communication and Sensory-Regulatory areas) and other instruments that measure similar areas of infant behavior in a sample of high-risk infant siblings of children with Autism Spectrum Disorder. These other instruments share some common aims and theoretical areas with the First-Year Inventory 2.0: the Autism Observation Scale for Infants, the Mullen Scales of Early Learning, the Vineland Adaptive Behavior Scales-II, and the Infant Behavior Questionnaire. Findings generally supported the validity of the First-Year Inventory 2.0 with other instruments. In particular, the Social-Communication area of the First-Year Inventory 2.0 showed greater commonality with other instruments than in the Sensory-Regulatory area. The Sensory-Regulatory area seemed to be a unique feature of the First-Year Inventory 2.0 instrument. Considering different aims and strengths of assessments, researchers and clinicians are encouraged to utilize a variety of instruments in a comprehensive evaluation of a child.

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.

Developmental and sequenced one-to-one educational intervention (DS1-EI) for autism spectrum disorder and intellectual disability: a two-year interim report of a randomized single-blind multicenter controlled trial

BackgroundChildren with autism spectrum disorder (ASD) and moderate to severe intellectual disability (ID) face many challenges. There is little evidence-based research into educational settings for children with ID and ASD and in France. Little is known about how this unserved population could benefit from intervention and education. This study assessed the feasibility and efficacy of a new intervention model using an individualized educational approach.MethodsWe conducted a randomized, single-blind controlled trial to assess a novel intervention: the “Developmental and Sequenced One-to-One Intervention (DS1-EI)”. In DS1-EI, trained teachers worked one-to-one with each child in a small classroom setting, offering 10 h per week of the intervention. The focus was on encouraging spontaneous communication, promoting skills through play with peers, supporting positive interactions, and developmental and sequenced learning. We enrolled 5- to 9-year-old children with ASD and ID across 11 French child care institutions for children with co-occurring ASD and ID. Participants were matched in dyads by developmental quotient and randomized to the treatment-as-usual (TAU) group or the DS1-EI group. Independent raters blindly assessed the primary variables: The Childhood Autism Rating scale (CARS) and the Psychoeducational Profile, third edition (PEP-3). The secondary variables included the Vineland Adaptive Behavior Scale II (VABS-II) and the Clinical Global Assessment Scale (CGAS). Here we perform interim analyses at 24 months.ResultsAt baseline, 72 participants were randomized. Nine patients (5 in the DS1-EI group and 4 in the TAU group) dropped out of the study. Using linear mixed models, both intent-to-treat (ITT) and per-protocol (PP) analyses at the 12-, 18- and 24-month outcomes showed no significant group nor group-by-time interaction effects. However, we found significant improvements in most primary and secondary variables over time in both groups.ConclusionsThe study did not show that DS1-EI was superior to TAU in treating children with ASD and ID over 24 months. However, the low dropout rate shows that DS1-EI is feasible, and well accepted. As the study is still ongoing, we need to wait for data at 36 months to ensure whether DS1-EI could be recommended.Trial registrationANSM130282B-31 (April 16, 2013) and ACTRN12616000592448. Registered 6 May 2016, retrospectively registered, http://www.anzctr.org.au/

Long-Term Neurobehavioral and Quality of Life Outcomes of Critically Ill Children after Glycemic Control

To investigate adaptive skills, behavior, and quality health-related quality of life in children from 32 centers enrolling in the Heart And Lung Failure-Pediatric INsulin Titration randomized controlled trial. This prospective longitudinal cohort study compared the effect of 2 tight glycemic control ranges (lower target, 80-100mg/dL vs higher target, 150-180mg/dL) 1-year neurobehavioral and health-related quality of life outcomes. Subjects had confirmed hyperglycemia and cardiac and/or respiratory failure. Patients aged 2-16years old enrolled between April 2012 and September 2016 were studied at 1year after intensive care discharge. The primary outcome, adaptive skills, was assessed using the Vineland Adaptive Behavior Scale. Behavior and health-related quality of life outcomes were assessed as secondary outcomes using the Pediatric Quality of Life and Child Behavior Checklist at baseline and 1-year follow-up. Group differences were evaluated using regression models adjusting for age category, baseline overall performance, and risk of mortality. Of 369 eligible children, 358 survived after hospital discharge and 214 (60%) completed follow-up. One-year Vineland Adaptive Behavior Scale-II composite scores were not different (mean±SD, 79.9±25.5 vs 79.4±26.9, lower vs higher target; P=.20). Improvement in Pediatric Quality of Life total health from baseline was greater in the higher target group (adjusted mean difference, 8.2; 95% CI, 1.1-15.3; P=.02). One-year adaptive behavior in critically ill children with lower vs higher target glycemic control did not differ. The higher target group demonstrated improvement from baseline in overall health. This study affirms the lack of benefit of lower glucose targeting. ClinicalTrials.gov: NCT01565941.