Improved Neurodevelopmental Outcomes at 5.5 Years of Age in Children Who Received Bovine Milk Fat Globule Membrane and Lactoferrin in Infant Formula Through 12 Months: A Randomized Controlled Trial.

OBJECTIVE: To evaluate the neurodevelopmental outcomes at 5.5 years of age in children who were previously randomized to cow milk-based infant formula (control) or similar formula (milk fat globule membrane + lactoferrin) with added sources of bovine milk fat globule membrane and bovine lactoferrin through 12 months of age. DESIGN: Children who completed study feeding were invited to participate in follow-up assessments: cognitive development across multiple domains (primary outcome; Wechsler Preschool & Primary Scale of Intelligence, 4th Edition), inhibitory control/rule learning (Stroop Task), flexibility/rule learning (Dimensional Change Card Sort), and behavior/emotion (Child Behavior Checklist). RESULTS: Of 292 eligible participants (control: 148, milk fat globule membrane + lactoferrin: 144), 116 enrolled and completed assessments (control: 59, milk fat globule membrane + LF: 57). There were no group demographic differences except family income (milk fat globule membrane + lactoferrin significantly higher). Wechsler Preschool & Primary Scale of Intelligence, 4th Edition composite scores (mean ± standard error) for Visual Spatial (100.6 ± 1.7 vs 95.3 ± 1.7; P = .027), Processing Speed (107.1 ± 1.4 vs 100.0 ± 1.4; P < .001), and Full-Scale IQ (98.7 ± 1.4 vs 93.5 ± 1.5; P = .012) were significantly higher for milk fat globule membrane + lactoferrin versus control, even after controlling for demographic/socioeconomic factors. Stroop Task scores were significantly higher in milk fat globule membrane + lactoferrin versus control (P < .001). Higher Dimensional Change Card Sort scores (P = .013) in the border phase (most complex/challenging) were detected, and more children passed the border phase (32% vs 12%; P = .039) for milk fat globule membrane versus control. No group differences in Child Behavior Checklist score were detected. CONCLUSIONS: Children who received infant formula to 12 months of age with added bovine milk fat globule membrane and bovine lactoferrin versus standard formula demonstrated improved cognitive outcomes in multiple domains at 5.5 years of age, including measures of intelligence and executive function. TRIAL REGISTRATION: Clinicaltrials.gov: https://clinicaltrials.gov/ct2/show/NCT04442477.

WPPSI-IV and NEPSY-II performance in mono- and bilingual 5-6-year-old children: Findings from The FinSwed Study.

Children's language background relates to their neurocognitive development. Knowledge of this relationship is important as bilingualism is common. However, research regarding language background in relation to performance on cognitive tests such as the WPPSI-IV and NEPSY-II is scarce. The present study compared WPPSI-IV and NEPSY-II performances between 5- and 6-year-old Swedish-speaking monolingual (n = 45) and Swedish-Finnish-speaking simultaneous bilingual (n = 34) children in Finland. The participants were gathered by stratified sampling and were assessed with the Swedish versions of the tests. In profile analyses, a significant monolingual advantage was found in some WPPSI-IV subtests and indexes requiring expressive vocabulary (Vocabulary, Similarities, Picture Naming, and Vocabulary Acquisition Index) and visuospatial skills (Object Assembly and Visual Spatial Index). No group differences were found between mono- and bilingual children in receptive language, visual memory, or fluid intelligence. Additionally, no differences were found on the Full Scale IQ. The performance on the WPPSI-IV Similarities subtest improved in a subgroup of bilinguals when answers in both Swedish and Finnish were accounted for, instead of accepting only answers in Swedish. No significant differences were found between mono- and bilinguals on the language and memory tasks of NEPSY-II. These findings highlight the importance of considering the child's language background when assessing expressive language in young children, as well as the benefits of assessing bilinguals in both of their languages.

Biomass fuel use for cooking in Nepalese families and child cognitive abilities, results from a community-based study.

BACKGROUND: Biomass fuel use for cooking is widespread in low to middle income countries. Studies on the association between biomass fuel use and cognitive abilities in children are limited. OBJECTIVE: To examine the association between biomass fuel use for cooking and cognitive abilities in Nepalese children at 4 years of age. METHODS: In a cohort design we have information on biomass fuel use in the households of 533 children in infancy and cognitive abilities when they were 4 years old from a community-based sample. Cognitive abilities were measured by the Wechsler Preschool and Primary Scale of Intelligence, 4th edition (WPPSI-IV) and the NEPSY-II. We examined the associations between biomass fuel use and scores on the WPPSI-IV Full-Scale IQ (FSIQ) (primary outcome), and WPPSI index and NEPSY-II subtest scores in multiple linear regression models. The associations were also examined in predefined subgroups. RESULTS: Ninety-nine (18.6%) of the families used biomass fuel for cooking. Children in these families had lower mean FSIQ than children in families with no biomass use (83.3 (95%CI 81.7, 85.0) vs. 85.3 (95%CI 84.5, 86.0)), with a mean difference of -2.2 (95%CI -3.9, -0.5) adjusting for demographics and socio-economic status. The association between biomass fuel use and cognitive abilities was strongest in subgroups of children from households with more than three rooms, with separate kitchen and bedroom, and with higher wealth-score. These interactions were significant for number of rooms in the home (p = 0.04), if the household had separate bedroom and kitchen (p = 0.05), and for the wealth-score (p = 0.03). CONCLUSION: Biomass fuel use for cooking in Nepalese families was associated with lower overall cognitive abilities at 4 years. Uncertainties include exposure misclassification and unmeasured confounding. The associations between biomass fuel use and neurodevelopment in children needs further investigation with more precise measurements of the exposure.

Neurocognitive development in isolated Robin sequence treated with the Tuebingen palatal plate.

OBJECTIVES: We aimed to determine the neurocognitive development of cleft palate patients with and without Robin sequence (RS). MATERIALS AND METHODS: Children with isolated RS with cleft palate and children with cleft palate only (CPO) were contacted at the age of 5-6 years. All RS children had undergone initial polygraphic sleep study (PG) with a mixed-obstructive apnea index (MOAI) of ≥ 3/h and were consequently treated with the Tuebingen palatal plate. A standardized clinical examination as well as a neuropediatric and neuropsychological examination included the Wechsler Pre-school and Primary Scale of Intelligence (WPPSI-III), Kaufman Assessment Battery for Children (K-ABC), and an assessment of developmental milestones. RESULTS: In total, 44 children (22RS, 22CPO) were included. RS children were younger at study (70.5 ± 7.3 and 75.2 ± 7.5 months; P = .035). Both groups achieved the evaluated milestones within the normed time frame. WPPSI-III and K-ABC results showed no group differences. Mean values for Verbal IQ (101.8 ± 11.1 vs. 97.1 ± 15.7), Performance IQ (102.9 ± 12.1 vs. 99.6 ± 14.5), Processing Speed Quotient (98.9 ± 15.6 vs. 94.5 ± 15.7), Full-Scale IQ (103.2 ± 12.1 vs. 98.4 ± 15.3), and Sequential Processing Scale (102.1 ± 13.1 vs. 94.2 ± 17.3) were within the reference range (IQ 85-115) for RS and CPO children, respectively, indicating average performance of both groups. CONCLUSION: No neurocognitive, physical, or mental impairments were detected suggesting that RS children having upper airway obstruction (UAO) treated early and effectively may use their potential for an age-appropriate neurocognitive development. CLINICAL RELEVANCE: Tuebingen palatal plate treatment successfully releases UAO. Thus, isolated RS does not necessarily result in developmental delay or an impaired neurocognitive outcome. TRIAL REGISTRATION: Deutsches Register Klinischer Studien, DRKS00006831, https://www.drks.de/drks_web/.

High Maternal Blood Mercury Level Is Associated with Low Verbal IQ in Children.

The objective of the present study was to investigate the relationship of IQ in children with maternal blood mercury concentration during late pregnancy. The present study is a component of the Mothers and Children's Environmental Health (MOCEH) study, a multi-center birth cohort project in Korea that began in 2006. The study cohort consisted of 553 children whose mothers underwent testing for blood mercury during late pregnancy. The children were given the Korean language version of the Wechsler Preschool and Primary Scale of Intelligence, revised edition (WPPSI-R) at 60 months of age. Multivariate linear regression analysis, with adjustment for covariates, was used to assess the relationship between verbal, performance, and total IQ in children and blood mercury concentration of mothers during late pregnancy. The results of multivariate linear regression analysis indicated that a doubling of blood mercury was associated with the decrease in verbal and total IQ by 2.482 (95% confidence interval [CI], 0.749-4.214) and 2.402 (95% CI, 0.526-4.279), respectively, after adjustment. This inverse association remained after further adjustment for blood lead concentration. Fish intake is an effect modifier of child IQ. In conclusion, high maternal blood mercury level is associated with low verbal IQ in children.

[Cognitive Profiles of Preschool Children with Developmental Coordination Disorders and ADHD]. / Kognitive Leistungsprofile von Kindern mit motorischen Entwicklungsstörungen und ADHS im Vorschulalter.

Cognitive Profiles of Preschool Children with Developmental Coordination Disorders and ADHD Studies confirm that developmental coordination disorders (DCD) are often accompanied by ADHD. It is important to know why children with combined disorders show a special profile in a common intelligence test (WPPSI-III). For this purpose, the WPPSI-III results of a total of 125 children aged five to six years with diagnosed isolated DCD, isolated ADHD, combined disorders and a normative sample were compared. Children with isolated ADHD showed the best cognitive profile. Children of all three diagnosis subgroups presented significantly poorer abilities in all WPPSI-III scales than the normative sample. In comparison with preschoolers showing isolated ADHD, children with DCD and ADHD have a significant lower Processing Speed Quotient.

Cognitive function in SMA patients with 2 or 3 SMN2 copies treated with SMN-modifying or gene addition therapy during the first year of life.

BACKGROUND: Spinal muscular atrophy (SMA) is a neuromuscular disease, causing progressive muscle weakness due to loss of lower motoneurons. Since 2017, three therapies, two modifying gene transcription and one adding the defective gene, have been approved with comparable efficacy on motor outcome. Data on cognitive outcomes of treated SMA type 1 patients is limited. The aim of this study was to evaluate cognitive function in symptomatic and presymptomatic SMA type 1 patients with two or three SMN2 copies who received SMN-modifying or gene-addition therapy in the first year of life. METHODS: Cognitive testing was performed in 20 patients, including 19 symptomatic SMA type 1 patients with up to three SMN2 copies and 1 pre-symptomatically treated patient. Children were tested using Bayley Scales of Infant Development (BSID-III) at the age of 2 or 3 years or the Wechsler Preschool and Primary Scale of Intelligence (WPSII-IV) at the of age of 5 years. RESULTS: 11/20 patients showed subnormal cognitive development. Boys had significantly lower cognitive scores. Patients requiring assisted ventilation or feeding support were more likely to have cognitive deficits. Achieving more motor milestones was associated with a better cognitive outcome. CONCLUSION: Treated patients with SMA type 1 have heterogeneous cognitive function with 55 % of patients showing deficits. Risk factors for cognitive impairment in our cohort were male gender and need for assisted ventilation or feeding support. Therefore, cognitive assessment should be included in the standard of care to allow early identification of deficits and potential therapeutic interventions.

The Effects of Language Background and Parental Education on Measures of Cognitive Ability: An Analysis of the WPPSI-IV Cognitive Profiles of Monolingual, Simultaneous Bilingual, and Sequential Bilingual German Children Aged 4 to 7 Years.

BACKGROUND: The present study investigated the possible effects of language background (monolinguals, simultaneous bilinguals, and sequential bilinguals) and parental education (no/low, medium, high, and highest parental education) on measures of cognitive ability provided by the Wechsler Primary and Preschool Scale-Fourth Edition (WPPSI-IV). METHODS: Statistical analyses were based on a sample of 290 children (130 females, 160 males). Three multivariate variance analyses were conducted to identify possible effects. In cases of statistically significant main effects, post hoc analyses were additionally performed to identify group differences. RESULTS: The results indicated that simultaneous bilinguals performed more similarly to monolinguals than sequential bilinguals. On average, sequential bilinguals achieved significantly lower scores on the Verbal Comprehension Index (VCI), the Vocabulary Acquisition Index (VAI), and the associated subtests than monolinguals and simultaneous bilinguals. Significantly lower average scores on VAI and the associated subtests were found for simultaneous bilinguals compared to monolinguals. Children with parents having no, a lower, or a medium educational level achieved significantly lower scores on VCI, VAI, and the FSIQ than children with parents having a high or highest educational level on average. CONCLUSIONS: The present findings suggest that the WPPSI-IV represents a suitable and reliable test battery for the assessment of cognitive skills in children with different language backgrounds and parental educational levels.

Every Newborn-Reach Up Early Education Intervention for All Children (EN-REACH)- a parent group intervention for school readiness in Bangladesh, Nepal, and Tanzania: study protocol for a cluster randomized controlled trial.

BACKGROUND: Vulnerable children, including those with neuro-developmental delays and disabilities, often face barriers in accessing early primary education, thus hindering progress toward Sustainable Development Goal 4.2. Evidence-based interventions are essential to enhancing inclusivity and establishing sustainable implementation strategies to address this challenge. This study, Every Newborn-Reach up Early Education Intervention for All Children (EN-REACH), builds on the previous Every Newborn- Simplified Measurement Integrating Longitudinal Neurodevelopmental and Growth (EN-SMILING) observational cohort study. This paper provides the protocol for a cluster randomized controlled trial (cRCT) to evaluate the effectiveness of a parenting group intervention program for enhancing school readiness in Bangladesh, Nepal, and Tanzania, and an embedded process evaluation to inform scalability and feasibility. METHODS: EN-REACH is a cRCT with at least 150 clusters to evaluate the impact of a parent training program led by trained parent-teacher facilitator pairs, focusing on children aged 4 ~ 6 years preparing for preschool. Approximately 500 participants from the EN-SMILING cohort at each site have been identified. A geographic information system will define ~ 50 clusters in each of the three countries, each with approximately ten parent-child dyads. Half the clusters will be randomly assigned to intervention and control groups. The primary outcome is "school readiness", assessed using the Measuring Early Learning Quality and Outcomes tool. Secondary outcomes include Intelligence Quotient, child functioning, growth, visual, and hearing assessments. Data will be collected at baseline, and post-intervention data following implementation of the parent group intervention sessions over approximately 5 months. Quantitative data on coverage and quality care, combined with qualitative insights from children, caregivers, facilitators, and stakeholders' perspectives, will be used to conduct a process evaluation applying the Reach, Effectiveness, Adoption, Implementation, and Maintenance (RE-AIM) framework.  DISCUSSION: This protocol details a trial focused on enhancing school readiness and cognitive abilities in young children, inclusive of those with disabilities, aiming to bridge gap from home to early primary education. EN-REACH aims to provide insights into the effectiveness and acceptability of a co-designed disability-inclusive school readiness program in three countries, potentially impacting national and global policies for all children, including those with disabilities. TRIAL REGISTRATION: The trial was retrospectively registered on clinicaltrials.gov on 29 February 2024 (NCT06334627).

The Global School Adaptation score: a new neurodevelopmental assessment tool for very preterm children at five years of age.

OBJECTIVE: To determine the usefulness of a neurodevelopmental assessment tool consisting of a questionnaire administered to teachers to measure the Global School Adaptation (GSA) scores of very preterm children at the age of 5 years. STUDY DESIGN: A sample of 445 very preterm children (<35 weeks of gestation) was assessed at 5 years of age using GSA and IQ scores. According to the consistency between the scores, children were determined to be well classified, intermediately classified, or misclassified. The differences between groups were assessed through univariate and multivariate logistic regression. RESULTS: The GSA score was highly or intermediately consistent with the IQ score for 89.2% of the children, and 10.8% were considered misclassified. Children with a higher GSA than IQ score had more autonomy and self-confidence (P < .01), and those with a lower GSA than IQ score had more behavioral problems (P < .01). Analysis by logistic regression showed that sex and gestational age significantly affected the consistency between the 2 scores. Thus, girls were less likely to have a lower GSA than IQ score (aOR = 0.45; 95% CI: 0.24-0.84; P = .01), and a lower gestational age significantly increased the likelihood of having a higher GSA than IQ score (for children born between 24 and 28 weeks of gestation: aOR = 2.70; 95% CI: 1.23-5.92; P = .01). CONCLUSIONS: The GSA score is a simple, inexpensive, and reliable screening tool for assessing neurodevelopment in very preterm children at 5 years of age.

Cognitive features that distinguish preschool-age children with neurofibromatosis type 1 from their peers: a matched case-control study.

OBJECTIVE: To examine the cognitive functioning of 40-month-old children with neurofibromatosis type 1 (NF1). STUDY DESIGN: In this case-control study, 43 children with NF1 and 43 comparison children (matched by age, sex, and maternal years of education) were assessed using the Wechsler Preschool and Primary Scale of Intelligence, Third Edition. Language, visual perception, preliteracy, and executive functioning were also examined. Parents completed questionnaires about their child's behavior. Group differences were examined using the paired-samples t test or the related Wilcoxon signed rank test. Conditional logistic regression was conducted to identify which cognitive variables predicted group membership (ie, NF1 or control). RESULTS: The NF1 group had significantly poorer general intelligence than matched comparisons. Preschool-age children with NF1 had significantly poorer language, visual perception, response inhibition, and preliteracy skills than comparison children. The Wechsler Preschool and Primary Scale of Intelligence, Third Edition, Object Assembly and Information subtests were significant predictors of group membership. Parent ratings indicated no group differences in behavior. CONCLUSIONS: After accounting for potentially confounding variables of age, sex, and maternal years of education, young children with NF1 have significantly poorer intellectual functioning, expressive language, and visual perception. These cognitive features that distinguish young children with NF1 from healthy peers can be detected in the preschool age group and are likely to impact on learning and performance during early school years. These areas should be targeted for intervention to maximize the developmental outcomes of young children with NF1.

Comparison of WPPSI-IV and WISC-V cognitive profiles in 6-7-year-old Finland-Swedish children - findings from the FinSwed study.

The Wechsler scales are among the most widely used tests in cognitive and neuropsychological assessments. When assessing children aged 6:0-7:7 years the clinician can choose between Wechsler Preschool and Primary Scale of Intelligence - Fourth Edition (WPPSI-IV) and Wechsler Intelligence Scale for Children - Fifth Edition (WISC-V). Information about how the tests function and differ in this overlapping age range is limited. Using a between-subjects design, the present study compared the cognitive profiles of typically developing Swedish-speaking children in Finland in this overlapping age range (6:1 - 7:2 years), assessed with the Swedish versions of either WPPSI-IV (n = 38) or WISC-V (n = 24). Profile analyses and one-way ANCOVA were performed to investigate differences in the comparable subtests, indexes and Full Scale IQ. On the subtest level, children assessed with WISC-V had significantly lower scores on the subtests Vocabulary, Matrix Reasoning, and Bug/Symbol Search compared to children assessed with WPPSI-IV. On the index level, scores for the Verbal Comprehension Index and the Fluid Reasoning Index were significantly lower for children assessed with WISC-V. The Full Scale IQ was significantly lower on WISC-V. Taken together, the findings indicate that WPPSI-IV and WISC-V produce partly different cognitive profiles. These differences are important to recognize when choosing which test to use and when interpreting the results of clinical assessments of children in this age group.

Verbal Abilities: Sex Differences in Children at Different Ages.

Background: The assertion of sex differences in verbal abilities is a highly controversial subject. Some studies have demonstrated a female advantage; other studies have found higher rates in males. The results depended on the type of verbal ability that was studied, the cultural context, and the ages of the subjects. There are two types of theories that have been developed to explain the existence of sex differences in cognitive abilities. Social theories explain the differences as caused by social determinants. Biological theories consider biological factors such as prenatal development conditions and hormone levels, among others, as the cause of sex differences. Objective: To investigate sex differences in verbal abilities in children of different ages. Design: Two different editions of Wechsler tests were used. For children age 2.5 to 5 years, the Wechsler Preschool Primary Scale of Intelligence (WPPSI-IV) was used. For children age 6 and older, we administered the Wechsler Intelligence Scale for Children (WISC-V). The total sample included 313 children. Results: The study found significant sex differences in performance on the Verbal Comprehension Scale in children of different ages. At the age of 2 to 4 years, the girls performed better than the boys. In the group of boys, there was a significant increase in verbal abilities at the age of 8-9 years. By the age of 10-11 years, boys began outperforming girls on the Verbal Comprehension Index. Scores on the Verbal Comprehension and Visual Spatial subtests for the boy sample showed stronger correlations than in the girl sample in all age groups. Conclusion: Sex differences in verbal abilities varied depending on the age of the children. The boys showed a stronger integration of their verbal abilities into the structure of their intelligence than the girls.

The significance of nonverbal performance in children with developmental language disorder.

Nonverbal deficits are frequently reported in children with developmental language disorder (DLD). In the new diagnostic criteria of DLD, the previous requirement of normal nonverbal performance has been removed and children with below average and even weak nonverbal skills now fit under the DLD definition. However, the significance of the nonverbal cognitive level, and the connection between nonverbal and verbal skills in these children diagnosed according to the new DLD classification is unclear. In the present study, the significance of nonverbal cognitive level on verbal performance was investigated among preschool-aged children with remarkable deficits in language development. Verbal skills were compared between average, below average, and weak nonverbal cognitive level groups. The connection between nonverbal and verbal skills was evaluated with Pearson correlations, and the covariance structure of the subtests used was modeled with Structural Modelling. The connection between nonverbal cognitive level and verbal skills was clear; weaker nonverbal cognitive levels were associated with lower verbal skills. While receptive language skills and verbal short-term-memory (STM) were the most profound weaknesses, relative strengths emerged for each nonverbal cognitive level group in fluid intelligence, especially in nonverbal reasoning tasks without time limits. In addition, fluid intelligence was strongly linked to verbal understanding and reasoning. These results suggest that the relative strength in nonverbal fluid intelligence with specific weaknesses in receptive language, verbal understanding, and verbal STM could be used as basic factors differentiating children with DLD from those with intellectual disability.

Intergenerational continuity of parent-child separation among mother-offspring dyads: Implication for child cognitive development in rural China.

The labor migration in China often leads to parent-child separation. Research to date has primarily focused on understanding mental and cognitive outcomes for children exposed to parent-child separation, with little consideration for the cumulative effects of intergenerational continuity of parent-child separation. A total of 2729 children between the ages of 4 and 6, along with one parent (primarily mothers, 86.2%) and/or one primary caregiver (if the child is separated from both parents), were recruited in the rural area of Anhui Province, China. A unique subsample of children (n = 249) with persistent experience of parent-child separation or whose mother reported being left behind by her parents during early childhood were enrolled for cognitive assessment. A total of 239 age-, gender- and residence-matched children without any parent-child separation experience were selected as the control group. Child cognitive performance was examined with the Chinese version of the Wechsler Preschool and Primary Scale of Intelligence, Fourth edition (WPPSI-IV). The association between intergenerational continuity of parent-child separation and child cognition was described using multivariate OLS regression models. Compared to mother-offspring dyads without any history of parent-child separation, girls (n = 236/450, 52.4%) who experienced the intergenerational continuity of parent-child separation showed a significant decrease of 5.73 points (95% CI: -9.83, -1.62; p = 0.006) on full-scale intelligence quotient (FSIQ) and a decrease of 5.71 points (95% CI: -9.80, 1.63; p = 0.006) on verbal comprehension index. No similar result was observed in boys. The cumulative effects of parent-child separation among mother-offspring dyads on child cognitive development highlight the need for effective early intervention to break the intergenerational cycle of disadvantage. Sex differences and possible epigenetic mechanisms underlying the intergenerational effects of parent-child separation warrant further investigation.

Assessing Long-Term Neurodevelopment among Children with Non-Syndromic Single Suture Craniosynostosis.

Background: Single suture craniosynostosis (SSC) is a disorder, affecting brain growth. Reviewing literature reveals controversialists of papers in this field. Methods: This prospective study was conducted from 2014 to 2016. All the individuals, aged 2 to 16 years, whose medical records files were complete, with SSC from 1999 to 2013 were included. All patients had undergone cranial vault remodeling at Mofid Hospital, Tehran, Iran. Wechsler questionnaires, WPPSI-III and WISC-IV, were completed for each child based on his/her age. Results: Seventy children were included, with the mean age of 6.7 (±2.9) years. Forty-six (65.7%) children were boys while 24 (34.3%) were girls. Mean FSIQ for all of children was 95.5 (±13.2). Mean verbal IQ, performance IQ, verbal comprehension, perceptual reasoning, processing speed, and working memory are 93.4 (±14.1), 96.1 (±13.3), 97.5 (±13.9), 102.2 (±12.5), 94.5 (±9.8), and 97.5 (±12.9), respectively. There was statistically significant difference between FSIQ of children with SSC and that of unaffected children (P-value<0.05). There was significant difference between verbal IQ of children with SSC and that of unaffected ones (P-value< 0.007). There was significant difference between in processing speed between affected children and unaffected children (P-value<0.012). Conclusion: Children, aged 2 to 6 years, with SSC had a significantly lower Verbal IQ, and children, aged 6 to 16 years, with SSC had a significantly lower processing speed than their healthy counterparts. Though FSIQ of children with SSC falls within normal range, it is a little lower than healthy peers.

Cognitive Profiles of Children with Low Motor Performance: A Contribution to the Validation of the WPPSI-IV.

(1) Background: Developmental Coordination Disorder (DCD) is a common developmental disorder of preschool age. Children often show cognitive deficits in addition to motor problems. Various studies point in particular to problems in visual perception, working memory and processing speed. In this context, it is investigated whether the Wechsler Preschool and Primary Scale-IV (WPPSI-IV) is a suitable instrument for mapping these deficits in a valid and economical way. (2) Methods: The WPPSI-IV profiles of children with DCD (n = 12), below-average motor performance (n = 22) and a control group (n = 32) were compared. (3) Results: Children with DCD achieved significantly poorer test performance in the primary indices Verbal Comprehension, Visual Spatial, Processing Speed and Full Scale compared to a control group. Children with below-average motor skills, on the other hand, do not differ from the children in the control group. (4) Conclusions: The WPPSI-IV is a suitable instrument for diagnosing cognitive deficits in the context of DCD. The Fluid Reasoning and Verbal Comprehension indices should be used as a supplement to assess cognitive performance levels.

The Role of Cognitive Skills, Sex, and Parental Education for Social-Emotional Skills: A Cross-Sectional Study on the WPPSI-IV Performances of Children Aged 3 to 5 Years.

BACKGROUND: Current research suggests that knowledge about the relationship between cognition and social-emotional skills in preschoolers is important to better understand child development. The present study investigated possible effects of cognitive skills measured by the Wechsler Primary and Preschool Scale-Fourth Edition (WPPSI-IV), children's sex, and parental educational level on social--emotional skills measured by the Developmental Test 6 Months to 6 Years-Revision (ET 6-6 R) for children aged 3 to 5. METHODS: Statistical analyses were based on a sample of N = 93 children (47 females, 46 males). First, bivariate correlations among relevant WPPSI-IV index scores, the ET social-emotional quotient, children's sex, and parental educational level were calculated to identify possible significant associations between the variables under investigation. Subsequently, two multiple regression analyses were conducted to test for the hypothesized main effects of cognitive skills, children's sex, and parental educational level on social-emotional skills. Finally, a moderated multiple regression analysis was carried out to investigate whether possible effects of cognitive skills on social-emotional skills were moderated by children's sex and parental educational level. RESULTS: Regression analyses indicated that visual-spatial skills measured by the WPPSI-IV and children's sex have both a small but significant main effect on social-emotional skills. The main effect of sex was due to the fact that, on average, females achieved higher scores on the measure of social-emotional skills than males. CONCLUSIONS: The present findings suggest that the WPPSI-IV represents a suitable test battery for the assessment of those cognitive skills, which might play a reasonable role in social-emotional development.

Structural Equation Modeling of Common Cognitive Abilities in Preschool-Aged Children Using WPPSI-IV and BRIEF-P.

Various studies have addressed the relationship between intelligence and executive functions (EF). There is widespread agreement that EF in preschool children is a unitary construct in which the subordinate factors of Updating, Inhibition, and Shifting are still undifferentiated and correlate moderately with a general factor of intelligence (g). The aim of this study is to investigate the common structural relationship between these two constructs using confirmatory factor analysis. Furthermore, we intend to close the gap of more daily life-associated executive functions and replicate findings in preschool-aged children. Data from a sample of N = 124 average developed children without severe impairments (aged 4 years 0 months-6 years 11 months) were analyzed using the data pool of the standardization and validation studies on the German Wechsler Preschool and Primary Scale of Intelligence-Fourth Edition. Additionally, Executive functions were assessed using a standardized parent-completed questionnaire (BRIEF-P) on their children's everyday behavior. A second-order factor solution revealed that a model with a loading of the common factor of general intelligence (g-factor) onto the EF factor fits the data best. To specify possible method effects due to different sources of measurements, a latent method factor was generated. The results indicate a heterogeneous method effect and a decreasing factor loading from g on to EF while controlling for the method factor.

Benefits of tailored disease management in improving tremor, white matter hyperintensities, and liver enzymes in a child with heterozygous X-linked ornithine transcarbamylase deficiency.

We report the case of a 19-month-old girl with late-onset ornithine transcarbamylase (OTC) deficiency initially referred to gastroenterology for intermittent vomiting lasting a year and abnormal liver enzymes (AST 730 U/L [reference range 26-55 U/L]; ALT 1213 U/L [reference range 11-30 U/L]) without hepatomegaly. While the patient was hospitalized for liver biopsy, intermittent tremors of the upper extremities with varying severity were noted. The patient was presumed to have hyperammonemia secondary to acute liver failure and was discharged after 5 days; follow-up monitoring led to readmission 7 days later. A brain MRI showed nonspecific bilateral pericallosal and bifrontal white matter FLAIR hyperintensities. These findings raised suspicion for a metabolic disease and prompted a genetics consultation. After inconclusive biochemical testing and worsening clinical status, rapid whole genome sequencing results were obtained identifying a novel, de novo, likely pathogenic, variant c.608C > T (p.Ser203Phe) in the OTC gene. The patient was promptly started on an oral nitrogen scavenger, citrulline supplementation, and protein restriction. Ammonia and glutamine levels normalized within 1 month of treatment and have stayed within the goal ranges with continued tailoring of treatment. Her parents noted resolution of vomiting and improved mood stability. Liver enzymes normalized after 2 months of treatment. The tremor, identified as asterixis, improved and a repeat brain MRI 3 months after the initial imaging showed near-complete resolution of previous white matter hyperintensities.