Improved Educational Achievement as a Path to Desistance.

In this article we present a summary of the literature on the associations between learning difficulties/disabilities and juvenile delinquency. This literature is almost a hundred years old, but, although reportedly demonstrating the low academic achievement-delinquency connection, contains numerous unanswered questions regarding the frequency, strength, direction, stability, and causality of these associations. We then use this literature to contextualize the research taking place at the Houston Learning Disabilities (LD) Hub, a member of the LD Centers and Hubs Network, supported by National Institute of Child Health and Human Development (NICHD). In doing so, we present our previous studies and our current research. We conclude by discussing a number of shortcomings in the literature, some-but far from all-of which we hope to address in our ongoing work.

Language development in rural and urban Russian-speaking children with and without developmental language disorder.

Using a newly developed Assessment of the Development of Russian Language (ORRIA), we investigated differences in language development between rural vs. urban Russian-speaking children (n = 100 with a mean age of 6.75) subdivided into groups with and without developmental language disorders. Using classical test theory and item response theory approaches, we found that while ORRIA displayed overall satisfactory psychometric properties, several of its items showed differential item functioning favoring rural children, and several others favoring urban children. After the removal of these items, rural children significantly underperformed on ORRIA compared to urban children. The urbanization factor did not significantly interact with language group. We discuss the latter finding in the context of the multiple additive risk factors for language development and emphasize the need for future studies of the mechanisms that underlie these influences and the implications of these findings for our understanding of the etiological architecture of children's language development.

Associations between household responsibilities and academic competencies in the context of education accessibility in Zambia.

The relationship between education and socioeconomic status has been demonstrated in studies of the developed and the developing world, yet there are communities in which schooling is either not available to all children or not a preferred activity for all children. In this study, we investigated the differences between children in-school and out-of-school in rural and peri-urban communities of Zambia. As expected, we found that the children in-school performed higher in domains of adaptive behavior and on assessments of academic achievement (i.e., mathematics, reading). Somewhat unexpectedly, however, when controlling for socioeconomic status, home responsibilities (i.e., chores, work) were a positive predictor for the performance of the children out-of-school, but a negative predictor for the children in-school. The relationship between home responsibilities and academic performance may be bidirectional and differential; for example, our findings allow for the hypothesis that for in-school children chores take time away from the studies, but for out-of-school children they provide some limited mathematics exposure.

The importance of contractile reserve in predicting exercise tolerance in asymptomatic patients with severe aortic stenosis.

BACKGROUND: Mortality dramatically rises with the onset of symptoms in patients with severe aortic stenosis (AS). Surgery is indicated when symptoms become apparent or when there is ventricular decompensation. Cardiopulmonary exercise testing (CPET) in combination with exercise echocardiography can unmask symptoms and provides valuable information regarding contractile reserve. The aim of the present study was to determine the prevalence of reduced exercise tolerance and the parameters predicting adverse cardiovascular events. METHODS: Thirty-two patients with asymptomatic severe AS were included in this study. Patients were followed up as part of an enhanced surveillance clinic. RESULTS: Age was 69 ± 15.7 years, 75% of patients were male. Patients had a raised NT-ProBNP of 301 pg/mL. VO2peak was 19.5 ± 6.2 mL/kg/min. Forty-one percent of patients had a reduced %VO2peak and this predicted unplanned cardiac hospitalisation (P = 0.005). Exercise systolic longitudinal velocity (S') and age were the strongest independent predictors for VO2peak (R 2 = 0.76; P < 0.0001). Exercise S' was the strongest independent predictor for NT-ProBNP (R 2 = 0.48; P = 0.001). CONCLUSION: A large proportion of patients had a lower than predicted VO2peak. The major determinant of exercise and NT-ProBNP is the ability of the left ventricle (LV) to augment S' on exercise rather than the severity of aortic valve obstruction or resting structural remodelling of the LV. Reduced exercise tolerance and more adverse remodelling, rather than valve obstruction predicted unplanned hospitalisation. This study demonstrates that for those patients, in whom a watchful waiting is an agreed strategy, a detailed assessment should be undertaken including CPET, exercise echocardiography and biomarkers to ensure those with exercise limitation and risk of decompensation are detected early and treated appropriately.

Clinical events and echocardiographic lesion progression rate in subjects with mild or moderate aortic regurgitation.

Background: The rate of progression of aortic regurgitation (AR) is not well described. Current guidelines state that asymptomatic patients with mild AR should be followed up every 3­5 years and 1­2 yearly for moderate AR. This study describes the lesion and clinical based progression of mild and moderate AR in a population of patients undergoing systematic follow-up. Methods and results: 341 patients with either mild or moderate AR were included. The rates of clinical events (death, aortic valve replacement and cardiac hospitalization) and progression of AR are reported. 341 patients were included; mean age was 71.1 years (IQR 66­80 years) and the median follow-up period was 4.6 (IQR 2­6.7) years. 292 patients did not have any events during follow-up. 3 patients required aortic valve replacement (2 of these due to severe aortic stenosis and 1 due to severe mitral regurgitation and co-existent moderate AR). 44 patients required cardiac hospitalization. 9 patients died during follow-up and 35 patients (10%) showed a progression of AR during follow-up with an average time of 4.0 ± 2.6 years. 8 patients (2.3% of the total) progressed to severe AR. Patients with mixed valvular pathology showed a greater increase in AR progression (27 (15%) vs 8 (5%); P = 0.004). Conclusions: Over medium term systematic follow-up progression and clinical events in patients with AR is rare, regardless of etiology. Patients who suffered from AR as an isolated valve pathology were less likely to show AR progression over time.

Sources of heterogeneity in developmental outcomes of children with past and current experiences of institutionalization in Russia: A four-group comparison.

The present study sought to compare 4 groups of age- and gender-matched children-(a) those reared in institutions for children without parental care in Russia; (b) those raised by their biological parents in Russia; (c) those adopted to the United States from Russian institutions; and (d) those born in the United States and raised by their biological parents-on indicators of cognition, language, and early learning. In addition, we aimed to compare the effects of the length of time spent in an institution, the age of initial placement in an institution, the age at adoption, and pre-institutional risk factors (i.e., prenatal substance exposure and prematurity and low birth weight) on the above-mentioned outcomes in the 2 groups of children with institutionalization experiences. Our results confirm previous reports demonstrating negative consequences of institutionalization and substantial ameliorating effects of adoption. They also underscore the complexity of the effects of institutionalization and adoption, showing that they are intertwined with the effects of pre-institutional risk factors. (PsycINFO Database Record

Word learning and individual differences in word learning reflected in event-related potentials.

Adults learned the meanings of rare words (e.g., gloaming) and then made meaning judgments on pairs of words. The 1st word was a trained rare word, an untrained rare word, or an untrained familiar word. Event-related potentials distinguished trained rare words from both untrained rare and familiar words, first at 140 ms and again at 400-600 ms after onset of the 1st word. These results may point to an episodic memory effect. The 2nd word produced an N400 that distinguished trained and familiar word pairs that were related in meaning from unrelated word pairs. Skilled comprehenders learned more words than less skilled comprehenders and showed a stronger episodic memory effect at 400-600 ms on the 1st word and a stronger N400 effect on the 2nd word. These results suggest that superior word learning among skilled comprehenders may arise from a stronger episodic trace that includes orthographic and meaning information and illustrate, how an episodic theory of word identification can explain reading skill.

Language development of internationally adopted children: Adverse early experiences outweigh the age of acquisition effect.

UNLABELLED: We compared English language and cognitive skills between internationally adopted children (IA; mean age at adoption=2.24, SD=1.8) and their non-adopted peers from the US reared in biological families (BF) at two time points. We also examined the relationships between outcome measures and age at initial institutionalization, length of institutionalization, and age at adoption. On measures of general language, early literacy, and non-verbal IQ, the IA group performed significantly below their age-peers reared in biological families at both time points, but the group differences disappeared on receptive vocabulary and kindergarten concept knowledge at the second time point. Furthermore, the majority of children reached normative age expectations between 1 and 2 years post-adoption on all standardized measures. Although the age at adoption, age of institutionalization, length of institutionalization, and time in the adoptive family all demonstrated significant correlations with one or more outcome measures, the negative relationship between length of institutionalization and child outcomes remained most robust after controlling for the other variables. Results point to much flexibility and resilience in children's capacity for language acquisition as well as the potential primacy of length of institutionalization in explaining individual variation in IA children's outcomes. LEARNING OUTCOMES: (1) Readers will be able to understand the importance of pre-adoption environment on language and early literacy development in internationally adopted children. (2) Readers will be able to compare the strength of the association between the length of institutionalization and language outcomes with the strength of the association between the latter and the age at adoption. (3) Readers will be able to understand that internationally adopted children are able to reach age expectations on expressive and receptive language measures despite adverse early experiences and a replacement of their first language with an adoptive language.

Academic Achievement Among Juvenile Detainees.

The literature has long pointed to heightened frequencies of learning disabilities (LD) within the population of law offenders; however, a systematic appraisal of these observations, careful estimation of these frequencies, and investigation of their correlates and causes have been lacking. Here we present data collected from all youth (1,337 unique admissions, mean age 14.81, 20.3% females) placed in detention in Connecticut (January 1, 2010-July 1, 2011). All youth completed a computerized educational screener designed to test a range of performance in reading (word and text levels) and mathematics. A subsample (n = 410) received the Wide Range Achievement Test, in addition to the educational screener. Quantitative (scale-based) and qualitative (grade-equivalence-based) indicators were then analyzed for both assessments. Results established the range of LD in this sample from 13% to 40%, averaging 24.9%. This work provides a systematic exploration of the type and severity of word and text reading and mathematics skill deficiencies among juvenile detainees and builds the foundation for subsequent efforts that may link these deficiencies to both more formal, structured, and variable definitions and classifications of LD, and to other types of disabilities (e.g., intellectual disability) and developmental disorders (e.g., ADHD) that need to be conducted in future research.

ERP evidence for the time course of graphic, phonological, and semantic information in Chinese meaning and pronunciation decisions.

Two words that varied in their relationship were presented sequentially to Chinese readers who made meaning and pronunciation decisions. In the meaning task, they decided whether the words had the same meaning. In the pronunciation task, they decided whether the words had the same pronunciation. In both tasks, the word pairs represented 1 of 4 relationships: graphically similar, homophonic, semantically related, or unrelated. Event related potentials (ERP) recordings made from the onset of the 2nd word suggested a temporal unfolding of graphic, phonological, and semantic effects. Specifically, graphically-related pairs produced a smaller P200 in the pronunciation task and a smaller N400 in the meaning task. Homophones produced reduced N400 component with bilateral sources in the meaning task.

LETM1 haploinsufficiency causes mitochondrial defects in cells from humans with Wolf-Hirschhorn syndrome: implications for dissecting the underlying pathomechanisms in this condition.

Wolf-Hirschhorn syndrome (WHS) represents an archetypical example of a contiguous gene deletion disorder - a condition comprising a complex set of developmental phenotypes with a multigenic origin. Epileptic seizures, intellectual disability, growth restriction, motor delay and hypotonia are major co-morbidities in WHS. Haploinsufficiency of LETM1, which encodes a mitochondrial inner-membrane protein functioning in ion transport, has been proposed as an underlying pathomechanism, principally for seizures but also for other core features of WHS, including growth and motor delay. Growing evidence derived from several model organisms suggests that reduced LETM1 expression is associated with some element of mitochondrial dysfunction. Surprisingly, LETM1-dependent mitochondrial functional deficits have not previously been described in cells from individuals with WHS. Here, using a unique panel of WHS-patient-derived cell lines with deletions of differing sizes, incorporating LETM1 or not, we show, for the first time, that LETM1 expression is reduced in mitochondria isolated from WHS-patient cells. Furthermore, we show that this is associated with distinct mitochondrial phenotypes, including altered intracellular [Ca(2+)] levels, dysfunctional mitochondrial transition-pore opening, hyperpolarization and superoxide leakage from resting mitochondria. Interestingly, we find that these phenotypes segregate with seizures in our WHS cohort. Our findings identify novel cellular phenotypes in WHS attributable to a 50% reduction in LETM1 expression level; these phenotypes could underlie and/or contribute to some of the core clinical features of this condition.

Examining the specific effects of context on adaptive behavior and achievement in a rural African community: six case studies from rural areas of Southern province, Zambia.

Generally accepted as universal, the construct of adaptive behavior differs in its manifestations across different cultures and settings. The Vineland-II (Sparrow et al. in Vineland Adaptive Behavior Scales, Second edn. AGS Publishing, Circle Pines, MN, 2005) was translated into Chitonga and adapted to the setting of rural Southern Province, Zambia. This version was administered to the parents/caregivers of 114 children (grades 3-7, mean age = 12.94, SD = 2.34). The relationships between these children's adaptive behavior, academic achievement and cognitive ability indicators are compared to those usually observed in US samples. Results reflect no association between adaptive behavior and cognitive ability indicators, but a strong relationship between high adaptive behavior and reading-related measures. Six case studies of children with high and low scores on the Vineland-II are presented to illustrate the possible factors affecting these outcomes.

Development and validation of the Delinquency Reduction Outcome Profile (DROP) in a sample of incarcerated juveniles: a multiconstruct/multisituational scoring approach.

The Delinquency Reduction Outcome Profile (DROP) is a novel situational-judgment test (SJT) designed to measure social decision making in delinquent youth. The DROP includes both a typical SJT scoring method, which captures the deviation of an individual response from an "ideal" expert-based response pattern, as well as a novel "Multiconstruct-Multisituational" (MCMS) factor-scoring method, enabling the assessment "in context" of latent dimensions reflecting stable decision-making tendencies. The authors present the development and validation of the DROP across 2 studies establishing its reliability and internal and concurrent validity using a sample of 1,922 young detainees and a sample of juveniles from the community. The authors also discuss the potential usefulness of the DROP as a prognostic tool to predict recidivism for delinquent youth and to monitor changes in intervention programs designed to improve social decision-making skills. Benefits of the MCMS scoring approach for SJT literature and psychological measurement are also discussed.

Searching for Potocki-Lupski syndrome phenotype: a patient with language impairment and no autism.

Potocki-Lupski syndrome (PTLS; OMIM 610883) is a genomic syndrome that arises as a result of a duplication of 17p11.2. Although numerous cases of individuals with PTLS have been presented in the literature, its behavioral characterization is still ambiguous. We present a male child with a de novo dup(17)(p11.2p11.2) and he does not possess any autistic features, but is characterized by severe speech and language impairment. In the context of the analyses of this patient and other cases of PTLS, we argue that the central feature of the syndrome appears to be related to diminished speech and language capacity, rather than the specific social deficits central to autism.

CUL4B-deficiency in humans: understanding the clinical consequences of impaired Cullin 4-RING E3 ubiquitin ligase function.

Cullin's encode the structural components for one of the most abundant E3 ubiquitin ligase families in eukaryotes accounting for as many as 400 distinct E3 ubiquitin ligases. Because of their modular assembly involving combinations of multiple distinct adaptor and substrate receptor proteins, it comes as no surprise that these E3's are implicated in a plethora of fundamental biochemical processes ranging from DNA replication and repair to transcription and development. Herein, we focus on one member of the cullin family, namely the Cullin 4-RING E3 ligases (CRL4's). More specifically, we overview what has been learned about some of the functions of CRL4's from various model systems. We discuss the unexpected association of defective CUL4B with syndromal X-linked mental retardation in humans and speculate on the biochemical consequences and clinical implications of defective CRL4 function. In particular, mutations in CUL4B highlight a previously unappreciated role for CRL4's in neuronal function and cognition in humans.

Education in Juvenile Detention Facilities in the State of Connecticut: A Glance at the System.

The state of Connecticut detained 7,444 children and youth and committed approximately 270 to the Department of Children and Families for out of home placement in the 2007-2008 calendar year. A significant number of children and youth have special education needs that are often unidentified by home school districts. State and federal law mandate the provision of special education and related services to this population. In addition, education of these individuals is imperative as research indicates educational success is a key component for decreasing recidivism (relapse into unlawful activity) rates and providing opportunities toward productive adulthood. The cost of recidivism to detention is not only monetary; criminal misconduct also threatens the safety of society members as well. The Yale University Child Study Center under the auspices of the Connecticut Judicial Branch, Court Support Services Division (CSSD) conducted a situational analysis of the juvenile detention centers and community residential centers. The focus of this analysis was to: (1) describe the educational characteristics of detained children and youth; (2) describe the educational programs currently used in detention and assess whether the educational programming provided is consistent with the framework of the State of Connecticut Department of Education; (3) typify the community of teachers working with students in detention, identify systemic obstacles and/or challenges to educating this population, ascertain the pathways of educational records of detained children and youth; and (4) identify system barriers or challenges to delivering education to this population and teaching in detention or alternative to detention settings. This report is a summary of findings.