Long-term neurodevelopmental outcomes following liver transplantation for metabolic disease-a single centre experience.

This study describes the neurodevelopmental outcome of children with urea cycle disorders (UCD) and organic acidemias (OA) preliver transplant (LT), 1-year, and 3-years post-LT. We performed a retrospective chart review of children with OA or UCD transplanted between January 2014 and December 2021. Standardized motor and cognitive assessment scores were collected from children who had ≥1 motor/cognitive assessment at any timepoint. Pre-LT brain magnetic resonance imaging (MRI) was graded. Associations between demographic/medical variables and neurodevelopmental outcomes were explored. Twenty-six children (64% male) underwent LT at a median age of 1.4 (interquartile range 0.71, 3.84) years. Fifteen (58%) had a UCD diagnosis, 14 (54%) required dialysis for hyperammonemia, and 10 (42%) had seizures typically around diagnosis. The proportion of children with gross motor scores >1 standard deviation (SD) below the mean increased across timepoints, and ≥50% demonstrated general intellect scores >2 SD below the mean at each timepoint. The following significant associations were noted: UCD diagnoses with lower general intellect scores (p = 0.019); arginosuccinate lyase deficiency diagnosis with lower visual motor scores at 3-years post-LT (p = 0.035); a history of seizures pre-LT with lower general intellect (>2SD below the mean) at 3-years post-LT (p = 0.020); dialysis pre-LT with lower motor scores (>1 SD below the mean) at 1-year post-LT (p = 0.039); pre-emptive LT with higher general intellect scores at 3-years post-LT (p = 0.001). MRI gradings were not associated with developmental scores. In our single centre study, children with UCD or OA had a higher prevalence of developmental impairment post-LT compared to population norms. Earlier screening, pre-emptive transplant, and rehabilitation may optimize long-term outcomes.

Allied health and nursing practices in pediatric solid organ transplantation: An international survey.

INTRODUCTION: With improved survival in pediatric solid organ transplantation (SOT) care has focused on optimizing functional, developmental, and psychosocial outcomes, roles often supported by Allied Health and Nursing professionals (AHNP). However, there is a scarcity of research examining frameworks of clinical practice. METHODS: The International Pediatric Transplant Association AHNP Committee developed and disseminated an online survey to transplant centers as a quality improvement project to explore AHNP practice issues. Participant responses were characterized using descriptive statistics, and free-text comments were thematically analyzed. Responses were compared across professional groups; Group 1: Advanced Practice Providers, Group 2: Nursing, Group 3: Allied Health. RESULTS: The survey was completed by 119 AHNP from across the globe, with responses predominantly (78%) from North America. Half of respondents had been working in pediatric transplant for 11+ years. Two-thirds of respondents were formally funded to provide transplant care; however, of these not funded, over half (57%) were allied health, compared to just 6% of advance practice providers. Advanced practice/nursing groups typically provided care to one organ program, with allied health providing care for multiple organ programs. Resource constraints were barriers to practice across all groups and countries. CONCLUSION: In this preliminary survey exploring AHNP roles, professionals provided a range of specialized clinical care. Challenges to practice were funding and breadth of care, highlighting the need for additional resources, alongside the development of clinical practice guidelines for defining, and supporting the role of AHNP within pediatric SOT. Professional organizations, such as IPTA, can offer professional advocacy.

Quality of life and psychosocial outcomes of adults who were pediatric solid organ transplant recipients: A systematic review.

BACKGROUND: The number of pediatric SOT recipients surviving into adulthood is increasing. Thus, understanding their psychosocial and QoL outcomes is important. We conducted a systematic review to collate existing literature examining QoL outcomes (physical functioning, psychological functioning, social functioning), as well as risk and protective factors associated with QoL, among adults who underwent SOT during childhood. METHODS: A systematic search of five databases, from inception to January 6, 2021, was conducted to identify articles that reported on QoL outcomes for adults (≥18-year of age) who received a SOT during childhood (<19-year of age). RESULTS: Twenty-five articles met inclusion criteria. Studies examined QoL across a range of SOT populations (liver, kidney, heart). QoL and psychosocial outcomes were variable; however, the majority of studies indicated QoL in this population to be similar to the general population, or at least similar to other chronic illness groups, with the exception of physical and social functioning. Factors related to a more optimal medical course, younger age at transplant and follow-up, and positive psychosocial functioning, were found to be predictive of better QoL outcomes. CONCLUSIONS: While several studies indicated QoL to be similar to the general population, the literature is limited in both quantity and quality. No study employed prospective, longitudinal methodologies to systematically evaluate QoL over time and few studies utilized normative-based measures of QoL. Furthermore, several SOT groups were under-represented in the literature (e.g., lung, intestine, multi-visceral). Nonetheless, findings have implications for intervention and clinical decision-making.

An international survey of allied health & nursing professionals during the COVID-19 pandemic: Perspectives on facilitators of & barriers to care.

BACKGROUND: Allied health and nursing professionals (AHNP) are integral members of transplant teams. During the COVID-19 pandemic, they were required to adapt to changes in their clinical practices. The goal of the present study was to describe AHNP perceptions concerning the impact of the pandemic on their roles, practice, and resource allocation. METHODS: An online survey was distributed globally via email by the International Pediatric Transplant Association to AHNP at transplant centers from September to December 2020. Responses to open-ended questions were collected using an electronic database. Using a thematic analysis approach, coding was conducted by three independent coders who identified patterns in responses, and discrepancies were resolved through discussion. RESULTS: The majority of respondents (n = 119) were from North America (78%), with many other countries represented (e.g., the United Kingdom, Europe, Australia, New Zealand, South Africa, and Central and South America). Four main categories of impacts were identified: (1) workflow changes, (2) the quality of the work environment, (3) patient care, and (4) resources. CONCLUSIONS: Participants indicated that the pandemic heightened existing barriers and resource challenges frequently experienced by AHNP; however, the value of team connections and opportunities afforded by technology were also highlighted. Virtual care was seen as increasing healthcare access but concerns about the quality and consistency of care were also expressed. A notable gap in participant responses was identified; the vast majority did not identify any personal challenges connected with the pandemic (e.g., caring for children while working remotely, personal stress) which likely further impacted their experiences.

Predictors of Social-Emotional Development and Adaptive Functioning in School-Age Children with Intestinal Failure.

OBJECTIVES: Despite a focus on neurocognition in pediatric intestinal failure (IF) to date, we examined social-emotional and adaptive functioning. METHODS: Children (N = 63) in our IF rehabilitation program underwent neuropsychological assessments including caregiver- and teacher-reported questionnaires. Results were compared to norms using z-tests. Caregiver and teacher reports were compared using t tests. Medical and demographic factors were examined in an exploratory manner using correlation and targeted regression analyses, adjusting for gestational age and full-scale IQ. RESULTS: Caregiver and teacher reports indicated poorer executive, internalizing, behavioral, and adaptive functioning compared to norms. Teachers reported more executive dysfunctions than caregivers. Necrotizing enterocolitis diagnosis predicted internalizing emotional problems. Immigrant status predicted poorer social and practical adaptive functioning. Living with biological parents predicted fewer externalizing emotional and behavioral problems. CONCLUSIONS: The group displayed social-emotional and adaptive functioning concerns. Identifying medical and demographic risks can allow for screening and intervention.

Exploring the potential for online peer support mentorship: Perspectives of pediatric solid organ transplant patients.

OBJECTIVE: Self-management for patients who have undergone solid organ transplantation is demanding and a challenge for adolescents transitioning into adult-oriented healthcare systems. This study explores whether adolescent and young adult solid organ transplant patients support the use of online peer support programs that encourage peer mentorship as an approach to improve disease self-management. METHODS: A qualitative descriptive design comprised of semi-structured interviews with adolescent and young adult transplant patients. Individual interviews were audio-recorded, transcribed verbatim, and subject to content analysis. Emergent categories and themes were refined through member checking and team consensus following saturation. RESULTS: Interviews were conducted across organ groups with 15 participants (60% female) ages 14 to 22 years. Participants expressed unanimous support for an online peer support mentorship program to aid disease self-management in the pediatric transplant patient population. Three themes emerged from the interviews: (a) self-management care can be "taxing"; (b) there would be value in peer mentorship for adolescent transplant patients; and (c) online peer mentorship is the "best" option but still requires relationship building. Logistical preferences of an online peer mentorship program were solicited. The preferred peer "match" was someone of the same organ transplant group and gender who was able to have weekly contact via texting. CONCLUSIONS: Creating tailored, online peer mentorship programs is gaining evidence to justify further development. Findings from this study will support program modifications for adolescent and young adult solid organ transplant patients. Next steps will involve usability and feasibility testing of an adapted online program for this patient group.

Early school-age cognitive performance post-pediatric heart transplantation.

BACKGROUND: As survival in pediatric heart transplantation (HTx) has improved due to medical advances, the analysis of long-term outcomes impacting quality of life such as cognition and development becomes increasingly important. Neuropsychological assessments provide a comprehensive understanding of individual needs, allowing for the development of tailored recommendations and interventions. METHODS: Routine neuropsychological assessment was completed between 5 and 7 years of age in this cohort of pediatric HTx recipients at our center (Jan 2014-Oct 2018), including tests of general intellect (WPPSI-IV, WISC-V), academics (WIAT-II/III), perceptual-motor abilities (Beery VMI), and memory (CMS). Relevant medical variables were collected. RESULTS: Among 25 children, the median age at testing was 6.7 (IQR:5.8-7.4) years, with a median time since HTx of 5.2 (IQR:4.8-6.8) years. Medical diagnoses included congenital heart disease (CHD; 56%) and cardiomyopathy (44%). Cognitive functioning across the intellectual, academic, and perceptual-motor domains fell within the low-average range, while memory abilities fell within the average range. DSM-5 clinical diagnoses were provided for 14 (56%) children: Intellectual Disability-Mild (20%), Learning Disability (20%), Language Disorder (8%), and Attention-Deficit/Hyperactivity Disorder (12%). The presence of neurological issues and/or CHD predicted poorer performance on various neuropsychological domains. CONCLUSIONS: Over 50% of this cohort of pediatric heart transplant recipients seen for routine post-HTx neuropsychological assessment received a clinical psychological diagnosis, notably higher than rates in the general population. This population requires monitoring to ensure that high risk children are identified and successfully supported in school and their community.

Neurocognitive Functioning in Early School-age Children With Intestinal Failure.

OBJECTIVES: Little is known about school-age functioning in children with intestinal failure (IF). This study examines neurocognitive outcomes of children with IF at ages 4 to 8 years treated at a single centre, along with relevant medical and demographic variables. METHODS: Between 2012 and 2016, neurocognitive assessments were administered to 28 children receiving treatment in our IF rehabilitation program, and included measures of intelligence, academics, learning/memory, language, visual-motor integration, and fine-motor dexterity. DSM-IV/V criteria were used to diagnose Learning Disability, Intellectual Disability, and/or Attention Deficit/Hyperactivity Disorder. RESULTS: Intellectual functioning ranged from extremely low to superior (Full Scale IQ range 53-123, mean = 89). Forty-six percentage received a cognitive/learning DSM diagnosis. Total number of first-year septic episodes correlated with poorer outcomes on the most cognitive measures. Adjusting for gestational age (61% were born <37 weeks), number of first-year septic episodes negatively predicted working memory, visual-motor integration, and visual memory scores. Additional factors correlating with poorer outcomes on ≥2 cognitive measures included length of first-year admissions, necrotizing enterocolitis diagnosis, and first-year sustained conjugated hyperbilirubinemia. Having ≥2 first-year septic episodes increased the likelihood of poorer outcome. Having a sibling was a significant positive predictor of working memory, processing speed, reading, and verbal learning skills. CONCLUSIONS: Our data provides preliminary evidence that children with IF are at risk of neurocognitive problems at early school age. Managing septic events during the first year is one strategy that may help reduce long-term neurocognitive risks in this population.

Assessing the acceptability and efficacy of teens taking charge: Transplant-A pilot randomized control trial.

BACKGROUND: Adolescents who have undergone SOT are at heightened risk for graft failure. This pilot randomized controlled trial aimed to determine the feasibility and obtain preliminary estimates of efficacy of the online TTC program. METHODS: TTC is a web-based self-management program for adolescent SOT patients. Participants-kidney or liver transplant candidates or recipients-were enrolled over a 2-year period and randomized to either an intervention group that accessed TTC or a control group that did not access the program. Outcome measures included feasibility, how much the program was utilized, and pre- and post-intervention health-related outcomes evaluating self-management, self-efficacy, mood, use of healthcare services, and knowledge. Semi-structured interviews were conducted with participants post-intervention. RESULTS: Forty-two participants were enrolled in the study. Participants in the intervention group expressed that they enjoyed learning about other teens' experiences, but reported barriers to accessing the site, such as being too busy, computer being too slow, and not being able to access the site on their mobile device. Time spent using the website was low for the majority of participants. Patients less than 1-year post-transplant accessed TTC more than patients who had their transplant for longer. No significant differences were found for health-related outcomes between groups. CONCLUSIONS: No differences in health-related outcomes were found between groups. Further studies that examine barriers to engagement with web-based education tools are warranted.

Transition to adult care in pediatric solid-organ transplant: development of a practice guideline.

BACKGROUND: Transition to adult-centered care is becoming an increasingly important area of practice in pediatric organ transplant. Standardized, best-practice guidelines are needed to assist transplant practitioners in providing optimal transitional care for this population of patients. OBJECTIVE: To describe the development and implementation of a practice guideline for the transitional care of pediatric transplant recipients. METHODS: A quality improvement project was undertaken in a pediatric multiorgan transplant program setting. Strategies employed included (1) creation of an interdisciplinary working group, (2) survey of transition-related practices and learning needs of transplant practitioners, (3) review of the literature and existing transition-related materials, and (4) creation of transition guidelines. RESULTS: An interdisciplinary survey of transplant practitioners at our institution identified practice strengths related to transitional care and learning needs. Review of relevant literature and other materials revealed limited but emerging research related to the transition of pediatric transplant recipients from pediatric to adult care. Existing transition tools were examined and applicable items identified. A practice guideline for use with pediatric transplant recipients transitioning to adult care was developed. Strategies to educate staff about the guideline and promote ongoing guideline use were implemented. CONCLUSION: Preparing pediatric transplant recipients and their families for transition to adult-centered care is an emerging challenge for transplant teams. These guidelines provide practitioners with a developmentally sensitive overview of important transition-related domains and strategies directed toward patients and their caregivers, who may experience the process of transition differently. Dissemination of the pediatric transplant transition guideline will make transition information more widely available to transplant practitioners.

The iPeer2Peer mentorship program for adolescent thoracic transplant recipients: An implementation-effectiveness evaluation.

BACKGROUND: An increase in self-management skills for adolescent thoracic transplant recipients may improve health outcomes and facilitate a successful transition to adulthood. The iPeer2Peer program is an online peer mentorship program that has been successfully implemented as a self-management intervention in multiple chronic disease populations. This study aimed to determine the implementation and effectiveness outcomes of the iPeer2Peer program for adolescent thoracic transplant recipients. METHODS: A type III, hybrid implementation-effectiveness pilot study that comprised a quasi-experimental single-arm pre-post design was used to evaluate the iPeer2Peer program. Participant mentees, ages 12-17, were recruited from 2 large Canadian transplant centers. Peer mentors, ages 18-25, were thoracic transplant recipients who had successfully transitioned to adult care and self-manage their condition. A mixed methods approach for data collection was used, including interviews, focus groups, and standardized questionnaires. RESULTS: Twenty mentees (median 15.0 years, IQR 3.3 years; 65% female) completed the iPeer2Peer program with 9 young adult mentors (median 21.0 years, IQR 3.0 years; 78% female). Implementation outcomes indicated that the iPeer2Peer program was perceived as feasible, adoptable, acceptable, and appropriate for adolescent thoracic transplant recipients. Significant findings were noted in mentees for increased self-management and a decrease in overall depression and anxiety symptoms. CONCLUSIONS: The successful implementation of the pilot iPeer2Peer program offers support to evaluate the scalability, sustainability, and cost-effectiveness of the program for adolescents with chronic illness, specifically thoracic transplant recipients. Changes to the iPeer2Peer program that facilitate a flexible delivery may help implementation and acceptance.

Mitigating work conditions that can inhibit learning from errors: Benefits of error management climate perceptions.

Introduction: Professionals do not always learn from their errors; rather, the way in which professionals experience errors and their work environment may not foster, but can rather inhibit error learning. In the wake of a series of accounting scandals, including Royal Ahold in Netherlands, Lehman Brothers in the United States, and Wirecard in Germany, within the context of financial auditing, we explore four audit-specific conditions at the workplace that could be negatively associated with learning: small error consequences, routine-type errors, negative emotions, and high time pressure. Then, we examine how perceptions of an open or blame error management climate (EMC) moderate the negative relationship between the four work conditions and learning from errors. Methods: Using an experiential questionnaire approach, we analyze data provided by 141 Dutch auditors across all hierarchical ranks from two audit firms. Results: Our results show that open EMC perceptions mitigate the negative relationship between negative emotions and error learning, as well as the negative relationship between time pressure and error learning. While we expected that blame EMC perceptions would exacerbate the negative relationship between negative emotions and error learning, we find a mitigating effect of low blame EMC perceptions. Further, and contrary to our expectations, we find that blame EMC perceptions mitigate the negative relationship between small error consequences and error learning, so that overall, more error learning takes place regardless of consequences when participants experience a blame EMC. Post-hoc analyses reveal that there is in fact an inverted- U-shaped relationship between time pressure and error learning. Discussion: We derive several recommendations for future research, and our findings generate specific implications on how (audit) organizations can foster learning from errors.

[Formula: see text]Examining parent and clinician views of a hospital-based pediatric neuropsychological service: a Canadian perspective.

Understanding how pediatric neuropsychological evaluations support families and the child's medical team is an important component of ensuring evidence-based care. For the first time within a Canadian context, we investigated the impact of neuropsychological assessments on parent knowledge, advocacy, and stress and the role of socioeconomic factors in parents' perceptions of the assessment. Responses from referring clinicians were also examined. As part of a hospital quality improvement project, 91 parents of children between the ages of 3 and 17 years (M = 8y7m; SD = 4y1m) and 45 clinician consumers (clinical staff who use neuropsychological services) completed one of two online questionnaires in English: Parent Overall Assessment of Supports and Testing, or Clinician Overall Assessment of Supports and Testing. Most parents indicated the neuropsychological evaluation promoted understanding of their child's cognitive profile and improved their ability to support their child at home and at school or in the community. Families characterized as being at higher social risk indicated that the evaluation led to more changes in how they approached their child at home than families with lower social risk status. Referring clinicians indicated neuropsychological reports were effective in communicating findings to them and patients/families. The most valuable sections of the report according to referring clinicians included the diagnosis/impression and recommendations sections. Parents and referral providers reported many benefits from the neuropsychological evaluation but also identified areas for service delivery improvement. Parent perceptions varied based on family and socio environmental factors, offering important targets for future research and clinical consideration.

Neuropsychological outcomes following pediatric lung transplantation.

OBJECTIVE: Lung transplantation (LTx) is a treatment option for eligible children with end-stage pulmonary diseases. Improving our understanding of longer-term developmental outcomes in pediatric LTx recipients is important for strategized interventions targeting cognitive difficulties. METHODS: Neuropsychological assessments were completed for children who received LTx at our center (2009-2017). Assessments comprised tasks of general intellect, memory, visual-perception, academics, and executive functioning as well as caregiver questionnaires of adaptive, executive, emotional, and behavioral functioning. Results were compared to age-matched population norms. Between-group nonparametric tests were performed pre-LTx vs post-LTx and for children with a primary diagnosis of cystic fibrosis (CF) vs other diagnoses (non-CF). RESULTS: Neuropsychological outcomes were assessed for 21 children post-LTx, with a median age (interquartile range) at the time of transplant of 11.52 (6.89, 14.12) years. Eleven children completed pre- and post-transplant assessments and within this group, improvements for verbal learning (P = .02), aspects of mood, behavior, and adaptive functioning were observed over time (all P < .05). Post-transplant whole group analysis suggested age-appropriate abilities across most cognitive domains, with a relative weakness for executive functioning. Emotional or behavioral difficulties were not endorsed by caregivers. Across pulmonary diagnoses, higher levels of emotional, behavioral, and executive functioning difficulty were reported in the non-CF group (all P < .05). CONCLUSIONS: Overall, LTx has a positive impact on cognitive functioning, particularly learning, adaptive functioning, mood, and behavior. Children transplanted for non-CF related diseases demonstrated greater challenges, highlighting the need for targeted assessments and interventions across the transplant process to support the complex needs of this population.

Neurodevelopmental outcomes of infants with intestinal failure at 12 and 26 months corrected age.

BACKGROUND: Survival rates of infants with intestinal failure (IF) are increasing, yet multiple medical factors may impact development. AIMS: This study examines neurodevelopmental outcomes at 12-15 and 26-32 months corrected age in a cohort of children with IF. STUDY DESIGN: Prospective single centre neurodevelopmental assessments of children admitted to an intestinal rehabilitation program between 2011 and 2013. Outcome measures include: Mullen Scales of Early Learning (12 and 26 months), and Vineland-II Adaptive Behaviour Scales - Caregiver Report (26 months). Standard scores were correlated with medical and demographic factors. RESULTS: Thirty-one children (17 males), median gestational age 34 weeks (IQR 30-36) with birth weight (BW) 2.12 kg (IQR 1.24-2.52). Ten (32.2%) were dependent on parental nutrition (PN) at the second assessment. At 12 months, 11/30 (36.7%) scored below average (>1SD below mean) on the early learning composite and 24/30 (80%) were below average on the gross motor subscale. Comparatively, at 26 months, mean early learning scores were significantly lower with >50% scoring below average. Mean gross motor scores remained low, and 14/27 (51.9%) were below average on the Vineland-II motor domain. At 12 months, factors significantly associated with lower scores are primarily related to prematurity. At 26 months, the impact of IF-related variables such as bowel length and PN days are more apparent. CONCLUSION: Children with IF are at risk of developmental delay in multiple domains. While gross motor skills appear to be most significantly affected, cognitive deficits become more apparent at 26 months, highlighting the importance of early developmental follow up.

Vertical air temperature gradients under the shade of two contrasting urban tree species during different types of summer days.

Moderation of thermal energy balance through the canopies of urban trees is well known. However, a more functional and quantitative view of the heterogeneous urban environment and their influence on the below-canopy vertical air temperature gradients is largely missing. Throughout the summer 2016 we continuously measured air temperature at three different heights (at 1.5, 3 and 4.5m from the ground) under the canopies of two common but contrasting street tree species in respect of eco-physiology and morphology in Munich, Germany: Robinia pseudoacacia L. (ring porous) and Tilia cordata Mill. (diffuse porous). Along with air and surface temperature we also measured meteorological and edaphic variables and categorized summer time as cool, mild, summer and hot days. Global radiation, vapour pressure deficit and soil temperature increased as the days got warmer but precipitation, soil moisture and wind speed showed the reversed pattern. Overall, T. cordata trees with higher leaf area index and sap-wood area provided three times more transpiration than R. pseudoacacia. On an average air temperature gradient of outside to inside canopy dropped from 1.8°C to 1.3°C for T. cordata but from 1.5°C to only 0.5°C for R. pseudoacacia as the days got warmer. Vertical decline of air cooling effect was around 1°C from canopy to the near-ground (1.5m). Lower soil moisture but higher soil temperature suggested that cool air from the canopy mixed with a higher amount of sensible heat flux under the canopies of T. cordata compared to the R. pseudoacacia as the days got warmer. The study indicated a threshold for extreme hot days when grass surface evapotranspirational cooling will not be as effective and act like built surfaces rather deep shading from tree canopies will be important.

The Impact of Sex Differences on Odor Identification and Facial Affect Recognition in Patients with Schizophrenia Spectrum Disorders.

BACKGROUND: Social interactive functions such as facial emotion recognition and smell identification have been shown to differ between women and men. However, little is known about how these differences are mirrored in patients with schizophrenia and how these abilities interact with each other and with other clinical variables in patients vs. healthy controls. METHODS: Standardized instruments were used to assess facial emotion recognition [Facially Expressed Emotion Labelling (FEEL)] and smell identification [University of Pennsylvania Smell Identification Test (UPSIT)] in 51 patients with schizophrenia spectrum disorders and 79 healthy controls; furthermore, working memory functions and clinical variables were assessed. RESULTS: In both the univariate and the multivariate results, illness showed a significant influence on UPSIT and FEEL. The inclusion of age and working memory in the MANOVA resulted in a differential effect with sex and working memory as remaining significant factors. Duration of illness was correlated with both emotion recognition and smell identification in men only, whereas immediate general psychopathology and negative symptoms were associated with emotion recognition only in women. CONCLUSION: Being affected by schizophrenia spectrum disorder impacts one's ability to correctly recognize facial affects and identify odors. Converging evidence suggests a link between the investigated basic and social cognitive abilities in patients with schizophrenia spectrum disorders with a strong contribution of working memory and differential effects of modulators in women vs. men.

Corrigendum to "Assessment of School Readiness in Chronic Cholestatic Liver Disease: A Pilot Study Examining Children with and without Liver Transplantation".

[This corrects the article DOI: 10.1155/2017/9873945.].

Assessment of School Readiness in Chronic Cholestatic Liver Disease: A Pilot Study Examining Children with and without Liver Transplantation.

Background. Assessment of school readiness evaluates physical, social-emotional, and neuropsychological domains essential for educational success. Cognitive testing of preschool aged children with chronic liver disease may guide more timely interventions and focused efforts by health care providers. Patients and Methods. Children with chronic cholestatic liver disease diagnosed as an infant and still with their native liver (NL) and children who received a liver transplant (LT) before age of 2 years underwent testing with a battery of well-validated pediatric psychometric measures. Results. Eighteen (13 LT, 5 NL) patients (median age of 4.45 and 4.05 years, resp.) were tested. Median Full-Scale IQ was 98 (range 102-116) for LT and 116 [(range 90-106), p = 0.35, NS] for NL subjects. LT recipients had significantly greater visual based difficulties, poorer caregiver rated daily living skills (p = 0.04), and higher levels of executive function based difficulties (e.g., inattention, inhibition). Conclusion. This pilot study highlights the risk of neuropsychological difficulties in early school age children who were under 2 years of age at time of LT. Comprehensive early school age assessment should integrate psychometric measures to identify children at greatest risk, thus allowing for proactive educational intervention.

Early neurodevelopmental outcomes of infants with intestinal failure.

BACKGROUND: The survival rate of infants and children with intestinal failure is increasing, necessitating a greater focus on their developmental trajectory. AIMS: To evaluate neurodevelopmental outcomes in children with intestinal failure at 0-15months corrected age. STUDY DESIGN: Analysis of clinical, demographic and developmental assessment results of 33 children followed in an intestinal rehabilitation program between 2011 and 2014. Outcome measures included: Prechtl's Assessment of General Movements, Movement Assessment of Infants, Alberta Infant Motor Scale and Mullen Scales of Early Learning. Clinical factors were correlated with poorer developmental outcomes at 12-15months corrected age. RESULTS: Thirty-three infants (17 males), median gestational age 34weeks (interquartile range 29.5-36.0) with birth weight 1.98kg (interquartile range 1.17-2.50). Twenty-nine (88%) infants had abnormal General Movements. More than half had suspect or abnormal scores on the Alberta Infant Motor Scale and medium to high-risk scores for future neuromotor delay on the Movement Assessment of Infants. Delays were seen across all Mullen subscales, most notably in gross motor skills. Factors significantly associated with poorer outcomes at 12-15months included: prematurity, low birth weight, central nervous system co-morbidity, longer neonatal intensive care admission, necrotizing enterocolitis diagnosis, number of operations and conjugated hyperbilirubinemia. CONCLUSION: Multiple risk factors contribute to early developmental delay in children with intestinal failure, highlighting the importance of close developmental follow-up.