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Background: Solid organ transplantation (SOT) offers improved long-term survival for youth with end-stage organ disease. From a neurodevelopmental, cognitive, and academic perspective, children with solid organ transplant have a number of unique risk factors. While cognitive functioning may improve post-transplantation, it is important to understand the trajectory of neurocognitive development starting in transplant candidacy to evaluate the implications of early deficits., Aim: The aim of this paper is to describe the neurocognitive risks and long-term implications for adolescent transplant recipients., Method: This paper provides an overview of neurocognitive functioning in youth with end-stage organ dysfunction with discussion of implications for adolescent transplant recipients., Results: Post-transplant, adolescent, and young adult solid organ transplant recipients exhibit significant levels of executive dysfunction, with implications for decision-making, regimen adherence, and transition to adult transplant care., Conclusion: Transplantation may reduce the risk for poor long-term neurocognitive effects, yet adolescent transplant recipients remain at increased risk, particularly in executive functioning, which has implications for adherence and transition to adulthood. Baseline and follow-up assessments for youth with end-stage organ disease and transplant are important for the monitoring of neurocognitive development and may be used to mitigate risk for low adherence to post-transplantation treatment regimens and reduce barriers to transitioning to adult transplant care., (© 2024 The Author(s). Pediatric Transplantation published by Wiley Periodicals LLC.)

Background: The process of transition to adult-based care encompasses a critical period in the life of an adolescent and young adult living with a chronic illness and one that comes with an increase in the risk of poor health outcomes. As yet, there is a dearth of empirical data to help optimize this process to ensure the best long-term outcome., Methods: This study used a principal components analysis to determine specific constructs measured by a revised version of the transition readiness survey used in our clinic. We investigated changes in these constructs over time. We further investigated the relationship between the change in these constructs over time spent in a focused transition program with adherence., Results: The primary component underlying our transition readiness survey for patients and parents represented self-efficacy. Time spent in the transition program was an independent predictor of change in self-efficacy (rho 0.299, p = .015); however, the magnitude of that change had no relationship to adherence. Change in parent-proxy self-efficacy was found to have a statistically significant relationship with tacrolimus standard deviation (rho -0.301, p = .026). There was disagreement identified between patient and parent responses on the survey. Neither change in patient nor parent reports of self-efficacy was found to have a relationship with post-transfer adherence., Conclusions: This study reaches the novel conclusion that self-efficacy and parent-proxy self-efficacy are dynamic concepts that change over time spent in a focused transition program. The patient-parent disagreement and the relationship between parent-proxy self-efficacy and adherence stress the importance of involving parents/guardians in the transition process as well., (© 2023 The Authors. Pediatric Transplantation published by Wiley Periodicals LLC.)

Objective: Racial and ethnic disparities are commonplace in health care. Research often relies on sociodemographic information recorded in the electronic health record (EHR). Little evidence is available about the accuracy of EHR-recorded sociodemographic information, and none in pediatrics. Our objective was to determine the accuracy of EHR-recorded race and ethnicity compared to self-report., Methods: Patients/guardians enrolled in two prospective observational studies (10/2014-1/2019) provided self-reported sociodemographic information. Corresponding EHR information was abstracted. EHR information was compared to self-report, considered "gold standard." Agreement was evaluated with Cohen's kappa., Results: A total of 503 patients (42% female, median age 12.8 years) were identified. Self-reported race (N = 484) was 73% White, 16% Black or African American (AA), 4% Asian, 5% multiracial, and 2% other. Self-reported ethnicity (N = 410) was 9% Hispanic/Latino, and 88% non-Hispanic/Latino. Agreement between self-reported and EHR-recorded race was substantial (kappa = 0.77, 95% CI 0.72-0.83). Race was discordant among 10% (47/476). Hispanic/Latino ethnicity also had strong agreement (kappa = 0.77, 95% CI 0.65-0.89). Among those who self-reported Hispanic/Latino and reported race (N = 21), race was less accurately recorded in the EHR (kappa = 0.26, 95% CI 0-0.54). Race did not match among 43% with recorded race (9/21). Among self-reported racial and/or ethnic minorities, 13% (12/164) were misclassified in the EHR as non-Hispanic White., Conclusions: We found race and ethnicity are often inaccurately recorded in the EHR for patients who self-identify as minorities, leading to under-representation of minorities in the EHR. Inaccurately recorded race and ethnicity has important implications for disparity research, and for informing health policy. Reliable processes are needed to incorporate self-reported race and ethnicity in the EHR at institutional and national levels., (© 2022. W. Montague Cobb-NMA Health Institute.)

Background: Inclusion of evidence-based behavior change techniques (e.g., self-monitoring) in mobile health apps has the potential to promote adherence to inflammatory bowel disease treatment. While inflammatory bowel disease management apps exist, the extent to which they incorporate behavior change techniques remains unknown., Aims: The present study systematically evaluated the content and quality of free, commercially available inflammatory bowel disease management apps., Methods: Apps were identified using a systematic search of the Apple App and Google Play stores. Apps were evaluated using Abraham and Michie's taxonomy of 26 behavior change techniques. A literature search was conducted to identify behavior change techniques specific and relevant for people with inflammatory bowel disease. App quality was assessed using the Mobile App Rating Scale with scores ranging from 1 (Inadequate) to 5 (Excellent)., Results: A total of 51 inflammatory bowel disease management apps were evaluated. Apps included 0-16 behavior change techniques (Mean = 4.55) and 0-10 inflammatory bowel disease management behavior change techniques (Mean = 3.43). App quality ranged from 2.03 to 4.62 (Mean = 3.39) out of 5.00. Two apps, My IBD Care: Crohn's & Colitis and MyGiHealth GI Symptom Tracker, included the highest number of overall and inflammatory bowel disease management behavior change techniques along with high-quality scores. Bezzy IBD was the only app with a high number of overall and inflammatory bowel disease management behavior change techniques with a primary focus on social support/change., Conclusion: Most inflammatory bowel disease management apps reviewed included evidence-based inflammatory bowel disease management behavior change techniques., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Importance: Despite high disease morbidity and mortality and complex treatment decisions, little is known about the medical and end-of-life decision-making preferences of adolescents and young adults (AYA) with advanced heart disease. AYA decision-making involvement is associated with important outcomes in other chronic illness groups., Objective: To characterize decision-making preferences of AYAs with advanced heart disease and their parents and determine factors associated with these preferences., Design, Setting, and Participants: Cross-sectional survey between July 2018 and April 2021 at a single-center heart failure/transplant service at a Midwestern US children's hospital. Participants were AYAs aged 12 to 24 years with heart failure, listed for heart transplantation, or posttransplant with life-limiting complications and a parent/caregiver. Data were analyzed from May 2021 to June 2022., Main Outcomes and Measures: Single-item measure of medical decision-making preferences, MyCHATT, and Lyon Family-Centered Advance Care Planning Survey., Results: Fifty-six of 63 eligible patients enrolled in the study (88.9%) with 53 AYA-parent dyads. Median (IQR) patient age was 17.8 (15.8-19.0) years; 34 (64.2%) patients were male, and 40 patients (75.5%) identified as White and 13 patients (24.5%) identified as members of a racial or ethnic minority group or multiracial. The greatest proportion of AYA participants (24 of 53 participants [45.3%]) indicated a preference for active, patient-led decision-making specific to heart disease management, while the greatest proportion of parents (18 of 51 participants [35.3%]) preferred they and physician(s) make shared medical decisions on behalf of their AYA, representing AYA-parent decision-making discordance (χ2 = 11.7; P = .01). Most AYA participants stated a preference to discuss adverse effects or risks of treatment (46 of 53 participants [86.8%]), procedural and/or surgical details (45 of 53 participants [84.9%]), impact of condition on daily activities (48 of 53 participants [90.6%]), and their prognosis (42 of 53 participants [79.2%]). More than half of AYAs preferred to be involved in end-of-life decisions if very ill (30 of 53 participants [56.6%]). Longer time since cardiac diagnosis (r = 0.32; P = .02) and worse functional status (mean [SD] 4.3 [1.4] in New York Heart Association class III or IV vs 2.8 [1.8] in New York Heart Association class I or II; t-value = 2.7; P = .01) were associated with a preference for more active, patient-led decision-making., Conclusions and Relevance: In this survey study, most AYAs with advanced heart disease favored active roles in medical decision-making. Interventions and educational efforts targeting clinicians, AYAs with heart disease, and their caregivers are needed to ensure they are meeting the decision-making and communication preferences of this patient population with complex disease and treatment courses.

Advances in medical therapies and liver transplantation have resulted in a greater number of pediatric patients reaching young adulthood. However, there is an increased risk for medical complications and morbidity surrounding transfer from pediatric to adult hepatology and transplant services. Health care transition (HCT) is the process of moving from a child/family-centered model of care to an adult or patient-centered model of health care. Successful HCT requires a partnership between pediatric and adult providers across all disciplines resulting in a transition process that does not end at the time of transfer but continues throughout early adulthood. Joint consensus guidelines in collaboration with the American Society of Transplantation are presented to facilitate the adoption of a structured, multidisciplinary approach to transition planning utilizing The Six Core Elements of Health Care Transition TM for use by both pediatric and adult specialists. This paper provides guidance and seeks support for the implementation of an HCT program which spans across both pediatric and adult hepatology and transplant centers., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by European Society for European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Objective: The COVID-19 pandemic increased economic, social, and health stressors for families, yet its impacts on families of youth with chronic conditions, such as type 1 diabetes (T1D), are not well understood. Self-regulation (SR)-or the capacities to control emotions, cognition, and behavior in response to challenge-is known to support T1D management and coping in the face of stress. Strong SR may have protected youth with T1D from the impacts of pandemic-related stressors. This study compared youth and parent emotional functioning and T1D management before and after the pandemic's onset in relation to family pandemic-related stress and youth SR., Methods: Parents of youth with T1D (N = 88) and a subset of these youth (N = 43; Mean age 15.3 years [SD 2.2]) completed surveys regarding SR, stress, emotional functioning, and T1D-related functioning prior to and after March 2020. Outcomes were compared using mixed effects models adjusting for covariates. Family pandemic-related stress experiences and youth SR were tested as moderators of change., Results: Parents' responsibility for T1D management increased across pandemic onset and their diabetes-related distress decreased. Family pandemic-related stress was associated with decreased emotional functioning over time. Youth SR, particularly emotional and behavioral aspects, predicted better emotional and T1D-related functioning., Discussion: While youth with T1D whose families experienced higher pandemic-related stress had poorer adjustment, strong emotional and behavioral SR appeared to protect against worsening youth mood and adherence across pandemic onset. Both social-contextual and individual factors are important to consider when working with families managing T1D., (© The Author(s) 2022. Published by Oxford University Press on behalf of the Society of Pediatric Psychology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Objective: To evaluate neurodevelopmental status among children with inherited cholestatic liver diseases with native liver and variables predictive of impairment., Methods: Participants with Alagille syndrome (ALGS), progressive familial intrahepatic cholestasis (PFIC), and alpha 1 antitrypsin deficiency (A1AT) enrolled in a longitudinal, multicenter study and completed the Wechsler Preschool and Primary Scale of Intelligence-III or Intelligence Scale for Children-IV. Full Scale Intelligence Quotient (FSIQ) was analyzed continuously and categorically (>100, 85-99, 70-84, <70). Univariate linear regression was performed to study association between FSIQ and risk factors, stratified by disease., Results: Two hundred and fifteen completed testing (ALGS n = 70, PFIC n = 43, A1AT n = 102); median age was 7.6 years (3.0-16.9). Mean FSIQ in ALGS was lower than A1AT (94 vs 101, P = 0.01). Frequency of FSIQ < 85 (>1 standard deviation [SD] below average) was highest in ALGS (29%) versus 18.6% in PFIC and 12.8% in A1AT, and was greater than expected in ALGS based on normal distribution (29% vs 15.9%, P = 0.003). ALGS scored significantly lower than test norms in almost all Wechsler composites; A1AT scored lower on Working Memory and Processing Speed; PFIC was not different from test norms. Total bilirubin, alkaline phosphatase, albumin, hemoglobin, and parental education were significantly associated with FSIQ., Conclusions: Patients with ALGS are at increased risk of lower FSIQ, whereas our data suggest A1AT and PFIC are not. A1AT and ALGS appear vulnerable to working memory and processing speed deficits suggestive of attention/executive function impairment. Malnutrition, liver disease severity, and sociodemographic factors appear related to FSIQ deficits, potentially identifying targets for early interventions., Competing Interests: Conflicts of Interest: D.H.L. reports grant/research support from Abbvie, Gilead, and CF Foundation; he also serves on advisory panels for Merck and Gilead. B.M.K. is a consultant for Mirum, Albireo and Audentes; she has unrestricted educational grants from Mirum and Albireo. V.L.N. is a consultant for Albireo. P.R. reports grants from Gilead, Merck, BMS, AbbVie, Travere Therapeutics, Albireo, and Mirum, Arrowhead; and serving as a consultant for Gilead, Mirum, Albireo, Audentes, and Vertex. R.J.S. reports consulting for Albireo, Mirum/Shire, and Retrophin (all <$10,000 per year). K.M.L. reports consulting for Albireo, Mirum and Retrophin (now known as Travere Therapeutics). The remaining authors report no conflicts of interest. The study sponsors did not have a role in study design; collection, analysis, and interpretation of data; writing of the report; or the decision to submit the paper for publication., (Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Self-regulation, known as the ability to harness cognitive, emotional, and motivational resources to achieve goals, is hypothesized to contribute to health behaviors across the lifespan. Enhancing self-regulation early in life may increase positive health outcomes. During pre-adolescence, children assume increased autonomy in health behaviors (e.g., eating; physical activity), many of which involve self-regulation. This article presents results from a clinical trial (NCT03060863) that used a factorial design to test behavioral interventions designed to enhance self-regulation, specifically targeting executive functioning, emotion regulation, future-oriented thinking, and approach biases. Participants were 118 children (9-12 years of age, M = 10.2 years) who had a history of living in poverty. They were randomized to receive up to four interventions that were delivered via home visits. Self-regulation was assayed using behavioral tasks, observations, interviews, and parent- and child-report surveys. Results were that self-regulation targets were reliably assessed and that interventions were delivered with high fidelity. Intervention effect sizes were very small to moderate (d range = .02-.65, median = .14), and most were not statistically significant. Intercorrelation analyses indicated that associations between measures within each target varied based on the self-regulation target evaluated. Results are discussed with regard to the role of self-regulation-focused interventions in child health promotion. Implications of findings are reviewed for informing next steps in behavioral self-regulation interventions among children from low-income backgrounds., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Background: Adverse effects of clinician burnout have been studied across multiple specialties; however, there have been no studies examining rates of burnout among pediatric solid organ transplant teams. This study aimed to measure burnout, work exhaustion, professional fulfillment, and post-traumatic stress symptoms among clinicians and administrators practicing in this high-stress field., Methods: This cross-sectional study utilized a 50 item web-based survey that included the Personal Fulfillment Index and the IES-R. This survey was distributed across four pediatric solid organ transplant centers in North America. Basic demographics, clinician characteristics, and information regarding wellness and self-care activities were collected. Descriptive and correlational analyses were performed., Results: One hundred and thirty five participants completed the survey, 76% were female and 78% were Caucasian. One-third (34%) of participants endorsed burnout, while 43% reported professional fulfillment. Approximately 15% of respondents endorsed clinically significant levels of post-traumatic stress symptoms related to patient deaths, with female clinicians more likely to endorse symptoms (p = .01). Nearly 80% of participants reported engaging in self-care activities outside of work and only 10% of participants reported participation in hospital-sponsored wellness programs., Conclusions: Pediatric solid organ transplant team members exhibited moderate levels of burnout, professional fulfillment, and post-traumatic stress. Female clinicians were the most likely to experience both work exhaustion and post-traumatic stress symptoms. Transplant centers are encouraged to consider interventions and programming to improve clinician wellness., (© 2021 Wiley Periodicals LLC.)

Objective: Non-adherence to pediatric regimens is a common concern. Low health literacy is correlated with poor outcomes in adults but is understudied in pediatrics. The current project aimed to determine the relationship between health literacy, adherence, and outcomes in pediatric liver transplant recipients. Hypotheses included a) parent and patient health literacy would be positively correlated; and b) low patient and/or parent health literacy would be negatively correlated with adherence and health outcomes., Patients and Methods: Eligible participants were recruited during routine follow-up visits in a pediatric liver transplant clinic. Parents and patients (>13 years old) completed 2 measures of health literacy. Patients ≥18 years completed health literacy measures without corresponding parent surveys. Adherence variables and health outcomes were obtained from medical records., Results: Seventy-nine patients across two sites completed the study. Variance in classification of health literacy between measures was observed; however, most parents (82%-100%) scored within an "adequate literacy" range. More adolescents scored in lower health literacy ranges relative to the parents. Markers of SES were positively correlated with health literacy. Parent health literacy was negatively associated with biopsy-proven rejection episodes and the number of hospitalizations; however, it was not associated with measures of tacrolimus adherence. There were no relationships observed between parent and adolescent health literacy., Conclusions: Health literacy is an important consideration in managing patient care; however, available measures demonstrate variability in capturing the skills of patients. Effective communication strategies may ameliorate admittedly small, but negative, impacts of limited health literacy on outcomes., (© 2020 Wiley Periodicals LLC.)

Purpose of Review: Youth with strong self-regulation (SR), or the ability to manage thoughts, emotions, and behaviors, engage in more effective type 1 diabetes (T1D) management. However, while parent support and engagement are critical to ensuring positive youth T1D outcomes, it is rarely considered that parents' SR may also influence youth T1D management. If this is the case, novel interventions to improve parents' SR or ensure adequate support for parents with SR challenges offer great potential to improve family functioning and youth T1D management., Recent Findings: Theoretical and preliminary empirical evidence suggests that parental SR impacts family processes that support youth T1D treatment regimen adherence. Furthermore, parent and youth SR likely interact, with high parent SR enhancing the positive effects of high youth SR or compensating for low youth SR. Continued research is needed to better understand the ways in which parent SR matters to youth T1D management and identify how to support improvements in T1D management among families of parents with low SR.

Pruritus is a debilitating symptom for patients with Alagille syndrome (ALGS). In a previously reported trial of maralixibat, an investigational antipruritic agent, itching was assessed using a digital diary based on twice-daily caregiver observation of itching severity (Itch Reported Outcome, ItchRO[Observer]). The goal of this study was to characterize pruritus in participants with ALGS at baseline in this trial, as assessed by the ItchRO instrument and the physician-observed clinician scratch scale (CSS), relative to biomarkers putatively associated with pruritus and health-related quality of life assessment. Thirty-seven participants with ALGS (median age of 6 years; range 1-17 years) were enrolled. No association was identified between CSS and ItchRO(Obs) ( r  = 0.22, P  = 0.2). Neither CSS nor ItchRO were associated with serum bile acids ( r  = -0.08, P  = 0.6 for both) or autotaxin ( r  = 0.22, P  = 0.2; r  = 0.28, P  = 0.12). There was no significant association between Pediatric Quality of Life Inventory total parent scores and CSS or ItchRO ( r  = -0.23, P  = 0.2; r  = -0.16, P  = 0.36). There was a significant association between ItchRO and Multidimensional Fatigue Scale and Family Impact Module total scores (Pearson correlation coefficient -0.575, P  = 0.0005; 0.504, P  = 0.002). In exploratory analysis, selected questions relating to fatigue and sleep disturbance (n = 12) from Pediatric Quality of Life Inventory, Multidimensional Fatigue Scale, and Family Impact Module were correlated with pruritus scores; positive associations were identified. Conclusion: Itching scores did not correlate with each other, nor with putative serum biomarkers of pruritus, and further, did not correlate with quality of life. Hypothesis-generating analyses implicate sleep disturbance and fatigue as key associations with caregiver observations of itching. This is highly relevant to the selection of surrogate endpoints for clinical trials of pruritus therapies., (© 2020 The Authors. Hepatology Communications published by Wiley Periodicals, Inc., on behalf of the American Association for the Study of Liver Diseases.)

Background: Self-regulation (SR), or the capacity to control one's thoughts, emotions, and behaviors in order to achieve a desired goal, shapes health outcomes through many pathways, including supporting adherence to medical treatment regimens. Type 1 Diabetes (T1D) is one specific condition that requires SR to ensure adherence to daily treatment regimens that can be arduous and effortful (e.g., monitoring blood glucose). Adolescents, in particular, have poor adherence to T1D treatment regimens, yet it is essential that they assume increased responsibility for managing their T1D as they approach young adulthood. Adolescence is also a time of rapid changes in SR capacity and thus a compelling period for intervention. Promoting SR among adolescents with T1D may thus be a novel method to improve treatment regimen adherence. The current study tests a behavioral intervention to enhance SR among adolescents with T1D. SR and T1D medical regimen adherence will be examined as primary and secondary outcomes, respectively., Methods: We will use a randomized control trial design to test the impact of a behavioral intervention on three SR targets: Executive Functioning (EF), Emotion Regulation (ER), and Future Orientation (FO); and T1D medical regimen adherence. Adolescents with T1D (n = 94) will be recruited from pediatric endocrinology clinics and randomly assigned to treatment or control group. The behavioral intervention consists of working memory training (to enhance EF), biofeedback and relaxation training (to enhance ER), and episodic future thinking training (to enhance FO) across an 8-week period. SR and treatment regimen adherence will be assessed at pre- and post-test using multiple methods (behavioral tasks, diabetes device downloads, self- and parent-report). We will use an intent-to-treat framework using generalized linear mixed models to test our hypotheses that: 1) the treatment group will demonstrate greater improvements in SR than the control group, and 2) the treatment group will demonstrate better treatment regimen adherence outcomes than the control group., Discussion: If successful, SR-focused behavioral interventions could improve health outcomes among adolescents with T1D and have transdiagnostic implications across multiple chronic conditions requiring treatment regimen adherence., Trial Registration: ClinicalTrials.gov: NCT03688919; registered September 28, 2018.

Self-regulation (SR), or the ability to manage thoughts, emotions, and behaviours in order to achieve a desired goal, is seen as underlying positive health behaviours. In adults, behaviour change techniques (BCTs) are recommended to promote SR across health domains; although establishing healthy habits early in life is important, studies of SR and health in children are rare. This conceptual review provides guidance on developmental considerations for applying BCTs to enhance SR capacity in children and youth with the goal of fostering positive behavioural health trajectories early in the lifespan. Key considerations include the nature of developmental changes in SR and interaction among SR processes; temporal associations between SR and health behaviours; and relevance of health goals for children and youth. Building on a meta-review of BCT's used to promote SR in adults and youth, this conceptual review highlights key SR milestones to consider in behaviour change-focused interventions from early childhood through adolescence and provides an overview of social-ecologic influences on SR development and associations between SR and health behaviours across these age periods. Implications for and examples of using developmentally-informed BCTs in interventions to enhance SR in children and youth are noted and suggestions for future research are discussed.

The overall objective of this paper was to systematically review and synthesize the emerging literature investigating the role of father involvement in pediatric outcomes among chronic illness populations. This review sought to answer the following questions: (1) what measures are used to assess father involvement in pediatric chronic illness populations, and who is the respondent, and (2) how is father involvement associated with child psychosocial and health related outcomes in pediatric chronic illness populations? Databases were searched using a key word search strategy. Articles were screened according to exclusion criteria, resulting in 15 identified articles that included a pediatric illness population, and assessed both father involvement and a child outcome variable. Qualitative analysis revealed that several measures have been used to assess father involvement in pediatric chronic illness populations. As a whole, the majority of findings indicate that better outcomes are associated with more father involvement in illness and non-illness related activities, and higher father-child relationship quality. Contradictory findings may be due to the quality of the involvement being assessed, or the possibility that father's become more involved with illness tasks in response to their child's poorer health outcomes. Future research should include the development and use of psychometrically sound measures of father involvement and employ more diverse samples with rigorous methodology.

Objectives: The aim of the study was to assess neurodevelopmental outcomes among children with biliary atresia (BA) surviving with their native liver at ages 3 to 12 years and evaluate variables that associate with neurodevelopment., Methods: Participants (ages 3-12 years) in a prospective, longitudinal, multicenter study underwent neurodevelopmental testing with Weschler Preschool and Primary Scale of Intelligence, 3rd edition (WPPSI-III, ages 3-5 years) and Weschler Intelligence Scale for Children, 4th edition (WISC-IV, ages 6-12 years). Continuous scores were analyzed using Kolmogorov-Smironov tests compared with a normal distribution (mean = 100 ± 15). Effect of covariates on Full-Scale Intelligence Quotient (FSIQ) was analyzed using linear regression., Results: Ninety-three participants completed 164 WPPSI-III (mean age 3.9) and 51 WISC-IV (mean age 6.9) tests. WPPSI-III FSIQ (104 ± 14, P < 0.02), Verbal IQ (106 ± 14, P < 0.001), and General Language Composite (107 ± 16, P < 0.001) distributions were shifted higher compared with test norms. WISC-IV FSIQ (105 ± 12, P < 0.01), Perceptual Reasoning Index (107 ± 12, P < 0.01), and Processing Speed Index (105 ± 10, P < 0.02) also shifted upwards. In univariate and multivariable analysis, parent education (P < 0.01) was a significant predictor of FSIQ on WPPSI-III and positively associated with WISC-IV FSIQ. Male sex and higher total bilirubin and gamma glutamyl transferase (GGT) predicted lower WPPSI-III FSIQ. Portal hypertension was predictive of lower WISC-IV FSIQ., Conclusions: This cohort of children with BA and native liver did not demonstrate higher prevalence of neurodevelopmental delays. Markers of advanced liver disease (higher total bilirubin and GGT for age ≤5 years; portal hypertension for age ≥6) correlate with lower FSIQ and may identify a vulnerable subset of patients who would benefit from intervention.

The Johns Hopkins University School of Medicine organized 2 multistakeholder symposia on February 2, 2018 and January 11, 2019 to address the problem of high graft failure in adolescent and young adult (AYA) solid organ transplant (SOT) recipients. Participants included international experts in transplantation, behavioral psychology, patient/parent advocacy, and technology. The objectives of the symposia were as follows: (1) to identify and discuss the barriers to and facilitators of effective transfer of care for AYA SOT recipients; (2) to actively explore strategies and digital solutions to promote their successful transfer of care; and (3) to develop meaningful partnerships for the successful development, evaluation, implementation, and dissemination of these digital solutions. Additionally, data were collected from 152 AYA SOT recipients demonstrating a substantial gap in how this population uses technologies for health-related activities, alongside an increased interest in an app to help them manage their transplant.

Parents of pediatric liver and kidney transplant recipients were surveyed regarding their current education plans (eg, Individualized Education Program, 504), satisfaction with these plans, and interest in educational support from the psychosocial transplant team. Survey results indicate high rates of IEP and 504 plans, academic and related services, and accommodations among this population. The majority of parents/guardians reported satisfaction with their child's current school plan and did not report need for additional transplant team support specific to school services on the survey measure. However, other information highlights the importance for pediatric transplant teams to consider other ways to support this population's educational needs., (© 2019 Wiley Periodicals, Inc.)

Background: Despite advancements in treatment and survival, pediatric organ failure and transplant populations continue to face significant risks of morbidity and mortality. Little scientific attention has been given to addressing the end-of-life care needs of this growing population of young people. This study characterized current practices, beliefs, and challenges specific to the disclosure of prognosis and end-of-life care topics among providers caring for pediatric organ failure and transplant populations., Methods: This cross-sectional study included 144 healthcare providers actively caring for children, adolescents, and young adults with organ failure or solid organ transplant history. Participants completed an electronic survey measuring frequency and comfort in discussing the following topics with patients and parents: prognosis/survival statistics, re-transplantation, advance care planning (ACP), and death/dying. Descriptive statistics, two-sample t tests, and analysis of variance were used., Results: Fewer than half of respondents regularly discuss prognosis/survival statistics and potential need for re-transplantation with their pediatric and young adult patients. Less than 20% of providers engage their pediatric patients in ACP discussions, and approximately 30% facilitate such discussions with young adult patients. Pediatric organ failure and transplant providers endorse a number of barriers specific to discussing these topics., Conclusion: Pediatric organ failure and transplant providers do not regularly discuss prognosis or end-of-life care topics with this patient population. Communication-focused intervention research is needed to improve honest and compassionate discussion of these topics that is aligned with both patients' and parents' needs and preferences., (© 2019 Wiley Periodicals, Inc.)

Objectives: To assess neurodevelopmental outcomes among participants with biliary atresia with their native liver at ages 12 months (group 1) and 24 months (group 2), and to evaluate variables predictive of neurodevelopmental impairment., Study Design: Participants enrolled in a prospective, longitudinal, multicenter study underwent neurodevelopmental testing with either the Bayley Scales of Infant Development, 2nd edition, or Bayley Scales of Infant and Toddler Development, 3rd edition. Scores (normative mean = 100 ± 15) were categorized as ≥100, 85-99, and <85 for χ 2 analysis. Risk for neurodevelopmental impairment (defined as ≥1 score of <85 on the Bayley Scales of Infant Development, 2nd edition, or Bayley Scales of Infant and Toddler Development, 3rd edition, scales) was analyzed using logistic regression., Results: There were 148 children who completed 217 Bayley Scales of Infant and Toddler Development, 3rd edition, examinations (group 1, n = 132; group 2, n = 85). Neurodevelopmental score distributions significantly shifted downward compared with test norms at 1 and 2 years of age. Multivariate analysis identified ascites (OR, 3.17; P = .01) and low length z-scores at time of testing (OR, 0.70; P < .04) as risk factors for physical/motor impairment; low weight z-score (OR, 0.57; P = .001) and ascites (OR, 2.89; P = .01) for mental/cognitive/language impairment at 1 year of age. An unsuccessful hepatoportoenterostomy was predictive of both physical/motor (OR, 4.88; P < .02) and mental/cognitive/language impairment (OR, 4.76; P = .02) at 2 years of age., Conclusion: Participants with biliary atresia surviving with native livers after hepatoportoenterostomy are at increased risk for neurodevelopmental delays at 12 and 24 months of age. Those with unsuccessful hepatoportoenterostomy are >4 times more likely to have neurodevelopmental impairment compared with those with successful hepatoportoenterostomy. Growth delays and/or complications indicating advanced liver disease should alert clinicians to the risk for neurodevelopmental delays, and expedite appropriate interventions., Trial Registration: Clinicaltrials.gov: NCT00061828 and NCT00294684., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Across pediatric organ transplant populations, patient and family psychosocial functioning is associated with important health-related outcomes. Research has suggested that pediatric heart transplant recipients and their families are at increased risk for adverse psychosocial outcomes; however, recent investigation of psychosocial functioning in this population is lacking. This study aimed to provide a contemporary characterization of psychosocial functioning in pediatric heart transplant recipients and their families. Associations between psychosocial function, demographic variables, and transplant-related variables were investigated. Fifty-six parents/guardians of pediatric heart transplant recipients completed a comprehensive psychosocial screening measure during transplant follow-up clinic visits. Descriptive statistics, correlational analyses, and independent samples t tests were performed. Forty percent of pediatric heart transplant recipients and their families endorsed clinically meaningful levels of total psychosocial risk. One-third of patients presented with clinically significant psychological problems per parent report. Psychosocial risk was unassociated with demographic or transplant-related factors. Despite notable improvements in the survival of pediatric heart transplant recipients over the past decade, patients and families present with sustained psychosocial risks well beyond the immediate post-transplant period, necessitating mental health intervention to mitigate adverse impact on health-related outcomes., (© 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Poor self-regulation (i.e., inability to harness cognitive, emotional, motivational resources to achieve goals) is hypothesized to contribute to unhealthy behaviors across the lifespan. Enhancing early self-regulation may increase positive health outcomes. Obesity is a major public health concern with early-emerging precursors related to self-regulation; it is therefore a good model for understanding self-regulation and health behavior. Preadolescence is a transition when children increase autonomy in health behaviors (e.g., eating, exercise habits), many of which involve self-regulation. This paper presents the scientific rationale for examining self-regulation mechanisms that are hypothesized to relate to health behaviors, specifically obesogenic eating, that have not been examined in children. We describe novel intervention protocols designed to enhance self-regulation skills, specifically executive functioning, emotion regulation, future-oriented thinking, and approach bias. Interventions are delivered via home visits. Assays of self-regulation and obesogenic eating behaviors using behavioral tasks and self-reports are implemented and evaluated to determine feasibility and psychometrics and to test intervention effects. Participants are low-income 9-12 year-old children who have been phenotyped for self-regulation, stress, eating behavior and adiposity through early childhood. Study goals are to examine intervention effects on self-regulation and whether change in self-regulation improves obesogenic eating., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

The process of pediatric solid organ transplantation (SOT) places new and increased stressors on patients and family members. Measures of family functioning may predict psychological and health outcomes for pediatric patients and their families, and provide opportunity for targeted intervention. This systematic review investigated parent and family functioning and factors associated with poorer functioning in the pediatric SOT population. Thirty-seven studies were identified and reviewed. Studies featured a range of organ populations (eg, heart, liver, kidney, lung, intestine) at various stages in the transplant process. Findings highlighted that parents of pediatric SOT populations commonly report increased stress and mental health symptoms, including posttraumatic stress disorder. Pediatric SOT is also associated with increased family stress and burden throughout the transplant process. Measures of parent and family functioning were associated with several important health-related factors, such as medication adherence, readiness for discharge, and number of hospitalizations. Overall, findings suggest that family stress and burden persists post-transplant, and parent and family functioning is associated with health-related factors in SOT, highlighting family-level functioning as an important target for future intervention., (© 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Background: Understanding the impact of effective paediatric adherence promotion interventions on patients, families and the healthcare system is necessary to inform efforts to improve healthcare quality and control costs. Building on previous research suggesting that improving adherence may have far-reaching benefits, the objective of this study was to quantify the impact of effective adherence promotion interventions for children and adolescents with a chronic medical condition on patients, families and the healthcare system., Methods: Authors systematically reviewed articles indexed in PubMed, PsycINFO and CINAHL to identify randomized controlled trials of paediatric adherence promotion interventions. Interventions that improved paediatric adherence and examined patient-level, family-level or healthcare system-level outcomes in children and adolescents (M age ≤ 18 years) with a chronic medical condition were included. Two authors independently extracted and classified outcome variables as patient-level (quality of life and disease-related activity restrictions), micro-level (family functioning, family conflict, caregiver quality of life, caregiver sleep interruption, caregiver days away from work and patient missed school days) or macro-level variables (emergency department visits, hospitalizations, outpatient visits and urgent care visits). Outcome variables detailed in previously published reviews (i.e. disease severity) were excluded., Results: Twenty studies representing 19 unique samples met inclusion criteria. An additional eight articles representing trials that did not significantly improve adherence were included in post hoc analyses. Compared with control interventions, effective paediatric adherence promotion interventions improved patient quality of life and family-level outcomes and decreased healthcare utilization among children and adolescents with a chronic medical condition., Conclusions: Interdisciplinary efforts to improve healthcare quality and reduce spending among children and adolescents with a chronic medical condition may be enhanced by incorporating effective paediatric adherence promotion interventions. As relatively few chronic medical conditions were represented in included studies, future research should examine the impact of paediatric adherence promotion interventions in other populations., (© 2015 John Wiley & Sons Ltd.)

The transition from pediatric to adult transplant care is a high risk period for non-adherence and poor health outcomes. This article describes a quality improvement initiative integrated into a pediatric liver transplant program that focused on improving outcomes following the transfer from pediatric to adult liver transplant care. Using improvement science methodology, we evaluated the impact of our center's transition readiness skills (TRS) program by conducting a chart review of 45 pediatric liver transplant recipients who transferred to adult transplant care. Medication adherence, clinic attendance, and health status variables were examined for the year pre-transfer and first year post-transfer. 19 recipients transferred without participating in the TRS program (control group) and 26 recipients participated in the program prior to transferring to the adult clinic (TRS group). The TRS group was significantly older at the time of transfer, more adherent with medications, and more likely to attend their first adult clinic visit compared to the control group. Among the TRS group, better adolescent and parent regimen knowledge were associated with greater adherence to post-transfer clinic appointments. Transition planning should focus on the gradual shift in responsibility for health management tasks, including clinic attendance, from parent to adolescent. There may be support for extending transition support for at least 1 year post-transfer to promote adherence.

Given the increased risk for non-adherence and poor health outcomes in late adolescence, there is a need for better methods to evaluate and improve the transition process as adolescent patients are prepared to be independent adults. This study assessed the psychometrics and concurrent validity of a newly developed measure of AoR for health management in pediatric liver transplant patients. A total of 48 patients and 37 parents completed a 13-item measure of AoR. We performed an exploratory PCA on survey results and used component scores to assess the relationship between AoR and age, age at transplant, adherence, and health outcomes. Two primary components were identified: communication with the healthcare system and self-management tasks. Parent perception of adolescent responsibility for tasks related to communicating with the healthcare system was correlated, in younger patients, with increased non-adherence while responsibility for tasks related to self-management was correlated, in older patients, with decreased non-adherence. These results support AoR as a two-domain construct, and they provide targets for monitoring and intervention as adolescent patients advance toward transfer., (© 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Objectives: To assess health-related quality of life (HRQOL) in children with Alagille syndrome (ALGS) in comparison with healthy and other liver disease cohorts, and to identify determinants of HRQOL in patients with ALGS., Study Design: Within the Childhood Liver Disease Research Network prospective study of cholestasis, Pediatric Quality of Life Inventory (PedsQL) questionnaires were administered to 70 children with ALGS, 95 children with alpha-1-antitrypsin deficiency (A1ATD), and 49 children with other causes of chronic intrahepatic cholestasis (IHC) aged 5-18 years. Parent proxy PedsQL scores were recorded for children aged 2-18 years (98 ALGS, 123 A1ATD, and 68 IHC)., Results: Mean ages and total bilirubin (mg/dL) were ALGS 9.4 years; 4.4, A1ATD 9.5 years; 0.7, and IHC 10.3 years; 2.9. ALGS child PedsQL scores were lower than in healthy children and children with A1ATD (mean 73 vs 83; P = .001). Children with ALGS and IHC were similar, except in physical scores (73 vs 79; P = .05). Parents of children with ALGS perceived their children to have worse HRQOL than A1ATD (P ≤ .001) and marginally lower compared with IHC. Univariate analysis revealed ALGS child-reported scores were positively associated with better growth and inversely with total bilirubin. Growth failure, elevated international normalized ratio, and an intracardiac defect were predictive of poor parental scores (P ≤ .05). In multivariate analysis, only weight z-score remained significant for child- and parent-reported scores., Conclusions: HRQOL is impaired in children with ALGS compared with healthy and children with A1ATD, similar to children with IHC and is associated with growth failure, which is a potentially treatable cause of impaired HRQOL., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Objective: Transition may be associated with poor health outcomes, but limited data exist regarding inflammatory bowel disease (IBD). Acquisition of self-management skills is believed to be important to this process. IBD-specific checklists of such skills have been developed to aid in transition, but none has been well studied or validated. This study aimed to describe self-assessment ability to perform tasks on one of these checklists and to explore the relation between patient age and disease duration., Methods: Patients ages 10 to 21 years with IBD were recruited. An iPad survey queried the patients for self-assessment of ability to perform specific self-management tasks. Task categories included basic knowledge of IBD, doctor visits, medications and other treatments, and disease management. Associations with age and disease duration were tested with Spearman rank correlation., Results: A total of 67 patients (31 boys) with Crohn disease (n = 40), ulcerative colitis (n = 25), and indeterminate colitis (n = 2) participated in the study. Mean patient age was 15.8 ± 2.5 years, with median disease duration of 5 years (2 months-14 years). The proportion of patients who self-reported ability to complete a task without help increased with age for most tasks, including "telling others my diagnosis" (ρ = 0.43, P = 0.003), "telling medical staff I do not like or am having trouble following a treatment" (ρ = 0.37, P = 0.003), and "naming my medications" (ρ = 0.28, P = 0.02). No task significantly improved with disease duration., Conclusions: Self-assessment of ability to perform some key tasks of transition appears to improve with age, but not with disease duration. More important, communication with the medical team did not improve with age, despite being of critical importance to functioning within an adult care model.

As survival rates for pediatric solid organ transplantation have continued to improve, researchers and healthcare providers have increasingly focused on understanding and enhancing the HRQOL and psychosocial functioning of their patients. This manuscript reviews the psychosocial functioning of pediatric transplant recipients during the "later years," defined as more than three yr post-transplant, and focuses on the day-to-day impact of living with a transplant after the immediate period of adjustment and early years after surgery. Key topics reviewed include HRQOL, cognitive functioning, impact on the family, regimen adherence, and transition of responsibility for self-management tasks. Overall, pediatric transplant recipients evidence impairment in HRQOL, neuropsychological outcomes, and family functioning as compared to non-transplant recipients. However, the degree of impairment is influenced by a variety of factors including, disease severity, age, solid organ type, and study methodologies. Studies are limited by small samples, cross-sectional design, and the lack of universal assessment battery to allow for comparisons across solid organ populations. Areas for future research are discussed., (© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

With advances in medical care, youth with chronic illness have the potential for higher quality of life; however, these treatments often come with cost (i.e., burden, financial) that can result in non-adherence. Pediatric non-adherence, on average, is approximately 50% across chronic health conditions. Research has identified effective, evidence-based assessment measures and intervention strategies to promote regimen adherence in youth. Yet, these measures and strategies typically are designed for clinical trials and thus may not be feasible or practical in typical clinic settings. As the field of adherence assessment and intervention expands, it will be important to devise evidence-based tools that are pragmatic and can be translated easily into practice. To guide this future direction, the goals of this paper are to review evidence-based adherence assessment and intervention strategies that can be used with youth and families in clinical practice, to illustrate the complexities of addressing adherence concerns in routine practice, and to discuss the challenges of disseminating and implementing evidence-based strategies in the real world.

Objective: To investigate the relationship between sleep disturbance in children with liver transplants and parent and family health-related quality of life (HRQOL)., Method: 47 parents of children with liver transplants completed measures of child sleep and family HRQOL. Relationships between sleep and HRQOL and differences in HRQOL between groups with scores above and below the cutoff on a pediatric sleep measure were examined., Results: Parents endorsed higher rates of sleep-related breathing disorder (SRBD) and restless legs syndrome and periodic limb movements during sleep (RLS/PLMS) and lower HRQOL compared with published data. Significant correlations were found between SRBD and RLS/PLMS and HRQOL, and significant group differences in HRQOL were found between groups above and below the cutoff for behavior problems and RLS/PLMS., Conclusion: There are significant relationships between symptoms of SRBD and RLS/PLMS in children with liver transplants and family HRQOL. Behavior problems may account for these strong relationships., (© The Author 2014. Published by Oxford University Press on behalf of the Society of Pediatric Psychology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Objective: Pediatricians are frequently asked to address parents' behavioral concerns. Time out (TO) is one of the few discipline strategies with empirical support and is recommended by the American Academy of Pediatrics. However, correctly performed, TO can be a complex procedure requiring training difficult to provide in clinic due to time and cost constraints. The Internet may be a resource for parents to supplement information provided by pediatricians. The present study included evaluation of information on TO contained in websites frequently accessed by parents. It was hypothesized that significant differences exist between the empirically supported parameters of TO and website-based information., Methods: Predefined search terms were entered into commonly used search engines. The information contained in each webpage (n = 102) was evaluated for completeness and accuracy based on research on TO. Data were also collected on the consistency of information about TO on the Internet., Results: None of the pages reviewed included accurate information about all empirically supported TO parameters. Only 1 parameter was accurately recommended by a majority of webpages. Inconsistent information was found within 29% of the pages. The use of TO to decrease problem behavior was inaccurately portrayed as possibly or wholly ineffective on 30% of webpages., Conclusions: A parent searching for information about TO on the Internet will find largely incomplete, inaccurate, and inconsistent information. Since nonadherence to any 1 parameter will decrease the efficacy of TO, it is not recommended that pediatricians suggest the Internet as a resource for supplemental information on TO. Alternative recommendations for pediatricians are provided.

This review focuses on active clinical research in pediatric liver transplantation with special emphasis on areas that could benefit from studies utilizing the SPLIT infrastructure and data repository. Ideas were solicited by members of the SPLIT Research Committee and sections were drafted by members of the committee with expertise in those given areas. This review is intended to highlight priorities for clinical research that could successfully be conducted through the SPLIT collaborative and would have significant impact in pediatric liver transplantation., (© 2013 John Wiley & Sons A/S.)

Objective: To document current clinical practices for medical regimen adherence assessment and intervention in the field of pediatric psychology., Methods: 113 members of the Society of Pediatric Psychology completed an anonymous online survey that assessed use of adherence assessments and interventions in clinical practice, barriers and facilitators to their use, and preferred resources for obtaining information on adherence assessments and interventions., Results: Respondents reported using a range of adherence assessment and intervention strategies, some of which are evidence-based. Barriers to implementing these clinical strategies included time constraints and lack of familiarity with available clinical tools. Respondents reported that education about effective clinical tools would facilitate their use of adherence assessments and interventions., Conclusions: Future research and clinical efforts in adherence should consider developing practical tools for clinical practice, making accessible resources to promote dissemination of these tools, and increase understanding of clinician implementation of adherence assessments and interventions.

Among adult liver transplant recipients (LTRs), sleep disturbances and fatigue are common. Sleep problems following pediatric liver transplantation may contribute to daytime fatigue and lower health-related quality of life (HRQOL). The aim of this cross-sectional study was to determine the impact of sleep problems on the HRQOL of pediatric LTRs using validated measures. Participants included 47 LTRs. Mean age of the LTRs was 10.9 ± 4.6 years, and mean time since transplantation was 6.2 ± 3.9 years. The primary indication for transplantation was biliary atresia (51%). According to parent reports, pediatric transplant recipients had symptoms of sleep-disordered breathing, excessive daytime sleepiness, daytime behavior problems, and restless legs; 40.4% of parents and 43.8% of children reported significantly lower total HRQOL for the recipients. Age, time since transplantation, and health status were not significantly related to the quality of life. Hierarchical regression analyses revealed that the sleep-disordered breathing subscale of the Pediatric Sleep Questionnaire accounted for significant variance in parent-proxy reports on the Pediatric Quality of Life (PedsQL) summary scales measuring children's psychosocial health (R(2) = 0.36, P < 0.001), physical health (R(2) = 0.19, P = 0.004), and total HRQOL (R(2) = 0.35, P < 0.001). Also, the sleep-disordered breathing subscale accounted for significant variance in the child self-reported school functioning scale (R(2) = 0.18, P = 0.03). Clinically significant sleep problems were more common among children with low total HRQOL. In conclusion, sleep problems were common in this cohort of pediatric LTRs and predicted significant variance in HRQOL. Prospective larger scale studies are needed to assess factors that contribute to sleep difficulties and low HRQOL in this population. The detection and treatment of significant sleep problems may benefit the HRQOL of pediatric LTRs., (Copyright © 2012 American Association for the Study of Liver Diseases.)

The need to prepare pediatric transplant recipients for the transfer to adult-centered transplant care has received increased attention. This study aimed to determine adolescent and young adult LTR and parent perceptions and attitudes about the transition process. LTR and their parents completed a survey assessing level of prior thought and interest in learning about transferring care, knowledge of the transition process, perceived importance of self-management skills, concerns about moving to the adult clinic, and responsibility for health management tasks. Responses were analyzed by age, gender, and time since transplantation. Participants included 46 LTR (mean age = 16.6 yr; range 12-21), and 31 parents. Recipients and parents reported moderate concern about transition, with leaving pediatric providers being a primary worry. LTR ≥16 yr reported greater health care responsibility and increased thought, interest, and knowledge about transition. There were significant differences between parent and LTR perceptions of health care responsibility, indicating that LTR perceive having more independence than what their parents report. Overall, results suggest that adolescent and young adult LTR and their parents perceive the importance of transitional care, but demonstrate poor knowledge of the process. There remains a need for improved transition planning for both adolescents and parents., (© 2011 John Wiley & Sons A/S.)

To examine transition readiness, adherence, and health outcomes in pediatric liver transplant recipients using a clinically administered screening measure. Seventy-one pediatric liver transplant recipients (11-20 yr) and 58 parents completed a clinic-based TRS measuring perceived and demonstrated self-management skills, AoR for health-related tasks, regimen knowledge, and psychosocial adjustment. Adherence was measured using s.d. of immunosuppressants, proportion of immunosuppressant blood levels out of target range, and clinic attendance. Health outcomes included liver test panels, biopsies, rejection episodes, and hospitalizations. Results indicate that all domains of transition readiness, with the exception of demonstrated skills, and non-adherence were positively correlated with age. Proportion of immunosuppressant blood levels below target range was positively correlated with self-management skills and increased responsibility for medication tasks. Parent regimen knowledge was associated with clinic attendance. Health outcomes were significantly related to medication non-adherence, but not to transition readiness domains. Medication adherence is considered to be a key factor in the transition from pediatric to adult-centered transplant care. Non-adherence is associated with an increased risk for medical complications and is potentially modifiable. Interventions to promote self-management skills and adherence should be an essential component of transition planning., (© 2010 John Wiley & Sons A/S.)

Purpose of Review: Nonadherence among pediatric transplant recipients is prevalent, with rates as high as 75% among adolescents. These rates are alarming given that adherence behavior has the potential to be modified. This review examines evidence from recent studies and position statements to determine the current issues related to improving medication adherence in adolescent transplant recipients., Recent Findings: To date, there are no empirically validated treatments to promote adherence in pediatric transplant recipients. Yet, interventions in other chronic illness populations suggest that combined education and behavioral strategies are the most effective in improving medication adherence. The use of technology, such as the Internet and cellphones, provides a promising mechanism for delivering adherence-promoting interventions to adolescents. Preliminary studies in pediatric solid organ transplant suggest that interventions focused on targeted education and behavioral cues using cellphone text messages are promising strategies for promoting medication adherence., Summary: Strategies for promoting adherence in adolescent transplant recipients should include developmentally appropriate models and should incorporate health-related education, motivational strategies, and behavioral skills. Interventions should be tailored to meet individual needs, and should be a collaborative effort between the adolescent, family and healthcare providers.

The measurement properties of the newly developed Pediatric Quality of Life Inventory (PedsQL) 3.0 Transplant Module in pediatric solid organ transplant recipients were evaluated. Participants included pediatric recipients of liver, kidney, heart and small bowel transplantation who were cared for at seven medical centers across the United States and their parents. Three hundred and thirty-eight parents of children ages 2-18 and 274 children ages 5-18 completed both the PedsQL 4.0 Generic Core Scales and the Transplant Module. Findings suggest that child self-report and parent proxy-report scales on the Transplant Module demonstrated excellent reliability (total scale score for child self-report alpha= 0.93; total scale score for parent proxy-report alpha= 0.94). Transplant-specific symptoms or problems were significantly correlated with lower generic HRQOL, supporting construct validity. Children with solid organ transplants and their parents reported statistically significant lower generic HRQOL than healthy children. Parent and child reports showed moderate to good agreement across the scales. In conclusion, the PedsQL Transplant Module demonstrated excellent initial feasibility, reliability and construct validity in pediatric patients with solid organ transplants.

1. Rates of nonadherence among pediatric transplant recipients are as high as 50% to 65%, and this places adolescent transplant recipients at increased risk. 2. Adherence to immunosuppressant medications is a critical factor in the transition process as poor medication adherence is associated with an increased risk of poor long-term health outcomes. 3. Before transferring to adult-centered care, the pediatric transplant recipient should demonstrate adequate regimen knowledge and self-management skills. 4. Future research is needed to identify factors and interventions that affect long-term health outcomes in pediatric transplant recipients, including factors related to nonadherence and the successful transfer from pediatric care to adult-centered care., ((c) 2009 AASLD.)

Adolescence is a particularly high-risk period for non-adherence with post-transplant medical regimens. There remains a lack of research investigating factors related to non-adherence in adolescent LT recipients. The present study empirically assessed the relationship between adherence and HRQOL in adolescent LT recipients. Participants included 25 adolescents (mean = 15.1 yr, range 12-17.9) and their parent/guardian(s). Adherence was assessed using multiple indices including clinician-conducted interviews, rate of clinic attendance, and s.d. of consecutive tacrolimus blood levels. HRQOL was examined using self-report and parent-proxy report on well-validated assessment measures. Results indicated that 76% of participants were non-adherent on at least one measure of adherence, and HRQOL was significantly lower than normative data for healthy children. Tacrolimus s.d. were significant related to poor HRQOL across domains of physical, school, and social functioning. Non-adherent adolescents reported poorer health perceptions, self-esteem, mental health, family cohesion, and more limitations in social and school activities related to physical, emotional, and behavioral problems. These results suggest that empirically based assessment of HRQOL may help identify those at highest risk for behavior, emotional and school difficulties, as well as non-adherence. The examination of tacrolimus s.d. may also help identify patients who may benefit from intervention to promote adherence and HRQOL. Prospective investigations are necessary to further identify the impact of HRQOL on adherence and long-term health outcomes to further guide clinical intervention.

Objective: To compare medical, nutritional, and psychosocial outcomes of continuous subcutaneous insulin infusion (CSII) therapy and multiple daily insulin injections (MDI) in preschoolers with type 1 diabetes mellitus (T1DM) in a randomized controlled trial., Study Design: Sixteen children (mean age 4.4 +/- 0.7 yr, range 3.1-5.3 yr) with T1DM were randomly assigned to CSII or MDI. Hemoglobin A1c (HbA1c) was measured monthly for 6 months. Glucose variability was measured at baseline and at 6 months using continuous blood glucose sensing. Quality of life, adverse events, and nutrition information were assessed., Results: Parents of the CSII group reported a significant decrease in diabetes-related worry, while parents of the MDI group reported an increased frequency of stress associated with their child's medical care. Mean HbA1c levels from baseline (CSII 8.3 +/- 1.4%, MDI 8.0 +/- 0.8%) to 6 months (CSII 8.4 +/- 0.8%, MDI 8.2 +/- 0.4%) remained stable, and group differences were not significant. There were no significant group differences in duration of hypo- or hyperglycemic events or frequency of adverse events., Conclusion(s): For young children with T1DM, CSII therapy is comparable to MDI therapy with regard to glucose control but is associated with higher treatment satisfaction and improved quality of life.

The present study empirically assessed the relationships between adherence behaviors and HRQOL, parent and child psychological functioning and family functioning, and investigated the relationship between adherence behaviors and health outcomes in children who were within 5 years of their liver transplantation. Participants included 38 children (mean = 8.5 years, range 28 months to 16 years) and their parent/guardian(s). HRQOL and psychological functioning were examined using well-validated assessment measures. Measures of adherence included the rate of clinic attendance and standard deviations (SDs) of consecutive tacrolimus blood levels, which were collected and evaluated retrospectively. Measures of child health status included the frequency of hospital admissions, liver biopsies, episodes of rejection and graft function for the year prior to study participation. Results indicated that nonadherence was related to lower physical HRQOL, more limitations in social and school activities related to emotional and behavioral problems, parental emotional distress and decreased family cohesion. Nonadherence was also related to frequency and duration of hospitalizations, liver biopsies and rejection episodes. These results suggest that empirically based assessment of HRQOL, parenting stress and family functioning may help identify patients at risk for nonadherence, and may allow for the need-based delivery of appropriate clinical interventions.

Rationale: Methylphenidate (MPH) is widely used in the treatment of attention deficit hyperactivity disorder (ADHD) and is associated with positive clinical effects across a wide range of domains. Despite the clinical effectiveness of MPH, concern has arisen with respect to its abuse potential., Objectives: To assess MPH preference in adults diagnosed with ADHD using a choice procedure and to evaluate the relationship among drug preference, therapeutic efficacy, and abuse potential in a clinical sample., Methods: Participants were ten volunteers (ages 18-22 years) with ADHD who were receiving MPH treatment. Preference was assessed using a double-blind choice procedure with four sampling sessions wherein subjects received either placebo or MPH and eight choice sessions when they chose either capsule or no capsules., Results: Overall, MPH was chosen significantly more often than placebo (chi2=52.5; P<0.001) and participants were equally separated into groups of those who chose MPH reliably (MPH choosers) and those who did not (MPH non-choosers). MPH decreased ADHD symptoms and resulted in lower ratings of stimulant effects among MPH choosers. MPH choosers also reported higher levels of baseline ADHD symptoms., Conclusions: Despite higher preference of MPH than placebo in this clinical sample, other measures of abuse potential were not elevated, and MPH choosers were more symptomatic than non-choosers. As such, MPH preference in ADHD populations likely reflects therapeutic efficacy rather than abuse potential. Future work should examine MPH choice in diagnosed and non-diagnosed populations to further explore the role of clinical efficacy in the preference of this stimulant drug.