Objective-To examine transition readiness, adherence and health outcomes in pediatric liver transplant recipients using a clinically administered screening measure.Methods-71 pediatric liver transplant recipients (11-20 years) and 58 parents completed a clinic-based transition readiness survey measuring perceived and demonstrated self-management skills, allocation of responsibility for health-related tasks, regimen knowledge, and psychosocial adjustment. Adherence was measured using standard deviations (SDs) 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-All domains of transition readiness, with the exception of demonstrated skills, and nonadherence 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 nonadherence, but not to transition readiness domains.Conclusions-Medication adherence is considered to be a key factor in the transition from pediatric to adult-centered transplant care. Nonadherence 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.

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.

The management of infants with heterotaxy and a functional single ventricle remains challenging. First-stage palliation is associated with high operative mortality and increased resource utilization owing to surgical morbidity. Nonetheless, outcomes beyond hospital discharge are comparable to those for patients with other single ventricle anomalies. Efforts to improve survival in those patients should focus on perioperative care.

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.

Rationale Among adult liver transplant recipients, sleep disturbances and fatigue are common. Sleep problems following pediatric liver transplantation may contribute to daytime fatigue and lower HRQoL. The aim of this cross-sectional study was to determine the impact of sleep problems on child HRQoL in pediatric liver transplant recipients using validated measures of sleep and quality of life. Methods Participants included 47 liver transplant recipients (LTR). The mean age of patients was 10.9 years ± 4.6, and the mean time since transplant was 6.2± 3.9 years. The primary indication for transplantation was biliary atresia (51%). Results Based on parent report, 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 child Total HRQoL. Age, time since transplantation and health status variables were not significantly related to 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 PedsQL summary scales measuring child Psychosocial Health (R2=0.38, p < 0.001), Physical Health (R2=0.19, p=0.004), and Total HRQoL (R2 =0.35, p < 0.0001). Across the subscales of the PedsQL, sleep-disordered breathing accounted for significant variance on the child self-reported school functioning scale (R2 = 0.18, p=0.033). Clinically significant sleep problems were more common among children with low Total HRQoL. There were no significant group differences based on child self-report. Conclusions Sleep problems were common in this cohort of pediatric liver transplant recipients and predicted significant variance in parent-proxy reports of HRQoL. Prospective larger scale studies are needed to assess factors that may lead to sleep difficulties and low HRQoL in this population. Appropriate detection of significant sleep problems may lead to interventions that could benefit the quality of life of pediatric LTR.

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We encountered the rare case of a 48-year-old Caucasian woman who developed Takayasu arteritis (TA) while suffering from seropositive rheumatoid arthritis (RA). Several studies have reported an association between TA and various autoimmune disorders, however, the concurrent presence of Takayasu arteritis and rheumatoid arthritis is described in only few cases in the literature to date. The exact nature of the relationship between TA and RA remains unknown. Perhaps the development of these two diseases represents non-specific systemic inflammatory changes in the presence of a hereditary background predisposing to both RA and TA.

ObjectiveNon‐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 MethodsEligible 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.ResultsSeventy‐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.ConclusionsHealth 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.