Health-Related Quality of Life and Cognitive Functioning in Pediatric Liver Transplant Recipients.

The goal of this work was to examine the change in health-related quality of life (HRQOL) and cognitive functioning from early childhood to adolescence in pediatric liver transplantation (LT) recipients. Patients were recruited from 8 North American centers through the Studies of Pediatric Liver Transplantation consortium. A total of 79 participants, ages 11-18 years, previously tested at age 5-6 years in the Functional Outcomes Group study were identified as surviving most recent LT by 2 years and in stable medical follow-up. The Pediatric Quality of Life 4.0 Generic Core Scale, Pediatric Quality of Life Cognitive Function Scale, and PROMIS Pediatric Cognitive Function tool were distributed to families electronically. Data were analyzed using repeated measures and paired t tests. Predictive variables were analyzed using univariate regression analysis. Of the 69 families contacted, 65 (94.2%) parents and 61 (88.4%) children completed surveys. Median age of participants was 16.1 years (range, 12.9-18.0 years), 55.4% were female, 33.8% were nonwhite, and 84.0% of primary caregivers had received at least some college education. Median age at LT was 1.1 years (range, 0.1-4.8 years). The majority of participants (86.2%) were not hospitalized in the last year. According to parents, adolescents had worse HRQOL and cognitive functioning compared with healthy children in all domains. Adolescents reported HRQOL similar to healthy children in all domains except psychosocial, school, and cognitive functioning (P = 0.02; P < 0.001; P = 0.04). Participants showed no improvement in HRQOL or cognitive functioning over time. For cognitive and school functioning, 60.0% and 50.8% of parents reported "poor" functioning, respectively (>1 standard deviation below the healthy mean). Deficits in HRQOL seem to persist in adolescence. Over half of adolescent LT recipients appear to be at risk for poor school and cognitive functioning, likely reflecting attention and executive function deficits.

A Pilot Study of a Screening Tool for Pediatric Minimal Hepatic Encephalopathy.

OBJECTIVES: Despite the need for monitoring cognition for minimal hepatic encephalopathy (MHE) in children with portal hypertension, few screening methods exist. The Patient-Reported Outcomes Measurement Information System (PROMIS) Pediatric Perceived Cognitive Function (PedsPCF) item bank, a 43-item parent- and self-report questionnaire, could be a useful screening tool. This study aimed to evaluate the PedsPCF item bank as a screening tool and explore its correlation with other neurocognitive measures and clinical indicators of portal hypertension. METHODS: Pediatric patients with portal hypertension were recruited at Lurie Children's Liver Clinic. A short battery of neuropsychological tests tapping attention, executive functioning, and fine motor speed was administered along with surveys of cognitive functioning (PedsPCF, Behavior Rating Inventory of Executive Function; BRIEF) and quality of life (PROMIS Pediatric-25 Profile). RESULTS: Eighteen patients participated (ages 8--17). The PedsPCF correlated well with the BRIEF but did not correlate with neurocognitive testing. Qualitative heatmap analysis of the relationship between z-scores and clinical signs of portal hypertension suggests the PedsPCF is less sensitive than the BRIEF. The fine motor task (Grooved Pegboard) appears to offer the highest sensitivity of the tests administered and is also relatively quick and easy to administer. CONCLUSIONS: Elements of the battery show promise in this small pilot sample. The BRIEF and the Grooved Pegboard may hold the most potential for screening in the clinical setting. Further study is necessary to examine this question in a larger multicenter sample.

The effect of angiotensin-converting enzyme inhibitors on the rate of ascending aorta dilation in patients with bicuspid aortic valve.

BACKGROUND: Bicuspid aortic valve (BAV), the most common congenital heart defect, is associated with progressive aortic dilation and increased risk of dissection and rupture. The use of an angiotensin-converting enzyme (ACE) inhibitor has recently proven effective in slowing the rate of aortic dilation in other aortopathies, suggesting it may also be useful in managing BAV. We sought to determine whether ACE inhibitors effectively reduce the rate of aortic dilation in adolescents and young adults with BAV. METHODS: A retrospective cohort study was undertaken. Subjects receiving ACE inhibitor therapy were compared with controls with BAV and matched for the degree of aortic regurgitation. The rate of change in ascending aorta diameter was compared between the two groups. RESULTS: Among the 141 subjects identified in the patient records, 103 were receiving no medication and 38 were taking ACE inhibitors. Over a mean follow-up of 37 months, the ascending aorta Z-score increased slightly among those receiving ACE inhibitor +4.5 ± 1.6 to +4.7 ± 1.6. The rate of change was no different than those subjects not receiving ACE inhibitors, P = .64. CONCLUSION: In an adolescent and young adult population with an isolated BAV, there is no proven benefit to ACE inhibitor use with regard to slowing rate of ascending aortic dilation.