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.

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.

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.