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Transplant-ineligible patients with advanced liver disease rarely receive timely advance care planning (ACP). Tools are needed to educate these patients about medical interventions available at the end of life to promote ACP.This single-site pilot randomized controlled trial assessed the feasibility, acceptability, and preliminary efficacy of an ACP video decision support tool for improving transplant-ineligible advanced liver disease patients' knowledge about and preferences for end-of-life care. Intervention participants watched a 5-minute video depicting 3 levels of goals of care: life-prolonging care (cardiopulmonary resuscitation [CPR] and intubation), life-limiting care (hospitalization, no CPR/intubation), and comfort care. Control subjects received only a verbal narrative of these 3 levels of goals of care. The primary outcome was feasibility (≥60% enrollment rate). Secondary outcomes included acceptability of the video, patients' knowledge of end-of-life care options (6-item test; range, 0-6), and postintervention goals-of-care and CPR or intubation preferences.We enrolled 85% (n = 50 of 59) of eligible patients randomized to the video (n = 26) or verbal (n = 24) arm. In the video arm, 81% of patients reported being very comfortable watching the video. Patients in the video arm had higher mean knowledge scores (5.7 vs 4.8; Plt; .001) and were less likely to prefer to receive CPR compared with patients in the verbal arm (35% vs 63%; P = .09).An ACP video decision support tool to improve knowledge about and preferences for end-of-life care is both feasible and highly acceptable to transplant-ineligible patients with advanced liver disease with a high enrollment rate and promising preliminary efficacy. Future studies should examine the efficacy of the ACP video for enhancing the quality of their end-of-life care. (ClinicalTrials.gov, Number: NCT03557086).

Motile cilia dysfunction causes primary ciliary dyskinesia (PCD), situs inversus totalis (SI), and a spectrum of laterality defects, yet the prevalence of laterality defects other than SI in PCD has not been prospectively studied.In this prospective study, participants with suspected PCD were referred to our multisite consortium. We measured nasal nitric oxide (nNO) level, examined cilia with electron microscopy, and analyzed PCD-causing gene mutations. Situs was classified as (1) situs solitus (SS), (2) SI, or (3) situs ambiguus (SA), including heterotaxy. Participants with hallmark electron microscopic defects, biallelic gene mutations, or both were considered to have classic PCD.Of 767 participants (median age, 8.1 years, range, 0.1-58 years), classic PCD was defined in 305, including 143 (46.9%), 125 (41.0%), and 37 (12.1%) with SS, SI, and SA, respectively. A spectrum of laterality defects was identified with classic PCD, including 2.6% and 2.3% with SA plus complex or simple cardiac defects, respectively; 4.6% with SA but no cardiac defect; and 2.6% with an isolated possible laterality defect. Participants with SA and classic PCD had a higher prevalence of PCD-associated respiratory symptoms vs SA control participants (year-round wet cough, P.001; year-round nasal congestion, P = .015; neonatal respiratory distress, P = .009; digital clubbing, P = .021) and lower nNO levels (median, 12 nL/min vs 252 nL/min; P.001).At least 12.1% of patients with classic PCD have SA and laterality defects ranging from classic heterotaxy to subtle laterality defects. Specific clinical features of PCD and low nNO levels help to identify PCD in patients with laterality defects.ClinicalTrials.gov; No.: NCT00323167; URL: www.clinicaltrials.gov.

Objectives The primary purpose of this research was to examine the influence of descriptive norms (perceptions of others' effort) on individual self-reported effort in sport. A secondary purpose was to examine the effect of the perceptions of norms associated with reference groups that differed on personal and contextual characteristics on an athlete's perceived effort, while controlling for an individual's reasons for working hard. Design A cross-sectional study (N = 50) was conducted with adolescent athletes playing on sport teams. Method Athletes completed a survey that assessed their reasons for putting forth effort, their self-perceived effort levels, and their perceptions about the effort of three different reference groups (friends on team, teammates, and other athletes within the same league). Results The results from a hierarchical regression analysis revealed that perceptions of descriptive norms predicted individual effort, after controlling for personal reasons for working hard. In terms of reference groups, individual effort was associated most with the norm of the friend group. Conclusions The influence of the perception of others' behavior on an individual's behavior was extended to the sport outcome of effort. Given that it was the norm of friends that emerged, preliminary evidence is provided suggesting that personal similarities may be more important than contextual similarities for predicting the reference group that will influence this individual behavior in an adolescent sport setting.