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Objectives To identify predictors of portal hypertension, liver transplantation, and death in North American youth with alpha-1-antitrypsin (AAT) deficiency, and compare with patients with AAT deficiency elsewhere. Study design The Childhood Liver Disease Research Network Longitudinal Observational Study of Genetic Causes of Intrahepatic Cholestasis is a prospective, cohort study of pediatric cholestatic liver diseases, including AAT deficiency, enrolling PIZZ and PISZ subjects 0-25 years of age seen since November 2007 at 17 tertiary care centers in the US and Canada. Data from standard-of-care baseline and annual follow-up visits were recorded from medical records, history, physical examination, and laboratory studies. Participants with portal hypertension were identified based on data collected. Results We enrolled 350 participants (60% male) with a native liver; 278 (79%) entered the cohort without portal hypertension and 18 developed portal hypertension during follow-up. Thirty participants required liver transplantation; 2 patients died during 1077 person-years of follow-up. There was no difference in participants with or without preceding neonatal cholestasis progressing to transplantation or death during the study (12% vs 7%; P = .09), or in experiencing portal hypertension (28% vs 21%; P = .16); the hazard ratio for neonatal cholestasis leading to portal hypertension was P = .04. Development of portal hypertension was associated with a reduced height Z-score. Conclusions Portal hypertension in youth with AAT deficiency impacts growth measures. Progression to liver transplantation is slow and death is rare, but the risk of complications and severe liver disease progression persists throughout childhood. A history of neonatal cholestasis is a weak predictor of severe disease.

Background and objectives Memories enter a labile state during recollection. Thus, memory changes that occur during recollection can affect future instances of its activation. Having subjects perform a secondary task that taxes working memory while they recall a negative emotional memory often reduces its vividness and emotional intensity during subsequent recollections. However, researchers have not manipulated the emotional valence of the secondary task itself. Methods Subjects viewed a video depicting the aftermath of three fatal road traffic accidents, establishing the same negative emotional memory for all subjects. We then tested their memory for the video after randomly assigning them to no secondary task or a delayed match-to-sample secondary task involving photographs of positive, negative, or neutral emotional valence. Results The positive secondary task reduced memory for details about the video, whereas negative and neutral tasks did not. Limitations We did not assess the vividness and emotionality of the subjects' memory of the video. Conclusions Having subjects recall a stressful experience while performing a positively valent secondary task can decrement details of the memory and perhaps its emotionality.

The mechanisms mediating the anxiolytic effects of attention bias modification (ABM) remain unclear. Accordingly, we randomly assigned speech-anxious subjects to receive four sessions of one of three training conditions: ABM, inverse ABM, and control. In the ABM condition, subjects viewed pairs of photographs of models displaying facial expressions of disgust and joy on a computer screen. Probes always replaced the positive face, and subjects pushed a button to indicate the identity of the probe (E or F) as rapidly as possible. In the inverse condition, the probes always replaced the negative face, and in the control condition, the probes replaced each face type equally often. After four training sessions, all groups exhibited statistically indistinguishable, but significant, reductions on self-report, behavioral, and physiological measures of speech anxiety. Self-report and behavioral measures of attentional control improved likewise. Contrary to early studies, ABM was not superior to control procedures in producing reductions on measures of social anxiety.