Dallcurran2359

PURPOSE To evaluate adverse events of fluorescein angiography (FA) in pediatric patients. DESIGN Single-institution retrospective chart review. PARTICIPANTS Patients 0 to 18 years of age who underwent FA between January 2010 and December 2015 at a single institution in the United States. METHODS Pediatric patients who underwent FA by 3 surgeons were included in the study. Patients with fewer than 24 hours of documented follow-up were excluded. Significant adverse events within 24 hours of FA were evaluated. Detailed intraoperative and perioperative physiological parameters, including heart rate, blood pressure, oxygen saturation, and ventilation parameters, in inpatients undergoing simultaneous examination under anesthesia were reviewed. Peri-injection effects of FA were evaluated by 2-tailed paired t test comparison of mean 5-minute preinjection and 5-minute postinjection physiological data. MAIN OUTCOME MEASURES Significant adverse events associated with FA. selleck inhibitor RESULTS One hundred fifteen patients with a totaled directly with systemic adverse events in pediatric patients in this study. Younger patients more commonly were found to require an inpatient FA, whereas older patients older than 4 years underwent outpatient UWFA. PURPOSE Mutations in the CLN2 gene lead to a neurodegenerative and blinding lysosomal storage disorder late infantile neuronal ceroid lipofucinosis, also known as "CLN2 disease." The purpose of the current study was to characterize the evolution of CLN2-associated retinal manifestations using the Weill Cornell Batten Scale (WCBS) and the age association of the retinal degeneration using central subfield thickness (CST) measurements and then correlate these findings with fundus photography and OCT to determine a critical period for retinal intervention. DESIGN Retrospective, single-center cohort. PARTICIPANTS Eighty-four eyes of 42 treatment-naïve patients with CLN2 disease. METHODS Clinical records, fundus photographs, and OCT imaging for patients with CLN2 disease collected during examinations under anesthesia were reviewed. Imaging was categorized per WCBS criteria by 3 masked graders. MAIN OUTCOME MEASURES CLN2-associated retinopathy assessed using WCBS scores, fundus photographs, and OCT imaging, correlat with retina-specific CLN2 gene therapy should occur ideally before or as early as possible within this critical period. The WCBS is a valuable tool and is highly correlated with the extent of retinal degeneration observed in OCT or fundus photographs; by using the fellow eye as a control, this grading scale can be used to monitor the effect of CLN2 gene therapy in future trials. In this study, the feasibility and efficacy of Acceptance and Commitment Therapy in Daily Life (ACT-DL), ACT augmented with a daily life application, was investigated in 55 emerging adults (age 16 to 25) with subthreshold depressive and/or psychotic complaints. Participants were randomized to ACT-DL (n = 27) or to active control (n = 28), with assessments completed at pre- and post-measurement and 6- and 12-months follow-up. It took up to five (ACT-DL) and 11 (control) months to start group-based interventions. Participants attended on average 4.32 out of 5 ACT-DL sessions. On the app, they filled in on average 69 (48%) of signal-contingent beep-questionnaires, agreed to 15 (41%) of offered beep-exercises, initiated 19 on-demand exercises, and rated ACT-DL metaphors moderately useful. Relative to active control, interviewer-rated depression scores decreased significantly in ACT-DL participants (p = .027). Decreases in self-reported depression, psychotic-related distress, anxiety, and general psychopathology did not differ between conditions. ACT-DL participants reported increased mean NA (p = .011), relative to active controls. Mean PA did not change in either group, nor did psychological flexibility. ACT-DL is a feasible intervention, although adaptations in future research may improve delivery of and compliance with the intervention. There were mixed findings for its efficacy in reducing subthreshold psychopathology in emerging adults. Dutch Trial Register no. NTR3808. AIM The impact of clinical characteristics for predicting patterns of ventricular involvement in arrhythmogenic right ventricular cardiomyopathy (ARVC) are not well defined. The aims of this study were to characterize different patterns of ventricular involvement in patients with ARVC and to stratify them based on clinical characteristics exercise and underlying genetic mutations. METHODS Sixty-four patients with definite ARVC from the Swiss ARVC Registry were enrolled. Right and left ventricular functions were assessed at baseline and most recent follow-up. All patients received genetic testing. Serum high-sensitivity cardiac Troponin T (hs-cTNT) and N-terminal of pro-brain natriuretic peptide (NT-proBNP) were determined at baseline. RESULTS Thirty-five patients (55%) had isolated right ventricular (RV) involvement, 12 patients (19%) had biventricular (BiV) involvement at baseline and 17 patients (26%) had no left ventricular (LV) involvement at baseline, but revealed new onset LV involvement at mean follow-up of 7.5 years. Patients with BiV involvement at baseline harbored significantly more desmoplakin and multiple mutations and patients with new-onset LV involvement at follow-up frequently showed non-desmosomal mutations. Patients engaging in competitive sports more often showed LV involvement during follow-up. Baseline hs-cTNT and NT-proBNP levels were higher in patients developing BiV involvement. CONCLUSION Multiple mutations are more common in ARVC patients with BiV involvement. Competitive exercise is associated with disease progression resulting in BiV involvement. Hs-cTNT and NT-proBNP are elevated in patients with BiV involvement and may help to identify ARVC patients at risk for developing BiV disease. OBJECTIVE Compare driving capacity of individuals with Amyotrophic Lateral Sclerosis (ALS) and healthy controls (HC) using a driving simulation program. METHODS A prospective study was performed on individuals with ALS who reported they were still driving, and a group of HCs. Demographic data included age and gender. Assessment included cognitive assessments (Montreal cognitive assessment [MoCA] and ALS Cognitive Behavioral Scale [ALS-CBS]); gait speed (m/s); ALS Functional Rating Scale-revised total score (ALSFRS-R); and simulated driving assessment (Lane Change Task [LCT]). The LCT is a simple assessment tool which simulates the visual, cognitive, and motor demands of driving to detect at-risk drivers and uses distractions (secondary tasks) to quantify the performance loss on the primary task (lane changes). RESULTS Twenty-eight individuals with ALS (22 males, mean age 64 years) and 20 HCs (7 males, mean age 59 years) were studied. Individuals with mild to moderate ALS (ALSFRS-R mean 36.2) were older, had mild cognitive difficulty (MoCA 24 vs 27; ALS-CBS 14.