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Stimuli that are physically salient—e.g., brighter or differently colored to others in the visual scene—capture eye gaze and attention. Many studies have shown that color-singleton distractors slow visual search for a target, even when participants are informed beforehand of the features (e.g., color) of the upcoming distractor. In those studies, however, participants may not have been particularly motivated to recruit attentional processes and try to prevent attentional distraction by upcoming stimuli. In the current study we investigated whether participants could use pre-trial information about the color of an upcoming distractor to prevent themselves from getting distracted by it, when a reward was at stake. Results showed that a performance-contingent reward reduced overall distraction by physically salient stimuli. However, reward did not increase the likelihood that participants would use information about the color of the upcoming distractor to further improve visual search performance. This study highlights the fast and reflexive nature of attentional capture by physically salient distractors, which is difficult to control strategically, even when motivated to do so.

Abstract Myotonic dystrophy type 2 (DM2) is a tetranucleotide CCTG repeat expansion disease associated with an increased prevalence of autoimmunity. Here, we identified an elevated type I interferon (IFN) signature in peripheral blood mononuclear cells and primary fibroblasts of DM2 patients as a trigger of chronic immune stimulation. Although RNA-repeat accumulation was prevalent in the cytosol of DM2-patient fibroblasts, type-I IFN release did not depend on innate RNA immune sensors but rather the DNA sensor cGAS and the prevalence of mitochondrial DNA (mtDNA) in the cytoplasm. Sublethal mtDNA release was promoted by a chronic activation of the ATF6 branch of the unfolded protein response (UPR) in reaction to RNA-repeat accumulation and non-AUG translated tetrapeptide expansion proteins. ATF6-dependent mtDNA release and resulting cGAS/STING activation could also be recapitulated in human THP-1 monocytes exposed to chronic endoplasmic reticulum (ER) stress. Altogether, our study demonstrates a novel mechanism by which large repeat expansions cause chronic endoplasmic reticulum stress and associated mtDNA leakage. This mtDNA is, in turn, sensed by the cGAS/STING pathway and induces a type-I IFN response predisposing to autoimmunity. Elucidating this pathway reveals new potential therapeutic targets for autoimmune disorders associated with repeat expansion diseases.

Objective: Height is a significant, yet under-studied dimension of body dissatisfaction in men. The present study examined the relationship between height dissatisfaction, height, muscle and fat dissatisfaction, body ideals, and eating disorder symptoms in men. Participants and methods: Participants were a sample of male undergraduate Australian students (N = 224) who were administered self-report measures of height, muscle, and fat dissatisfaction, eating disorder symptoms, and muscle and body fat body-ideals, and reported their height. Results: Results showed that height, muscle dissatisfaction, and desired muscularity were important for explaining height dissatisfaction. Additionally, although eating disorder symptoms did not uniquely predict height dissatisfaction, there were small positive correlations between height dissatisfaction and eating disorder symptoms. Conclusions: This study highlights the importance of height in male body dissatisfaction and its association with muscular dissatisfaction and desired muscularity, as well as the potential significance of height in male eating disorders.

Background The purpose of this study was to investigate a therapeutic approach targeting the inflammatory response and consequent remodeling from ischemic myocardial injury. Methods and Results Coronary thrombus aspirates were collected from patients at the time of ST‐segment–elevation myocardial infarction and subjected to array‐based proteome analysis. Clinically indistinguishable at myocardial infarction (MI), patients were stratified into vulnerable and resilient on the basis of 1‐year left ventricular ejection fraction and death. Network analysis from coronary aspirates revealed prioritization of tumor necrosis factor‐α signaling in patients with worse clinical outcomes. Infliximab, a tumor necrosis factor‐α inhibitor, was infused intravenously at reperfusion in a porcine MI model to assess whether infliximab‐mediated immune modulation impacts post‐MI injury. At 3 days after MI (n=7), infliximab infusion increased proregenerative M2 macrophages in the myocardial border zone as quantified by immunofluorescence (24.1%±23.3% in infliximab versus 9.29%±8.7% in sham; P

Current quantitative methods to evaluate pavement conditions in the United States are most commonly focused on construction acceptance using the International Roughness Index (IRI). However, from an asset management perspective, qualitative visual inspection techniques are the most prevalent. Modern vehicles with factory-equipped sensors drive these roadways daily and can passively assess the condition of infrastructure at an accuracy level somewhere between qualitative assessment and rigorous construction acceptance techniques. This paper compares crowdsourced ride quality data with an industry standard inertial profiler on a 7-mile bi-directional construction zone. A linear correlation was performed on 14 miles of I-65 that resulted in an R2 of 0.7 and a p-value of

Motivationally salient stimuli, such as those associated with reward, can automatically gain attentional prioritisation – even when individuals are motivated to ignore such stimuli. This ‘attentional bias for reward’ has often been interpreted as evidence for involuntary Pavlovian ‘sign tracking’ behaviour. The prioritisation of reward-signalling distractors may additionally reflect a drive to gain information about the state of the world, irrespective of the particular reward that is being signalled. In the current study we assessed whether forewarning participants on each trial as to the upcoming features of a distractor would reduce reward-related attentional capture. This manipulation reduces the information provided by the distractor, without affecting the magnitude of the signalled reward. Using eye tracking in Experiment 1, we found that reward-related attentional capture was virtually eliminated when participants were informed of the upcoming distractor colour (relative to the baseline condition when no information was provided). In Experiment 2, using a response-time version of the task, we again found a significant reduction in reward-related attentional capture when participants received information about the colour of an upcoming distractor, or information about the value of the upcoming reward. Finally, in Experiment 3 we assessed whether participants were using the pre-trial information to strategically inhibit attention to the upcoming distractor colour. The results of these experiments are discussed within the context of information-seeking accounts of reward-related attentional capture effects.

Background: Trastuzumab given intravenously in combination with chemotherapy is standard of care for patients with early HER2-positive breast cancer (BC). Different randomised studies have shown equivalent efficacy of a subcutaneous injection into the thigh compared to the intravenous formulation. Other body regions for injection have not been investigated but might be more convenient for patients. Methods: After surgery, patients were randomised to receive either subcutaneous trastuzumab into the thigh or into the abdominal wall (AW). Patient preferences were evaluated using validated questionnaires (PINT). Primary objectives of this multicentre, non-blinded, randomised substudy of the GAIN-2 study were to investigate pharmacokinetics of the injection into the thigh versus AW and to determine patients' preferences of either administration site versus the previously received intravenous application. Results: 226 patients were randomised and 219 patients (thigh: N = 110; AW: N = 109) formed the modified intent-to-treat (mITT). Overall, 83.5% (out of N = 182 with information about patients’ preference) preferred subcutaneous over previous intravenous application or had no preference. Preference was similar between both administration sites (thigh: 80.6%; AW: 86.5; p = 0.322). Pharmacokinetic analysis included 30 patients. Geometric means of Cmax and AUC0-21d were higher in thigh than in AW group (geometric mean ratio with body weight adjustment: Cmax: 1.291, 90%-CI 1.052–1.584; AUC0-21d: 1.291, 90%-CI 1.026–1.626). Safety profile was in line with previous reports of subcutaneous trastuzumab. Conclusion: Subcutaneous trastuzumab into the thigh showed an approximately 30% higher bioavailability. Injections were well tolerated and preferred over intravenous administration. The subcutaneous injection into the thigh should remain the standard of care.

Summary: Background: Treatment with elexacaftor/tezacaftor/ivacaftor (ETI) improves multiple clinical outcomes in people with cystic fibrosis (pwCF) with at least one F508del allele. This study evaluated the real-world impact of ETI on lung function, nutritional status, pulmonary exacerbation frequency, and sweat chloride concentrations in a large group of pwCF. Methods: This observational cohort study used data from the German CF Registry for pwCF who received ETI therapy and were followed up for a period of 12 months. Findings: The study included 2645 pwCF from 67 centres in Germany (mean age 28.0 ± 11.5 years). Over the first year after ETI was initiated, percent predicted forced expiratory volume in 1 s (ppFEV1) increased by 11.3% (95% confidence interval [CI] 10.8–11.8, p

UNFOLDER (Unfavorable Young Low-Risk Densification of R-Chemo Regimens) is an international phase-3 trial in patients 18–60 years with aggressive B-cell lymphoma and intermediate prognosis defined by age-adjusted International Prognostic Index (aaIPI) of 0 and bulky disease (≥7.5 cm) or aaIPI of 1. In a 2 × 2 factorial design patients were randomized to 6× R-CHOP-14 or 6× R-CHOP-21 (rituximab, cyclophosphamide, doxorubicin, vincristine, and prediso[lo]ne) and to consolidation radiotherapy to extralymphatic and bulky disease or observation. Response was assessed according to the standardized response criteria published in 1999, not including F-18 fluordesoxyglucose positron emission tomography/computed tomography (FDG-PET). Primary endpoint was event-free survival (EFS). A total of 695 of 700 patients were eligible for the intention-to-treat analysis. Totally 467 patients qualified for radiotherapy of whom 305 patients were randomized to receive radiotherapy (R-CHOP-21: 155; R-CHOP-14: 150) and 162 to observation (R-CHOP-21: 81, R-CHOP-14: 81). Two hundred twenty-eight patients not qualifying for radiotherapy were randomized for R-CHOP-14 versus R-CHOP-21. After a median observation of 66 months 3-year EFS was superior in the radiotherapy-arm versus observation-arm (84% versus 68%; P = 0.0012), due to a lower rate of partial responses (PR) (2% versus 11%). PR often triggered additional treatment, mostly radiotherapy. No significant difference was observed in progression-free survival (PFS) (89% versus 81%; P = 0.22) and overall survival (OS) (93% versus 93%; P = 0.51). Comparing R-CHOP-14 and R-CHOP-21 EFS, PFS and OS were not different. Patients randomized to radiotherapy had a superior EFS, largely due to a lower PR rate requiring less additional treatment (NCT00278408, EUDRACT 2005-005218-19).

The state of Indiana maintains approximately 120 salt storage facilities strategically distributed across the state for winter operations. In April 2023, those facilities contained approximately 217,000 tons of salt with an estimated value of USD 21 million. Accurate inventories at each facility during the winter season are important for scheduling re-supply so the facilities do not run out of salt. Inventories are also important at the end of the season for restocking to provide balanced inventories. This paper describes the implementation of a portable pole-mounted LiDAR system to measure salt stockpile inventory at 120 salt storage facilities in Indiana. Using two INDOT staff members, the end-of-season inventory took 9 working days, with volumetric inventories provided within 24 h of data collection. To provide an independent evaluation of the methodologies, the Hovermap ST backpack was used at selected facilities to provide control volumes. This system has a range of 100 m and an accuracy of ±3 cm, which reduces the occlusion to less than 8%. The pre-season facility capacity ranged from 0% to 100%, with an average of 66% full across all facilities. The post-season facility percentage ranged from 3% to 100%, with an average of 70% full. In addition, permanent roof-mounted LiDAR systems were deployed at two facilities to evaluate the effectiveness of monitoring salt stockpile inventories during winter operation activities. Plans are now underway to install fixed LiDAR systems at 15 additional facilities for the 2023–2024 winter season.

Social media offer an unprecedented opportunity for companies to interact more closely with customers and market their products and services. But social media also present reputational risks as negative word-of-mouth can spread more quickly and widely through these platforms than ever before. This study investigates how companies respond to customer complaints on Twitter. We propose an innovative mixed methods approach (i) to identify the key features that mark the styles used by a sample of companies in their replies to customers and (ii) to determine the most effective strategies for responding to complaints. Our results reveal that an affective style, expressed through devices such as stance markers, emphatics, and amplifiers, elicits the most positive response from complainants, regardless of the formality of the message. The study advances our understanding of the features and effects of corporate social media discourse. It also provides business communication practitioners with linguistically grounded insights that can inform the development of appropriate strategies for dealing with negative word-of-mouth online.

Der Beitrag untersucht mittels kritischer Diskursanalyse die mediale Bearbeitung des rechtsterroristischen Anschlags von Halle (Saale) im Oktober 2019 in den drei größten Qualitätstageszeitungen Deutschlands. Ausgehend von Theorien der Zivilreligion und des kollektiven Gedächtnisses argumentieren wir, dass Journalist*innen als memory agents angesichts von Rechtsterrorismus im Sinn der deutschen Basiserzählung handeln. Dazu zeigen wir anhand identifizierter Diskursstränge, wie Schuldabwehr, positive Wir- Konstruktion und securitisation von Rechtsextremismus zusammenhängen. Wir argumentieren folglich, dass die mediale Bearbeitung dieses Anschlags gerade nicht dazu dient, politische Antworten auf Rechtsextremismus zu finden, sondern Diskurse (re-)produziert, die eine gesamtgesellschaftliche Auseinandersetzung damit behindern.

As the country moves toward electric vehicles (EV), the United States is in the process of investing over USD 7.5 billion in EV charging stations, and Indiana has been allocated $100 million to invest in their EV charging network. In contrast to traditional “gas stations”, EV charging times, depending on the charger power delivery rating, can require considerably longer dwell times. As a result, drivers tend to pair charging with other activities. This study looks at two EV public charging locations and monitors driver activity while charging, charge time, and station utilization over a 2-month period in Lafayette, Indiana. Over 4000 charging sessions at stations with varying power levels (350 kW, 150 kW, and 50 kW) were monitored, and the median charge time was between 28 and 36 min. A large variation in station utilization was observed at Electrify America charging stations that had a range of stations with 350 kW, 150 kW, and 50 kW available. The highest utilization rates by hour of day on average were observed at 25% at the 150 kW Tesla station. Driver activity during charging influenced dwell times, with the average dwell time of drivers who waited in their vehicles to charge being 10 min shorter than those who would travel to the shops. Rain in the forecast also impacted the number of users per day. Although there are no published metrics for EV utilization and associated driver activities, we believe examining this relationship will produce best practices for planning future investments in EV charging infrastructure as public and private sector partners develop a nationwide charging network.

Right-side diverticulitis (RSD) is an uncommon disease in Western countries. We conducted a case-matched comparison of surgically managed right-side and left-side diverticulitis (LSD) from the Southern California Kaiser Permanente database (2007-2014). Of 995 patients undergoing emergent surgery for diverticulitis, 33 RSD (3.3%) met our inclusion criteria and were matched (1:1) to LSD based on age, gender, year of diagnosis, and Hinchey class. Mean age of the RSD group was 56 ± 13.9 years, and 24.2 per cent were Asian. RSD was classified as Hinchey class III or IV in 28.1 per cent and 9.4 per cent of cases, respectively. Right hemicolectomy was performed in 87.9 per cent and laparoscopy was used in 24.2 per cent of the cases. Surgically managed RSD patients were more likely to be Asian (25% vs 3.1%, P = 0.03) and have body mass index < 25 (31.3% vs 6.3%, P = 0.02) compared with LSD patients. Diverting stoma was less common in the RSD (6.3% vs 62.5%) (P < 0.001). Hospital stay was shorter in RSD (7.6 ± 4.2 vs 12.8 ± 9.4 days, P = 0.006) and more common in the RSD group (P < 0.01). Open surgery (90.6% vs 71.9%) and postoperative complications (37.5% vs 25%) were more common in the LSD group, but that was not statistically significant (P > 0.05). Surgery for complicated RSD was associated with shorter hospital stay and decreased likelihood of diverting ostomy.

Large dermatology centers such as the Veterans Affairs health care system carry the challenge of providing adequate care for patients within an appropriate timeline. Herein we begin a discussion about the tracking systems in place at busy hospitals, such as the VA, where numerous biopsies are referred to outside providers for further treatment. The complex psychosocial aspect of providing care specifically to veterans is also addressed. Finally, we describe our system, which monitors malignant skin biopsies that are referred to outside clinics for Mohs Micrographic Surgery (MMS).

The United States has over 8.8 million lane miles nationwide, which require regular maintenance and evaluations of sign retroreflectivity, pavement markings, and other pavement information. Pavement markings convey crucial information to drivers as well as connected and autonomous vehicles for lane delineations. Current means of evaluation are by human inspection or semi-automated dedicated vehicles, which often capture one to two pavement lines at a time. Mobile LiDAR is also frequently used by agencies to map signs and infrastructure as well as assess pavement conditions and drainage profiles. This paper presents a case study where over 70 miles of US-52 and US-41 in Indiana were assessed, utilizing both a mobile retroreflectometer and a LiDAR mobile mapping system. Comparing the intensity data from LiDAR data and the retroreflective readings, there was a linear correlation for right edge pavement markings with an R2 of 0.87 and for the center skip line a linear correlation with an R2 of 0.63. The p-values were 0.000 and 0.000, respectively. Although there are no published standards for using LiDAR to evaluate pavement marking retroreflectivity, these results suggest that mobile LiDAR is a viable tool for network level monitoring of retroreflectivity.

The National Household Travel Survey (NHTS), conducted by the Federal Highway Administration, has historically been used for documenting personal mobility trends. Current techniques using surveys to collect this data are labor-intensive and difficult to scale. Emerging connected vehicle (CV) data can provide an alternative data source to potentially provide a more scalable method to measure the temporal and spatial usage of passenger vehicles in near real-time. With an impending shift in the automobile industry towards alternative fuel vehicles (AFV), agile monitoring of trip trends is important to help guide state and national investments in AFV infrastructure. This study presents methodologies and visualizations summarizing observed trip characteristics using a sample of more than 500 billion CV records and nearly 1 billion CV trips for December 2022 in the United States. The analysis found very close agreement between trip lengths for internal combustion engine vehicles (ICEV) for CVs and those reported by the 2017 NHTS. Mean trip lengths and trip durations from CVs and NHTS for ICEVs are within 7.8% and 6.6% of each other. The 85th percentile comparison was similarly close, within 0.7% and 8.3%. A comparison of trip trends among states for ICEVs and AFVs as well as US census places and temporal trends for a selection of states, including Indiana, Texas, Wyoming, and California, is provided. The paper concludes that CV data is an important source to monitor trip characteristics across ICEVs and AFVs in near real-time, which will be particularly important to track during the anticipated change to AFVs.

Chronic obstructive pulmonary disease (COPD) is a prevalent lung disease usually resulting from cigarette smoking (CS). Cigarette smoking induces oxidative stress, which causes inflammation and alveolar epithelial cell apoptosis and represents a compelling therapeutic target for COPD. Purified human platelet-derived exosome product (PEP) is endowed with antioxidant enzymes and immunomodulatory molecules that mediate tissue repair. In this study, a murine model of CS-induced emphysema was used to determine whether nebulized PEP can influence the development of CS-induced emphysema through the mitigation of oxidative stress and inflammation in the lung. Nebulization of PEP effectively delivered the PEP vesicles into the alveolar region, with evidence of their uptake by type I and type II alveolar epithelial cells and macrophages. Lung function testing and morphometric assessment showed a significant attenuation of CS-induced emphysema in mice treated with nebulized PEP thrice weekly for 4 weeks. Whole lung immuno-oncology RNA sequencing analysis revealed that PEP suppressed several CS-induced cell injuries and inflammatory pathways. Validation of inflammatory cytokines and apoptotic protein expression on the lung tissue revealed that mice treated with PEP had significantly lower levels of S100A8/A9 expressing macrophages, higher levels of CD4+/FOXP3+ Treg cells, and reduced NF-κB activation, inflammatory cytokine production, and apoptotic proteins expression. Further validation using in vitro cell culture showed that pretreatment of alveolar epithelial cells with PEP significantly attenuated CS extract-induced apoptotic cell death. These data show that nebulization of exosomes like PEP can effectively deliver exosome cargo into the lung, mitigate CS-induced emphysema in mice, and suppress oxidative lung injury, inflammation, and apoptotic alveolar epithelial cell death. [ABSTRACT FROM AUTHOR]

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The pineal hormone melatonin is the natural transducer of the environmental light–dark signal to the body. Although the responsiveness to photoperiod is well-conserved in humans, only about 25 percent of the human population experiences seasonal changes in behavior. As a consequence, humans seem to have adapted—at least partly—to the seasonal changes in day length. The aim of the study was to demonstrate that the individual melatonin deficit marker DOC (degree of pineal calcification) is related to variation of seasonal phenomena in humans. Out of 3,011 patients in which cranial computer tomography (cCT) was performed for diagnostic reasons, 97 consecutive “healthy” subjects (43 female, 54 male; age 18–68 yrs, mean ± SD: 35.0 ± 13.1) were included. Exclusion criteria were pathological finding in cCT, acute/chronic illness including alcohol/drug abuse, shift work, and medication, which are known to influence melatonin excretion. The degree of pineal calcification (DOC) was semiquantitatively determined using the previously validated method. The Seasonal Pattern Assessment Questionnaire (SPAQ) was performed in a telephone interview. Twenty-six subjects fulfilled the criteria for seasonal affective disorder (SAD) or subsyndromal (S) SAD. Seasonality was more pronounced in women than in men (SPAQ seasonality score: 7.8 ± 4.0 vs. 4.9 ± 4.5; p = 0.001) and negatively and significantly associated with age (r = −0.178; p = 0.04). The subjective sleep length significantly varied between seasons (one-way repeated measures ANOVA: F = 45.75; p < 0.0001), with sleep during winter being 53 min (±70 min) longer than during summer. Controlling for age, the total seasonality score was negatively and significantly associated with DOC (r94 = −0.214; p = 0.036). Data confirm earlier studies with respect to distribution of seasonality with sex and age. The survival of seasonality in the sleep length of people living in an urban environment underlines functionality of the circadian timing system in modern societies. Moreover, data confirm for the first time that diminished experience of seasonality in behavior is associated with a reduced individual capacity to produce melatonin.

Modern vehicles have extensive instrumentation that can be used to actively assess the condition of infrastructure such as pavement markings, signs, and pavement smoothness. Currently, pavement condition evaluations are performed by state and federal officials typically using the industry standard of the International Roughness Index (IRI) or visual inspections. This paper looks at the use of on-board sensors integrated in Original Equipment Manufacturer (OEM) connected vehicles to obtain crowdsource estimates of ride quality using the International Rough Index (IRI). This paper presents a case study where over 112 km (70 mi) of Interstate-65 in Indiana were assessed, utilizing both an inertial profiler and connected production vehicle data. By comparing the inertial profiler to crowdsourced connected vehicle data, there was a linear correlation with an R2 of 0.79 and a p-value of

Abstract A nanosized coating was designed to reduce fouling on the surface of a new type of cochlear implant relying on optoacoustic stimulation. This kind of device imposes novel design principles for antifouling coatings, such as optical transparency and resistance to significant constant bending. To reach this goal we deposited on poly(dimethylsiloxane) a PEO‐based layer with negligible thickness compared to the curvature radius of the cochlea. Its antifouling performance was monitored upon storage by quartz crystal microbalance, and its resistance upon bending was tested by fluorescence microscopy under geometrical constraints similar to those of implantation. The coating displayed excellent antifouling features and good stability, and proved suitable for further testing in real‐environment conditions.

Transportation agencies in northern environments spend a considerable amount of their budget on salt for winter operations. For example, in the state of Indiana, there are approximately 140 salt storage facilities distributed throughout the state and the state expends between USD 30 M and USD 60 M on inventory and delivery each year. Historical techniques of relying on visual estimates of salt stockpiles can be inaccurate and do not scale well for managing the supply chain during the winter or planning for re-supply during summer months. This paper describes the implementation of a portable pole mounted LiDAR system that can be used to inventory a large barn in under 15 min and describes how this system has been deployed over 90 times at 30 facilities. A quick and easy accuracy test, based upon conservation of volume, was used to provide an independent check on the system performance by repositioning portions of the salt pile. Those tests indicated stockpile volumes can be estimated with an accuracy of approximately 0.1%. The paper concludes by discussing how this technology can be permanently installed near the roof for systematic monitoring throughout the year.

The United States has over three trillion vehicle miles of travel annually on over four million miles of public roadways, which require regular maintenance. To maintain and improve these facilities, agencies often temporarily close lanes, reconfigure lane geometry, or completely close the road depending on the scope of the construction project. Lane widths of less than 11 feet in construction zones can impact highway capacity and crash rates. Crash data can be used to identify locations where the road geometry could be improved. However, this is a manual process that does not scale well. This paper describes findings for using data from onboard sensors in production vehicles for measuring lane widths. Over 200 miles of roadway on US-52, US-41, and I-65 in Indiana were measured using vehicle sensor data and compared with mobile LiDAR point clouds as ground truth and had a root mean square error of approximately 0.24 feet. The novelty of these results is that vehicle sensors can identify when work zones use lane widths substantially narrower than the 11 foot standard at a network level and can be used to aid in the inspection and verification of construction specification conformity. This information would contribute to the construction inspection performed by agencies in a safer, more efficient way.

Introduction Youth with brain-based disabilities (BBDs), as well as their parents/caregivers, often feel ill-prepared for the transfer from paediatric to adult healthcare services. To address this pressing issue, we developed the MyREADY TransitionTM BBD App, a patient-facing e-health intervention. The primary aim of this randomised controlled trial (RCT) was to determine whether the App will result in greater transition readiness compared with usual care for youth with BBD. Secondary aims included exploring the contextual experiences of youth using the App, as well as the interactive processes of youth, their parents/caregivers and healthcare providers around use of the intervention.Methods and analysis We aimed to randomise 264 youth with BBD between 15 and 17 years of age, to receive existing services/usual care (control group) or to receive usual care along with the App (intervention group). Our recruitment strategy includes remote and virtual options in response to the current requirements for physical distancing due to the COVID-19 pandemic. We will use an embedded experimental model design which involves embedding a qualitative study within a RCT. The Transition Readiness Assessment Questionnaire will be administered as the primary outcome measure. Analysis of covariance will be used to compare change in the two groups on the primary outcome measure; analysis will be intention-to-treat. Interviews will be conducted with subsets of youth in the intervention group, as well as parents/caregivers and healthcare providers.Ethics and dissemination The study has been approved by the research ethics board of each participating site in four different regions in Canada. We will leverage our patient and family partnerships to find novel dissemination strategies. Study findings will be shared with the academic and stakeholder community, including dissemination of teaching and training tools through patient associations, and patient and family advocacy groups.Trial registration number NCT03852550.