Preoperative Crohn's Disease Exclusion Diet and Exclusive Enteral Nutrition in Adults with Crohn's Disease: A Feasibility Randomised Controlled Trial.

Preoperative exclusive enteral nutrition (EEN) improves nutritional status, reduces intestinal inflammation, and likely improves surgical outcomes. Crohn's disease exclusion diet with partial enteral nutrition (CDED) also reduces intestinal inflammation but its safety preoperatively is unknown. This single-blinded, multicentre, randomised controlled trial of three preoperative nutritional therapies aimed to assess the feasibility of recruiting and retaining patients and collecting primary and secondary effectiveness outcomes. Adults undergoing elective Crohn's disease surgery with a body mass index (BMI) > 18.5 kg/m2 and without significant weight loss were eligible to participate. Patients were randomly assigned to six weeks of preoperative EEN, CDED, or standard care. Feasibility, nutritional, radiological, and surgical outcomes were recorded. Over 18 months, 48 patients were screened, 17 (35%) were randomised, and 13/17 (76%) patients were retained in the intervention phase. It was feasible to collect primary and secondary effectiveness data; at day 30, three patients had Clavien Dindo Grade 2 complications, and 10 had no complications. Nutritional therapy adherence of patients retained in the study was high. Recruitment and retention of patients who need elective Crohn's disease surgery for preoperative nutritional therapy is possible, although a shorter duration may improve EEN completion. The impact on surgical outcomes should be assessed in a larger study.

Clinical and immunophenotype correlating with response to immunotherapy in paediatric patients with primary liver carcinoma. A case series.

BACKGROUND: Paediatric hepatocellular carcinomas (HCC) traditionally arise in the context of a normal structural and functional liver and carry a dismal prognosis. While chemotherapy is the frontline standard, there is emerging interest in the study of immunotherapies for paediatric patients with relapsed/refractory disease. There is limited data to support whether immunotherapies will be of utility in this patient population. METHODS: Six paediatric patients (median age:16 years, range: 12-17 at the time of treatment) with advanced hepatocellular neosplams, either conventional hepatocellular or fibrolamellar carcinoma, were treated with immunotherapy. Patients were consented to institutional genomic profiling and biobanking protocols. Baseline samples and serial tissue samples, when available, were evaluated for somatic mutation rate, actionable gene mutations, and pan-immune bulk RNA expression profiling. Results were correlated with clinical course. FINDINGS: Three patients responded to checkpoint inhibition: one achieved a complete, durable response and the other two, prolonged stable disease. Three additional patients progressed. Diagnostic tissue from the complete responder demonstrated a higher relative mutational burden and robust immune infiltrate. Pre-treatment samples from the three responders demonstrated decreased expression of genes associated with T-cell dysfunction. INTERPRETATION: A subset of patients with primary paediatric hepatocellular tumours will respond to immunotherapy. Immunotherapies are currently under prospective study for relapsed/refractory liver tumours in paediatric patients. Results from this report support the prospective collection of serial serum and tissue samples which may further identify genomic and immunophenotypic patterns predictive of response. FUNDING: This work was supported by Philanthropic funds (Pan Mass Challenge, Team Angus and Team Perspective).

What Constitutes Quality of Life? Perspectives of Adolescents and Young Adults With Advanced Cancer.

BACKGROUND: Adolescents and young adults (AYAs) with advanced cancer identify maintaining a good quality of life (QoL) as a central goal of end-of-life care. QoL is a dynamic and subjective overarching concept that refers to an individual's relative satisfaction with their own life. Despite its importance to AYAs with advanced cancer, a patient-centered definition of QoL is lacking in this population. PATIENTS AND METHODS: This qualitative secondary analysis of semistructured interviews was conducted across 3 institutions and 1 online support community among AYA patients with advanced cancer, family caregivers, and health care providers who cared for living or recently deceased AYAs. Interviewees were asked about priorities in receipt of care. Interviews were transcribed using NVivo software for primary analysis, and previously coded excerpts were screened for references to QoL. Relevant excerpts were sorted into organizing domains. RESULTS: Participants included 23 AYA patients, 28 family caregivers, and 29 health care providers (including physicians, nurses, nurse practitioners, social workers, and psychologists). Four domains of QoL were identified: psychosocial and physical well-being, dignity, normalcy, and personal and family relationships. Within each domain there was agreement across AYAs, caregivers, and health care providers, with nuanced perspectives provided by AYAs of different ages. Personal and family relationships was the most frequently referenced domain of QoL among all participants. A common feature of each domain was that adaptation to current circumstances impacted perspectives on QoL. Patients valued active participation in the development of a care plan that supported these domains. CONCLUSIONS: AYAs with advanced cancer, their caregivers, and health care providers agree on several broad domains of QoL in this population. To provide high-quality, patient-centered care, care plans should integrate these domains to enable AYAs to maximize their QoL throughout their advanced cancer care.

Patient, Caregiver, and Clinician Perspectives on Core Components of Therapeutic Alliance for Adolescents and Young Adults With Advanced Cancer: A Qualitative Study.

Importance: The patient-clinician therapeutic alliance is an important aspect of high-quality cancer care. However, components of the therapeutic alliance in adolescents and young adults (AYAs, aged 12-39 years) with cancer have not been defined. Objective: To identify components of and barriers to the therapeutic alliance between AYAs, caregivers, and clinicians from the perspective of all key stakeholders. Design, Setting, and Participants: In this qualitative study, semistructured in-depth interviews with patients, caregivers, and clinicians were conducted from 2018 to 2021 with no additional follow-up, with content analysis of resulting transcripts. Participants were recruited from Dana-Farber Cancer Institute, Kaiser Permanente Northern California, Kaiser Permanente Southern California, and an online cancer support community (Cactus Cancer Society). Eligible participants were English- or Spanish-speaking. Eligible patients were aged 12 to 39 years with stage IV or recurrent cancer. Eligible caregivers cared for an AYA living with advanced cancer or one who had died within 5 years. Eligible clinicians routinely provided care for AYAs with cancer. Main Outcomes and Measures: Perspectives on therapeutic alliance. Results: Interviews were conducted with 80 participants: 23 were patients (48% were female; 78% were White), 28 were caregivers (82% were female; 50% were White), and 29 were clinicians (69% were female; 45% were White). The mean (SD) age of patients was 29 (7.3) years. Interviews identified 6 components of therapeutic alliance: (1) compassion; (2) sense of connection; (3) clinician presence; (4) information sharing; (5) shared goals; and (6) individualization of care. While some domains were represented in prior models of therapeutic alliance, a unique domain was identified related to the need for individualization of the approach to care for AYA patients and their caregivers. Interviews also identified potential barriers to building the therapeutic alliance specific to the AYA population, including managing discordant needs between patients and caregivers and communication challenges at the end of life. Conclusions and Relevance: This study identified core components and barriers to building therapeutic alliance in the AYA advanced cancer population from the perspective of all the key stakeholders in the relationship. A novel component highlighting the need for individualization was identified. This model enables a deeper understanding of how to build therapeutic alliance in the AYA advanced cancer population, which may guide clinician training and facilitate improved care for this vulnerable population.

A single-institution pediatric and young adult interventional oncology collaborative: Novel therapeutic options for relapsed/refractory solid tumors.

BACKGROUND: Pediatric interventional oncology (PIO) is a growing field intended to provide additional or alternative treatment options for pediatric patients with benign or malignant tumors. Large series of patients treated uniformly and subjected to rigorous endpoints for efficacy are not available. METHODS: We designed a collaborative initiative to capture data from pediatric patients with benign and malignant tumors who underwent a therapeutic interventional radiology procedure. Modified Response Evaluation Criteria in Solid Tumors (mRECIST) was utilized as a measure of radiologic response and data were collected regarding improvement in pain and functional endpoints. Cumulative incidence of progressive disease was calculated using both the treated site and the patient as the analytic unit. FINDINGS: Forty patients, 16 with malignant tumors and 24 with benign tumors, underwent a total of 88 procedures. Cryo- and radiofrequency ablation were the most frequently utilized techniques for both cohorts of patients. A complete or partial response, or prolonged disease stability, were achieved in approximately 40% of patients with malignant tumors and 60% of patients with benign tumors. No patients had progressive disease as their best response. Resolution of pain and improved mobility with return-to-baseline activity were demonstrated across patients from both cohorts. Only minor complications were experienced. INTERPRETATION: Interventional radiology-guided interventions can serve as an alternative or complementary approach to the treatment of benign and malignant tumors in pediatric patients. Prospective, multi-institutional trials are required to adequately study utility, treatment endpoints, and durability of response.

Current state of dietetic services for inflammatory bowel disease patients in New Zealand: an observational study.

AIM: Nutritional therapies for inflammatory bowel disease are increasingly recommended. This study aimed to gain insight from patients, dietitians and gastroenterologists into inflammatory bowel disease dietetic care in New Zealand. METHODS: Mixed-methods surveys were developed and then distributed online to patients with inflammatory bowel disease and dietitians and gastroenterologists that care for patients with inflammatory bowel disease. Quantitative survey data were analysed using nonparametric statistical tests. Qualitative survey data were analysed using thematic analysis. RESULTS: Responses were received from 406 inflammatory bowel disease patients, 79 dietitians and 40 gastroenterologists. Half of the patients (52%) had seen a dietitian for nutrition advice. Patients more likely to have seen a dietitian were/had: Crohn's disease (p = 0.001), previous bowel surgery (p < 0.001), younger (p < 0.001) or receiving biologic therapy (p = 0.005). Two-thirds (66%) of patients found the dietitian advice at least moderately useful. A common theme from patient comments was that dietitians needed better knowledge of inflammatory bowel disease. Almost all (97%) gastroenterologists reported that their inflammatory bowel disease patients ask about nutrition; 57% reported that there were inadequate dietitians to meet patient needs. Over 50% of dietitians saw inflammatory bowel disease patients infrequently and 39% were not confident that their knowledge of the nutritional management of inflammatory bowel disease was current. Dietitians desired greater links with the inflammatory bowel disease multidisciplinary team. CONCLUSION: Current inflammatory bowel disease dietetic services in New Zealand are inadequate. Standardised care, increased resourcing, dietitian training in inflammatory bowel disease, and stronger links with the multidisciplinary team are suggested to improve services.

Quality Indicators for Adolescents and Young Adults With Advanced Cancer: A Modified Delphi Process With Patients, Family Members, and Clinicians.

CONTEXT: Quality measures have been devised for end-of-life care of older adults with cancer, but are lacking for adolescents and young adults (AYAs). OBJECTIVE: We previously conducted interviews with AYAs, family caregivers, and clinicians to identify priority domains for high quality care of AYAs with advanced cancer. The goal of this study was to use a modified Delphi process to form consensus around the highest priority quality indicators. METHODS: A modified Delphi process was conducted with 10 AYAs with recurrent or metastatic cancer, 11 family caregivers, and 29 multidisciplinary clinicians, using small group web conferences. Participants were asked to rate the importance of each of 41 potential quality indicators, rank the 10 most important, and engage in discussion to reconcile differences. RESULTS: Of 41 initial indicators, 34 were rated as highly important (rating seven, eight, or nine on a nine-point scale) by >70% of participants. The panel was unable to reach consensus around the 10 most important indicators. Instead, participants recommended retaining a larger set of indicators to reflect potential for different priorities across the population, resulting in a final set of 32 indicators. Recommended indicators broadly encompassed attention to physical symptoms; quality of life; psychosocial, and spiritual care; communication and decision-making; relationships with clinicians; care and treatment; and independence. CONCLUSION: A patient- and family-centered process for quality indicator development led to strong endorsement of multiple potential indicators by Delphi participants. Further validation and refinement will be performed using a survey of bereaved family members.

Use of substances to cope during the COVID-19 pandemic among transgender and gender diverse adults.

Background: Studies have found changes in substance use during the COVID-19 pandemic in specific populations. Transgender and gender diverse (TGD) individuals have experienced greater distress compared to cisgender individuals during the pandemic; however, there is little research on substance use among TGD individuals during this sensitive time period.Objectives: The objective of this study is to examine distress from COVID-19 and coping via substance use including alcohol, cannabis, tobacco, and non-medical use of prescription drugs (NMUPD) among TGD adults.Method: An online survey assessing substance use, general psychiatric symptoms, and COVID-19 anxiety was completed by 342 TGD individuals (16.4% transfeminine, 19.6% transmasculine, 64.0% Gender Diverse) in June/July 2020. Chi-square and structural equation modeling (SEM) analyses examined the connections between distress, coping, and substance use.Results: Seventy-one percent of participants reported no changes in substance use since the start of the pandemic and 22% reported an increase in substance use. Increased substance use was associated with alcohol (p < .001), cannabis (p < .001), and combustible tobacco (p < .001) use in the prior three months. SEM showed significant direct effects between distress and substance use coping, substance use coping and recent drug use, and an indirect effect of distress on recent drug use through substance use coping (ß = .31, p = .001).Conclusion: Results highlight the risk of substance use to cope with COVID-19-related stress in a large sample of a minoritized population with mental health disparities. Transmasculine and gender diverse participants were especially likely to report using substances to cope.

Acute effects of flavored Black and mild cigars among young adult cigarette smokers.

The U.S. Food and Drug Administration proposed new product standards that would ban characterizing flavors (other than tobacco) in cigars. To inform this regulatory action, we compared physiological effects, use behavior, and subjective effects of four popular cigar flavors in cigar-naïve young adult cigarette smokers. Across five laboratory visits, participants (n = 25) used and evaluated own brand (OB) cigarettes or Black & Mild cigars (original, wine, apple, and cream flavors). Linear mixed models tested differences in saliva nicotine, exhaled carbon monoxide (CO), heart rate (HR), blood pressure (BP), puff topography, and subjective effects (p < .05). Compared to all cigars, OB resulted in higher nicotine boost (953 vs. < 300 ng/ml) and lower CO boost (4 vs. 8-9 ppm). Nicotine boost for original cigars (283 ng/ml) was significantly higher than wine (190 ng/ml). All products significantly increased HR/BP relative to baseline, but across time wine and apple cigars were associated with significantly lower HR than OB and BP effects varied. Relative to OB, participants took approximately 0.5 s longer puffs for all cigars and took significantly larger puffs (+ 21%-24%) of original, wine, and apple cigars. OB was rated more positively than all cigars, which had similar subjective effects. Wine cigars were disliked most and were less effective in reducing tobacco abstinence symptoms than OB; cream cigars were harsher and had stronger flavor intensity than original. The consistency in toxicant exposure, use behavior, and subjective effects across cigar flavors, including original, highlights the need for product standards to interpret characterizing flavors subject to prohibition broadly. (PsycInfo Database Record (c) 2023 APA, all rights reserved).

Evaluation of Nutritional Characteristics Reveals Similar Prevalence of Malnutrition in Patients with Ulcerative Colitis and Crohn's Disease.

BACKGROUND: Inflammatory bowel disease (IBD) is associated with an increased risk of malnutrition and sarcopenia. AIMS: To evaluate the nutritional status of patients with IBD and determine the threshold values of different parameters of nutritional assessment to identify malnutrition. METHODS: This was a single-centre cross-sectional analysis of adult patients with IBD [ulcerative colitis (UC) and Crohn's disease (CD)] who underwent anthropometry [body mass index (BMI), mid upper arm circumference (MUAC) and triceps-fold thickness (TSF)], body composition analysis and assessment for sarcopenia [hand-grip strength and skeletal muscle index (SMI) at L3 vertebral level)]. Age- and gender-matched healthy adults served as controls. Malnutrition was defined according to the European Society of Clinical Nutrition and Metabolism (ESPEN) criteria. RESULTS: A total of 406 patients [336 (82.76%) UC and 70 (17.24%) CD; mean age 40.56 ± 13.67 years; 215 (52.95%) males] with IBD and 100 healthy controls (mean age 38.69 ± 10.90 years; 56 (56%) males) were enrolled. The mean BMI, MUAC, TSF thickness, fat and lean mass, hand-grip strength, and SMI at L3 vertebral level were lower in patients with IBD compared to controls. The prevalence of malnutrition was similar in UC and CD [24.40% (n = 82) and 28.57% (n = 20), respectively (p = 0.46)]. Thresholds for fat mass in females (15.8 kg) and visceral fat index in males (0.26) were both sensitive and specific to detect malnutrition. The cutoff values of MUAC and TSF thickness to identify malnutrition were 23.25 cm and 25.25 cm, and 16.50 mm and 8.50 mm, in females and males, respectively. CONCLUSION: Malnutrition and sarcopenia were common in patients with IBD, with the prevalence being similar in patients with both UC and CD.

Multidisciplinary Perinatal Care in IBD.

BACKGROUND AND AIMS: Patients with inflammatory bowel disease [IBD] are often affected during their reproductive years and may have many perinatal queries that require the comprehensive perspectives of a multidisciplinary team [MDT]. The purpose of this topical review is to assess the scientific evidence and provide expert opinion related to nutritional, psychological and supportive care of women and their infants throughout the prenatal, antenatal and infant periods. METHODS: A consensus expert panel of a paediatrician, gastroenterologists, nurses and dietitians was convened by the European Crohn's and Colitis Organisation. This panel critically reviewed literature related to the non-medical management of patients with IBD during preconception, pregnancy, the postnatal period and the first years of the infant's life. Statements were developed using an e-Delphi process over two rounds and were confirmed when ≥80% of experts agreed with the statements. RESULTS: A total of 19 current practice positions were developed that cover the preconception period, pregnancy and lactation, and early-life exposures associated with risk of IBD. Development of the infant microbiome and its role in the immune system and topics including nutritional optimization, psychological support and education relating to early life were reviewed. CONCLUSIONS: Patients with IBD have unique nutritional and psychosocial needs that may affect fertility and pregnancy outcomes. The early-life environment of infants born to parents with IBD may be associated with subsequent development of IBD in offspring. An MDT is the optimal setting to support and counsel patients throughout the perinatal period.

"The simple life experiences that every other human gets": Desire for normalcy among adolescents and young adults with advanced cancer.

BACKGROUND: Adolescents and young adults (AYAs) with advanced cancer identify normalcy as an important component of quality end-of-life care. We sought to define domains of normalcy and identify ways in which clinicians facilitate or hinder normalcy during advanced cancer care. PROCEDURE: This was a secondary analysis of a qualitative study that aimed to identify priority domains for end-of-life care. Content analysis of semi-structured interviews among AYAs aged 12-39 years with advanced cancer, caregivers, and clinicians was used to evaluate transcripts. Coded excerpts were reviewed to identify themes related to normalcy. RESULTS: Participants included 23 AYAs with advanced cancer, 28 caregivers, and 29 clinicians. Participants identified five domains of normalcy including relationships, activities, career/school, milestones, and appearance. AYAs and caregivers identified that clinicians facilitate normalcy through exploration of these domains with AYAs, allowing flexibility in care plans, identification of short-term and long-term goals across normalcy domains, and recognizing losses of normalcy that occur during cancer care. CONCLUSIONS: AYAs with cancer experience multiple threats to normalcy during advanced cancer care. Clinicians can attend to normalcy and improve AYA quality of life by acknowledging these losses through ongoing discussions on how best to support domains of normalcy and by reinforcing AYA identities beyond a cancer diagnosis.

Improving Food-Related Quality of Life in Inflammatory Bowel Disease through a Novel Web Resource: A Feasibility Randomised Controlled Trial.

Food-related quality of life (FR-QoL) is impaired in inflammatory bowel disease (IBD) and education and support on food-related issues in IBD is needed. This feasibility trial aimed to investigate the effectiveness and acceptability of a web resource in enhancing FR-QoL in newly diagnosed IBD. Patients diagnosed with Crohn's disease or ulcerative colitis in the preceding 12 months, with an impaired FR-QoL, were recruited and randomised to either receive access to the web resource (covering IBD-specific diet concerns) or no access (control group) for 12 weeks, while receiving usual clinical care. FR-QoL, health-related quality of life, psychological outcomes, and clinical disease activity were assessed. Web resource usage was assessed, and patients' experiences of the web resource were investigated in semi-structured interviews. Of 81 patients screened, 50 participants were randomised, 30 to the web resource and 20 to control. FR-QoL increased more in the web resource (+11.7 SD 18.2) than control group (+1.4 SD 20.4) (p = 0.067), while IBD distress reduced in the web resource (-6.8 SD 26.6) and increased in the control group (+8.3 SD 25.5) (p = 0.052), albeit not statistically significantly. End of trial Crohn's disease clinical activity (PRO-2) was significantly lower in the web resource than control group (p = 0.046). Participants most frequently accessed web resource content discussing dietary management of gut symptoms and in semi-structured interviews, reported the website to contain relevant information. This feasibility study demonstrates potential effectiveness of the web resource on improving FR-QoL and psychological outcomes in IBD. An adequately powered effectiveness RCT is feasible to conduct and is now warranted. NCT03884686.

Dietary Nutrient Intake and Blood Micronutrient Status of Children with Crohn's Disease Compared with Their Shared-Home Environment, Healthy Siblings.

(1) The nutritional status of children with Crohn's disease (CD) is rarely described. This study aimed to assess the dietary intake and blood micronutrient status of children with CD compared with their healthy, shared-environment siblings. (2) Methods: This observational study included children with CD (cases) and their shared-environment siblings (controls). The dietary nutrient intake was assessed with a four-day food/beverage diary and was compared with the recommended daily intakes (RDI). Blood micronutrient concentrations were measured using laboratory methods. The nutritional analyses were completed through a multivariate analysis of variance between groups. Between-group comparisons of single-nutrients were assessed using a Mann−Whitney U-test. Chi-squared analyses compared the proportion of children who did not meet the RDI for each nutrient. The results were significant at 0.05. (3) Results: The dietary intake was similar for most nutrients, except the controls had a lower intake of vitamins A and E, copper, zinc, iron, and selenium (p < 0.05). Children using partial enteral nutrition had significantly higher intakes of many micronutrients. It was common for both groups to not meet the RDI's­more than 50% of cases for 9 nutrients and more than 50% of controls for 13 nutrients. (4) Conclusion: New Zealand children with CD and their shared-environment siblings did not meet the RDI for several micronutrients. Dietary education and/or micronutrient supplementation may be required.

Patient, Family, and Clinician Perspectives on Location of Death for Adolescents and Young Adults With Cancer.

PURPOSE: Adolescents and young adults (AYAs) with cancer have high rates of hospital deaths. It is not clear if this reflects their preferences or barriers to dying at home. METHODS: Between December 2018 and January 2021, we conducted in-depth interviews with AYAs (age 12-39 years) with stage IV or recurrent cancer, family caregivers including bereaved caregivers, and clinicians of AYAs with cancer. Patients were asked about their priorities for care including location of death, caregivers were asked what was most important in the care of their AYA family member, and clinicians were asked to reflect on priorities identified through caring for AYAs. Directed content analysis was applied to interview data, and themes regarding location of death were developed. RESULTS: Eighty individuals (23 AYAs, 28 caregivers, and 29 clinicians) participated in interviews. Most AYAs and caregivers preferred a home death. However, some AYAs and caregivers opted for a hospital death to alleviate caregiver burden or protect siblings from the perceived trauma of witnessing a home death. Lack of adequate services to manage intractable symptoms at home and insufficient caregiver support led some AYAs/caregivers to opt for hospital death despite a preference for home death. Participants acknowledged the value of hospice while also pointing out its limitations in attaining a home death. CONCLUSION: Although most AYAs prefer to die at home, this preference is not always achieved. Robust home-based services for effective symptom management and caregiver support are needed to close the gap between preferred and actual location of death for AYAs.

Food-Related Quality of Life in Children and Adolescents With Crohn's Disease.

BACKGROUND: Food-related quality of life (FRQoL) encompasses the psychosocial elements of eating and drinking. The FRQoL of children and adolescents with inflammatory bowel disease has not yet been assessed. This study aimed to evaluate the utility of the validated FR-Qol-29 instrument in children with Crohn's disease (CD). METHODS: Children diagnosed with CD, a shared home environment healthy sibling, and healthy control subjects 6 to 17 years of age were recruited to this single-center, prospective, cross-sectional study. Children or their parent or guardian completed the FR-QoL-29 instrument. Internal consistency was assessed by completing Cronbach's α. Construct validity was established by correlating the CD FR-QoL-29 sum scores with the Physician Global Assessment and Pediatric Crohn Disease Activity Index scores. The discriminant validity was analyzed using a 1-way analysis of variance, and a Spearman's correlation coefficient test was completed to identify any correlations associated with FRQoL. RESULTS: Sixty children or their parent or guardian completed the FR-QoL-29 instrument (10 children in each subgroup). The internal consistency was excellent (Cronbach's α = 0.938). The mean FR-QoL-29 sum scores were 94.3 ± 27.6 for CD, 107.6 ± 20 for siblings, and 113.7 ± 13.8 for control subjects (P = .005). Those with higher disease activity had worse FRQoL (Physician Global Assessment P = .021 and Pediatric Crohn Disease Activity Index P = .004). Inflammatory bowel disease FR-QoL-29 sum scores correlated with weight (P = .027), height (P = .035), body mass index (P = .023), and age (P = .015). CONCLUSIONS: FRQoL is impaired in children with CD. Healthy siblings also have poorer FRQoL than control subjects. Several clinical factors are associated with poorer FRQoL in children with CD including age and level of nutritional risk (weight, height, and body mass index). Further research is required validate these findings and to develop strategies for the prevention or treatment of impaired FRQoL in children with CD.

Food-related quality of life is impaired in children with Crohn's disease compared with their healthy, shared environment sibling and healthy control subjects. Several clinical factors are associated with poorer food-related quality of life in children with Crohn's disease including age and nutritional risk (weight, height, and body mass index).

Behavioral economic assessment of abuse liability for Black & Mild cigar flavors among young adults.

Cigars are available in a variety of flavors that may impact uptake and use, but little is known about how different flavors affect abuse liability. This study used 3 behavioral economic tasks to examine abuse liability of Black & Mild cigars differing in flavor among young adult cigarette smokers. Participants were 25 cigar-naïve young adults (aged 18-25 years) who smoked ≥ 5 cigarettes/day. In 5 Latin square-ordered laboratory visits, participants completed 3 abuse liability tasks (drug purchase task, cross-price purchase task, and multiple-choice procedure) for each of 4 cigar flavors (original, cream, wine, or apple) and own-brand cigarettes. In the drug purchase task, relative to own-brand cigarettes, all cigar flavors were associated with lower abuse liability using most measures (intensity, breakpoint, maximum total tobacco expenditure for 1 day [ps < .05]), although only wine-flavored cigars scored significantly lower using 1 measure (price at maximum total tobacco expenditure for 1 day). When cigars and cigarettes were available concurrently in the cross-price purchase task, all cigar flavors functioned as substitutes for cigarettes. Using the multiple-choice procedure, crossover points for wine- (mean = $0.61) and apple-flavored cigars (mean = $0.71) were significantly lower than own-brand cigarettes (mean = $0.86) and original-flavored cigars (mean = $1.00); no significant differences existed between own-brand cigarettes and original-flavored cigars. Thus, whereas abuse liability may be highest for participants' own-brand cigarette, young adult smokers may be willing to use flavored cigars. Furthermore, abuse liability varies by cigar flavor, with original- and cream-flavored cigars appearing to have the highest abuse liability. Characterizing flavors and flavor additives in cigars represent an important tobacco regulatory target. (PsycInfo Database Record (c) 2022 APA, all rights reserved).

Content appealing to youth and spend characteristics of electronic cigarette video advertisements.

BACKGROUND: The US electronic cigarette (ECIG) market and use behavior continues to rise, warranting investigation of ECIG advertisement (ad) content within media channels frequented by youth including internet and television (TV). In order to inform potential policy regulations, this content analysis sought to assess the prevalence of youth-appealing content and spend characteristics among ECIG video ads. METHODS: Between 2015 and 2016, 46 ECIG video ads were identified using an ad-tracking firm and were coded using the Content Appealing to Youth (CAY) index. Bivariate analyses and analysis of variance were used to compare CAY indices between online and TV ads and by ECIG brands. Ad-specific spend and source information, including website/TV program targeted were examined descriptively. RESULTS: Common youth-appealing features included use of animation (56.5%) and content related to positive sensations (52.2%), promoting mood (34.8%), individuality/freedom (23.9%) and addiction (19.6%). Features not associated with youth appeal also were prevalent. Few differences were observed by media source or ECIG brand. TV ads accounted for the largest spend, and Vuse was the top spending brand. Websites/TV programs were diverse. CONCLUSIONS: Findings support regulatory efforts to restrict the use of youth-appealing content within ECIG ads as well as the reduction of media sources available for ECIG marketing.

Belzutifan, a Potent HIF2α Inhibitor, in the Pacak-Zhuang Syndrome.

The integration of genomic testing into clinical care enables the use of individualized approaches to the management of rare diseases. We describe the use of belzutifan, a potent and selective small-molecule inhibitor of the protein hypoxia-inducible factor 2α (HIF2α), in a patient with polycythemia and multiple paragangliomas (the Pacak-Zhuang syndrome). The syndrome was caused in this patient by somatic mosaicism for an activating mutation in EPAS1. Treatment with belzutifan led to a rapid and sustained tumor response along with resolution of hypertension, headaches, and long-standing polycythemia. This case shows the application of a targeted therapy for the treatment of a patient with a rare tumor-predisposition syndrome. (Funded by the Morin Family Fund for Pediatric Cancer and Alex's Lemonade Stand Foundation.).

Patient, Family, and Clinician Perspectives on End-of-Life Care Quality Domains and Candidate Indicators for Adolescents and Young Adults With Cancer.

Importance: End-of-life care quality indicators specific to adolescents and young adults (AYAs) aged 12 to 39 years with cancer have not been developed. Objective: To identify priority domains for end-of-life care from the perspectives of AYAs, family caregivers, and clinicians, and to propose candidate quality indicators reflecting priorities. Design, Setting, and Participants: This qualitative study was conducted from December 6, 2018, to January 5, 2021, with no additional follow-up. In-depth interviews were conducted with patients, family caregivers, and clinicians and included a content analysis of resulting transcripts. A multidisciplinary advisory group translated priorities into proposed quality indicators. Interviews were conducted at the Dana-Farber Cancer Institute, Kaiser Permanente Northern California, Kaiser Permanente Southern California, and an AYA cancer support community (lacunaloft.org). Participants included 23 AYAs, 28 caregivers, and 29 clinicians. Exposure: Stage IV or recurrent cancer. Main Outcomes and Measures: Care priorities. Results: Interviews were conducted with 23 patients (mean [SD] age, 29.3 [7.3] years; 12 men [52%]; 18 White participants [78%]), 28 family caregivers (23 women [82%]; 14 White participants [50%]), and 29 clinicians (20 women [69%]; 13 White participants [45%]). Caregivers included 22 parents (79%), 5 spouses or partners (18%), and 1 other family member (4%); the 29 clinicians included 15 physicians (52%), 6 nurses or nurse practitioners (21%), and 8 social workers or psychologists (28%). Interviews identified 7 end-of-life priority domains: attention to physical symptoms, attention to quality of life, psychosocial and spiritual care, communication and decision-making, relationships with clinicians, care and treatment, and independence. Themes were consistent across the AYA age range and participant type. Although some domains were represented in quality indicators developed for adults, unique domains were identified, as well as AYA-specific manifestations of existing domains. For example, quality of life included global quality of life; attainment of life goals, legacy, and meaning; support of personal relationships; and normalcy. Within communication and decision-making, domains included communication early in the disease course, addressing prognosis and what to expect at the end of life, and opportunity for AYAs to hold desired roles in decision-making. Care and treatment domains relevant to cancer therapy, use of life-prolonging measures, and location of death emphasized the need for preference sensitivity rather than a standard path. This finding differs from existing adult indicators that propose that late-life chemotherapy, intensive measures, and hospital death should be rare. Conclusions and Relevance: The findings of this qualitative study suggest that AYAs with cancer have priorities for care at the end of life that are not fully encompassed in existing indicators for adults. Use of new indicators for this young population may better reflect patient- and family-centered experiences of quality care.