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.).

Factors Associated with College Students' Attitudes Toward Telehealth for Primary Care.

Introduction: Establishing routine primary care visits helps to prevent serious health issues. College students are less likely than the general population to have a regular primary care provider and engage in routine health visits. Recent research provides evidence that telehealth is a convenient alternative to in-person primary care and that college students are comfortable using this technology, suggesting that telehealth has the potential to mitigate this disparity. As attitudes toward telehealth are one critical precursor to behavioral intention and actual utilization of telehealth, the goal of this study was to investigate which factors predict positive or negative attitudes toward telehealth. Methods: Data for this study were collected from a sample of 621 college students at a large southeastern university between September 19, 2022 and December 19, 2022. Results: The study found that college students who reported more trust in physicians, less medical mistrust, and less discrimination in health care settings reported more positive attitudes toward telehealth. Conclusions: These findings suggest that health care providers' skills in delivering patient-centered culturally informed care and building trust and rapport with patients might promote more positive attitudes toward telehealth and, potentially, greater overall utilization of health care services (including both telehealth and in-person services) among college students. This study lays the foundation for future research to examine psychological mechanisms underlying individuals' utilization of telehealth.

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.

"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.

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.

British Dietetic Association consensus guidelines on the nutritional assessment and dietary management of patients with inflammatory bowel disease.

BACKGROUND: Despite increased awareness of diet and nutrition being integral to the management of patients with inflammatory bowel disease (IBD), there are gaps in the knowledge of IBD healthcare providers regarding nutrition. Furthermore, high quality evidence on nutritional assessment and dietary management of IBD is limited. A Delphi consensus from a panel of experts allows for best-practice guidelines to be developed, especially where high quality evidence is limited. The aim was to develop guidelines for the nutritional assessment and dietary management of IBD using an eDelphi online consensus agreement platform. METHODS: Seventeen research topics related to IBD and nutrition were systematically reviewed. Searches in Cochrane, Embase®, Medline® and Scopus® electronic databases were performed. GRADE was used to develop recommendations. Experts from the IBD community (healthcare professionals and patients with IBD) were invited to vote anonymously on the recommendations in a custom-built online platform. Three rounds of voting were carried out with updated iterations of the recommendations and evaluative text based on feedback from the previous round. RESULTS: From 23,824 non-duplicated papers, 167 were critically appraised. Fifty-five participants completed three rounds of voting and 14 GRADE statements and 42 practice statements achieved 80% consensus. Comprehensive guidance related to nutrition assessment, nutrition screening and dietary management is provided. CONCLUSIONS: Guidelines on the nutritional assessment and dietary management of IBD have been developed using evidence-based consensus to improve equality of care. The statements and practice statements developed demonstrate the level of agreement and the quality and strength of the guidelines.

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.

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.

Colchicine toxicity in renal patients - Are we paying attention?

Colchicine is an approved agent in the management and prophylaxis of gout and familial Mediterranean fever but its therapeutic value is limited by its narrow therapeutic index. Multisystem toxicity is uncommonly reported; and is often associated with renal impairment and/or specific drug interactions. We report two cases of colchicine toxicity marked by severe neuromyopathy in a diabetic with stage 4 chronic kidney disease (CKD) and a renal transplant recipient. Both patients presented with diarrhea, acute on chronic kidney injury and progressive muscle weakness while on colchicine for several weeks or longer. In addition to kidney disease, risk factors for colchicine toxicity included maintenance therapy with simvastatin in the first patient and cyclosporine in the second. Creatine phosphokinase (CPK) was elevated in both cases at presentation and neurophysiologic studies showed a pattern of severe myopathy with axonal sensorimotor neuropathy. The first patient recovered from neurological weakness in a few weeks, but the second patient suffered an extraordinarily protracted and severe neuromuscular disability for a year. The two cases reinforce the need for extra vigilance in prescribing and monitoring colchicine therapy in renal patients with specific attention to drug interactions known to increase the risk of toxicity, thus avoiding such combinations in patients with renal impairment.

Correlation of knee and hindfoot deformities in advanced knee OA: compensatory hindfoot alignment and where it occurs.

BACKGROUND: Many patients undergoing TKA have both knee and ankle pathology, and it seems likely that some compensatory changes occur at each joint in response to deformity at the other. However, it is not fully understood how the foot and ankle compensate for a given varus or valgus deformity of the knee. QUESTIONS/PURPOSES: (1) What is the compensatory hindfoot alignment in patients with end-stage osteoarthritis who undergo total knee arthroplasty (TKA)? (2) Where in the hindfoot does the compensation occur? METHODS: Between January 1, 2005, and December 31, 2009, one surgeon (JJC) obtained full-length radiographs on all patients undergoing primary TKA (N=518) as part of routine practice; patients were analyzed for the current study and after meeting inclusion criteria, a total of 401 knees in 324 patients were reviewed for this analysis. Preoperative standing long-leg AP radiographs and Saltzman hindfoot views were analyzed for the following measurements: mechanical axis angle, Saltzman hindfoot alignment and angle, anatomic lateral distal tibial angle, and the ankle line convergence angle. Statistical analysis included two-tailed Pearson correlations and linear regression models. Intraobserver and interobserver intraclass coefficients for the measurements considered were evaluated and all were excellent (in excess of 0.8). RESULTS: As the mechanical axis angle becomes either more varus or valgus, the hindfoot will subsequently orient in more valgus or varus position, respectively. For every degree increase in the valgus mechanical axis angle, the hindfoot shifts into varus by -0.43° (95% confidence interval [CI], -0.76° to -0.1°; r=-0.302, p=0.0012). For every degree increase in the varus mechanical axis angle, the hindfoot shifts into valgus by -0.49° (95% CI, -0.67° to -0.31°; r=-0.347, p<0.0001). In addition, the subtalar joint had a strong positive correlation (r=0.848, r2=0.72, p<0.0001) with the Saltzman hindfoot angle, whereas the anatomic lateral distal tibial angle (r=0.450, r2=0.20, p<0.0001) and the ankle line convergence angle (r=0.319, r2=0.10, p<0.0001) had a moderate positive correlation. The coefficient of determination (r2) shows that 72% of the variance in the overall hindfoot angle can be explained by changes in the subtalar joint orientation. CONCLUSIONS: These findings have implications for treating patients with both knee and foot/ankle problems. For example, a patient with varus arthritis of the knee should be examined for fixed hindfoot valgus deformity. The concern is that patients undergoing TKA, who also present with a stiff subtalar joint, may have exacerbated, post-TKA foot/ankle pain or disability or malalignment of the lower extremity mechanical axis as a result of the inability of the subtalar joint to reorient itself after knee realignment. A prospective study is underway to confirm this speculation. LEVEL OF EVIDENCE: Level III, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

A systematic review and meta-analysis of the dietary fiber menu provision and consumption for older adults living in residential care facilities.

BACKGROUND: Older adults living in residential care facilities are commonly given laxatives to treat constipation; however, these may not always provide full relief, and side effects include diarrhea. Dietary fiber effectively prevents constipation, and international guidelines recommend 25 g/d for optimal laxation. Older adults in residential care rely on the facility menu to provide their nutritional requirements, including adequate dietary fiber. Little is known about how much dietary fiber is provided and consumed. OBJECTIVES: We aimed to determine the provision and consumption of dietary fiber for older adults living in residential care facilities. METHODS: We systematically searched available literature for studies reporting the analysis of residential care menus and meals consumed by residents aged over 65 y. A meta-analysis was performed on the studies that provided the mean amount of dietary fiber provided and consumed by residents. A random effect model was applied due to the heterogeneity of study methodologies. RESULTS: The literature search yielded 4406 publications, but only 28 studies were eligible for our meta-analysis. The study sample comprised 4817 residents. The mean amount of fiber provided to residents was 21.4 g/d [standard error (SE): 1.2; 95% confidence interval: 18.8, 24.2 g/d], the mean amount of fiber consumed by residents was 15.8 g/d (SE: 0.6; 95% confidence interval: 14.7, 16.9 g/d). CONCLUSIONS: Older adults living in care facilities are provided with dietary fiber below the recommended guidelines. Compounding this is that residents consume much less than what is provided and do not meet the recommendations for dietary fiber consumption. There is scope to improve dietary fiber provision, promote consumption to residents to aid laxation, and potentially reduce laxative use and the unwanted side effects of diarrhea. This trial was registered at PROSPERO as CRD42023427265.

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.

Effects of Defatted Rice Bran-Fortified Bread on the Gut Microbiota Composition of Healthy Adults With Low Dietary Fiber Intake: Protocol for a Crossover Randomized Controlled Trial.

BACKGROUND: Inadequate dietary fiber (DF) intake is associated with several human diseases. Bread is commonly consumed, and its DF content can be increased by incorporating defatted rice bran (DRB). OBJECTIVE: This first human study on DRB-fortified bread primarily aims to assess the effect of DRB-fortified bread on the relative abundance of a composite of key microbial genera and species in fecal samples. Secondary outcomes include clinical (cardiovascular risk profile), patient-reported (daily bread consumption and bowel movement, gut comfort, general well-being, and total DF intake), biological (fecal microbiota gene abundances, and fecal and plasma metabolites), and physiome (whole-gut and regional transit time and gas fermentation profiles) outcomes in healthy adults with low DF intake. METHODS: This is a 2-armed, placebo-controlled, double-blinded, crossover randomized controlled trial. The study duration is 14 weeks: 2 weeks of lead-in, 4 weeks of intervention per phase, 2 weeks of washout, and 2 weeks of follow-up. Overall, 60 healthy adults with low DF intake (<18 g [female individuals] or <22 g [male individuals] per day) were recruited in Christchurch, New Zealand, between June and December 2022. Randomly assigned participants consumed 3 (female individuals) or 4 (male individuals) slices of DRB-fortified bread per day and then placebo bread, and vice versa. The DRB-fortified bread provided 8 g (female individuals) or 10.6 g (male individuals) of total DF, whereas the placebo (a matched commercial white toast bread) provided 2.7 g (female individuals) or 3.6 g (male individuals) of total DF. Before and after each intervention phase, participants provided fecal and blood samples to assess biological responses; completed a 3-day food diary to assess usual intakes and web-based questionnaires to assess gut comfort, general and mental well-being, daily bread intake, and bowel movement via an app; underwent anthropometry and blood pressure measurements; and drank blue food dye to assess whole-gut transit time. Additionally, 25% (15/60) of the participants ingested Atmo gas-sensing capsules to assess colonic gas fermentation profile and whole-gut and regional transit time. Mean differences from baseline will be compared between the DRB and placebo groups, as well as within groups (after the intervention vs baseline). For metabolome analyses, comparisons will be made within and between groups using postintervention values. RESULTS: Preliminary analysis included 56 participants (n=33, 59% female; n=23, 41% male). Due to the large dataset, data analysis was planned to be fully completed by the last quarter of 2024, with full results expected to be published in peer-reviewed journals by the end of 2024. CONCLUSIONS: This first human study offers insights into the prospect of consuming DRB-fortified bread to effectively modulate health-promoting gut microbes, their metabolism, and DF intake in healthy adults with low DF intake. TRIAL REGISTRATION: Australian New Zealand Clinical Trials Registry ACTRN12622000884707; https://www.anzctr.org.au/Trial/Registration/TrialReview.aspx?id=383814. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/59227.

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).

Malnutrition screening tool use in a New Zealand hospital: Reliability and rates of malnutrition screening on admission.

AIMS: This research aimed to assess the rate and reliability of routine nurse-completed malnutrition screening and report the prevalence of malnutrition risk on admission to Christchurch Hospital. METHODS: Student dietitians administered the Malnutrition Screening Tool to patients in three speciality wards within 48 h of admission. Student dietitians' Malnutrition Screening Tool scores were compared against documented nurse-completed Malnutrition Screening Tool scores. Data were analysed using descriptive statistics, Fisher's exact test, and Cohen's kappa tests (interrater reliability ⱪ). A p-value <0.05 was considered statistically significant. RESULTS: Student dietitians, using the Malnutrition Screening Tool, screened 360 (96%) of 377 eligible patients while nurses screened 332 (88%) patients. Student dietitians and nurses screened 119 patients (33%) and 63 patients (18%) respectively at risk of malnutrition. There was fair agreement (ⱪ = 0.311) in Malnutrition Screening Tool total scores between nurses and student dietitians. There was a significant difference in the proportion of patients screened at risk of malnutrition between nurses and student dietitians (p < 0.0001). CONCLUSION: Malnutrition risk remains high in acute care settings. Nurses were less likely to screen patients as at risk of malnutrition than student dietitians. Regular support and training in using Malnutrition Screening Tool may help improve the rate and reliability of routine malnutrition screening.

Development and validation of an inflammatory bowel disease nutrition self-screening tool (IBD-NST) for digital use.

Background and aim: The identification of, and timely intervention for, patients with impaired nutritional status may reduce inflammatory bowel disease (IBD) complications. This study aimed to develop and validate an IBD-specific nutrition self-screening tool (IBD-NST) that identifies patients at nutrition risk. Methods: An expert IBD panel was consulted to support development of an IBD-NST. The tool was assessed in different cohorts of patients attending IBD outpatient clinics for face, content and convergent validity and repeat reliability. The tool was compared with (i) the malnutrition universal screening tool to assess face validity and (ii) subjective global assessment (SGA), hand-grip strength (HGS) and mid-arm muscle circumference to assess convergent validity. Tool content was informed by agreement between assessment tools, sensitivity analysis and chi-squared tests. The IBD-NST was completed electronically twice, 1 week apart to assess repeat reliability using observed agreement and kappa statistic. Statistical significance assumed at p < 0.05. Results: In total, 282 IBD patients (175 with Crohn's disease) were recruited to validate the IBD-NST. The final validated IBD-NST includes body mass index (BMI), weight loss and IBD-specific nutrition-focussed questions which were acceptable to patients. It identified patients at risk of malnutrition, moderately or severely malnourished patients and patients at nutritional risk. The IBD-NST identified 54/179 (30%) patients at moderate or high nutrition risk and had excellent repeat reliability in 85 patients [r = 0.77 (95% CI 0.669 to 0.746)]. Conclusion: The IBD-NST is a self-screening tool, validated for use as either a paper or e-health version, that identifies patients at nutrition risk who are likely to benefit from dietetic assessment and intervention. Furthermore, patients with IBD symptoms who are concerned about their dietary intake can potentially access dietetic care more easily therefore encouraging greater self-management of IBD-related symptoms. The routine use of the IBD-NST as a self-screening tool would enable patient-led care in the outpatient setting and may facilitate timely access to dietetic care.

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.

Trans broken arm syndrome: A mixed-methods exploration of gender-related medical misattribution and invasive questioning.

RATIONALE: Gender-related medical misattribution and invasive questioning (GRMMIQ), colloquially known as "trans broken arm syndrome," is a form of medical discrimination faced by transgender and gender diverse (TGD) patients wherein a provider incorrectly assumes that a medical condition results from a patient's gender identity or medical transition. This phenomenon may take one of two forms: (1) the incorrect and explicit misattribution of gender identity or medical transition as being the cause of an acute complaint, or (2) invasive and unnecessary questions regarding a patient's gender identity or gender transition status. OBJECTIVE: Using mixed-methods procedures, this study aims to explore the incidence, some common correlates, and manifestations of GRMMIQ. METHODS: American TGD participants (N = 147), recruited through an online recruitment platform, completed questions assessing their experiences in the healthcare system including lifetime incidence of GRMMIQ, outness to healthcare providers, and additional experiences of gender-related discrimination in a medical setting. Participants who indicated experiences of GRMMIQ were asked open-ended questions about one such experience. RESULTS: Nearly one-third of participants reported experiencing GRMMIQ. Experiences were associated with outness to acute care providers and other types of gender-related discrimination in healthcare settings. Analysis of qualitative data revealed four primary themes: (1) assumptions of disordered thinking and being, (2) hyperfocus on aspects of medical transition, (3) cultural ignorance and incompetence, and (4) dismissiveness of the patient. CONCLUSION: Together, these results enhance the understanding of an underexplored aspect of medical discrimination faced by TGD individuals while highlighting commonalities across different experiences.

Exclusive Enteral Nutrition for the Treatment of Pediatric Crohn's Disease: The Patient Perspective.

Crohn's disease (CD) is a chronic, incurable and relapsing disease involving any part of the gastrointestinal tract and exclusive enteral nutrition (EEN) is first-line therapy. Few studies have examined the patient experience of EEN. The aim of this study was to assess the child' s experiences of EEN, to identify problematic themes and understand the child's mindset. Children with CD who previously completed EEN were recruited to complete a survey. All data were analyzed using Microsoft Excel and reported as N (%). Forty-four children (mean age 11.3 years) consented to participate. Sixty-eight percent of children reported limited formula flavors as the most challenging aspect and 68% of children identified 'support' to be important. This study highlights the psychological impact of chronic disease and its therapies on children. Providing adequate support is essential to insure EEN is successful. Further studies are required to determine psychological support strategies for children taking EEN.