Nutrition Management in Children Less than 5 Years of Age with Glycogen Storage Disease Type I: Survey Results.

Background: Nutrition management for GSD Type I (GSDI; OMIM #232200, 232220) is complex, with the goal being to maintain euglycemia while minimizing metabolic derangements. Management guidelines were published in 2002 and 2014. However, there is limited information on the nuances of nutrition management and the unique feeding challenges of children. Methods: A REDCap survey focusing on staffing and current practices in the nutrition management of children with GSD I who were <5 years of age was sent to the metabolic dietitian's listserv and GMDI membership in 8/2023. Results: There were 21 North American respondents. In 17/21 clinics (81%), Prosobee® was the primary choice for infant formula. Dietitians used different methods to determine hourly glucose needs. Fasting recommendations ranged from 1 to 3 h, and the use of nighttime continuous feeding was common. Cornstarch was started between 6 and 12 months of age. Most clinics did not use Glycosade® for children <5 years of age. Oral motor dysfunction, gagging, and lack of interest in food were common. Continuous glucose monitoring (CGM) devices were recommended in 20 clinics (95%). Most clinics followed patients on an outpatient basis. All clinics provided a hypoglycemia management plan; however, there was wide variability in practice. Conclusion: This survey highlights the variability in the care of individuals <5 years of age with GSD I. Updated guidelines are needed to help address the unique nutrition challenges in this age group.

Increased Intrauterine Transfusion Blood Volume Needed to Correct Fetal Anemia due to Placental Chorioangioma.

INTRODUCTION: To compare the blood volume transfused for fetal anemia in cases of placental chorioangioma versus red blood cell (RBC) alloimmunization in patients matched for gestational age (GA) and hydrops. METHODS: Study patients had intrauterine transfusions and were obtained from 3 sources: group (1) placental chorioangioma patients treated at our center (2016-2023); group (2) placental chorioangioma patients reported in the medical literature; and group (3) RBC alloimmunization patients treated for fetal anemia at our center (2016-2023) matched (2:1) to patients in groups 1 and 2 by GA at procedure and presence of hydrops. The expected volume (cc) of transfusion was calculated for all patients based on a formula commonly used for fetal anemia in cases of RBC alloimmunization that includes the GA at procedure, pre-transfusion hemoglobin, donor hemoglobin, and target hemoglobin. The ratio of the volume actually transfused to the expected volume was calculated for each group and for groups 1 and 2 combined; comparisons were made using nonparametric testing. RESULTS: By group, the patients studied included: (1) 7 treated chorioangioma patients, (2) 8 chorioangioma patients described in the literature, and (3) 30 matched RBC alloimmunization patients. The median (range) GA at procedure for groups 1, 2, and 3 was 29.6 (22.7-32.7), 27.0 (24.0-30.0), 28.4 (22.7-34.3) weeks. The median pre-procedure hemoglobin for each group was 8.5 (5.8-12.5), 6.7 (5.6-12.0), and 5.3 (2.2-10.7) g/dL, and the median post-procedure hemoglobin for each group was 12.9 (11.5-14.0), 12.7 (9.6-14.7), and 13.6 (8.0-15.7) g/dL. The median (range) ratio of the actual to the expected volume transfused for each group was 2.50 (1.79-8.33), 1.64 (1.11-3.85), and 1.10 (0.69-1.86) (p < 0.001). When groups 1 and 2 were combined, the median ratio was 1.89 (1.11-8.33), which remained statistically significant when compared to group 3 (p < 0.001). CONCLUSION: Intrauterine transfusion for fetal anemia in cases of large placental chorioangiomas appeared to require nearly twice the blood volume that was normally anticipated for cases of RBC alloimmunization, although the actual amount transfused varied widely.

Achieving gender equity in academic psychiatry - barriers to involvement and solutions for success.

OBJECTIVE: Women face considerable barriers in pursuing careers in academic psychiatry. METHODS: A group of Australian and New Zealand academic women psychiatrists convened in September 2022 to identify and propose solutions to increase opportunities for women in academic psychiatry. RESULTS: Limiting factors were identified in pathways to academia including financial support, engagement and coordination between academia and clinical services, and flexible working conditions. Gender biases and the risk of burnout were additional and fundamental barriers. Potential solutions include offering advanced training certificates to enable trainees to commence a PhD and Fellowship contemporaneously; improved financial support; expanding opportunities for research involvement; establishing mentoring opportunities and communities of practice; and strategies to enhance safety at work and redress gender bias and imbalance in academia. CONCLUSIONS: Support for women in research careers will decrease gender disparity in academic psychiatry and may decrease problematic gender bias in research. Fellows and trainees, the RANZCP, universities, research institutes, governments, industry and health services should collaborate to develop and implement policies supporting changes in working conditions and training. Facilitating the entry and retention of women to careers in academic psychiatry requires mentoring and development of a community of practice to provide and enable support, role modelling, and inspiration.

Development of the Children's ACuTe UltraSound (CACTUS) point-of-care ultrasound (POCUS)-accredited training in the UK: a descriptive study.

Point-of-care ultrasound (POCUS) is an established, evidence-supported tool that can be used in neonatal and paediatric medicine, offering clinicians immediate diagnostic insights, assessment of interventions and improved safety profiles and success rate of various procedures. Its effective use requires an established education programme, governance and standardisation to ensure competence in this skill. While adult clinical practice has established POCUS training protocols, this had not been replicated in paediatrics. This article describes the development and launch of the UK's inaugural accredited paediatric-specific POCUS curriculum and training pathway: the 'Children's ACuTe UltraSound' course, addressing this significant gap in paediatric healthcare education and describing the training delivered and available for paediatricians and allied health professionals working with children.

Nurturing Attentiveness: A Naturalistic Observation Study of Personal Care Interactions Between People With Advanced Dementia and Their Caregivers.

BACKGROUND AND OBJECTIVES: Personal care interactions can provide vital opportunities for caregivers to engage with a person living with advanced dementia. However, interactions may also be a contentious experience, what makes this so is not fully understood. We aimed to examine features of personal care interactions between caregivers and people with advanced dementia to understand how care may be improved. RESEARCH DESIGN AND METHODS: This was a naturalistic observation study using one-off video-recorded observations of personal care interactions between 14 people with advanced dementia and 12 caregivers (n = 7 care-home staff, n = 5 family carers) in the United Kingdom (total observation time 03:01:52). Observations were analyzed with observational video coding to determine the frequency of actions of people with dementia and qualitative content analysis for in-depth examination. RESULTS: Refusals of care were present in 32% of video sections. Active engagement of people with dementia was observed in 66% of sections. Rare contentious interactional components were characterized by the person with dementia appearing to show uneasiness and caregivers being flustered and uncertain. However, caregivers typically emanated a nurturing attentiveness, were attuned to the person, and skilled in seamlessly supporting them through care activities. DISCUSSION AND IMPLICATIONS: Findings draw on real-world empirical evidence to reinvigorate the notion of person-centeredness in dementia care. The findings provide much needed insight into practical ways to improve care interactions for people with advanced dementia and enhance their personhood. Appropriate training/guidance for caregivers could support positive personal care experiences for both the person with dementia and caregiver.

Ethics rounds in the ambulance service: a qualitative evaluation.

BACKGROUND: It is a common ethical challenge for ambulance clinicians to care for patients with impaired decision-making capacities while assessing and determining the degree of decision-making ability and considering ethical values. Ambulance clinicians' ethical competence seems to be increasingly important in coping with such varied ethical dilemmas. Ethics rounds is a model designed to promote the development of ethical competence among clinicians. While standard in other contexts, to the best of our knowledge, it has not been applied within the ambulance service context. Thus, the aim of this study was to describe ambulance clinicians' experiences of participating in ethics rounds. METHODS: This was a qualitative descriptive study, evaluating an intervention. Data were collected through sixteen interviews with ambulance clinicians who had participated in an intervention involving ethics rounds. The analysis was performed by use of content analysis. RESULTS: Two themes describe the participants' experiences: (1) Reflecting freely within a given framework, and (2) Being surprised by new insights. The following categories form the basis of the themes; 1a) Gentle guidance by the facilitator, 1b) A comprehensible structure, 2a) New awareness in the face of ethical problems, and 2b) Shared learning through dialogue. CONCLUSION: Incorporating structured ethics rounds seems to create a continuous development in ethical competence that may improve the quality of care in the ambulance service. Structured guidance and facilitated group reflections offer ambulance clinicians opportunities for both personal and professional development. An important prerequisite for the development of ethical competence is a well-educated facilitator. Consequently, this type of ethics rounds may be considered a useful pedagogical model for the development of ethical competence in the ambulance service.

Predictors of severe and recurrent adult anaphylaxis, and gaps in the cascade of care: a retrospective, single-centre study 2009-2018.

BACKGROUND: Anaphylaxis is a severe, potentially fatal, systemic allergic reaction. Understanding predictors of recurrent and severe anaphylaxis in adults, and identifying gaps in ongoing anaphylaxis care, is needed to minimise its impact. AIMS: To evaluate the risk factors in adults with severe and recurrent anaphylaxis presentations and to evaluate the management of patients in regard to the recommended cascade of care. METHODS: We completed a retrospective audit of adults with confirmed anaphylaxis who presented to an inner-city emergency department from 1 January 2009 through 31 December 2018. Data recorded included demographics, background history, medication use, severity, co-factors, triggers, management, discharge disposition and referral for follow-up. Data were managed in REDCap and analysed using Stata. Associations were assessed through odds ratios (ORs) and t tests. RESULTS: Six hundred sixteen individuals had 689 episodes of anaphylaxis over the audit period. Age over 65 (OR: 5.4 (95% confidence interval, CI: 2.3-13.2), P < 0.0001) and history of asthma (OR: 1.6 (95% CI: 1.03-2.5), P = 0.03) were independent risk factors for severe anaphylaxis. History of food allergy (P < 0.001) and food as the trigger were associated with recurrent presentations (OR: 2.1, 95% CI: 1.1-3.9, P = 0.01). Only 19% of patients met the recommended cascade of care, with post-adrenaline monitoring and recommending follow-up with an allergy specialist demonstrating the largest gaps. There were increased presentations with time but no difference in triggers or severity. CONCLUSIONS: Increased age and asthma were identified as risk factors for severe presentations. History of food allergy was a risk factor for recurrent presentations. Further research is needed on the gaps in care for adults with anaphylaxis to identify the reasons why, so we can better care for these patients.

Effects of childhood trauma on sleep quality and stress-related variables in adulthood: evidence from two multilevel studies.

Childhood trauma has been found to have serious negative consequences for mental and physical health. However, the precise mechanisms through which trauma influences health outcomes are unclear. Childhood trauma-related disruptions to sleep in adulthood represent an important potential mechanism. Two 7-day multilevel studies investigated the effects of childhood trauma on daily sleep outcomes and stress-related variables and whether the effects of trauma on sleep outcomes were mediated through these stress-related variables (or vice versa). Participants completed the Childhood Trauma Questionnaire before a 7-day online daily diary study. Measures of daily stress, perseverative cognition, and sleep were completed daily. Multi-level modelling found that higher levels of childhood neglect were associated with poorer daily sleep quality, shorter sleep duration, longer sleep onset latency, and higher daily stress and rumination levels. Higher childhood abuse was associated with shorter sleep duration, greater morning tiredness, and higher levels of daily stress, rumination, and worry. Childhood trauma was found also to have bidirectional, indirect effects on sleep quality and morning tiredness through daily stress-related variables. The current findings suggest that interventions aimed at mitigating the negative effects of childhood trauma should also incorporate components that target modifiable risk factors, such as sleep, stress, worry, and rumination.

Influence of growth and metabolic markers on hs-troponin T and NT-proBNP levels in healthy children.

Background and objectives: As part of the LIFE Child study, we previously described the associations between N-terminal-pro-hormone brain natriuretic peptide (NT-proBNP) and hs-troponin T (hs-TnT) levels and an individual's sex, age and pubertal status, as well as with body mass index (BMI) and serum lipid levels. For NT-proBNP, we found inverse associations with advancing puberty, increasing BMI and serum lipid levels. These findings led us to further question the putative influences of the developing individual's metabolic and growth status as represented by levels of insulin-like growth factor-1 (IGF-1) and IGF-1-binding protein-3 (IGF-BP3) as well as hemoglobin A1c (HbA1c) and Cystatin C (CysC). Material and methods: Serum values, medical history and anthropometric data provided by 2522 children aged 0.25-18 years were collected and analyzed as per study protocol. Results: A strong negative association between NT-proBNP values and IGF-1, IGF-BP3 and HbA1c levels was identified. For IGF-BP3, this interaction was modulated by sex and age, for HbA1c only by age. For hs-TnT, a positive association was found with IGF-BP3, IGF-1 and CysC. The association between hs-TnT and IGF-1 was sex dependent. The association between CysC and hs-TnT was stronger in girls, but the interaction with age was only seen in boys. Between hs-TnT and HbA1c, the association was significantly negative and modulated by age. Conclusion: Based on our large pediatric cohort, we could identify age- and sex-dependent interactions between the metabolic status represented by IGF-1, IGF-BP3, CysC and HbA1c levels and the cardiac markers NT-proBNP and hs-TnT.

Trends, Characteristics, and Maternal Morbidity Associated With Unhoused Status in Pregnancy.

Importance: Unhoused status is a substantial problem in the US. Pregnancy characteristics and maternal outcomes of individuals experiencing homelessness are currently under active investigation to optimize health outcomes for this population. Objective: To assess the trends, characteristics, and maternal outcomes associated with unhoused status in pregnancy. Design, Setting, and Participants: This cross-sectional study analyzed data from the Healthcare Cost and Utilization Project National (Nationwide) Inpatient Sample. The study population included hospitalizations for vaginal and cesarean deliveries from January 1, 2016, to December 31, 2020. Unhoused status of these patients was identified from use of International Statistical Classification of Diseases, Tenth Revision, Clinical Modification code Z59.0. Statistical analysis was conducted from December 2022 to June 2023. Main Outcomes and Measures: Primary outcomes were (1) temporal trends; (2) patient and pregnancy characteristics associated with unhoused status, which were assessed with a multivariable logistic regression model; (3) delivery outcomes, including severe maternal morbidity (SMM) and mortality at delivery, which used the Centers for Disease Control and Prevention definition for SMM indicators and were assessed with a propensity score-adjusted model; and (4) choice of long-acting reversible contraception method and surgical sterilization at delivery. Results: A total of 18 076 440 hospital deliveries were included, of which 18 970 involved pregnant patients who were experiencing homelessness at the time of delivery, for a prevalence rate of 104.9 per 100 000 hospital deliveries. These patients had a median (IQR) age of 29 (25-33) years. The prevalence of unhoused patients increased by 72.1% over a 5-year period from 76.1 in 2016 to 131.0 in 2020 per 100 000 deliveries (P for trend < .001). This association remained independent in multivariable analysis. In addition, (1) substance use disorder (tobacco, illicit drugs, and alcohol use disorder), (2) mental health conditions (schizophrenia, bipolar, depressive, and anxiety disorders, including suicidal ideation and past suicide attempt), (3) infectious diseases (hepatitis, gonorrhea, syphilis, herpes, and COVID-19), (4) patient characteristics (Black and Native American race and ethnicity, younger and older age, low or unknown household income, obesity, pregestational hypertension, pregestational diabetes, and asthma), and (5) pregnancy characteristics (prior uterine scar, excess weight gain during pregnancy, and preeclampsia) were associated with unhoused status in pregnancy. Unhoused status was associated with extreme preterm delivery (<28-week gestation: 34.3 vs 10.8 per 1000 deliveries; adjusted odds ratio [AOR], 2.76 [95% CI, 2.55-2.99]); SMM at in-hospital delivery (any morbidity: 53.8 vs 17.7 per 1000 deliveries; AOR, 2.30 [95% CI, 2.15-2.45]); and in-hospital mortality (0.8 vs <0.1 per 1000 deliveries; AOR, 10.17 [95% CI, 6.10-16.94]), including case fatality risk after SMM (1.5% vs 0.3%; AOR, 4.46 [95% CI, 2.67-7.45]). Individual morbidity indicators associated with unhoused status included cardiac arrest (AOR, 12.43; 95% CI, 8.66-17.85), cardiac rhythm conversion (AOR, 6.62; 95% CI, 3.98-11.01), ventilation (AOR, 6.24; 95% CI, 5.03-7.74), and sepsis (AOR, 5.37; 95% CI, 4.53-6.36). Conclusions and Relevance: Results of this national cross-sectional study suggest that unhoused status in pregnancy gradually increased in the US during the 5-year study period and that pregnant patients with unhoused status were a high-risk pregnancy group.

Severe Maternal Morbidity and Mortality of Pregnant Patients With COVID-19 Infection During the Early Pandemic Period in the US.

This cohort study examines the patient characteristics and maternal outcomes associated with COVID-19 infection among pregnant patients at delivery during the early pandemic period in the US.

Multinational Pharmaceutical Companies Shortchange Canada in Research and Development Investments: Is It Time to Pursue Other Options?

In 1987, the government passed legislation to protect brand-name pharmaceutical firms against competition from generic drug brands in exchange for economic investment in Canadian pharmaceutical research and development (R&D). Since 2002, brand-name pharmaceutical companies' R&D investments have fallen short of their commitment, while Canadians now pay the fourth highest drug prices of all the Organisation for Economic Co-operation and Development member countries. In this article, we examine the degree to which brand-name pharmaceutical companies have fallen short of their promises, discuss whether a patent policy is the best strategy to secure Canadian pharmaceutical R&D funding and propose practical alternatives to this arrangement.

It's not all about control: challenging mainstream framing of eating disorders.

BACKGROUND: The concept of control has long been suggested as a central factor in eating disorder (ED) aetiology. The concept is now so mainstream that it risks being used in a potentially reductionist, stigmatising or otherwise harmful manner. In this paper, we explore and discuss our positions on the use of control-related terminology for EDs. METHODS: The authors of this auto-ethnographic position paper include academic researchers, individuals with lived experience and clinicians (not mutually exclusive). In sharing our experiences and observations, we aim to raise awareness of the wider impacts that control framing can have on ED perceptions, treatment, recovery and individuals' lived experience. RESULTS: We argue that although control can play a role in some ED experiences, an overemphasis upon this factor to the exclusion of other conceptualisations is not beneficial. CONCLUSIONS: To mitigate against pathologisation of an individual, it is important to challenge a discourse that can lead to EDs being perceived as something 'wrong' with the individual, rather than a consequence of life events or other environmental influences. We identify priorities for the future for researchers, clinicians, policy makers and the wider public.

Control has often been described as a central factor within Eating Disorders (EDs). Whilst control can play a role in ED experiences, we argue that overemphasis upon this factor can result in other important factors being overlooked. For many individuals, EDs are the consequence of life events and/or other environmental influences. With this in mind, discourse which overemphasises control (e.g., rather than coping) can inaccurately portray EDs as something 'wrong' with the individual. It is important to challenge this discourse to encourage more appropriate perceptions of EDs. In turn, this could improve understanding and treatment of EDs, reduce stigma, and promote recovery.

Feasibility, Acceptability, and Safety of Faecal Microbiota Transplantation in the Treatment of Major Depressive Disorder: A Pilot Randomized Controlled Trial.

OBJECTIVES: Perturbations of the intestinal microbiota have been associated with mental health disorders, including major depressive disorder (MDD). Therefore, faecal microbiota transplantation (FMT) holds promise as a microbiota-modulating treatment for MDD. Yet, to date, there are no published controlled studies evaluating the use of FMT for MDD. This study aimed to address this gap by evaluating the feasibility, acceptability, and safety of FMT for MDD. METHODS: The study was an 8-week, double-blind, 2:1 parallel group, randomized controlled pilot trial (n = 15) of enema-delivered FMT (n = 10) compared with a placebo enema (n = 5) in adults with moderate-to-severe MDD. RESULTS: Recruitment was completed within 2 months, with 0% attrition and 100% attendance at key study appointments. There were no major protocol deviations. The placebo and blinding strategies were considered successful; nurses and participants correctly guessing their treatment allocation at a rate similar to that anticipated by chance. No serious or severe adverse events were reported in either group, and there were no significant differences in mild-to-moderate adverse events between groups (median of 2 adverse events per participant reported in both groups). Furthermore, the 12/15 participants who completed the Week 2 participant satisfaction survey agreed or strongly agreed that the enema delivery was tolerable and that they would have the treatment again if required. Whilst the study was not designed to measure clinical outcomes, exploratory data also suggested that the active FMT treatment may lead to improvements in gastrointestinal symptoms and quality of life in this population, noting that irritable bowel syndrome is commonly comorbid with MDD. CONCLUSIONS: All feasibility targets were met or exceeded. This study found that enema-delivered FMT is feasible, acceptable, well-tolerated, and safe in patients with MDD. The findings of this study support further research to evaluate clinical efficacy, and the use of this protocol is supported.

Safety and feasibility of faecal microbiota transplant for major depressive disorder: study protocol for a pilot randomised controlled trial.

BACKGROUND: Mental disorders, including major depressive disorder (MDD), are a leading cause of non-fatal burden of disease globally. Current conventional treatments for depression have significant limitations, and there have been few new treatments in decades. The microbiota-gut-brain-axis is now recognised as playing a role in mental and brain health, and promising preclinical and clinical data suggest Faecal Microbiota Transplants (FMT) may be efficacious for treating a range of mental illnesses. However, there are no existing published studies in humans evaluating the efficacy of FMT for MDD. METHODS AND DESIGN: This protocol describes an 8-week, triple-blind, 2:1 parallel group, randomised controlled pilot trial (n = 15), of enema-delivered FMT treatment (n = 10) compared with a placebo enema (n = 5) in adults with moderate-to-severe MDD. There will be a further 26-week follow-up to monitor longer-term safety. Participants will receive four FMT or placebo enemas over four consecutive days. The primary aims of the study are to evaluate feasibility and safety of FMT as an adjunctive treatment for MDD in adults. Changes in gut microbiota will be assessed as a secondary outcome. Other data will be collected, including changes in depression and anxiety symptoms, and safety parameters. DISCUSSION: Modification of the microbiota-gut-brain axis via FMT is a promising potential treatment for MDD, but there are no published rigorous clinical trials evaluating its use. If this study finds that our FMT strategy is safe and feasible, a larger fully powered RCT is planned. Further high-quality research in this field is urgently needed to address unmet need. TRIAL REGISTRATION: Australian and New Zealand Clinical Trials Registry: ACTRN12621000932864.

Audiovisual integration in the human brain: a coordinate-based meta-analysis.

People can seamlessly integrate a vast array of information from what they see and hear in the noisy and uncertain world. However, the neural underpinnings of audiovisual integration continue to be a topic of debate. Using strict inclusion criteria, we performed an activation likelihood estimation meta-analysis on 121 neuroimaging experiments with a total of 2,092 participants. We found that audiovisual integration is linked with the coexistence of multiple integration sites, including early cortical, subcortical, and higher association areas. Although activity was consistently found within the superior temporal cortex, different portions of this cortical region were identified depending on the analytical contrast used, complexity of the stimuli, and modality within which attention was directed. The context-dependent neural activity related to audiovisual integration suggests a flexible rather than fixed neural pathway for audiovisual integration. Together, our findings highlight a flexible multiple pathways model for audiovisual integration, with superior temporal cortex as the central node in these neural assemblies.

Pest population dynamics are related to a continental overwintering gradient.

Overwintering success is an important determinant of arthropod populations that must be considered as climate change continues to influence the spatiotemporal population dynamics of agricultural pests. Using a long-term monitoring database and biologically relevant overwintering zones, we modeled the annual and seasonal population dynamics of a common pest, Helicoverpa zea (Boddie), based on three overwintering suitability zones throughout North America using four decades of soil temperatures: the southern range (able to persist through winter), transitional zone (uncertain overwintering survivorship), and northern limits (unable to survive winter). Our model indicates H. zea population dynamics are hierarchically structured with continental-level effects that are partitioned into three geographic zones. Seasonal populations were initially detected in the southern range, where they experienced multiple large population peaks. All three zones experienced a final peak between late July (southern range) and mid-August to mid-September (transitional zone and northern limits). The southern range expanded by 3% since 1981 and is projected to increase by twofold by 2099 but the areas of other zones are expected to decrease in the future. These changes suggest larger populations may persist at higher latitudes in the future due to reduced low-temperature lethal events during winter. Because H. zea is a highly migratory pest, predicting when populations accumulate in one region can inform synchronous or lagged population development in other regions. We show the value of combining long-term datasets, remotely sensed data, and laboratory findings to inform forecasting of insect pests.