Anaphylaxis: A 2023 practice parameter update.

This practice parameter update focuses on 7 areas in which there are new evidence and new recommendations. Diagnostic criteria for anaphylaxis have been revised, and patterns of anaphylaxis are defined. Measurement of serum tryptase is important for diagnosis of anaphylaxis and to identify underlying mast cell disorders. In infants and toddlers, age-specific symptoms may differ from older children and adults, patient age is not correlated with reaction severity, and anaphylaxis is unlikely to be the initial reaction to an allergen on first exposure. Different community settings for anaphylaxis require specific measures for prevention and treatment of anaphylaxis. Optimal prescribing and use of epinephrine autoinjector devices require specific counseling and training of patients and caregivers, including when and how to administer the epinephrine autoinjector and whether and when to call 911. If epinephrine is used promptly, immediate activation of emergency medical services may not be required if the patient experiences a prompt, complete, and durable response. For most medical indications, the risk of stopping or changing beta-blocker or angiotensin-converting enzyme inhibitor medication may exceed the risk of more severe anaphylaxis if the medication is continued, especially in patients with insect sting anaphylaxis. Evaluation for mastocytosis, including a bone marrow biopsy, should be considered for adult patients with severe insect sting anaphylaxis or recurrent idiopathic anaphylaxis. After perioperative anaphylaxis, repeat anesthesia may proceed in the context of shared decision-making and based on the history and results of diagnostic evaluation with skin tests or in vitro tests when available, and supervised challenge when necessary.

Prevention and management of allergic reactions to food in child care centers and schools: Practice guidelines.

Food allergy management in child care centers and schools is a controversial topic, for which evidence-based guidance is needed. Following the Grading of Recommendations Assessment, Development, and Evaluation approach, we conducted systematic literature reviews of the anticipated health effects of selected interventions for managing food allergy in child care centers and schools; we compiled data about the costs, feasibility, acceptability, and effects on health equity of the selected interventions; and we developed the following conditional recommendations: we suggest that child care centers and schools implement allergy training and action plans; we suggest that they use epinephrine (adrenaline) to treat suspected anaphylaxis; we suggest that they stock unassigned epinephrine autoinjectors, instead of requiring students to supply their own personal autoinjectors to be stored on site for designated at-school use; and we suggest that they do not implement site-wide food prohibitions (eg, "nut-free" schools) or allergen-restricted zones (eg, "milk-free" tables), except in the special circumstances identified in this document. The recommendations are labeled "conditional" due to the low quality of available evidence. More research is needed to determine with greater certainty which interventions are likely to be the most beneficial. Policymakers might need to adapt the recommendations to fit local circumstances.

The known burden of Huntington disease in the North of Scotland: prevalence of manifest and identified pre-symptomatic gene expansion carriers in the molecular era.

BACKGROUND: Huntington disease prevalence was first estimated in Grampian, northern Scotland in 1984. Molecular testing has since increased ascertainment. OBJECTIVE: To estimate the prevalence of manifest Huntington disease and identified pre-symptomatic gene expansion carriers (IPGEC) in northern Scotland, and estimate the magnitude of biases in prevalence studies that rely upon routine coding in primary care records. METHODS: Cases were ascertained using North of Scotland genetic laboratory, clinic, and hospital records. Prevalence was calculated for manifest and IPGEC on 01/07/2016 and 01/01/2020 and compared with local published data. RESULTS: The prevalence of manifest Huntington disease in northern Scotland in 2020 was 14.6 (95% CI 14.3-15.3) per 100,000, and of IPGEC was 8.3 (95% CI 7.8-9.2) per 100,000. Whilst the population of northern Scotland decreased by 0.05% between 2016 and 2020, the number of manifest and identified pre-symptomatic gene expansion carriers increased by 7.4% and 23.3%, respectively. Manifest disease in Grampian increased by 45.9% between 1984 and 2020. More women than men had a diagnosis. General Practice coding underestimated symptomatic molecularly confirmed prevalence by 2.2 per 100,000 people. CONCLUSION: Even in an area with previously high ascertainment, there has been a 45.9% increase in manifest Huntington disease over the last 30 years. Within our catchment area, prevalence varies between health board regions with similar community-based services. Such variation in prevalence could have major drug cost and service delivery implications, especially if expensive, complexly administered therapies prove successful. Health services should gather accurate population-based data on a regional basis to inform service planning.

Youth and parents' experiences of a new inter-agency transition model for spina bifida compared to youth who did not take part in the model.

BACKGROUND: Young adults with spina bifida are underserved in health care and are at risk as they transition to adult health care. A pediatric rehabilitation hospital and an adult community health center partnered to help address this gap. Although some research has explored general transition experiences of youth with disabilities, little is known about their experiences in a transition model that involves an inter-agency partnership, continuous, age-appropriate, and client-centered care. OBJECTIVE: To explore youth and parent experiences of a new transition model for youth with spina bifida, compared to the experiences of young adults with spina bifida who did not participate in the model. METHODS: Using a descriptive, qualitative design involving a thematic analysis we conducted semi-structured interviews with 32 participants (9 youth, 11 parents, 12 young adults). RESULTS: Most youth and parents in our sample who took part in the new model felt supported by pediatric providers and benefitted from gradually transferring responsibility from parents to youth. They also reported experiencing challenges, including lack of support from primary care providers and lack of clarity about the new model. Many young adults who did not take part in the model reported receiving some transition-related thought support from pediatric specialists, parents, and in some cases, primary care providers. However, they also reported experiencing gaps in their continuity of care and needed more support with employment, relationships, finances, and housing. CONCLUSIONS: Our findings show the new transition model for youth with spina bifida can help enhance participants' transition experiences and preparation for adulthood. However, the model needs further development to address the varied abilities and support needs of youth with spina bifida.

Experiencing a first food allergic reaction: a survey of parent and caregiver perspectives.

BACKGROUND: Insufficient knowledge of food allergy and anaphylaxis has been identified by caregivers as an important barrier to coping, and a potential cause of fear and anxiety, particularly for those with children newly diagnosed with food allergy. The purpose of the study was to better understand the experiences of caregivers of children with a first allergic reaction to food, and to identify any deficiencies in the information received at diagnosis. METHODS: A mixed-methods study consisting of an online survey administered to the Anaphylaxis Canada online registry (a patient support group database of approximately 10,000 members), and a follow-up qualitative interview with a subset of survey participants. Analysis consisted of frequency analysis (quantitative and qualitative data) and descriptive statistics to calculate proportions and means with standard deviations. Qualitative analyses were guided by the constant comparative method of grounded theory methodology. RESULTS: Of 293 survey respondents, 208 were eligible to complete the survey (first allergic reaction to food within 12 months of the study), and 184 respondents consented. Identified gaps included education about food allergy, anaphylaxis management, for example, how to use epinephrine auto- injectors, and coping strategies for fear and anxiety. The qualitative follow-up study supported these findings, yielding 3 major themes: 1) lack of provision of information following the episode on the recognition and management of food allergy related allergic reactions, 2) prolonged wait times for an allergist, and 3) significant family anxiety. CONCLUSIONS: The online survey highlighted multiple deficiencies at diagnosis, findings which were supported by the follow up qualitative study. Results will inform the development of educational strategies for patients newly diagnosed with food allergy.