Introduction to immunology and immune disorders.

The body has a collection of physical barriers to prevent infection, but once these are overcome, we rely on our immune systems to protect us against a wide variety of infections. The complex mechanisms through which this is achieved are grouped into two lines of defense called the "innate" and "adaptive" immune systems. The innate immune system provides a rapid and tailored response to infection or injury often associated with inflammation.

Evaluating family-centred care at BC Children's Hospital: Healthcare providers' perspectives.

Background: Family-centred care (FCC) emphasizes a partnership approach to care between healthcare providers (HCPs), patients, and families. FCC provides significant benefits to both children and families; however, challenges exist in implementing FCC into practice. This study aimed to explore HCPs' FCC behaviours in multidisciplinary specialty clinics at a tertiary pediatric health care center in Canada.

Revaccination outcomes among adolescents and adults with suspected hypersensitivity reactions following COVID-19 vaccination: A Canadian immunization research network study.

Background: COVID-19 vaccination has been associated with anaphylaxis and hypersensitivity reactions. Infectious disease physicians and allergists in the Canadian Special Immunization Clinic (SIC) Network developed guidance for evaluating patients with adverse events following immunization (AEFI) including suspected hypersensitivity. This study evaluated management and adverse event recurrence following subsequent COVID-19 vaccinations.

Inborn errors of immunity in adulthood.

Inborn errors of immunity (IEIs) are a group of conditions whereby parts of the immune system are missing or dysfunctional. Once thought to primarily be a pediatric disorder, it is now estimated that more than 50% of worldwide incident IEI cases are accounted for by adults. Delayed diagnosis, late symptom onset, and IEI phenocopies can all lead to adult-onset recognition of IEIs.

Summary of the NACI Statement on Public Health Level Recommendations on the Use of Pneumococcal Vaccines in Adults, Including the Use of 15-valent and 20-valent Conjugate Vaccines.

Background: Age and certain medical/social conditions are risk factors for invasive pneumococcal disease (IPD). For prevention of IPD, the National Advisory Committee on Immunization (NACI) has recommended the 23-valent polysaccharide pneumococcal vaccine, PNEU-P-23, for adults 65 years of age and older and adults over 18 years of age living with certain underlying conditions. NACI has also recommended 13-valent conjugate pneumococcal vaccine, PNEU-C-13, for adults; however, in publicly funded programs, this recommendation is limited to individuals with risk factors for IPD.

Trends in the proportion of women speakers at North American Allergy and Immunology conferences, 2008 to 2020.

Background: Women in medicine continue to be underrepresented at medical conferences. Previous studies have evaluated the proportion of invited female speakers across multiple specialties and evaluated factors that may have led to this disparity. The field of Allergy and Immunology has often been excluded and analyses have not illustrated how the trends have changed over the past decade.

Dominant negative variants in cause ICHAD syndrome, a new disorder characterised by immunodysregulation, craniofacial anomalies, hearing impairment, athelia and developmental delay.

Background: Helios (encoded by ), a member of the Ikaros family of transcription factors, is a zinc finger protein involved in embryogenesis and immune function. Although predominantly recognised for its role in the development and function of T lymphocytes, particularly the CD4 regulatory T cells (Tregs), the expression and function of Helios extends beyond the immune system. During embryogenesis, Helios is expressed in a wide range of tissues, making genetic variants that disrupt the function of Helios strong candidates for causing widespread immune-related and developmental abnormalities in humans.

Cow's milk alternatives for children with cow's milk allergy and beyond.

Cow's milk allergy (CMA) is one of the most common food allergies in the first years of life, with worldwide prevalence estimated to range from 2% to 5%. While the majority of children with CMA will eventually develop tolerance to cow's milk proteins (it is estimated that >75% of children with CMA develop tolerance to cow's milk proteins by the age of 3 years, and >90% develop tolerance by the age of 6 years), the selection of an appropriate cow's milk (CM) alternative for those with CMA is vital to ensure adequate growth and development during childhood. The increasing number of CM alternative products on the commercial market with markedly different nutritional content and micronutrient fortification adds a layer of complexity that can be challenging for both families and clinicians to navigate.

Septic arthritis due to in a young patient with hypogammaglobulinemia.

is an obligate intracellular bacterium usually found as a commensal in the human oral cavity. Symptomatic infections with this organism are rare, but severe disease has been described in the setting of impaired humoral immunity. Here, we describe a case in which was identified from the joint fluid of a patient with septic arthritis, splenic lesions, and agammaglobulinemia.

Revaccination and Adverse Event Recurrence in Patients with Adverse Events following Immunization.

Objectives: To estimate the risk of recurrence of adverse events following immunization (AEFIs) upon revaccination and to determine among patients with suspected vaccine allergy whether allergy skin test positivity was associated with AEFI recurrence.

Study Design: This prospective observational study included patients assessed in the Canadian Special Immunization Clinic Network from 2013 to 2019 with AEFIs who required revaccination with the vaccine temporally associated with their AEFI. Participants underwent standardized assessment and data collection.

Inborn errors of immunity manifesting as atopic disorders.

Inborn errors of immunity are traditionally best known for enhancing susceptibility to infections. However, allergic inflammation, among other types of immune dysregulation, occurs frequently in patients with inborn errors of immunity. As such, the term primary atopic disorders (PADs) was recently coined to describe the group of heritable monogenic allergic disorders.

Diverse clinical features and diagnostic delay in monogenic inborn errors of immunity: A call for access to genetic testing.

Background: Inborn errors of immunity (IEIs) are a group of conditions affecting immune system development and function. Due to their clinical heterogeneity and lack of provider awareness, patients suffer from long diagnostic delays that increase morbidity and mortality. Next-generation sequencing facilitates earlier diagnosis and treatment of IEIs, but too often patients are unable to see the benefit of this technology due to gaps in providers' knowledge regarding which patients to test and barriers to accessing sequencing.

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.

L'évaluation et la prise en charge des allergies alimentaires non induites par les IgE.

Le syndrome d'entérocolite induite par les protéines alimentaires et la proctocolite allergique induite par les protéines alimentaires sont les principaux types d'allergies alimentaires non induites par les immunoglobines E. Le syndrome d'entérocolite induit par les protéines alimentaires se manifeste par des vomissements réfractaires tardifs, tandis que la proctocolite allergique induite par les protéines alimentaires se révèle par une hématochézie chez des nourrissons autrement en santé. La prise en charge immédiate du syndrome d'entérocolite induite par les protéines alimentaires inclut la réhydratation, l'ondansétron ou ces deux traitements, mais est inutile pour soigner la proctocolite allergique induite par les protéines alimentaires.