Bridging Knowledge Gaps in Anaphylaxis Management Through a Video-Based Educational Tool.

Introduction: We aimed to develop and test the effectiveness of an education tool to help pediatric patients and their families better understand anaphylaxis and its management, and to improve current knowledge and treatment guidelines adherence. Methods: From June 2019 to May 2022, 128 pediatric patients with history of food-triggered anaphylaxis who presented to the allergy outpatient clinics at the study institution were recruited. Consenting families were asked to complete 6 questions related to the triggers, recognition, and management of anaphylaxis at the time of presentation to the clinic. Participants were shown a 5-min animated video on the causes, presentation, and management of anaphylaxis. At the end of the video, the participants were redirected to the same 6 questions to respond again. The scores were recorded in proportion of correct answers (minimum 0.0; maximum 1.0). Results: The mean age of the patients was 5.8 ± 4.5 years (range: 0.5-18.8 years). The majority were males (70 patients; 54.7%). The mean baseline prevideo education questionnaire score was 0.76 ± 0.2 (range: 0.3-1.0), whereas the mean follow-up score was 0.82 ± 0.2 (range: 0.3-1.0). This score difference of 0.06 was statistically significant (P < 0.001). There were no significant associations between change in scores and age or gender of the participants. Conclusion: Our video teaching method was successful in educating patients and their families to better understand anaphylaxis and its management at the moment of the clinical encounter. Retention of knowledge at long-term follow-up should be assessed.

Anaphylaxis to patent blue dye in a 17-year-old boy.

Patent blue V dye (PBV) is frequently used as a perioperative drug for lymphangiography, as well as a food additive. Hypersensitivity to PBV is poorly documented in adults and had not been previously described in children. The diagnosis of PBV allergy depends on corroboration of history consistent with an IgE-mediated reaction and confirmatory skin tests. We present in this paper a paediatric case of PBV anaphylaxis and of biphasic reaction that exemplifies the challenges involved in diagnosing and managing this rare but potentially life-threatening allergic reaction.

A Comprehensive Review on Kiwifruit Allergy: Pathogenesis, Diagnosis, Management, and Potential Modification of Allergens Through Processing.

Kiwifruit is rich in bioactive components including dietary fibers, carbohydrates, natural sugars, vitamins, minerals, omega-3 fatty acids, and antioxidants. These components are beneficial to boost the human immune system and prevent cancer and heart diseases. However, kiwifruit is emerging as one of the most common elicitors of food allergies worldwide. Kiwifruit allergy results from an abnormal immune response to kiwifruit proteins and occur after consuming this fruit. Symptoms range from the oral allergy syndrome (OAS) to the life-threatening anaphylaxis. Thirteen different allergens have been identified in green kiwifruit and, among these allergens, Act d 1, Act d 2, Act d 8, Act d 11, and Act d 12 are defined as the "major allergens." Act d 1 and Act d 2 are ripening-related allergens and are found in abundance in fully ripe kiwifruit. Structures of several kiwifruit allergens may be altered under high temperatures or strong acidic conditions. This review discusses the pathogenesis, clinical features, and diagnosis of kiwifruit allergy and evaluates food processing methods including thermal, ultrasound, and chemical processing which may be used to reduce the allergenicity of kiwifruit. Management and medical treatments for kiwifruit allergy are also summarized.

Modulation of the Interleukin-21 Pathway with Interleukin-4 Distinguishes Common Variable Immunodeficiency Patients with More Non-infectious Clinical Complications.

PURPOSE: Common variable immunodeficiency (CVID) is characterized by hypogammaglobulinemia and clinical manifestations such as infections, autoimmunity, and malignancy. We sought to determine if responsiveness to interleukin-21 (IL-21), a key cytokine for B cell differentiation, correlates with distinct clinical phenotypes in CVID. METHODS: CVID subjects were recruited through the Canadian Primary Immunodeficiency Evaluative Survey registry. Peripheral blood mononuclear cells were cultured with anti-CD40 ± interferon-gamma, interleukin-4 (IL-4), IL-21, and/or IL-4+IL-21. B cell subpopulations and IgG production were determined at baseline and day 7 by flow cytometry and ELISA. Clinical complications were compared using contingency tables. RESULTS: CVID subjects exhibited decreased CD27+ B cells and IgG production after 7 days of stimulation with anti-CD40+IL-21 (p < 0.05). In a subset of subjects [CVID responders (R)], the addition of IL-4 led to significant increases in CD27+ B cells and IgG (p < 0.05). In CVID non-responders (NR), CD27+ B cells and IgG remained lower despite the addition of IL-4. CVID NR experienced significantly more non-infectious clinical complications of CVID than R [OR 8.8, 95% confidence interval (CI) 1.6 to 48.13]. Previous studies reported that CVID subjects with ≤ 2% class-switched memory B cells were more at risk of these complications, but no significant association was found among this cohort of subjects [OR 3.5, CI 0.9 to 13.3]. In fact, 34.6% of CVID NR had > 2% class-switched memory B cells at baseline. CONCLUSIONS: The IL-4 and IL-21 in vitro assays distinguish two groups of CVID subjects and can be used with baseline B cell subpopulation phenotyping to better identify patients experiencing more vs. fewer clinical non-infectious complications and potentially to modulate therapy.

Autoimmune hemolytic anemia in a teenager: a wolf in sheep's clothing.

This case report describes a 14-year-old boy who presented to the emergency department with symptoms of severe anemia. He was diagnosed with autoimmune hemolytic anemia, and on further investigation, it was noted that he had no functioning T cells. Despite no antecedent history of severe infection, he was worked up for a severe combined immunodeficiency, and was found to have a compound hypomorphic mutation in an enzyme responsible for recombination of the B- and T-cell receptors. He was subsequently diagnosed with severe combined immunodeficiency, presenting with autoimmunity, and received a bone marrow transplant. As our knowledge of the immune system continues to expand, we are learning that dysregulation can occur in any one of the complex immune pathways, and may have a variety of clinical presentations. A high index of suspicion for immune defects should be maintained in cases of atypical or severe infections, autoimmunity or malignancy, particularly by the general practitioner, who is often the first to encounter these challenging patients.

Young patients with chest pain: 1-year outcomes.

OBJECTIVE: Prior studies found that young adult chest pain patients without known cardiac disease with either no cardiac risk factors or a normal electrocardiogram (ECG) are at low risk (<1%) for acute coronary syndromes (ACS) and 30-day cardiovascular events. Longer-term event rates in this subset of patients are unknown. We hypothesized that patients younger than 40 years without past cardiac history and a normal ECG are at less than 1% risk for 1-year adverse cardiovascular events. METHODS: We conducted a prospective cohort study in an urban university emergency department evaluating patients younger than 40 years who received an ECG for evaluation of potential ACS. Cocaine users, cancer patients, and patients with a history of myocardial infarction or revascularization were excluded. Structured data collection at presentation included demographics, chest pain description, history, laboratory results, and ECG data. Hospital course was followed. Follow-up was obtained by telephone, record review, and social security death index search. Our main outcome was 1-year adverse cardiovascular events (death; acute myocardial infarction [AMI]; or revascularization-percutaneous coronary intervention [PCI] or coronary artery bypass graft). Descriptive statistics and 95% confidence intervals were used. RESULTS: Of 3846 chest pain patients, 609 met criteria. Of those, 35.5% were admitted. Patients had a mean age of 34.8 years (SD, 3.8 years). They were most often female (57.6%) and black (69.5%). There were 7 patients (1.1%; 95% CI, 0.5%-2.4%) with adverse cardiovascular events over the year. Of the subset of 560 patients with a normal/nonspecific ECG, there were 2 deaths (0.4%), 3 AMI (0.5%), and 2 PCIs (0.4%) by 1 year for a composite adverse cardiovascular event rate of 6 (1.1%; 95% CI, 0.4%-2.3%). Of the subset of 269 patients with no cardiac risk factors and a normal/nonspecific ECG, there were no deaths, 1 AMI, and 1 PCI for a composite adverse cardiovascular event rate at 1 year of 0.3% (0.01%-2.1%). The addition of an initial cardiac marker to this group resulted in a cohort that was event-free at 1 year (95% CI, 0%-1.4%). CONCLUSIONS: Patients younger than 40 years without a cardiac history who present to the ED with symptoms consistent with ACS but have either no risk factors or a normal or nonspecific ECG have a very low rate of adverse events during the subsequent year.

Multicentric castleman disease in a child with primary immunodeficiency.

Multicentric Castleman disease is a rare lymphoproliferative disorder mostly seen in adults with HIV. It presents with fever and systemic symptoms and is extremely uncommon in children. We describe a novel case of multicentric Castleman disease associated with primary immunodeficiency (common variable immunodeficiency) and discuss pathophysiologic mechanisms and recent advances in understanding this disease.

One-year outcomes following coronary computerized tomographic angiography for evaluation of emergency department patients with potential acute coronary syndrome.

OBJECTIVES: Coronary computerized tomographic angiography (CTA) has high correlation with cardiac catheterization and has been shown to be safe and cost-effective when used for rapid evaluation of low-risk chest pain patients from the emergency department (ED). The long-term outcome of patients discharged from the ED with negative coronary CTA has not been well studied. METHODS: The authors prospectively evaluated consecutive low- to intermediate-risk patients who received coronary CTA in the ED for evaluation of a potential acute coronary syndrome (ACS). Patients with cocaine use, known cancer, and significant comorbidity reducing life expectancy and those found to have significant disease (stenosis > or = 50% or ejection fraction < 30%) were excluded. Demographics, medical and cardiac history, labs, and electrocardiogram (ECG) results were collected. Patients were followed by telephone contact and record review for 1 year. The main outcome was 1-year cardiovascular death or nonfatal acute myocardial infarction (AMI). RESULTS: Of 588 patients who received coronary CTA in the ED, 481 met study criteria. They had a mean (+/-SD) age of 46.1 (+/-8.8) years, 63% were black or African American, and 60% were female. There were 53 patients (11%) rehospitalized and 51 patients (11%) who received further diagnostic testing (stress or catheterization) over the subsequent year. There was one death (0.2%; 95% confidence interval [CI] = 0.01% to 1.15%) with unclear etiology, no AMI (0%; 95% CI = 0 to 0.76%), and no revascularization procedures (0%; 95% CI = 0 to 0.76%) during this time period. CONCLUSIONS: Low- to intermediate-risk patients with a Thrombosis In Myocardial Infarction (TIMI) score of 0 to 2 who present to the ED with potential ACS and have a negative coronary CTA have a very low likelihood of cardiovascular events over the ensuing year.

Inhibition of experimental allergic airways disease by local application of a cell-penetrating dominant-negative STAT-6 peptide.

Allergic airways disease is initiated and perpetuated by an aberrant Th2 inflammatory response regulated in part by the cytokines IL-4 and IL-13, each of which induces activation of the STAT-6 transcription factor. Data from murine models indicate that the clinical manifestations of acute asthma are STAT-6 dependent, and thus, STAT-6 is a target for drug development in allergic airways disease. We designed a novel chimeric peptide (STAT-6 inhibitory peptide (STAT-6-IP)) comprised of a sequence predicted to bind to and inhibit STAT-6, fused to a protein transduction domain, to facilitate cellular uptake of the STAT-6-binding peptide. Our data demonstrate that the STAT-6-IP inhibited OVA-induced production of Th2 cytokines IL-4 and IL-13 in vitro. In contrast, the STAT-6-IP did not affect production of IFN-gamma, demonstrating specificity for Th2 cytokine inhibition. Following intranasal administration, the STAT-6-IP was localized to epithelial cells in the airways. Finally, in in vivo murine models of allergic rhinitis and asthma, intranasal delivery of the STAT-6-IP inhibited OVA-induced lung inflammation and mucus production as well as accumulation of eosinophils and IL-13 in bronchoalveolar lavage fluid and OVA-dependent airway hyperresponsiveness. Together these data show that local application of cell-penetrating peptide inhibitors of STAT-6 has significant potential for the treatment of allergic rhinitis and asthma.

Long-term therapy with aerosolized ribavirin for parainfluenza 3 virus respiratory tract infection in an infant with severe combined immunodeficiency.

We report the case of an infant with severe combined immunodeficiency who was presented with PIV3 infection. Aerosolized ribavirin was administered for 10 months until the child gained a functional immune system through an allogeneic hematopoietic stem cell transplant and cleared PIV3 infection. No adverse effect was observed in the child and in healthcare personnel, with a follow-up of three years. Despite the burden of aerosolized administration, early and prolonged administration of aerosolized ribavirin was feasible, well tolerated, and safe for the patient and the caregivers. This is a case report and no definite conclusions can be drawn. However, our experience suggests that prolonged aerosolized ribavirin administration should be considered for the treatment of PIV3 infection in the context of primary immunodeficiency, where there is no currently available alternative treatment, until a functional immune system is gained.