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Michelle Le, Sofianne Gabrielli, Sarah De Schryver, Moshe Ben-Shoshan Division of Allergy, Immunology and Dermatology, Department of Pediatrics, McGill University Health Center, Montreal, QC, CanadaCorrespondence: Michelle LeDivision of Pediatric Allergy, Clinical Immunology and Dermatology, Department of Pediatrics, McGill University Health Center, 1001 Boul. Decarie, Montreal, Quebec H4A 3J1, CanadaTel +1 514-412-4400 (23151)Fax +1 514-412-4390Email michelle.le@mail.mcgill.caBackground: Idiopathic anaphylaxis (IA) is a diagnosis of exclusion and represents a major diagnostic and management challenge. There are no current guidelines for diagnosis and management of IA. We aim to present a systematic review of the literature on adult and pediatric IA.Methods: We conducted a systematic review of original articles published in the past 22 years regarding diagnosis and management strategies of adult and pediatric IA.Results: The current proposed diagnostic approach and treatment regimens are based on a few small studies. Future large-scale studies are required. IA is a diagnosis of exclusion and should be made only after extensive evaluation excludes potential anaphylaxis triggers as well as non-allergic conditions with a similar presentation. There is currently no diagnostic consensus for IA. Furthermore, the current proposed treatment regimens are limited and rely on prophylactic treatment with antihistamines and prednisone for patients with frequent episodes. However, daily treatment with systemic steroids has well-recognized serious adverse effects. More recently, the use of biologics was suggested to benefit patients with IA, although the optimal management protocol is not yet established.Conclusion: Future studies are needed to optimize diagnosis and treatment strategies in adult and pediatric cases of IA. Omalizumab may be a promising novel therapeutic option for adult and pediatric IA.Keywords: anaphylaxis, diagnosis, management, treatment

Andrew W O'Keefe,1,2 Sarah De Schryver,1 Jennifer Mill,3 Christopher Mill,3 Alizee Dery,1 Moshe Ben-Shoshan1 1Division of Pediatric Allergy and Clinical Immunology, Department of Pediatrics, Montreal Children's Hospital, McGill University Health Centre, Montreal, QC, Canada; 2Department of Pediatrics, Faculty of Medicine, Memorial University of Newfoundland, St John's, NL, Canada; 3Division of Clinical Epidemiology, Department of Medicine, McGill University Health Centre, Montreal, QC, Canada Abstract: It is reported that 6% of children and 3% of adults have food allergies, with studies suggesting increased prevalence worldwide over the last few decades. Despite this, our diagnostic capabilities and techniques for managing patients with food allergies remain limited. We have conducted a systematic review of literature published within the last 5 years on the diagnosis and management of food allergies. While the gold standard for diagnosis remains the double-blind, placebo-controlled food challenge, this assessment is resource intensive and impractical in most clinical situations. In an effort to reduce the need for the double-blind, placebo-controlled food challenge, several risk-stratifying tests are employed, namely skin prick testing, measurement of serum-specific immunoglobulin E levels, component testing, and open food challenges. Management of food allergies typically involves allergen avoidance and carrying an epinephrine autoinjector. Clinical research trials of oral immunotherapy for some foods, including peanut, milk, egg, and peach, are under way. While oral immunotherapy is promising, its readiness for clinical application is controversial. In this review, we assess the latest studies published on the above diagnostic and management modalities, as well as novel strategies in the diagnosis and management of food allergy. Keywords: skin prick testing, oral challenge, specific IgE, component testing, oral immunotherapy, epinephrine autoinjector

Background: There is a lack of patient educational resources about chronic urticaria (CU)., Aims: To develop and test the effectiveness of an education tool to help paediatric patients and their families better understand CU and its management., Methods: From July 2020 to May 2022, paediatric patients with a history of CU who presented to the allergy outpatient clinics at our institution were recruited. Consenting families and patients were asked to complete five questions related to the definition, causes and management of CU at the time of presentation to the clinic. Participants were shown a 5-min animated video addressing the main knowledge gaps about CU. At the end of the video, participants were redirected to the same five questions to respond again. The scores were recorded as a proportion of correct answers (range 0·0-1·0)., Results: In total, 53 patients [30 girls (56·6%), 23 boys (43·4%); mean age 9·7 ± 5·1 years, range 1·4-18·5 years] were recruited. The mean baseline pre-video education questionnaire score was 0·67 ± 0·2 (range 0·2-1·0), while the mean post-video score was 0·94 ± 0·1 (range 0·4-1·0), a mean score difference of 0·27, which was statistically significant (P < 0·001). At the 1-year follow-up, 14 (26·4%) patients answered the questionnaire again to assess retention of knowledge; the mean score was 0·83 ± 0·2 (range 0·2-1·0)., Conclusions: Our educational video was successful in educating patients and their families to better understand urticaria. Future studies should aim to optimize patient education through nontraditional tools such as videos, and compare knowledge gain using different methods of education., Competing Interests: Conflict of interest The authors declare that they have no conflict of interest., (© The Author(s) 2022. Published by Oxford University Press on behalf of British Association of Dermatologists. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

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.

Background: This study focuses on the side effects of cow's milk oral immunotherapy (CM-OIT) using consensus definitions of food-induced anaphylaxis., Objective: To evaluate the risk of allergic reactions (ARs) and to identify risk factors associated with higher risk of anaphylactic ARs (AARs) during CM-OIT in children., Methods: Clinical charts of children receiving CM-OIT were carefully reviewed. ARs were defined as single-organ ARs, and AARs were defined as involvement of 2 organ systems and/or hypotension in response to CM protein. Descriptive statistics were used to represent demographics, occurrence, reaction characteristics, and comorbidities. Poisson analysis was performed to evaluate risk factors associated with AARs., Results: Among 41 children undergoing CM-OIT, 11 discontinued the treatment (N = 26.8%). The mean age at challenge was 12.1 years (standard deviation [SD], 3.6) and half were male (56.1%). The mean number of AARs per patient was 6.0 (SD, 3.5) versus a mean of 17.4 (SD, 11.9) non-AARs per patient. Among withdrawals from OIT, the mean number of AARs per patient was 8.3 versus 5.1 in nonwithdrawals. AARs were more frequent in children with higher specific IgE (sIgE) for α-lactalbumine and casein at baseline (1.11 [95% confidence interval (CI): 1.01, 1.22] and 1.01 [1.0, 1.03], respectively). Children with resolved eczema and higher sIgE for β-lactoglobuline at baseline (0.13 [95% CI: 0.04, 0.46] and 0.96 [95% CI: 0.94, 0.99], respectively) were less likely to develop AARs., Conclusions: Although the majority of ARs during OIT are nonanaphylactic, AARs occur frequently. Children with higher sIgE for α-lactalbumine and casein at baseline seem to be at higher risk for AARs during OIT., (Copyright © 2019 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)

Syndromic primary immunodeficiencies are rare genetic disorders that affect both the immune system and other organ systems. More often, the immune defect is not the major clinical problem and is sometimes only recognized after a diagnosis has been made based on extra-immunological abnormalities. Here, we report two sibling pairs with syndromic primary immunodeficiencies that exceptionally presented with a phenotype resembling early-onset common variable immunodeficiency, while extra-immunological characteristics were not apparent at that time. Additional features not typically associated with common variable immunodeficiency were diagnosed only later, including skeletal and organ anomalies and mild facial dysmorphism. Whole exome sequencing revealed KMT2A-associated Wiedemann-Steiner syndrome in one sibling pair and their mother. In the other sibling pair, targeted testing of the known disease gene for Roifman syndrome (RNU4ATAC) provided a definite diagnosis. With this study, we underline the importance of an early-stage and thorough genetic assessment in paediatric patients with a common variable immunodeficiency phenotype, to establish a conclusive diagnosis and guide patient management. In addition, this study extends the mutational and immunophenotypical spectrum of Wiedemann-Steiner and Roifman syndromes and highlights potential directions for future pathophysiological research.

Background: The diagnosis of anaphylaxis currently relies on suggestive clinical history after exposure to a potential triggering factor because no reliable diagnostic marker is available to confirm the diagnosis., Objectives: We aimed to evaluate tryptase levels in children with anaphylaxis and to examine predictors of elevated tryptase level (defined as ≥11.4 μg/L during reaction and for those with a baseline level, defined as a reaction level of at least 2 ng/mL + 1.2 × [postreaction tryptase level])., Methods: Children presenting with anaphylaxis to the Montreal Children's Hospital were recruited over a 4-year period. Symptoms, triggers, and management of anaphylaxis were documented. Levels during the reaction and approximately 9 months after the reaction were compared on the basis of paired means using the t distribution. Multivariate linear and logistic regressions were used to evaluate the association between tryptase levels and risk factors., Results: Over a 4-year period, 203 children had serum tryptase levels measured. Among these, 39 children (19.2%; 95% CI, 14.1%-25.4%) had elevated levels. Only severe reactions were associated with reaction levels of 11.4 μg/L or more (odds ratio, 6.5; 95% CI, 2.2-19.0). Milk-induced anaphylaxis and severe reactions were more likely associated with increased tryptase levels (beta-adjusted, 4.0; 95% CI, 0.95-7.0, and 7.5; 95% CI, 4.8-10.3, respectively). Reaction levels exceeding the threshold level of 2 ng/mL + 1.2 × (postreaction tryptase level) detected most of the anaphylactic reactions, particularly if baseline levels were taken within 2 months of the reaction., Conclusions: Tryptase levels are particularly useful for the diagnosis of severe and/or milk-induced anaphylaxis. Assessing the difference between reaction and postreaction tryptase levels may improve diagnostic sensitivity., (Copyright © 2015 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)

It is reported that 6% of children and 3% of adults have food allergies, with studies suggesting increased prevalence worldwide over the last few decades. Despite this, our diagnostic capabilities and techniques for managing patients with food allergies remain limited. We have conducted a systematic review of literature published within the last 5 years on the diagnosis and management of food allergies. While the gold standard for diagnosis remains the double-blind, placebo-controlled food challenge, this assessment is resource intensive and impractical in most clinical situations. In an effort to reduce the need for the double-blind, placebo-controlled food challenge, several risk-stratifying tests are employed, namely skin prick testing, measurement of serum-specific immunoglobulin E levels, component testing, and open food challenges. Management of food allergies typically involves allergen avoidance and carrying an epinephrine autoinjector. Clinical research trials of oral immunotherapy for some foods, including peanut, milk, egg, and peach, are under way. While oral immunotherapy is promising, its readiness for clinical application is controversial. In this review, we assess the latest studies published on the above diagnostic and management modalities, as well as novel strategies in the diagnosis and management of food allergy.