Food protein-induced enterocolitis syndrome in a tertiary pediatric center: safety of guideline-conforming food challenges.

Food protein-induced enterocolitis syndrome is a non-IgE-mediated reaction to food that is poorly understood, and underdiagnosed. Trigger foods can belong to any food group, but are most commonly milk, soy, rice, oat, egg, and fish. In this retrospective study (2015-2020), we describe the clinical presentations and triggers of 37 children referred to tertiary hospital with a confirmed or suspected diagnosis of food protein-inducted enterocolitis. We reviewed the safety of current recommendations by looking at the outcome of 24 oral food challenges. All of these patients presented with clear cut systemic reactions including lethargy. We also assessed the severity of the reactions. Oral food challenges occurred in the hospital day unit with the majority of patients having IV access in place. Despite a clear history of FPIES with lethargy and the requirement for re-hydration of the challenged population, 21/24 (88%) of the FPIES OFCs were successful. Of the three patients who reacted, symptoms were of moderate nature, mainly vomiting. This highlights the importance of early diagnosis and a pro-active approach to performing guideline-directed oral food challenges in patients with food protein-induced enterocolitis syndrome.

"Call Me by My Name": Improving Communication with Family Members at the Bedside via the Caregiver Identification Badge.

BACKGROUND: Patient and family experience are integral to the care that we provide. In the pediatric hospital setting, multiple family members are directly involved in patient care. We identified the need for greater caregiver name recognition at The Hospital for Sick Children, Toronto, ON. OBJECTIVE: We aimed to improve communication between healthcare providers and families via the optimization of caregiver identification badges. METHODS: We used a qualitative, narrative study design to explore perceptions surrounding caregiver identification badges via unstructured interviews. RESULTS: We identified key hospital and family stakeholders. Unstructured interviews supported the theory that badge optimization could improve communication and patient care. Our initiative, however, was abruptly interrupted by the emergence of the COVID-19 pandemic. CONCLUSION: Communication with patients and families is crucial across medical disciplines. The optimization of caregiver identification badges to facilitate the use of preferred names and pronouns will ultimately lead to the more effective and safer delivery of high-quality care.

Basophil activation test shows high accuracy in the diagnosis of peanut and tree nut allergy: The Markers of Nut Allergy Study.

BACKGROUND: Peanut and tree nut allergies are the most important causes of anaphylaxis. Co-reactivity to more than one nut is frequent, and co-sensitization in the absence of clinical data is often obtained. Confirmatory oral food challenges (OFCs) are inconsistently performed. OBJECTIVE: To investigate the utility of the basophil activation test (BAT) in diagnosing peanut and tree nut allergies. METHODS: The Markers Of Nut Allergy Study (MONAS) prospectively enrolled patients aged 0.5-17 years with confirmed peanut and/or tree nut (almond, cashew, hazelnut, pistachio, walnut) allergy or sensitization from Canadian (n = 150) and Austrian (n = 50) tertiary pediatric centers. BAT using %CD63+ basophils (SSClow/CCR3pos) as outcome was performed with whole blood samples stimulated with allergen extracts of each nut (0.001-1000 ng/mL protein). BAT results were assessed against confirmed allergic status in a blinded fashion to develop a generalizable statistical model for comparison to extract and marker allergen-specific IgE. RESULTS: A mixed effect model integrating BAT results for 10 and 100 ng/mL of peanut and individual tree nut extracts was optimal. The area under the ROC curve (AUROC) was 0.98 for peanut, 0.97 for cashew, 0.92 for hazelnut, 0.95 for pistachio, and 0.97 for walnut. The BAT outperformed sIgE testing for peanut or hazelnut and was comparable for walnut (AUROC 0.95, 0.94, 0.92) in a sub-analysis in sensitized patients undergoing OFC. CONCLUSIONS: Basophil activation test can predict allergic clinical status to peanut and tree nuts in multi-nut-sensitized children and may reduce the need for high-risk OFCs in patients.

Modeling the conversion between specific IgE test platforms for nut allergens in children and adolescents.

BACKGROUND: Multiplex tests allow for measurement of allergen-specific IgE responses to multiple extracts and molecular allergens and have several advantages for large cohort studies. Due to significant methodological differences, test systems are difficult to integrate in meta-analyses/systematic reviews since there is a lack of datasets with direct comparison. We aimed to create models for statistical integration of allergen-specific IgE to peanut/tree nut allergens from three IgE test platforms. METHODS: Plasma from Canadian and Austrian children/adolescents with peanut/tree nut sensitization and a cohort of sensitized, high-risk, pre-school asthmatics (total n = 166) were measured with three R&D multiplex IgE test platforms: Allergy Explorer version 1 (ALEX) (Macro Array Dx), MeDALL-chip (Mechanisms of Development of Allergy) (Thermo Fisher), and EUROLINE (EUROIMMUN). Skin prick test (n = 51) and ImmunoCAP (Thermo Fisher) (n = 62) results for extracts were available in a subset. Regression models (Multivariate Adaptive Regression Splines, local polynomial regression) were applied if >30% of samples were positive to the allergen. Intra-test correlations between PR-10 and nsLTP allergens were assessed. RESULTS: Using two regression methods, we demonstrated the ability to model allergen-specific relationships with acceptable measures of fit (r2  = 94%-56%) for peanut and tree nut sIgE testing at the extract and molecular-level, in order from highest to lowest: Ara h 2, Ara h 6, Jug r 1, Ana o 3, Ara h 1, Jug r 2, and Cor a 9. CONCLUSION: Our models support the notion that quantitative conversion is possible between sIgE multiplex platforms for extracts and molecular allergens and may provide options to aggregate data for future meta-analysis.

Psychosocial impacts of adult strabismus and strabismus surgery: a review of the literature.

BACKGROUND: Patients with strabismus may suffer from both reduced visual functioning and the psychosocial effects of ocular misalignment. For adults with strabismus, there is a strong body of evidence supporting the advantages of surgical restoration of binocular visual function. However, even in the absence of binocular visual potential, patients may benefit from reconstructive surgery to restore an aesthetically acceptable ocular alignment. OBJECTIVE: To review the psychosocial implications of strabismus in the adult population and to summarize the psychosocial impact of surgical correction. METHODS: A Medline search (1946-2013) was conducted to identify the psychosocial implications of strabismus, its effects on employment, and the impact of surgical correction on strabismic adults using the following keywords: strabismus, surgery, quality of life, life style, employment/employability effects, job satisfaction, social support/social isolation/social marginalization, adaptation, stress, depression, family relations, interpersonal relations, friends, and peer group. The search was limited to patients older than 18 years and studies published in English. Of the initial 87 articles for which abstracts were reviewed, 20 studies were of sufficient relevance to be included in the review. RESULTS: Eight studies examined the psychosocial impact of strabismus, 2 of which explored the effect of strabismus on finding partners, and 4 studies examined the effect of strabismus on employment. These studies examined perceptions of both patients with strabismus and the general population. Eight studies evaluated the impact of strabismus surgery. Strabismus was shown to have negative psychosocial functioning and employability in both patients' and the public's perceptions. Surgical correction was associated with long-lasting improvement in psychological and social functioning. CONCLUSIONS: There is a fairly strong body of evidence in the literature to suggest that adult strabismus patients suffer significant social, psychological, and economic effects because of their condition. Surgery can be effective in improving these disadvantages. Clinicians should be aware of the importance of offering surgery to patients with significant strabismus, even in patients for whom it is not expected to improve visual function.

Sirolimus Treatment of an Infant With Intrathoracic Kaposiform Hemangioendothelioma Complicated by Life-threatening Pleural and Pericardial Effusions.

Kaposiform hemangioendothelioma (KHE) is a rare infiltrative vascular tumor that may be associated with Kasabach-Merritt Phenomenon (KMP), which is a consumptive coagulopathy with potentially life-threatening thrombocytopenia. Management of KHE and KMP is challenging, and currently, there are no standardized validated treatment protocols. Mammalian target of rapamycin inhibitors have been shown to be effective in the treatment of KHE. We describe a term male who presented as a diagnostic dilemma with life-threatening pleural and pericardial effusions and severe thrombocytopenia. After extensive work-up the etiology for his condition was determined to be KHE with KMP. The patient was commenced on sirolimus and responded well to therapy with resolution of KMP.

Hematological Malignancies Associated With Primary Immunodeficiency Disorders.

Primary Immunodeficiency disorders (PID) have been increasingly recognized in association with hematologic malignancies. To better appreciate this association, a systemic search of the Ovid MEDLINE database was performed with terms related of hematologic malignancies and all PID described in the 2017 International Union of Immunological Societies Expert Committee for Primary Immunodeficiency. More than 60 PID distinct PID, caused by cell-intrinsic and extrinsic mechanisms were associated with diverse hematologic malignancies. These occurred among all subgroups of PID, including syndromic and non-syndromic combined PID affecting cellular and humoral immunity, predominantly antibody deficiencies and defects of immune regulation. In addition, defects in phagocyte numbers or functions, or in innate immunity were associated with hematologic malignancies. Increased awareness and vigilance for the possibility of malignancy is required when caring for patients with PID.

Postthrombotic syndrome following upper extremity deep vein thrombosis in children.

Despite its relatively estimated high occurrence, the characterization of pediatric upper extremity deep vein thrombosis (UE-DVT) and of UE postthrombotic syndrome (PTS) is still lacking. We investigated the occurrence, characteristics, and predictors of UE-PTS in a cohort of children with objectively confirmed UE-DVT. Patients were analyzed in 3 groups according to DVT pathogenesis and neonatal status: primary (G1), secondary neonates (G2neonates), and non-neonates (G2non-neonates). A total of 158 children (23 G1, 25 G2neonates, and 110 G2non-neonates) were included. The most common triggering factors were effort-related (87%) in G1 and central lines in G2neonates (100%) and in G2non-neonates (92%). PTS scores ≥1, as per the Modified Villalta Scale, were identified in 87% of primary patients, 16% of G2neonates, and 49% of G2non-neonates. Survival analysis showed that the time to PTS score ≥1 significantly differed among group (log-rank test P < .0001). A multivariable logistic regression showed that DVT pathogenesis and imaging-determined degree of thrombus resolution at the end of therapy were independent predictors of a PTS score ≥2. In conclusion, pediatric UE-PTS frequency and severity depend on UE-DVT pathogenesis (primary/secondary) and, within the secondary group, on patient's age. Line-related UE-PTS has a more benign course, particularly in neonates.