Journal of Allergy and Clinical Immunology-In Practice最新文献

Background: Oral immunotherapy (OIT) for food allergy has been largely studied in older children within the context of clinical trials, and its availability has historically been limited for younger patients with food allergy. Data have shown that the most impact may actually be seen with the use of OIT in younger infants and toddlers.

Objective: To evaluate the safety and feasibility of OIT in subjects 24 months and younger in a real-world setting using commercially available food products.

Methods: This was a retrospective study of subjects 24 months and younger initiated on OIT for peanut, tree nut, or sesame allergy within the Scripps Clinic allergy department. Medical records were reviewed for data regarding initial oral food challenges, OIT, and adverse outcomes.

Results: Fifty-two subjects 24 months and younger were initiated on OIT. Most subjects (84.6%) were on single-food OIT, and some (15.4%) were on multifood OIT. No increased adverse outcomes were observed on multifood OIT. Of the 59 initial oral food challenges, objective reactions occurred during 42 challenges, most being low-grade reactions. During initial oral food challenges, 86.1% of peanut-allergic children tolerated 1/8 of 1 Bamba stick with no reaction. Most subjects (73.1%) updosed at home, and most (51.9%) had no reactions while updosing. Some had low-grade cutaneous reactions, none requiring epinephrine or emergency evaluation.

Conclusions: OIT in infants is safe and feasible to perform in a real-world setting using commercially available food products with at-home updosing, thus increasing the availability of OIT for patients.

Background: Hymenoptera venom is one of the most frequent causes of anaphylaxis. Studies from adults indicate the clinical profiles and risk factors of Hymenoptera venom-induced anaphylaxis (VIA). Much less is known about pediatric VIA.

Objective: To understand elicitor- and age-related factors determining pediatric VIA by analyzing data from the anaphylaxis registry.

Methods: We selected pediatric VIA, pediatric food-induced anaphylaxis (FIA), and adult VIA cohorts from the anaphylaxis registry and performed a comparative data analysis regarding elicitors, symptoms, and management.

Results: We identified 725 pediatric patients with VIA, 3,149 with pediatric FIA, and 5,534 with adult VIA. In pediatric VIA, boys were more frequently affected, atopy was not increased, and the onset of the reaction after exposure was fast (≤30 min; 91%) compared with pediatric FIA. Symptoms in pediatric VIA were age dependent, and although respiratory symptoms occurred most frequently besides skin symptoms in both pediatric patients with VIA and FIA, cardiovascular symptoms were more frequently reported in pediatric patients with VIA than pediatric patients with FIA. The analysis of pediatric versus adult VIA revealed clear differences in the frequency of involved organ systems (skin: 93% vs 78%; respiratory: 77% vs 64%; and cardiovascular: 61% vs 85%). For both pediatric and adult VIA, the rates of adrenaline application by a professional were low (29% vs 31%) but hospitalization rates were higher in children than in adults (61% vs 42%). Venom immunotherapy was frequently initiated regardless of age (78% each).

Conclusions: Pediatric VIA is more frequent in boys, symptoms are age dependent, and hospitalization is often required. Adrenaline should be applied according to current guidelines. Venom immunotherapy is an important treatment option in pediatric VIA and should be considered in severely affected children.

Insect stings can cause large local reactions (LLRs) that are IgE-mediated and associated with considerable morbidity. A risk for systemic reactions including anaphylaxis to subsequent stings has been reported and is often noted by patients and health care providers. Guidelines do not recommend venom immunotherapy (VIT) for LLRs based on the relatively low risk of anaphylaxis, but this is debated in this review. On the pro side: the risk of anaphylaxis may be higher than reported in the limited literature, especially in patients who had only 1 LLR; new species with more potent stings are spreading into new areas; the quality of life can be markedly impaired by LLRs; and VIT is generally safe and highly effective. On the con side: LLRs are benign, stings occur infrequently, VIT has significant cost, systemic reactions occur more often to VIT than to stings in patients with LLRs, and Food and Drug Administration approval and published guidelines do not recommend VIT for LLRs. In practice, shared decision-making is appropriate to incorporate knowledge of the natural history and known high-risk factors in the context of the patient's personal values and preferences.

Background: Food allergy (FA) affects around 5.6 million children in the United States, conferring risk for negative impacts on growth and psychosocial functioning. While evidence suggests a higher prevalence of feeding difficulties in children with FA, the link between FA and feeding dysfunction remains unclear.

Objective: To investigate feeding problems in children with parent-reported IgE-mediated FA and/or pediatric feeding disorder (PFD) compared with healthy children in a community-based sample and explore factors associated with feeding problems in children with FA.

Methods: A matched cross-sectional cohort study used survey data from 352 parents of children aged 6 months to 7 years who reported a diagnosed IgE-mediated FA and/or PFD (50 with FA only, 67 with FA and PFD, and 235 with PFD only). Healthy children were matched based on age, sex, race, and socioeconomic factors. Feeding problems were assessed using the Pediatric Eating Assessment Tool. Matched-pairs generalized linear regressions compared each cohort and explored factors associated with feeding problems in children with FA.

Results: Children with FA exhibited significantly higher feeding problems compared with healthy children. Children with a diagnosed PFD had more severe feeding problems, irrespective of comorbid FA. Factors linked with feeding problems in children with FA included older age, non-White race, increased medical comorbidity, and limited dietary variety.

Conclusions: This study adds to evidence indicating a higher prevalence of feeding problems in children with FA. Clinicians treating FA patients should routinely screen for feeding and growth concerns and consider referral to multidisciplinary feeding programs as needed.

Background: The noncanonical NF-κB2 (nuclear factor kappa B subunit 2) pathway is integral in regulating immunologic responses, supervising immune function, development, and homeostasis. NFKB2 encodes the cytoplasmic precursor p100, which undergoes processing of its inhibitory C-terminal half to generate p52. Impeding C-terminal defects are well established to cause primary immunodeficiency disorder. In contrast, the mechanism of truncating N-terminal defects remains obscure.

Objective: We characterized clinical phenotypes associated with 3 distinct protein-defect types: (1) early truncations: typically occurring N-terminal relative to the nuclear localization sequence and affecting the Rel homology domain, predicting p100 expression to be halved and subsequent p52 generation by processing to be diminished; (2) central truncations: mainly affecting the ARD and predicting immediate expression of p52-like proteins and a 50% reduction of p100; and (3) C-terminal phosphorylation-/ubiquitination domain defects: causing expression of nonprocessable p100 with retained IκB-like activity and subsequently reducing generation of p52.

Methods: We performed literature research on PubMed, Clinvar, and Human Gene Mutation Database collecting clinical and immunologic data on NFKB2 patients, focusing on comparing protein-defect-specific impacts.

Results: The highest prevalence of early-onset primary immunodeficiency disorder and antibody deficiency occurred in the CTD-defect group. In addition, endocrinological abnormalities and T-cell-mediated autoimmunity were common and frequently required immunosuppression. An extensive immunologic workup revealed patients with C-terminal defects to have pan-hypogammaglobulinemia and reduced specific antibody responses and markedly impaired B-cell differentiation, but normal to elevated T-cell counts. In contrast, pathogenic NFKB2 variants causing central or early-truncating protein defects were only partially penetrant, with ameliorated symptoms and diminished T-cell-mediated autoimmunity.

Conclusions: Our work defines a clear genotype-phenotype correlation for NFKB2 mutations.