Allergy Asthma and Clinical Immunology最新文献

Background: An evidence-based standardized ED asthma care pathway (EDACP) was developed and implemented in Ontario, Canada.

Objective: To determine the impact of EDACP implementation and access to ED asthma management resources and specialists on return ED visits.

Methods: All 173 Ontario hospitals were surveyed regarding their access to community and ED asthma specialists and ED asthma management resources, including EDACP implementation date and status as of August 2017. Survey data were linked to provincial health administrative data to quantify acute health services utilization. A Poisson regression interrupted time series analysis was conducted.

Results: Of the 123 hospitals responding to the survey, 44 (35.8%) had approved the EDACP. Data were analyzed for the 5 years preceding (30,028 asthma visits) and 17 months following (7,916 asthma visits) implementation, with a 3-month implementation black-out period. After controlling for auto-regressive factors, EDACP implementation was associated with a 2% reduction in the absolute rate of return ED visits within 72 h (p = 0.0124), and within 7 days (p = 0.0295) at teaching hospitals. The same effect was not seen at community hospitals. Peak expiratory flow testing (available at 77% of sites) and spirometry (available at 45% of sites) were associated with 34% (p = 0.0071) and 23% (p = 0.028) reductions in the odds of return ED visits within 72 h, respectively.

Conclusion: The positive results from this large-scale effort to implement an evidence-based knowledge translation initiative in diverse settings, suggests there is merit in continuing to invest time and resources to overcome barriers to adoption and implementation of this EDACP.

Background: Atopic dermatitis (AD) is a chronic and inflammatory skin disease which requires continuous self-management by patients and caregivers. Patient education in AD can improve the self-management practices, treatment adherence rates, and clinical outcomes of patients. Patient-reported outcome measures and objective clinical outcome measures have been used to assess the effectiveness of AD patient education interventions, however they have limited use in assessing learning outcomes, such as knowledge. The literature on knowledge outcome measures for AD patient education interventions has not been examined to date. MAIN: We performed a scoping review of the literature on knowledge assessment tools for AD patient education interventions following the PRISMA-ScR framework. Search databases included MEDLINE, Embase, CINAHL, Education Source, Web of Science, Grey Matters, Clinical Trials.gov, and the International Clinical Trials Registry Platform (ICTRP). Of the 3914 articles identified from the search strategy, 20 studies were eligible for data extraction and summarized by narrative synthesis. Most studies were randomised controlled trials originating in the United States, Europe, and Asia, and published in the years of 2003-2023. Researchers commonly evaluated caregivers' knowledge of AD and included assessments of clinical outcome measures. Similar methods were employed for assessing subjective knowledge across studies. Likewise, studies measuring AD patient/caregiver objective knowledge used comparable methods. Multiple-choice and true/false question formats were used in objective knowledge assessments, and Likert-type scales were common for evaluating subjective knowledge. Objective knowledge assessments consisted of more questions than subjective knowledge outcome measures. Content assessed in knowledge outcome measures was relatively consistent across studies. Delivery of subjective and objective AD knowledge assessments was by telephone, in clinic, and/or online. In pre- and post-test study designs, identical knowledge outcome measures were administered.

Conclusion: This scoping review highlights the diverse components of knowledge assessment tools for AD patient education interventions. Further studies on developing and validating high-quality AD knowledge outcome measures are needed for assessing the true effects of patient education interventions on improving patient/caregiver knowledge.

Background: Hereditary angioedema (HAE) is a rare genetic disorder characterized by painful and potentially life-threatening tissue swelling due to a deficiency or dysfunction of the C1 esterase inhibitor protein. Despite the availability of comprehensive on-demand treatment guidelines, compliance to guideline recommendations remains suboptimal, resulting in persisting unmet need.

Methods: This observational, online survey was conducted between September 6, 2022, and October 19, 2022 to understand the behaviors and perspectives of individuals in the US with hereditary angioedema (HAE). Participants were recruited by the US Hereditary Angioedema Association and were eligible if they were US residents with clinician-diagnosed HAE type I or II and had experienced at least one HAE attack. The survey included multiple-choice, rank-order, and scale-based responses using a 5-point Likert scale for agreement and an 11-point Likert scale for anxiety. Statistical analysis was performed using Microsoft Excel, summarizing continuous variables as means, medians, and ranges, and categorical variables as frequency distributions and percentages.

Results: A total of 107 out of 155 participants completed the survey (mean age = 41 years; 80.4% female). Half of the respondents used both prophylaxis and on-demand therapy, while the other half used on-demand therapy only. Icatibant was the most commonly used on-demand treatment (78.5%). The survey revealed that 57% of respondents did not treat all HAE attacks, and only 14% treated attacks immediately. Delays in treatment were common, with a mean time to treatment of 2.4 h, and younger patients were less likely to carry on-demand treatment. Reasons for delaying treatment included the perceived severity of the attack, lack of on-demand treatment availability, and pain associated with treatment. Additionally, 32.7% of respondents experienced the return of an HAE attack after initial treatment, with those delaying treatment more likely to experience recurrence. The survey also found that delayed treatment led to more severe attacks and longer recovery times, impacting work, social activities, and overall quality of life.

Conclusions: Although guidelines recommend early treatment of HAE attacks, many respondents do not treat immediately. This finding underscores the importance of incorporating open patient-physician communication to improve guideline compliance and the management of HAE.

Background: Food protein-induced enterocolitis syndrome (FPIES) is an understudied non-IgE-mediated food allergy, which is distinct from and lacks diagnostic testing akin to IgE testing. FPIES affects infants and toddlers but can persist into adulthood. As there are no extant methods to identify safe foods for FPIES patients, food ingestion trials are performed at home and often lead to reactions and development of food aversions, which may lead to failure-to-thrive and gastric feeding tube requirements. We hypothesized that foods that fail to elicit responses in immune cells of FPIES patients would be safe to ingest, which could support development of a diagnostic method to headstart safe food identification in patients.

Methods: We developed an ex vivo model of FPIES using food-stimulated white blood cells (WBCs) from pediatric FPIES patients and controls by defining a 9-gene panel representative of FPIES ex vivo responses and conducted a single-arm pilot clinical trial.

Results: Myeloid cells of FPIES patients displayed variable individual-specific myeloid cell reactivity patterns (iMCRPs) to different foods. Foods that failed to elicit repsonses in patients' immune cells were safe to ingest with a negative predictive value of 98.5%. This, when utilized in prospective predictions, reduced newly introduced food reaction rates from 19.5 to 0% while increasing food repertoire diversity.

Conclusions: iMCRPs represent a novel and potentially useful tool that associates with safe food ingestion in FPIES patients for foods that fail to elicit immune cell reactions. Trial Registration The trial has been registered at registered at ClinicalTrials.gov # NCT04644783.

Background: Hypereosinophilic syndrome is a group of disorders characterized by organ dysfunction caused by hypereosinophilia, which frequently leads to thromboembolic complications with potentially fatal outcomes. Interleukin-5, a key cytokine that promotes the differentiation and activation of eosinophils, has been identified as a therapeutic target. Anti-interleukin-5 antibody therapy has demonstrated efficacy in reducing eosinophil counts and enabling steroid dose tapering in patients with hypereosinophilic syndrome. This report describes the case of a patient with severe thromboembolism associated with hypereosinophilic syndrome during the acute phase who was successfully treated with benralizumab, an anti-interleukin-5 receptor alpha antibody.

Case presentation: A 22-year-old woman presented with a persistent cough and was diagnosed with eosinophilic pneumonia and portal vein thrombosis. Although eosinophilic pneumonia improved with corticosteroid therapy, thrombotic complications worsened despite additional anticoagulant treatment. The administration of benralizumab led to marked improvement in thrombosis, resulting in clinical recovery.

Conclusions: This case suggests that the early administration of anti-interleukin-5 receptor antibody therapy may be a valuable treatment option for refractory thrombosis.

Background: Perflutren lipid microsphere suspension, sold under the brand name Definity®, is a microbubble ultrasound contrast agent. The microspheres contain octafluoropropane (C₃F₈) gas encapsulated by an outer lipid shell of phospholipids and a polyethylene glycol (PEG)ylated phospholipid. Anaphylaxis to perflutren lipid microsphere is very rare, with only one case report clearly attributing the reaction to the PEG excipient. We report a novel case of anaphylaxis likely caused by a non-PEGylated component of Definity®.

Case presentation: Our patient is a healthy 54-year-old female, who underwent an exercise stress transthoracic echocardiogram using Definity® as an enhancing agent. She experienced anaphylaxis within 15 min of injection. Symptoms resolved after she was treated with diphenhydramine and epinephrine, followed by a systemic corticosteroid and ondansetron in the Emergency Department. The patient underwent allergy testing at our clinic for Definity® and various PEG-containing substances. While all PEG products tested negative, she had positive intradermal tests to Definity®. She also had negative skin prick testing to PEG 8000 and passed an oral challenge to PEG 3350, thus ruling out PEG as the causative agent of anaphylaxis.

Conclusions: Our case report highlights a previously undocumented instance of anaphylaxis to Definity® not caused by PEG. We suspect the reaction to be an IgE-mediated response to a non-PEGylated component of Definity®. An alternative explanation for the reaction could be a complement activation-related pseudoallergy. This report provides critical information to physicians on the potential risks of using Definity® and contributes to growing research surrounding the profile of Definity®.

Background: Pitaya, commonly known as dragon fruit, is increasingly available and has allergenic potential. Pollens have been found to have cross-reactivity and thus induce allergies to several fruits, however, to our knowledge this is first report of pitaya anaphylaxis in a patient without co-sensitization to other fruit or environmental allergens.

Case presentation: A 26-year-old male presented to the emergency department with anaphylaxis after consumption of pitaya (dragon fruit). He had no prior history of atopy. Epicutaneous skin testing demonstrated positive to pitaya and negative to all other cross-reactive food and environmental allergens, suggesting his pitaya allergy did not derive from cross-sensitization.

Conclusions: Our case is unique in demonstrating the potential for pitaya allergy to occur independent of other allergies and cross-sensitization. Future research is warranted into suspected allergenic proteins in pitaya and quantifying their structural similarity to other known allergens.

Background: Hyper-IgE Syndrome, also known as Job's syndrome, is a rare primary immunodeficiency disorder characterized by recurrent infections and elevated levels of immunoglobulin E. While respiratory and systemic manifestations have been more emphasized, dermatological manifestations in Hyper-IgE Syndrome also play a significant role in disease presentation.

Methods: This narrative review explores the dermatologic presentations of Hyper-IgE Syndrome in pediatric populations, including descriptions, associated symptoms/findings, and available treatment options.

Results and conclusion: Neonatal rash, mucocutaneous candidiasis, noma neonatorum, psoriasis, cold staphylococcal abscesses, and candida onychomycosis are among the dermatological manifestations of Hyper-IgE Syndrome. Each manifestation has unique characteristics and treatment considerations, necessitating accurate recognition and diagnosis for effective management. Optimal treatment strategies involve a combination of supportive care, topical/systemic therapies, antifungal medications, and surgical interventions when necessary. Further research is needed to enhance our understanding of these manifestations and evaluate treatment modalities for individuals affected by Hyper-IgE Syndrome.

Background: X-linked inhibitor of apoptosis (XIAP) deficiency is a rare inborn error of immunity which occurs secondary to mutations in the XIAP/BIRC4 gene. Disease onset usually manifests within the first few years of life, and is associated with a spectrum of clinical features, secondary to immune dysregulation. Males typically present with refractory chronic colitis, hemophagocytic lymphohistiocytosis, and severe and/or recurrent infections. Laboratory analysis may reveal hypogammaglobulinemia and cytopenias. At present, the only curative treatment is allogenic hematopoietic stem cell transplantation.

Case presentation: A 24-year-old gentleman, immigrant from the Democratic Republic of Congo, was referred to outpatient immunology for evaluation of an inborn error of immunity given a past medical history significant for refractory fistulizing Crohn's disease, arthritis, liver abscesses, prior disseminated tuberculosis, anemia, and recurrent infections. He had been asymptomatic throughout his childhood and adolescence, with no infections or symptoms of inflammatory disease until the age of 19, when he was diagnosed with Crohn's disease. He was soon after admitted to hospital and was diagnosed with hemophagocytic lymphohistiocytosis. Primary immunodeficiency gene panel testing revealed a nonsense variant XIAP c833C > G p.(Ser278*), which generates a premature stop codon at exon 2 (of total 7 exons). On flow cytometry analysis, XIAP protein expression was significantly reduced, confirming the diagnosis of XIAP deficiency.

Conclusion: This is one of the only documented reports of a patient with XIAP deficiency, presenting with symptom-onset in adulthood. This case highlights the need to maintain a high index of suspicion for XIAP deficiency in patients with the appropriate clinical presentation, despite advanced age of presentation.

Background: Allogenic hematopoietic stem cell transplantation (HSCT) is the optimal treatment of hematologic diseases and various malignancies. Development of allergic disease in a transplant patient has been reported.

Case presentations: A 49-year-old male with no history of atopy underwent two allogenic HSCTs for aplastic anemia from his brother with severe atopic dermatitis 11 years ago. The patient developed eczema on whole body and an elevated peripheral blood eosinophil count of 5775 cells/µL at 3 months after the second HSCT. Despite prolonged treatment with systemic corticosteroids and immunomodulators the skin rash and elevated blood eosinophil count persisted. However, after 4 months of dupilumab therapy, the patient showed near-complete clearance of symptoms. The sustained clinical improvement was observed during 36 months treatment without adverse drug reactions.

Conclusions: Although rare, atopic dermatitis can occur after HSCT, and dupilumab may be safe and effective for refractory conditions.