Allergy and asthma proceedings最新文献

Background: Various formulations of dog allergen extracts, including conventional dog (also known as dog epithelium) and acetone precipitated (AP) dog, have been used for skin-prick testing (SPT), with AP dog showing improved antigen content but experiencing stability issues due to precipitant formation. Ultrafiltered (UF) dog extract has been developed to address these concerns by offering comparable allergen content to AP dog. This study retrospectively compared UF dog with conventional dog and AP dog in SPT. Objective: To compare the efficacy of UF dog extract with conventional dog and AP dog extracts in detecting dog sensitization through SPT. Methods: Retrospective analysis of SPT results from a single U.S. allergy clinic was conducted. Tests performed between October 2022 and March 2024 were included. Primary and secondary outcomes were analyzed by using descriptive statistics and statistical tests. Results: UF dog, AP dog, and conventional dog showed positivity rates of 24.2%, 23.5%, and 16.3%, respectively. UF dog demonstrated significantly higher average wheal and erythema sizes compared with conventional dog and AP dog, but UF dog was not statistically different from AP dog in terms of test positivity. Conclusion: UF dog extract showed comparable number of positive tests to AP dog and a greater number of positive tests to conventional dog. Results of the study suggest UF dog as a viable alternative to AP dog, which offered improved stability and similar test responses. Further research with larger sample sizes is recommended to confirm these findings.

Background: Symptomatic dermographism (SD) is the most common form of chronic inducible urticaria. SD disease activity increases with food intake in adult patients. Whether this is also so in children with SD is currently unknown. Objective: To assess children with SD for their disease activity by standardized provocation testing before and after eating. Methods: We subjected 44 children with SD (29 girls; median [interquartile range] age 12.5 years [8.3-15 years]), before and after eating, to standardized skin provocation testing with a dermographometer. Dermographometer scores were calculated based on responses evaluated at 1-minute intervals for 10 minutes and recorded as negative (-) or positive (+ to ++++). Clinical characteristics and urticaria control test scores were documented. Results: Dermographometer scores before eating were 2.3 of 4 on average and inversely correlated with urticaria control test scores. Dermographometer scores were higher after eating than before eating. Of 44 children with SD, 35 had increased dermographometer scores after eating and 9 patients had a postprandial increase of ≥1 point. Eating-induced increases in dermographometer scores were linked to earlier whealing in 17 of 35 patients, and differences in preprandial versus postprandial dermographometer responses were more pronounced at earlier than later time points after testing. Conclusion: Disease activity, as assessed by provocation testing, is increased in most pediatric patients with SD after eating. Future studies should explore the prevalence of food-exacerbated SD in larger pediatric SD populations. Most pediatric patients with symptomatic dermographism have higher disease activity, assessed by provocation testing, after eating as compared to before eating. Standardized provocation testing and trigger threshold assessments in children with symptomatic dermographism should be performed before and after eating. Knowledge of food-exacerbated disease may help patients with the management of their symptomatic dermographism.

Shared decision-making (SDM) requires a clear-eyed view of evidence certainty, context, and equipoise in clinical care. This paradigm of care builds on the foundational ethical principle of patient autonomy, further leveraging beneficence, nonmaleficence, and justice to provide bespoke care in the appropriate clinical setting. When evidence is carefully evaluated together with acceptability and feasibility, equity, cost-effectiveness, resources, and patient preferences, an individualized assessment of the trade-off between possible benefits and harms can optimize patient management. In the setting of a conditional recommendation, it is appropriate to engage in SDM with patient partners, to the extent each patient is willing and able to engage in the SDM process. Three conversations inform SDM and include team talk, option talk, and decision talk with discussion of the plan of care. During these conversations, clear communication strategies that are specific, measurable, achievable, realistic, time sensitive, and provide assessment of absolute (not just relative) risk are important to provide necessary education to patient partners. Follow-up is key to ensure that decisions lead to effective treatment. Through this process, it is necessary to minimize cognitive overload and promote a minimally disruptive medicine approach. The acronym "HOW" promotes a holistic appraisal of evidence in context, open-minded teamwork with patients and families, and willingness to be a listening presence while serving as a partner and guide and appreciating the multidimensional and unique nature of each individual. SDM is and will continue to remain a cornerstone of appropriate medical care in settings of clinical equipoise.

In contrast to inborn errors of immunity (IEI), which are inherited disorders of the immune system that predispose to infections, malignancy, atopy, and immune dysregulation, secondary immunodeficiencies and immune dysregulation states (SID) are acquired impairments in immune cell function and/or regulation, and may be transient, reversible, or permanent. SIDs can derive from a variety of medical comorbidities, including protein-losing conditions, malnutrition, malignancy, certain genetic syndromes, prematurity, and chronic infections. Medications, including immunosuppressive and chemotherapeutic drugs, can have profound effects on immunity and biologic agents used in rheumatology, neurology, and hematology/oncology practice are increasingly common causes of SID. Iatrogenic factors, including surgical procedures (thymectomy, splenectomy) can also contribute to SID. A thorough case history, medication review, and laboratory evaluation are necessary to identify the primary driver and determine proper management of SID. Careful consideration should be given to whether a primary IEI could be contributing to autoimmunity, malignancy, and posttreatment complications (e.g., antibody deficiency). SID management consists of addressing the driving condition and/or removing the offending agent if feasible. If SID is suspected to be permanent, then antibiotic prophylaxis, additional immunization, and immunoglobulin replacement should be considered.

Primary antibody deficiencies are characterized by the inability to effectively produce antibodies and may involve defects in B-cell development or maturation. Primary antibody deficiencies can occur at any age, depending on the disease pathology. Certain primary antibody deficiencies affect males and females equally, whereas others affect males more often. Patients typically present with recurrent sinopulmonary and gastrointestinal infections, and some patients can experience an increased risk of opportunistic infections. Multidisciplinary collaboration is important in the management of patients with primary antibody deficiencies because these patients require heightened monitoring for atopic, autoimmune, and malignant comorbidities and complications. The underlying genetic defects associated with many primary antibody deficiencies have been discovered, but, in some diseases, the underlying genetic defect and inheritance are still unknown. The diagnosis of primary antibody deficiencies is often made through the evaluation of immunoglobulin levels, lymphocyte levels, and antibody responses. A definitive diagnosis is obtained through genetic testing, which offers specific management options and may inform future family planning. Treatment varies but generally includes antibiotic prophylaxis, vaccination, and immunoglobulin replacement. Hematopoietic stem cell transplantation is also an option for certain primary antibody deficiencies.

Primary immunodeficiencies, also commonly called inborn errors of immunity (IEI), are commonly due to developmental or functional defects in peripheral blood cells derived from hematopoietic stem cells. In light of this, for the past 50 years, hematopoietic stem cell transplantation (HSCT) has been used as a definitive therapy for IEI. The fields of both clinical immunology and transplantation medicine have had significant advances. This, in turn, has allowed for both an increasing ability to determine a monogenic etiology for many IEIs and an increasing ability to successfully treat these patients with HSCT. Therefore, it has become more common for the practicing allergist/immunologist to diagnose and manage a broad range of patients with IEI before and after HSCT. This review aims to provide practical guidance for the clinical allergist/immunologist on the basics of HSCT and known outcomes in selected forms of IEI, the importance of pre-HSCT supportive care, and the critical importance of and guidance for life-long immunologic and medical monitoring of these patients.

Immunoglobulin replacement is donor-derived pooled immunoglobulin G, which provides passive immunity to patients with antibody deficiency or dysfunction. It may be administered via either intravenous or subcutaneous routes. Intravenous immunoglobulin is administered at higher doses every 3-4 weeks, whereas most forms of subcutaneous immunoglobulin are administered at lower doses, usually every 1-2 weeks. Benefits and risks, including adverse effects, convenience, and cost vary according to route of administration. Immunoglobulin products also differ in their composition, so patient-specific comorbidities are important to consider when selecting an immunoglobulin product. We discuss adverse effects associated with immunoglobulin therapy, their associated risk factors, treatment, and ways to mitigate these risks. Finally, the laboratory monitoring and vaccination recommendations for patients on immunoglobulin replacement therapy are reviewed.

Immunodeficiency disorders pose substantial burdens on the health-care system and the patients affected. Broadly, immunodeficiencies can be divided into primary immunodeficiency disorders (PIDDs) and secondary immunodeficiency disorders. This review will focus on PIDDs. The overall prevalence for PIDDs is estimated to be ∼1-2% of the population but may be underestimated due to underdiagnosis of these conditions. PIDDs affect males slightly more often than females. The mortality rates differ based on the specific condition but can be extremely high if the condition is left undiagnosed or untreated. The most common causes of death are infections, respiratory complications, and cancers (e.g., lymphoma). Comorbidities and complications include infection, chronic lung disease, granulomatous lymphocytic interstitial lung disease, and autoimmune disorders. The disease burden of patients with common variable immunodeficiency (CVID) is estimated to be greater than patients with diabetes mellitus and chronic obstructive pulmonary disease. PIDDs have a serious impact on the quality of life of the patients, including sleep disturbance, anxiety, and social participation as well as other psychosocial burdens associated with these disorders. The financial cost of PIDDs can be substantial, with the cost of untreated CVID estimated to be $111,053 per patient per year. Indirect costs include productivity loss and time lost due to infusion and hospital visits. Secondary immunodeficiency is not fully discussed in this review but likely contributes equally to the burden of overall immunodeficiency disorders. Management of patients with PIDDs should use a comprehensive approach, including medical, nursing, psychiatric, and quality of life, to improve the outcome.

The primary immunodeficiency diseases are often accompanied by autoimmunity, autoinflammatory, or aberrant lymphoproliferation. The paradoxical nature of this association can be explained by the multiple cells and molecules involved in immune networks that interact with each other in synergistic, redundant, antagonistic, and parallel arrangements. Because progressively more immunodeficiencies are found to have a genetic etiology, in many cases, a monogenic pathology, an understanding of why immunodeficiency is really an immune dysfunction becomes evident. Understanding the role of specific genes allows us to better understand the complete nature of the inborn error of immunity (IEI); the latter is a term generally used when a clear genetic etiology can be discerned. Autoimmune cytopenias, inflammatory bowel disease, autoimmune thyroiditis, and autoimmune liver diseases as well as lymphomas and cancers frequently accompany primary immunodeficiencies, and it is important that the practitioner be aware of this association and to expect that this is more common than not. The treatment of autoimmune or immunodysregulation in primary immunodeficiencies often involves further immunosuppression, which places the patient at even greater risk of infection. Mitigating measures to prevent such an infection should be considered as part of the treatment regimen. Treatment of immunodysregulation should be mechanism based, as much as we understand the pathways that lead to the dysfunction. Focusing on abnormalities in specific cells or molecules, e.g., cytokines, will become increasingly used to provide a targeted approach to therapy, a prelude to the success of personalized medicine in the treatment of IEIs.