Clinical updates in inborn errors of immunity: a focus on the noninfectious clinical manifestations.

PURPOSE OF REVIEW: In the last 5 years, several new inborn errors of immunity (IEI) have been described, especially in the areas of immune dysregulation and autoinflammation. As a result, the clinical presentation of IEIs has broadened. We review the heterogeneous presentation of IEIs and detail several of the recently described IEIs with a focus on the noninfectious manifestations commonly seen. RECENT FINDINGS: IEIs may present with early onset and/or multiple autoimmune manifestations, increased risk for malignancy, lymphoproliferation, severe atopy, autoinflammation and/or hyperinflammation. Because of this, patients can present to a wide array of providers ranging from primary care to various pediatric subspecialists. The International Union of Immunological Societies (IUIS) expert committee has created a phenotypic classification of IEIs in order to help clinicians narrow their evaluation based on the laboratory and clinical findings. SUMMARY: Both primary care pediatricians and pediatric subspecialists need to be aware of the common clinical features associated with IEI and recognize when to refer to allergy-immunology for further evaluation. Early diagnosis can lead to earlier treatment initiation and improve clinical outcomes for our patients.

Anaphylaxis: A 2023 practice parameter update.

This practice parameter update focuses on 7 areas in which there are new evidence and new recommendations. Diagnostic criteria for anaphylaxis have been revised, and patterns of anaphylaxis are defined. Measurement of serum tryptase is important for diagnosis of anaphylaxis and to identify underlying mast cell disorders. In infants and toddlers, age-specific symptoms may differ from older children and adults, patient age is not correlated with reaction severity, and anaphylaxis is unlikely to be the initial reaction to an allergen on first exposure. Different community settings for anaphylaxis require specific measures for prevention and treatment of anaphylaxis. Optimal prescribing and use of epinephrine autoinjector devices require specific counseling and training of patients and caregivers, including when and how to administer the epinephrine autoinjector and whether and when to call 911. If epinephrine is used promptly, immediate activation of emergency medical services may not be required if the patient experiences a prompt, complete, and durable response. For most medical indications, the risk of stopping or changing beta-blocker or angiotensin-converting enzyme inhibitor medication may exceed the risk of more severe anaphylaxis if the medication is continued, especially in patients with insect sting anaphylaxis. Evaluation for mastocytosis, including a bone marrow biopsy, should be considered for adult patients with severe insect sting anaphylaxis or recurrent idiopathic anaphylaxis. After perioperative anaphylaxis, repeat anesthesia may proceed in the context of shared decision-making and based on the history and results of diagnostic evaluation with skin tests or in vitro tests when available, and supervised challenge when necessary.

An update on the impact of food allergy on anxiety and quality of life.

PURPOSE OF REVIEW: Food allergies have become more common, and management involves dietary avoidance that can impair quality of life. Patients and families must manage the daily risk of anaphylaxis at each meal. The purpose of this review is to describe the impact of food allergies on quality of life and to provide an update on new developments in food allergy management, particularly peanut allergy. RECENT FINDINGS: Food allergy requires careful avoidance of common and ubiquitous dietary allergens. Living with food allergy is associated with annual economic costs in excess of $4000 per child, in addition to risks of anxiety and depressive symptoms. An expert panel sponsored by the 2017 National Institute of Allergy and Infectious Diseases published addendum guidelines for the prevention of peanut allergy, which suggest three separate approaches to peanut protein introduction for infants at various levels of risk. SUMMARY: Clinicians must be aware of underappreciated burdens faced by children and families with food allergies. Management involves a partnership between primary and specialty care. Mitigation strategies to improve quality of life for patients include efforts to avoid overdiagnosis in synergy with balanced counseling about the risks of food allergies. Experimental food allergen desensitization can improve quality of life but remains investigational at this time. For patients with significant anxiety, interdisciplinary management involving professional counseling may be helpful. Risk stratification and early introduction of peanut protein can help prevent the development of peanut allergy.

Prevention of food allergy in infancy: the role of maternal interventions and exposures during pregnancy and lactation.

There is increased focus on the role of maternal interventions in the prevention of food allergy in infancy. There is no role for maternal dietary modifications during pregnancy or lactation, such as allergen avoidance, as a means of infant allergy prevention. Although exclusive breastfeeding is the recommended infant nutrition source globally, the effect of breastfeeding on infant allergy prevention remains unclear. There is emerging evidence that irregular cow's milk exposure (ie, infrequent formula supplementation) might increase the risk of cow's milk allergy. Although further studies are required, there is also emerging evidence that maternal peanut ingestion during breastfeeding along with early peanut introduction in infancy might have a preventive role. The effect of maternal dietary supplementation with vitamin D, omega-3, and prebiotics or probiotics remains unclear.

Provision of Food Allergy Care in the United Kingdom and United States: Current Issues and Future Directions.

Food allergy (FA) is a growing issue worldwide. The United Kingdom and United States are high-income, industrialized countries with reported increases in FA prevalence over the past few decades. This review compares delivery of FA care in the United Kingdom and United States and each country's response to the heightened demand and disparities for FA services. In the United Kingdom, allergy specialists are scarce and general practitioners (GPs) provide most allergy care. Whereas the United States has more allergists per capita than the United Kingdom, there is still a shortage of allergy services owing to the greater reliance on specialist care for FA in America and wide geographic variation in access to allergist services. Currently, generalists in these countries lack the specialty training and equipment to diagnose and manage FA optimally. Moving forward, the United Kingdom aims to enhance training for GPs so they may provide better quality frontline allergy care. In addition, the United Kingdom is implementing a new tier of semi-specialized GPs and increasing cross-center collaboration through clinical networks. The United Kingdom and United States aim to increase the number of FA specialists, which is critical at a time of rapidly expanding management options for allergic and immunologic diseases requiring clinical expertise and shared decision-making to select appropriate therapies. While these countries aim to grow their supply of quality FA services actively, further efforts to build clinical networks and perhaps recruit international medical graduates and expand telehealth services are necessary to reduce disparities in access to care. For the United Kingdom in particular, increasing quality services will require additional support from the leadership of the centralized National Health Service, which remains challenging.

COVID-19: Pandemic Contingency Planning for the Allergy and Immunology Clinic.

In the event of a global infectious pandemic, drastic measures may be needed that limit or require adjustment of ambulatory allergy services. However, no rationale for how to prioritize service shut down and patient care exists. A consensus-based ad-hoc expert panel of allergy/immunology specialists from the United States and Canada developed a service and patient prioritization schematic to temporarily triage allergy/immunology services. Recommendations and feedback were developed iteratively, using an adapted modified Delphi methodology to achieve consensus. During the ongoing pandemic while social distancing is being encouraged, most allergy/immunology care could be postponed/delayed or handled through virtual care. With the exception of many patients with primary immunodeficiency, patients on venom immunotherapy, and patients with asthma of a certain severity, there is limited need for face-to-face visits under such conditions. These suggestions are intended to help provide a logical approach to quickly adjust service to mitigate risk to both medical staff and patients. Importantly, individual community circumstances may be unique and require contextual consideration. The decision to enact any of these measures rests with the judgment of each clinician and individual health care system. Pandemics are unanticipated, and enforced social distancing/quarantining is highly unusual. This expert panel consensus document offers a prioritization rational to help guide decision making when such situations arise and an allergist/immunologist is forced to reduce services or makes the decision on his or her own to do so.

A Phased Approach to Resuming Suspended Allergy/Immunology Clinical Services.

In early 2020, the first US and Canadian cases of the novel severe acute respiratory syndrome coronavirus 2 infection were detected. In the ensuing months, there has been rapid spread of the infection. In March 2020, in response to the virus, state/provincial and local governments instituted shelter-in-place orders, and nonessential ambulatory care was significantly curtailed, including allergy/immunology services. With rates of new infections and fatalities potentially reaching a plateau and/or declining, restrictions on provision of routine ambulatory care are lifting, and there is a need to help guide the allergy/immunology clinician on how to reinitiate services. Given the fact that coronavirus disease 2019 will circulate within our communities for months or longer, we present a flexible, algorithmic best-practices planning approach on how to prioritize services, in 4 stratified phases of reopening according to community risk level, as well as highlight key considerations for how to safely do so. The decisions on what services to offer and how fast to proceed are left to the discretion of the individual clinician and practice, operating in accordance with state and local ordinances with respect to the level of nonessential ambulatory care that can be provided. Clear communication with staff and patients before and after all changes should be incorporated into this new paradigm on continual change, given the movement may be forward and even backward through the phases because this is an evolving situation.