Vivian Hernandez-Trujillo

Section on allergy and immunology

Founded in 1948, the Section on Allergy and Immunology is dedicated to ensuring that children receive the highest quality of allergy and immunology care. To accomplish its mission, the Section provides a number of educational, training, and research programs and continually advocates for improved allergy and immunology care and services. The Section sponsors educational programs for both pediatric generalists and subspecialists at the American Academy of Pediatrics (AAP) National Conference and Exhibition (NCE) each fall and at the American Academy of Allergy Asthma & Immunology annual meeting each spring. The Section’s other educational endeavors include this annual “Best Articles Relevant to Pediatric Allergy and Immunology” supplement to Pediatrics, Visiting Professor Program, Pediatric Asthma Speaker’s Kit, online continuing medical education course on “asthma gadgets,” electronic quality improvement in practice program on asthma diagnosis and management (Education in Quality Improvement for Pediatric Practice [eQIPP], which meets the American Board of Pediatrics maintenance-ofcertification criteria), school nurse allergy tool kit, and a number of public education materials. The Section is also active in contributing to educational programs and resources such as AAP News, educational brochures, clinical reports, and many other endeavors. To support training and promote research in pediatric allergy and immunology, the Section awards travel grants to residents and training fellows to participate and present cases at the AAP NCE and provides outstanding abstract awards for training fellows and junior faculty for presentation at the American Academy of Allergy Asthma & Immunology annual meeting. In close collaboration with other subspecialty societies, the Section is actively involved with initiatives to improve subspecialty education such as the American Board of Allergy and Immunology maintenance-of-certification requirements. Section members represent the AAP in national and government conferences and provide input on federal legislation on behalf of the AAP. For more information on all AAP allergy and immunology resources and initiatives, visit www.aap.org/sections/allergy. The reviews contained in the 2011 synopsis were written by Fellows of the AAP Section on Allergy and Immunology and fellows in allergy and immunology training programs who contributed reviews with their mentors. The editor selected the journals to be reviewed on the basis of the likelihood that they would contain articles on allergy and immunology that would be of value and interest to the pediatrician. Each journal was assigned to a voluntary reviewer who was responsible for selecting articles and writing reviews of their articles. Only articles of original research were selected for review. Final selection of the articles to be included was made by the editor. The 2010–2011 journals chosen for review were Allergy, American Journal of Asthma & Allergy for Pediatricians, Archives of Pediatric and Adolescent Medicine, American Journal of Medicine, American Journal of Respiratory and Critical Care Medicine, Annals of Allergy, Asthma, and Immunology, Annals of Internal Medicine, Archives of Disease in Childhood, Archives of Internal Medicine, Blood, British Journal of Dermatology, British Medical Journal, Chest, Clinical and Experimental Allergy, Clinical Pharmacology and Therapeutics, Critical Care Medicine, European Journal of Pediatrics, European Respiratory Journal, Immunology, Journal of Allergy and Clinical Immunology, Journal of the American Academy of Dermatology, Journal of the American Medical Association, Journal of Applied Physiology, Journal of Experimental Medicine, Journal of Immunology, Journal of Infectious Diseases, Journal of Pediatric Gastroenterology and Nutrition, Journal of Pediatrics, Journal of Pharmacology and Experimental Therapeutics, Lancet, Nature, New England Journal of Medicine, Pediatrics, Medicine, Pediatric Allergy and Immunology, Pediatric Asthma, Allergy & Immunology, Pediatric Dermatology, Pediatric Infectious Disease Journal, and Science. The editor and the Section on Allergy and Immunology gratefully acknowledge the work of the reviewers and their trainees who assisted. The reviewers were Stuart L. Abramson, MD, PhD, Sugar Land, TX; James R. Banks, MD, Arnold, MD; Theresa A. Bingemann, MD, Rochester,

Integration of retroviral vectors induces minor changes in the transcriptional activity of T cells from ADA-SCID patients treated with gene therapy

Gene transfer into hematopoietic stem cells by gamma-retroviral vectors (RVs) is an effective treatment for inherited blood disorders, although potentially limited by the risk of insertional mutagenesis. We evaluated the genomic impact of RV integration in T lymphocytes from adenosine deaminase-deficient severe combined immunodeficiency (ADA-SCID) patients 10 to 30 months after infusion of autologous, genetically corrected CD34(+) cells. Expression profiling on ex vivo T-cell bulk population revealed no difference with respect to healthy controls. To assess the effect of vector integration on gene expression at the single-cell level, primary T-cell clones were isolated from 2 patients. T-cell clones harbored either 1 (89.8%) or 2 (10.2%) vector copies per cell and displayed partial to full correction of ADA expression, purine metabolism, and T-cell receptor-driven functions. Analysis of RV integration sites indicated a high diversity in T-cell origin, consistently with the polyclonal T-cell receptor-Vbeta repertoire. Quantitative transcript analysis of 120 genes within a 200-kb window around RV integration sites showed modest (2.8- to 5.2-fold) dysregulation of 5.8% genes in 18.6% of the T-cell clones compared with controls. Nonetheless, affected clones maintained a stable phenotype and normal in vitro functions. These results confirm that RV-mediated gene transfer for ADA-SCID is safe, and provide crucial information for the development of future gene therapy protocols. The trials described herein have been registered at http://www.clinicaltrials.gov as #NCT00598481 and #NCT00599781.

Recommendations for live viral and bacterial vaccines in immunodeficient patients and their close contacts

The present uncertainty of which live viral or bacterial vaccines can be given to immunodeficient patients and the growing neglect of societal adherence to routine immunizations has prompted the Medical Advisory Committee of the Immune Deficiency Foundation to issue recommendations based on published literature and the collective experience of the committee members. These recommendations address the concern for immunodeficient patients acquiring infections from healthy subjects who have not been immunized or who are shedding live vaccine-derived viral or bacterial organisms. Such transmission of infectious agents can occur within the hospital, clinic, or home or at any public gathering. Collectively, we define this type of transmission as close-contact spread of infectious disease that is particularly relevant in patients with impaired immunity who might have an infection when exposed to subjects carrying vaccine-preventable infectious diseases or who have recently received a live vaccine. Immunodeficient patients who have received therapeutic hematopoietic stem transplantation are also at risk during the time when immune reconstitution is incomplete or while they are receiving immunosuppressive agents to prevent or treat graft-versus-host disease. This review recommends the general education of what is known about vaccine-preventable or vaccine-derived diseases being spread to immunodeficient patients at risk for close-contact spread of infection and describes the relative risks for a child with severe immunodeficiency. The review also recommends a balance between the need to protect vulnerable subjects and their social needs to integrate into society, attend school, and benefit from peer education.

Diagnosing primary immunodeficiency: a practical approach for the non-immunologist

Abstract Objective: This review will provide an overview of the most common clinical presentations of primary immunodeficiency (PI), navigating through various affected organ systems. The goal is to accurately portray the high variability of this disease and provide a resource that helps to raise the index of suspicion of PI among physicians, aid in recognition of various PI disorders, and trigger more frequent screenings with appropriate referrals to immunologists for further evaluation and treatment. Summary: Patients with PI comprise more than 200 defined genetic abnormalities. Patients have an array of clinical manifestations, ranging from the most widely associated recurrent and chronic bacterial infections to other associated comorbid conditions involving many organ systems. There is still considerable delay between the onset of symptoms and the time of diagnosis of PI. This review will present an overview of the clinical manifestations that will enhance a physician’s recognition of a possible PI. Particular emphasis is placed on the pathogens associated with the specific arm of the immune system that is related to each particular type of PI. The initial immune evaluation is described, which together with the history and physical exam can help focus the physician on the immune compartment most likely associated with a PI. Conclusions: Understanding the types of PI and the related clinical manifestations can help physicians see beyond the presenting symptoms and lead to improved recognition and diagnosis of PI. Timely diagnosis is of utmost importance in PI, as recent advances in bone transplantation and immunoglobulin replacement therapy, as well as future gene therapies, provide effective ways to prevent significant mortality and morbidity.

New Genetic Discoveries and Primary Immune Deficiencies

The field of immunology has undergone recent discoveries of genetic causes for many primary immunodeficiency diseases (PIDD). The ever-expanding knowledge has led to increased understanding behind the pathophysiology of these diseases. Since these diseases are rare, the patients are frequently misdiagnosed early in the presentation of their illnesses. The identification of new genes has increased our opportunities for recognizing and making the diagnosis in patients with PIDD before they succumb to infections that may result secondary to their PIDD. Some mutations lead to a variety of presentations of severe combined immunodeficiency (SCID). The myriad and ever-growing genetic mutations which lead to SCID phenotypes have been identified in recent years. Other mutations associated with some genetic syndromes have associated immunodeficiency and are important for making the diagnosis of primary immunodeficiency in patients with some syndromes, who may otherwise be missed within the larger context of their syndromes. A variety of mutations also lead to increased susceptibility to infections due to particular organisms. These patterns of infections due to specific organisms are important keys in properly identifying the part of the immune system which is affected in these patients. This review will discuss recent genetic discoveries that enhance our understanding of these complex diseases.

Prospective Evaluation of an Anaphylaxis Education Mini-Handout: The AAAAI Anaphylaxis Wallet Card

BACKGROUND Few anaphylaxis education materials have been prospectively evaluated in randomized controlled trials. OBJECTIVE Our objective was to evaluate the American Academy of Allergy, Asthma & Immunology Anaphylaxis Wallet Card (AAAAI-AWC) as an anaphylaxis education mini-handout for health care professionals. METHODS We performed a randomized controlled study of the AAAAI-AWC with residents in general pediatrics at Miami Childrens Hospital. Participants in the intervention group completed a pretest about anaphylaxis, heard a 3-minute PowerPoint presentation based on the AAAAI-AWC, reviewed the AAAAI-AWC, and discussed it with the presenter. After this, participants took a post-test immediately and a follow-up test 4 weeks later. Participants in the control group took the pretest, were handed an AAAAI-AWC, studied it briefly, then took the post-test immediately and the follow-up test 4 weeks later. RESULTS Fifty-five residents participated. Regardless of the amount of time spent studying the AAAAI-AWC, when the pretests were compared with the post-tests and follow-up tests, residents in both the intervention and control groups were more likely to recognize anaphylaxis symptoms (P < .05), name asthma as the most common comorbid disease in children with fatal or near-fatal anaphylaxis (P < .05), and recall the names of epinephrine auto injectors (P < .05) and the epinephrine doses available in these auto injectors (P < .05). When the pretests were compared with the post-tests and the follow-up tests, residents in the intervention group were more likely than controls to identify the body organ systems involved in severe or fatal anaphylaxis correctly (P < .05). CONCLUSION The AAAAI-AWC is a practical, concise anaphylaxis education mini-handout for pediatric residents, a time-challenged group of health care professionals.

Antihistamines treatment for allergic rhinitis: different routes, different mechanisms?

Antihistamines have been used in the treatment of rhinitis since ancient times. Antihistamines can be used via oral or topical route for the treatment of allergic rhinitis, as well as nonallergic forms of rhinitis. The topical formulation of medications is the preferred treatment route used for many diseases. This article will investigate the differences in the route of antihistamines in the treatment of allergic rhinitis. A review of the literature was performed to investigate the differences between the use of oral and topical antihistamines in the treatment of allergic rhinitis. Antihistamines inhibit the mediators of inflammation that worsen the symptoms of allergic rhinitis. Intranasal and oral antihistamines are important options in the therapy of allergic rhinitis, and topical use may result in unexpected benefits to patients with allergic rhinitis.

Cow's milk allergy in a patient with hyper-IgE syndrome

BACKGROUND Both hyper-IgE syndrome and food allergies can result in the early onset of skin rash, eosinophilia, and markedly elevated serum IgE. Occasionally, it can be difficult to distinguish the 2 disorders. Most patients with hyper-IgE syndrome do not have food allergy. OBJECTIVE To describe a child with cows milk allergy associated with hyper-IgE syndrome manifesting as failure to thrive (FTT). METHODS Epicutaneous skin prick test to cows milk, CAP radioallergosorbent test, atopy patch tests, and double-blind, placebo-controlled milk challenge (DBPCMC) were performed. RESULTS During initial presentation at 3 weeks of age, the circulating eosinophil count increased from 13,800/mm3 to 44,254/mm3 within 2 weeks while taking cephalexin. Despite treatment, he had worsening rash and FTT at 10 weeks of age with an IgE level of 8,454 U/mL. After changing from an infant milk formula with whey protein to an amino acid-based formula in combination with oral antibiotic treatment, his rash and growth velocity improved markedly within 2 months. IgE decreased to 2,747 U/mL. He remained clinically well for 12 months. He subsequently developed additional food and inhalant allergies with an increase in IgE to 12,150 U/mL. Cows milk allergy was confirmed by epicutaneous skin prick test, atopy patch test, and DBPCMC. CONCLUSIONS Traditional prophylactic antistaphylococcal antibiotics, in combination with Neocate formula, were effective in treating the early skin manifestations of hyper-IgE syndrome and FTT in this infant. Cows milk protein allergy should be considered in patients with hyper-IgE syndrome and FTT.

Approach to Children with Recurrent Infections.

Recurrent infections in children are a cause for concern. It is essential to distinguish simple recurrent infections caused by exposures in the day care or school settings from those caused by inherent deficiencies in the immune system or other systemic diseases. Multiple diagnostic tools are available for the evaluation of recurrent infections. The sites of infections and organisms responsible are important in guiding clinicians in the appropriate laboratory work-up and diagnosis of these patients. Once a diagnosis is made, proper treatment and management decisions can be made to treat the patients appropriately and ensure their lifelong health.

Primary Immunodeficiency Diseases: An Opportunity in Pediatrics for Improving Patient Outcomes

Objectives. Primary immunodeficiency diseases (PIDDs) are caused by inherent deficits in immune defenses that result in abnormal susceptibility to infection. In most cases, early and appropriate diagnosis can improve patient outcomes. The objective of this study was to evaluate understanding, recognition, and diagnosis of PIDD among pediatricians. Methods. A mail survey sent to a sample of pediatricians obtained from the American Medical Association and American Osteopathic Association. Results were compared with a similar survey of specialists who are members of the American Academy of Asthma, Allergy and Immunology. Results. More than a third (35%) of pediatricians were uncomfortable with the recognition and diagnosis of PIDD despite 95% having ordered screening tests or referring patients to specialists to be evaluated for PIDD, and 77% having followed at leastone patient with PIDD. In all, 84% of pediatricians were unaware that professional guidelines for PIDD exist. Conclusions. Patients with PIDD would benefit from improved recognition of the diseases by pediatricians in order to facilitate earlier diagnosis and optimize ongoing therapy.