Allergy and Immunology Commons^™

Chronic Granulomatous Disease-Like Presentation Of A Child With Autosomal Recessive Pkcδ Deficiency, Anna-Lena Neehus, Karen Tuano, Tom Le Voyer, Sarada L Nandiwada, Kruthi Murthy, Anne Puel, Jean-Laurent Casanova, Javier Chinen, Jacinta Bustamante

Faculty, Staff and Students Publications

BACKGROUND: Autosomal recessive (AR) PKCδ deficiency is a rare inborn error of immunity (IEI) characterized by autoimmunity and susceptibility to bacterial, fungal, and viral infections. PKCδ is involved in the intracellular production of reactive oxidative species (ROS).

MATERIAL AND METHODS: We studied a 5-year old girl presenting with a history of Burkholderia cepacia infection. She had no history of autoimmunity, lymphocyte counts were normal, and no auto-antibodies were detected in her plasma. We performed a targeted panel analysis of 407 immunity-related genes and immunological investigations of the underlying genetic condition in this patient.

RESULTS: Consistent with a history suggestive of …

A Practical Focus On Milk Oral Immunotherapy, Aikaterini Anagnostou

Faculty, Staff and Students Publications

Cow's milk allergy (CMA) is a common childhood food allergy associated with a significant burden for those children who are affected and their families, including unintentional exposures that result in allergic reactions, severe allergic reactions, and anaphylaxis. In young children, cow's milk is one of the most frequent food triggers in anaphylactic episodes, and fatalities have also been described as a result of unintentional exposures, which reinforces the notion that milk allergy can be severe in some individuals. The natural history of CMA is favorable, with the allergy resolving over time in the majority of individuals, although some will have …

A Practical Focus On Egg Oral Immunotherapy, Aikaterini Anagnostou

Faculty, Staff and Students Publications

Egg allergy usually develops in the first year of life, with an estimated prevalence of 2.6%-9%. Rates of resolution and timing vary, with approximately one-third of patients outgrowing their egg allergy by age 6 years. Persistent egg allergy has been associated with high egg white specific immunoglobulin E levels and sensitization to the egg allergen component Gal d 1, which is resistant to heat and digestion. These individuals seem to have a more severe and persistent phenotype of egg allergy. Recently, an active approach has emerged for patients with food allergy, including those with egg allergy, in the form of …

Evaluating Eosinophilic Colitis As A Unique Disease Using Colonic Molecular Profiles: A Multi-Site Study, Tetsuo Shoda, Margaret H Collins, Mark Rochman, Ting Wen, Julie M Caldwell, Lydia E Mack, Garrett A Osswald, John A Besse, Yael Haberman, Seema S Aceves, Nicoleta C Arva, Kelley E Capocelli, Mirna Chehade, Carla M Davis, Evan S Dellon, Gary W Falk, Nirmala Gonsalves, Sandeep K Gupta, Ikuo Hirano, Paneez Khoury, Amy Klion, Calies Menard-Katcher, John Leung, Vincent A Mukkada, Philip E Putnam, Jonathan M Spergel, Joshua B Wechsler, Guang-Yu Yang, Glenn T Furuta, Lee A Denson, Marc E Rothenberg, Consortium Of Eosinophilic Gastrointestinal Diseases Researchers (Cegir)

Faculty, Staff and Students Publications

BACKGROUND & AIMS: Colonic eosinophilia, an enigmatic finding often referred to as eosinophilic colitis (EoC), is a poorly understood condition. Whether EoC is a distinct disease or a colonic manifestation of eosinophilic gastrointestinal diseases (EGIDs) or inflammatory bowel disease (IBD) is undetermined.

METHODS: Subjects with EoC (n = 27) and controls (normal [NL, n = 20], Crohn's disease [CD, n = 14]) were enrolled across sites associated with the Consortium of Eosinophilic Gastrointestinal Disease Researchers. EoC was diagnosed as colonic eosinophilia (ascending ≥100, descending ≥85, sigmoid ≥65 eosinophils/high-power field) with related symptoms. Colon biopsies were subjected to RNA sequencing. Associations …

Methods For Cross-Cultural Communication In Clinic Encounters, Princess U Ogbogu, Lenora Mendoza Noroski, Kimberly Arcoleo, Benjamin D Reese, Andrea J Apter

Faculty, Staff and Students Publications

Successful cross-cultural communication is critical for adequate exchange of ideas with our patients. Our communities have become more diverse, and thus, the necessity has increased. The murder of George Floyd and other atrocities have sparked recognition of the need to address social injustice and racism and as we fight the ongoing coronavirus disease 2019 (COVID-19) pandemic. Allergist-immunologists are uniquely trained to explain the complex immunology of COVID-19 to patients, but they have less experience discussing issues of health equity. Here, we explore critical components of patient-provider communication: communicating with those for whom English is a second language, advising patients with …

Impact Of Social Determinants On The Burden Of Asthma And Eczema: Results From A Us Patient Survey., Don A. Bukstein, Adam Friedman, Erika Gonzalez Reyes, Mary Hart, Bridgette Jones, Tonya Winders

Manuscripts, Articles, Book Chapters and Other Papers

INTRODUCTION: Little is known about how patients with asthma and eczema perceive their medical care and burden of disease. A survey was conducted to evaluate the perceptions among the general patient population with asthma and/or eczema regarding disease and treatment burden and barriers to adequate care.

METHODS: An online survey was completed by market research panelists in the USA between March 24, 2020 and April 6, 2020. Eligible participants were at least 18 years of age and endorsed a diagnosis of asthma and/or eczema. Survey responses are described for all participants, by designated racial/ethnic groups, and by income level.

RESULTS: …

Pathologic Inflammation In Malnutrition Is Driven By Proinflammatory Intestinal Microbiota, Large Intestine Barrier Dysfunction, And Translocation Of Bacterial Lipopolysaccharide, Grace T Patterson, Elvia Y Osorio, Alex Peniche, Sara M Dann, Erika Cordova, Geoffrey A Preidis, Ji Ho Suh, Ichiaki Ito, Omar A Saldarriaga, Michael Loeffelholz, Nadim J Ajami, Bruno L Travi, Peter C Melby

Faculty, Staff and Students Publications

Acute malnutrition, or wasting, is implicated in over half of all deaths in children under five and increases risk of infectious disease. Studies in humans and preclinical models have demonstrated that malnutrition is linked to an immature intestinal microbiota characterized by increased prevalence of Enterobacteriaceae. Observational studies in children with moderate acute malnutrition (MAM) have also observed heightened systemic inflammation and increased circulating bacterial lipopolysaccharides (LPS; endotoxin). However, the mechanisms that underpin the systemic inflammatory state and endotoxemia, and their pathophysiological consequences, remain uncertain. Understanding these pathophysiological mechanisms is necessary to design targeted treatments that will improve the unacceptable rate …

A Case Of An Elevated Tryptase, Sonya Parashar, Nikita Raje

Posters

Hereditary Alpha Tryptasemia (HαT) is an autosomal dominant disorder characterized by an elevated baseline tryptase that occurs up to 3% of the population and clinically resembles mast cell activation syndrome.

Delayed-Onset Anaphylactic Reaction With High Fever After Amoxicillin Oral Challenge And Negative Penicillin Skin Testing, Jordan Pitt, Paul J. Dowling, Christopher Miller, Aarti Pandya

Posters

Introduction:  Immunologic adverse drug reactions can be categorized based on Gell and Coombs’s classification system. Anaphylaxis is generally considered a type I, immediate, IgE-mediated reaction and typically occurs independent of other immunologic reactions. However, the child presented here reacted after amoxicillin challenge with features of type I and type III or IV hypersensitivity reactions. Case Description:  A 12-year-old female presented for amoxicillin allergy evaluation after treatment for scarlet fever with amoxicillin. After the second dose she developed rash with varied features, fatigue, edema, and joint swelling. Labs included a persistently low C4 level, eosinophilia, normal inflammatory markers, and normal tryptase …

Personalized Digital Phenotype Score, Healthcare Management And Intervention Strategies Using Knowledge Enabled Digital Health Framework For Pediatric Asthma, Utkarshani Jaimini, Amit Sheth

Publications

Asthma is a personalized, and multi-trigger respiratory condition which requires continuous monitoring and management of symptoms and medication adherence. We developed kHealth: Knowledge-enabled Digital Healthcare Framework to monitor and manage the asthma symptoms, medication adherence, lung function, daily activity, sleep quality, indoor, and outdoor environmental triggers of pediatric asthma patients. The kHealth framework collects up to 1852 data points per patient per day. It is practically impossible for the clinicians, parents, and the patient to analyze this vast amount of multimodal data collected from the kHealth framework. In this chapter, we describe the personalized scores, clinically relevant asthma categorization using …

Recurrent Neonatal Herpes Simplex Virus Infection Associated With Irf7 And Unc93b1 Variants, Venkatesh Sampath, Megan H. Tucker, Heather Menden, Sheng Xia, Wei Yu, Nikita Raje

Posters

Background: Neonatal herpes simplex virus (HSV) is a devastating disease with high mortality. In adults and children, genetic variants in the toll-like receptor 3 (TLR3) pathway increase susceptibility to herpes simplex encephalitis (HSE), but the genetic basis of susceptibility to neonatal HSV is unknown. We hypothesized that deleterious variants in the TLR3 pathway increased vulnerability to HSE in neonates. We investigated immunogenetic studies in an infant with neonatal skin, eye, mouth (SEM) HSV followed by HSE.


Objective: To combine exome sequencing with in vivo and in vitro immune functional analysis to discover the immunogenetic basis of HSV vulnerability in proband. …

An International Study Of Interobserver Variability Of “String Sign” Of Pancreatic Cysts Among Experienced Endosonographers, Wanda Phipatanakul, David T Mauger, Theresa W Guilbert, Leonard B Bacharier, Sandy Durrani, Daniel J Jackson, Fernando D Martinez, Anne M Fitzpatrick, Amparito Cunningham, Susan Kunselman, Lisa M Wheatley, Cindy Bauer, Carla M Davis, Bob Geng, Kirsten M Kloepfer, Craig Lapin, Andrew H Liu, Jacqueline A Pongracic, Stephen J Teach, James Chmiel, Jonathan M Gaffin, Matthew Greenhawt, Meera R Gupta, Peggy S Lai, Robert F Lemanske, Wayne J Morgan, William J Sheehan, Jeffrey Stokes, Peter S Thorne, Hans C Oettgen, Elliot Israel

Faculty, Staff and Students Publications

Asthma remains one of the most important challenges to pediatric public health in the US. A large majority of children with persistent and chronic asthma demonstrate aeroallergen sensitization, which remains a pivotal risk factor associated with the development of persistent, progressive asthma throughout life. In individuals with a tendency toward Type 2 inflammation, sensitization and exposure to high concentrations of offending allergens is associated with increased risk for development of, and impairment from, asthma. The cascade of biological responses to allergens is primarily mediated through IgE antibodies and their production is further stimulated by IgE responses to antigen exposure. In …

The Spectrum Of Primary Immunodeficiencies At A Tertiary Care Hospital In Pakistan, Sonia Qureshi, Fatima Mir, Samina Junejo, Khalid Saleem, Samreen Zaidi, Abdullah B. Naveed, Khalil Ahmad, Farah Naz Qamar

Department of Paediatrics and Child Health

Background: Primary Immunodeficiency Disorders (PIDs) are well-known disorders in the West. but the recognition and diagnosis of these disorders is challenging in developing countries. We present the spectrum of PIDs seen at a tertiary care center in Pakistan, identified using clinical case definitions and molecular methods.
Methods: A retrospective chart review of children suspected to have PID was conducted at the Aga Khan University Hospital (AKUH) Karachi, Pakistan from 2010 to 2016. Data on demographics, clinical features, family history of consanguinity, sibling death, details of laboratory workup done for PID and molecular tests targeted panel next generation sequencing (NGS) or …

Diagnosis Of Peanut Allergy Using Continuous Likelihood Ratios, Nalin Ug

Research Days

No abstract provided.

The Reliability Of Histamine Pharmacodynamic Response Phenotype Classification In Children With Allergic Disease., Shaun S Kumar, Xiaoxi Liu, Catherine M Sherwin, Bridgette Jones

Manuscripts, Articles, Book Chapters and Other Papers

We have identified distinct histamine pharmacodynamic response phenotypes in children with allergic disease utilizing histamine iontophoresis with laser Doppler (HILD). These response phenotypes may be relevant in guiding therapeutic decision making for agents targeting the allergic response pathways. However, the reliability of these response phenotypes has not been assessed. Therefore, we performed HILD in children with allergic rhinitis and/or asthma on two to three separate occasions. HILD response-time data were analyzed in NONMEM using a linked effect PKPD model. Examination of observed vs. classified response phenotypes predicted response plots and the sum of residuals. The intraclass correlation coefficient (ICC) was …

How To Use Peanut Ige Levels To Predict Challenge Outcomes In The Pediatric Population, Nalin U.G., Jay Portnoy, Jodi Shroba, Salman Aljubran

Posters

Introduction: The accepted clinical practice is to consider oral challenge when peanut IgE levels are 2.0 kUA/L or less in patients with a history of a major reaction or less than 5.0 kUA/L in patients with only sensitization. Certain populations are predisposed to having increased IgE levels, and these patients are often not offered challenges due to elevated numbers.

Methods: We reviewed unidentified patients information from January 2017 to July 2019 on peanut IgE levels and oral challenge outcomes through REDCap database. A ROC using SPSS from IBM was used and the points from the ROC curve were used to …

Parental Observation Of Environmental Exposures In The Home Of Children With Asthma, Hannah N. Neuhaus

Research Days

No abstract provided.

Effects Of Allergen Sensitization On Response To Therapy In Children With Eosinophilic Esophagitis, Robert D. Pesek, Mallikarjuna Rettiganti, Erin O'Brien, Sarah Beckwith, Caroline Daniel, Chunqiao Luo, Amy M. Scurlock, Peggy Chandler, Rebecca A Levy, Tamara T. Perry, Joshua L. Kennedy, Sheva Chervinskiy, Maryelle Vonlanthen, Helen Casteel, Stephen C. Fiedorek, Troy Gibbons, Stacie M. Jones

Pediatrics Faculty Publications

Background: In children with eosinophilic esophagitis (EoE) foods are the most common disease triggers, but environmental allergens are also suspected culprits.

Objective: To determine the effects of environmental allergen sensitization on response to treatment in children with EoE in the southeastern United States.

Methods: Patients 2 to 18 years old who were referred to the Arkansas Children’s Hospital Eosinophilic Gastrointestinal Disorders Clinic from January 2012 to January 2016 were enrolled in a prospective, longitudinal cohort study with collection of demographics, clinical symptoms, medical history, allergy sensitization profiles, and response to treatment over time. Comparisons were made between complete responders (peak …

Addendum Guidelines For The Prevention Of Peanut Allergy In The United States: Report Of The National Institute Of Allergy And Infectious Diseases–Sponsored Expert Panel, Alkis Togias, Susan F. Cooper, Maria L. Acebal, Amal Assa'ad, James R. Baker, Lisa A. Beck, Julie Block, Carol Byrd-Bredbenner, Edmond S. Chan, Lawrence F. Eichenfield, David M Fleischer, George J. Fuchs Iii, Glenn T. Furuta, Matthew J. Greenhawt, Ruchi S. Gupta, Michele Habich, Stacie M. Jones, Kari Keaton, Antonella Muraro, Marshall Plaut, Lanny J. Rosenwasser, Daniel Rotrosen, Hugh A. Sampson, Lynda C. Schneider, Scott H. Sicherer, Robert Sidbury, Jonathan Spergel, David R. Stukus, Carina Venter, Joshua A. Boyce

Pediatrics Faculty Publications

Background: Food allergy is an important public health problem because it affects children and adults, can be severe and even life-threatening, and may be increasing in prevalence. Beginning in 2008, the National Institute of Allergy and Infectious Diseases, working with other organizations and advocacy groups, led the development of the first clinical guidelines for the diagnosis and management of food allergy. A recent landmark clinical trial and other emerging data suggest that peanut allergy can be prevented through introduction of peanut-containing foods beginning in infancy.

Objectives: Prompted by these findings, along with 25 professional organizations, federal agencies, and patient advocacy …

A Case Of Difficult-To-Treat Asthma Due To Uncontrolled Atypical Gastroesophageal Reflux, Alissa Welsh, Andrey Leonov

Research Day

Introduction: 
In uncontrolled asthma, comorbid conditions should be treated in order to achieve better asthma control. The contribution of gastro-esophageal reflux (GER) may be overlooked when its presentation is atypical. 
Case Report: 
11 year-old male with moderate persistent asthma and allergic rhinitis presented with persistent barky cough, diagnosed as spasmodic croup after bronchoscopy, esophagram, swallow study and magnified airway x-ray failed to show anatomical abnormality. Empiric proton pump inhibitor (PPI) treatment was started with improvement in cough. Patient was referred to Gastroenterology for evaluation of GER, despite lack of heartburn or vomiting. Endoscopy revealed esophageal candida infection which was treated. …

Case Report: Recurrent Staphylococcal Scalded Skin Syndrome In Healthy Term Neonate Despite Full Course Of Antibiotic Therapy, Indira Bhavsar, Rebecca Hayes, Amy Vaughan

Marshall Journal of Medicine

Background

Staphylococcal scalded skin syndrome (SSSS) describes a blistering skin infection caused by the exfoliative toxin in the bacterium Staphylococcus aureus. It more commonly affects the infant population and is characterized by large blistering bullae that rupture upon application of pressure.

Case description

We describe a case of recurrent SSSS in a healthy term neonate who initially presented with a perioral rash on day of life (DOL) 11 that quickly became vesicular with new lesions on the sternum and extremities. The patient’s rash began to resolve upon administration of culture-specific IV antibiotics. She was appropriately treated with a 14-day …

Allergic Diseases And Internalizing Behaviors In Early Childhood., Maya K. Nanda, Grace K. Lemasters, Linda Levin, Marc E. Rothenberg, Amal H. Assa'ad, Nicholas Newman, David Bernstein, Gurjit Khurana-Hershey, James E. Lockey, Patrick H. Ryan

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND AND OBJECTIVES: The relationship between allergic diseases and internalizing disorders has not been well characterized with regard to multiple allergic diseases or longitudinal study. The objective of this study was to examine the association between multiple allergic diseases in early childhood with validated measures of internalizing disorders in the school-age years.

METHODS: Children enrolled in the Cincinnati Childhood Allergy and Air Pollution Study underwent skin testing and examinations at ages 1, 2, 3, 4, and 7 years. At age 7, parents completed the Behavior Assessment System for Children, Second Edition (BASC-2), a validated measure of childhood behavior and emotion. …

World Allergy Organization-Mcmaster University Guidelines For Allergic Disease Prevention (Glad-P): Prebiotics., Carlos A. Cuello-Garcia, Alessandro Fiocchi, Ruby Pawankar, Juan José Yepes-Nuñez, Gian Paolo Morgano, Yuan Zhang, Kangmo Ahn, Suleiman Al-Hammadi, Arnav Agarwal, Shreyas Gandhi, Kirsten Beyer, Wesley Burks, Giorgio W. Canonica, Motohiro Ebisawa, Rose Kamenwa, Bee Wah Lee, Haiqi Li, Susan Prescott, Lanny Rosenwasser, Hugh Sampson, Michael Spigler, Luigi Terracciano, Andrea Vereda, Susan Waserman, Holger J. Schünemann, Jan L. Brożek

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: The prevalence of allergic diseases in infants, whose parents and siblings do not have allergy, is approximately 10 % and reaches 20-30 % in those with an allergic first-degree relative. Intestinal microbiota may modulate immunologic and inflammatory systemic responses and, thus, influence development of sensitization and allergy. Prebiotics - non-digestible oligosaccharides that stimulate growth of probiotic bacteria - have been reported to modulate immune responses and their supplementation has been proposed as a preventive intervention.

OBJECTIVE: The World Allergy Organization (WAO) convened a guideline panel to develop evidence-based recommendations about the use of prebiotics in the prevention of allergy. …

A Program To Change The Approach To Care Of Children With Asthma In The Primary Care Setting Did Not Reduce Rates Of Hospital Admissions: Lessons Learned From A Descriptive Study, Sheniz Moonie, Robert C. Strunk, Mario Castro

Environmental & Global Health Faculty Research

Asthma is a critical global health issue. It affects people of all ages in countries throughout the world. The prevalence of asthma is increasing in most countries among young children who also represent the greatest proportion of health care utilization. Outpatient asthma-treatment programs managed by chest physicians or allergists have reduced hospitalizations, yet programs in pediatric offices have not successfully impacted hospitalizations. The Community Asthma Program (CAP) was designed to support pediatrician use of clinical guidelines in their everyday office practice. The goal was to reduce asthma hospitalizations by 15 percent from selected pediatric practices. A study was done in …