Allergy and Immunology Commons^™

Perioperative Anaphylaxis In A Healthy Adolescent After First Lifetime Surgery, Shayan Sazdar, Salman Aljubran

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Introduction: Perioperative anaphylaxis is a rare life-threatening event. Its evaluation poses complex considerations inherent to the use of multiple medications. Patient presentation: A healthy 11-year-old boy developed anaphylaxis shortly after discharge home from first lifetime surgery for orchiopexy. Perioperative medications included rocuronium, propofol, fentanyl, bupivacaine, sugammadex, ondansetron, dexamethasone, dexmedetomidine, and ketorolac. No antibiotics. After surgery, he consumed fast food dinner and ice cream. Within 2 hours after discharge home, he returned to our Emergency Department (ED) with facial and tongue angioedema, hives, and respiratory distress. He received multiple doses of intramuscular epinephrine and briefly required epinephrine drip in the ED. …

Asthma Phenotypes And Control In 22q11.2 Deletion Syndrome: A Single-Center Retrospective Study, Cody Shopper, Katey Campbell, Nikita S. Raje

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Background: Patients with 22q11.2 deletion syndrome have increased rates of atopy and immune dysregulation and may experience a higher burden of asthma; however, real-world data describing asthma control, severity, and treatment patterns in this population are limited. We hypothesized that asthma control and severity patterns in patients with 22q11.2 deletion syndrome differ from those observed in the general asthma population. Objectives/Goal: To characterize asthma control, severity, and treatment patterns among patients with 22q11.2 deletion syndrome and to compare observed patterns with those typically reported in the general asthma population. Methods/Design: We conducted a retrospective observational cohort study of patients with …

Allergies Coinciding With Central Sensitization Syndromes (Access), Jacqueline Morey, Alemu Fite, Carl Lauter, Matthew Sims

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Significance of Reported Allergies: Many reported drug allergies are mislabeled side effects or misinterpretations, leading to suboptimal treatment, antibiotic resistance, and worse patient outcomes.¹

Central Sensitization Syndromes (CSS): CSSs are chronic pain conditions linked to altered sensory perception, including pain, fatigue, and neurologic dysfunction.² These patients may be more likely to misattribute medication side effects as allergies.

Previous Findings & Hypothesis: Prior research found high allergy reporting in interstitial cystitis and fibromyalgia—both CSS conditions.⁴ We hypothesized that other CSS diagnoses also correlate with increased allergy reporting, emphasizing the need for better allergy evaluation.

An Early Successful Hematopoietic Stem Cell Transplant (Hsct) For A Boy With Ipex Syndrome, Ali H. Alnajim, Salman Aljubran

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Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a rare inborn error of immunity. It affects about one in every 1.6 million people. It is caused by mutations in the forkhead box P3 (FOXP3) gene on chromosome Xp11.23. FOXP3 is a vital regulator of T cell development and function. Dysfunctional FOXP3 allows the hyperactivation of T cells, resulting in skin rash, enteropathy, diabetes, thyroiditis, hemolytic anemia, and thrombocytopenia. The classic clinical presentation is intractable diarrhea, growth problems, dermatitis, and autoimmune endocrinopathy. Case Description: A 4-week-old male was transferred to our hospital’s NICU due to respiratory distress and feeding difficulties. Neurology …

Elevated Tryptase Level Identifies A Case Of Hereditary Alpha Tryptasemia, Ali H. Alnajim, Salman Aljubran

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Introduction Hereditary alpha tryptasemia (HaT) is a rare genetic disorder characterized by elevated serum tryptase levels due to duplication or triplication of the TPSAB1 gene. Affecting 6 to 8 percent of the population, HaT presents with diverse symptoms, including idiopathic anaphylaxis, skin rashes, and gastrointestinal issues. This report highlights a case that underscores the complexities and diagnostic challenges of HaT. Case Description A 4-year-old male presented with recurrent skin rashes and gastrointestinal symptoms following ingestion of certain foods, despite negative allergy tests. Persistent symptoms led to further investigations, revealing elevated tryptase levels of 10.3 ng/ml, 12.1 ng/ml, and 8.3 ng/ml …

Ada Deficiency: Improvement In Immune Function After Enzyme Replacement Therapy, Esosa Adah, Nikita Raje

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Introduction: Adenosine deaminase (ADA)-deficient severe combined immunodeficiency (SCID) is characterized by severe lymphocytopenia affecting T-, and B-,lymphocytes, with non-immunological manifestations including neurodevelopmental deficits, sensorineural deafness, and skeletal abnormalities. Treatment modalities include enzyme replacement therapy(ERT) and/or autologous gene therapy or hematopoietic stem cell treatment. We report a 9-month-old female with ADA deficient T-B-NK+ SCID treated with ERT while awaiting stem cell treatment. Case Description: A 9-month-old female with a history of failure to thrive, hypotonia and loss of developmental milestones had genetic testing done that showed variants ADA c.911 T>G, c.302G>A. She was admitted for further work up. Lymphocyte …

Investigating The Association Between Mast Cell Activation Syndrome And Irritable Bowel Syndrome, Ishaan Jakhar, Maggie Urschler, Medha Singh, Suman Sahil, Hassan Ahmad

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Investigating the Association between Mast Cell Activation Syndrome and Irritable Bowel Syndrome Introduction Mast Cell Activation Syndrome (MCAS) involves the inappropriate release of chemical mediators resulting in varied episodic symptoms. Irritable Bowel Syndrome (IBS) is a common functional abdominal disorder where mucosal immune alterations play a key role. Since mast cells function as gatekeepers for the intestinal interface, we examined what proportion of patients diagnosed with MCAS also had IBS along with comorbid risk factors. Methods All patients in our study were 18 years of age or older. We used Cerner Healthfacts to gather our cohorts of patients with MCAS, …

Evaluating A Newborn Infant With Pancytopenia For Whim Syndrome, Shayan Sazdar, Thao Le, Nikita Raje

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Introduction Warts, hypogammaglobulinemia, infections, and myelokathexis (WHIM) syndrome is a rare immunodeficiency caused by a gain-of-function mutation in CXCR4. A complication of WHIM syndrome is severe neutropenia, which can result in fatal bacterial infections. Given the rarity of the disease and variability of clinical presentations, WHIM syndrome can be difficult to diagnose and manage. Case Description A full term male infant was hospitalized for hypoxic ischemic encephalopathy after delivery. Physical exam was unremarkable. During his hospitalization, he was found to have progressive pancytopenia. Initial lymphocyte subsets showed a significant T cell lymphopenia of 1325 mm3 CD3+, 920 mm3 CD4+, 390 …

Evaluating Patients With Recurrent Fever And Elevated Igd, Thao Le, Nikita Raje

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Introduction: Hyperimmunoglobulinemia D with periodic fever syndrome (HIDS) often presents within the first year of life. Symptoms include fevers, chills, headache, lymphadenopathy, and abdominal pain. Patients may be misdiagnosed as other autoinflammatory syndromes, resulting in delay of treatment. Case: A 16-year-old female presented for evaluation of recurrent fever. Her fevers started at two months of age, occurred one to two times a month, and were associated with headache, abdominal pain, and fatigue . At 18-months, she was diagnosed with periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA). She was treated with prednisone and an adenotonsillectomy but her fevers persisted. . At …

Clinical Course Of A Patient With Agammaglobulinemia Caused By Slc39a7 Defect, Thao Le, Emily Farrow, Alvin Singh, Isabelle Thiffault, Nikita Raje

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Case Report: A 10-year-old unimmunized boy initially presented to the hospital at 18-months of age with pneumonia and failure to thrive. He had multiple infections including Escherichia coli urosepsis, viral croup, chronic otitis media with bilateral ruptured tympanic membranes, and bacterial pneumonia. On physical examination, he was ill appearing and had diffuse crackles. His laboratory work-up showed leukocytosis, normocytic anemia, undetectable immunoglobulin (Ig) G, A, and E, low IgM (28 mg/dL), absent B cell with normal T cell (7800 mm3) and NK cell (527 mm3) counts, and low zinc level (63 mcg/dL). Genetic testing was negative for Bruton tyrosine kinase …

Experiences Of Racism Among Black And African Children With Asthma, Esosa Adah, Rayanna Tucker, Bridgette Jones

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Rationale: The relationship between adverse childhood experiences, toxic stress and asthma risk has been previously described in the literature among adult and pediatric populations. Studies have identified experiences of racism as a chronic stressor associated with asthma morbidity however, most studies have focused primarily on adults or parental perspectives. We initiated a pilot study to characterize described experiences of racism among Black/African American children with persistent asthma and describe some of our initial results. Methods: Children were asked to complete the “Perceptions of Racism in Children and Youth,” a validated questionnaire, measuring perceptions of racism and discrimination. Responses were analyzed …

Malignancy Masquerading As Steroid Resistant Hypereosinophilic Syndrome, Sonya Parashar, Aarti Pandya

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Introduction: Hypereosinophilia syndrome (HES) is defined as 1500 eosinophils/mL or above with evidence of end-organ damage due to the eosinophils at least 4 weeks apart. Case description: A 3-year-old male was admitted for newly found anterior mediastinal mass and left pleural effusion. About 6 weeks prior to admission, he was seen in his primary care office for difficulty breathing. Despite the appropriate treatment for his symptoms, the patient continued to have significant respiratory symptoms and a chest x-ray showed a large left pleural effusion. He was immediately transferred to a tertiary care center and a CT chest showed large left …

A Rare Cause Of Urticaria In An Nsaid Tolerant Patient, Thao Le, Mary K. Mcleod, Laurianne Wild

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Introduction: Acetaminophen is one of the most used anti-pyretics to mitigate fever. Few cases have been reported of hypersensitivity reactions to acetaminophen without non-steroidal anti-inflammatory drug (NSAID) intolerance. It is recommended that if there is suspected drug hypersensitivity then the patient should undergo an oral drug challenge. Case Description: A 49-year-old male presented for evaluation of urticaria that occurred with acetaminophen ingestion. He reported several episodes where he developed diffuse urticaria within 15 minutes of ingestion, but no other symptoms concerning for anaphylaxis. He was unsure of the dose. He also noted urticaria with Excedrin migraine, which contains acetaminophen, aspirin, …

Timing Of Testing For Ige-Mediated Food Allergy After Systemic Reaction, Jordan Pitt, Paul J. Dowling, Christopher Miller, Ashley Sherman, Salman Aljubran

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Rationale: A refractory period of falsely negative testing can occur following a systemic allergic reaction to Hymenoptera sting. As a result, blood specific IgE and/or skin prick tests (SPT) for other allergens are often delayed. This retrospective chart review aims to identify the proportion of patients with falsely negative test results in the 6 weeks following an allergic reaction to food, and factors that may affect it. Methods: One hundred fourteen pediatric subjects met inclusion criteria. Each had a convincing history of food allergy with a systemic allergic reaction and was tested to the culprit food within 6 weeks. The …

Evaluation Of The Outcomes Of Oral Challenges To Azithromycin, Cephalexin And Trimethoprim-Sulfamethoxazole In Pediatrics, Hannah N. Neuhaus, Jordan Heath Md, Salman Aljubran

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Rationale: Antibiotic hypersensitivity complicates treatment for various infections and leads to long-term healthcare costs and antibiotic resistance. Data regarding the outcomes of oral challenges to trimethoprim-sulfamethoxazole, azithromycin and cephalexin are limited in Pediatrics.  The goal of this study was to characterize the outcomes and safety of oral challenges to these antibiotics in Pediatrics.

Methods: A retrospective chart review was performed of pediatric patients who underwent oral challenges to cephalexin, azithromycin and trimethoprim-sulfamethoxazole in Allergy Clinic over the last 12 years.

Results: Ten patients underwent oral challenge to trimethoprim-sulfamethoxazole and all were successful. Thirteen patients underwent oral challenge to azithromycin and …

Evaluation Of The Outcomes Of Trimethoprim-Sulfamethoxazole Oral Challenges In The Pediatric Population, Hannah N. Neuhaus, Salman Aljubran

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Introduction: Trimethoprim-sulfamethoxazole allergy can complicate treatment for various infections. While trimethoprim-sulfamethoxazole hypersensitivity, desensitization/oral challenges are frequently reported in adults, data is limited on the outcomes/safety of oral challenges to trimethoprim-sulfamethoxazole in Pediatrics. The goal of this study was to characterize the outcomes and safety of trimethoprim-sulfamethoxazole oral challenges in Pediatrics.

Methods: An IRB-exempt retrospective chart review was performed of pediatric patients who underwent oral challenge to trimethoprim-sulfamethoxazole in Allergy Clinic over the last 12 years. We assessed characteristics including age, sex, reaction (IgE-mediated/non-IgE-mediated/indeterminate), skin testing, challenge outcome and complications to draw a conclusion regarding the overall safety of the procedure …

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

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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

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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 …

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

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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. …

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

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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 …