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CASE SERIES OF INFANTS WITH CHRONIC FPIES
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Abstracts: Medically Challenging Cases / Ann Allergy Asthma Immunol 121 (2018) S63−S134
bronchovascular markings without consolidation or hyperinflation have been investigated. Positive milk, egg, and mango specific IgE results have been detected in infant’s mother. Management: Antibiotics; cold medications including anti-histamines, bronchodilators, frequent nebulization; anti-reflux medications (Esomeprazole) have been used without positive response. After careful elimination of milk, dairy products, mango and egg, the condition had improved within 2 weeks. Anti-reflux medications have been eliminated 2 months later. Planned Food challenge has been tested with recurrence of the same symptoms. Elimination of these foods for 6 months was done. Planned re-introduction of milk, egg (at 1.3 year) and mango (at 2 years) has been done with successful results. Discussion: In this case, the challenge was to suspect the rule of the food as a trigger of cough, especially with the onset before start of weaning in an exclusively breast fed infant. We recommend careful food allergy testing in any unexplained chronic cough in infants.
M317 TREE NUT ALLERGY MASQUERADING AS FOREIGN BODY ASPIRATION: PISTACHIO ALLERGY PRESENTING WITH ISOLATED SUPRAGLOTTIC EDEMA M. Alpern*, E. O'Brien, R. Campbell, M. Park, J. Hagan, Rochester, MN Introduction: Foreign body aspiration and food allergy are common causes of respiratory distress in pediatric patients. We present a case of respiratory distress in a 19 month old male who reportedly choked on a pistachio. However, during evaluation for foreign body aspiration, he was found to have isolated supraglottic edema due to pistachio IgE mediated hypersensitivity. Case Description: A previously healthy 19 month old male presented with coughing and difficulty breathing immediately after reportedly choking on a pistachio. His respiratory rate was 50, heart rate was 180 bpm, and pulse oximetry was 30% which improved to 100% with supplemental oxygen. Exam was notable for grunting, accessory respiratory muscle use, and the absence of rash, angioedema, or wheezing. He was given intramuscular epinephrine 0.15mg without improvement. Chest X-ray revealed no radio-opaque foreign body or pneumothorax. Otolaryngology performed immediate laryngoscopy/ bronchoscopy that revealed significant supraglottic edema of epiglottis, aryepiglottic folds, and bilateral arytenoids (Figure 1). There was no evidence of foreign body, and he was intubated for airway protection. Intravenous epinephrine 9mcg, dexamethasone 7mg, and diphenhydramine 12.5mg were administered with improvement of edema. He was successfully extubated and discharged. Six weeks later, IgE to pistachio was 4.91 KU/L and cashew was 3.25 KU/L. IgE testing to other tree nuts was negative. He has subsequently had no further episodes with strict avoidance of tree nuts. Discussion: Food allergy must be considered in the differential diagnosis of foreign body aspiration, despite the lack of cutaneous findings and particularly following the ingestion of commonly allergic foods.
Supraglottic edema of epiglottis, aryepiglottic folds, and bilateral arytenoids.
M318 CASE SERIES OF INFANTS WITH CHRONIC FPIES M. Baker*, J. Flom, A. Nowak-Wegrzyn, New York, NY Introduction: Acute and chronic FPIES are two phenotypes that may occur in the same patient. Due to the heterogeneous presentation and lack of specific biomarkers, the FPIES diagnosis may prove elusive. At our practice, we recently saw three infants with chronic FPIES. Case Description: Patient A developed watery/mucousy stools and frequent spit-up on day 3 of life. She was switched from cow’s milk formula (CMF) to extensively hydrolyzed formula. Symptoms returned within 3 weeks, and she was hospitalized with dehydration, leukocytosis, thrombocytosis, and metabolic acidosis. Sepsis work-up was negative. Her symptoms resolved with an elemental formula, suggesting FPIES to milk. Patients B and C are twins who presented at 3 weeks. They were fed CMF and had chronic diarrhea, with Patient B requiring intensive care unit admission for dehydration. Symptoms resolved on elemental formula, and FPIES to milk was suspected. They remained asymptomatic until age 11 months, when they were fed cow’s milk yogurt and within 3 hours developed repetitive vomiting, followed by diarrhea and dehydration requiring intravenous fluids, confirming the FPIES diagnosis. They similarly developed vomiting and diarrhea one week after introducing soy yogurt, suggesting concomitant FPIES to soy. Discussion: Diagnosis of FPIES requires recognition of a constellation of symptoms. In these infants, the diagnosis of FPIES to milk was suggested by chronic diarrhea that resolved with dietary elimination. An acute FPIES reaction in the twins confirmed the diagnosis. The diagnosis of FPIES continues to require a detailed history and keen clinical acumen.
M319 URTICARIA, NAUSEA, AND VOMITING, IS THIS THE GALT ENZYME DEFICIENCY? R. Villarreal*,1, F. Tabatabaian2, D. Ledford1, 1. Tampa, FL; 2. St. Petersburg, FL Introduction: Galactosemia is a rare genetic disorder not frequently encountered in clinical practice. GALT enzyme deficiency is in the spectrum of non IgE mediated food intolerance. However, when encountered with symptoms consistent anaphylaxis, the possibility of an IgE mediated reaction should not be excluded. Case Description: Our patient is a 17-year old female who presented for evaluation of food intolerance after ingestion of milk. She was diagnosed as a carrier, or having a mild variant of galactosemia, at birth. She had been strictly avoiding milk since infancy, until her recent exposure to baked goods made with milk. After ingestion she developed immediate urticaria, nausea and vomiting. Physical exam was found to be unremarkable. Her in-vitro testing demonstrated a serum IgE to milk >100 kU/L (normal < 0.1 kU/L). Discussion: This is a unique case that demonstrates both non IgE mediated and IgE mediated intolerance to milk in the same patient. Classic galactosemia and Duarte variant galactosemia are autosomal recessive disorders that lead to the deficiency of the enzyme galactose-1-phosphate uridyltransferase. Diagnosis is made on the newborn screen. Classic galactosemia presents with feeding problems, failure to thrive, liver damage, bleeding, and infections. Duarte variant galactosemia presents as jaundice in infancy that resolves when switching to low-galactose formula. Our patient had symptoms upon introduction of milk later in life that was not fully explained by her diagnosis of Duarte variant of galactosemia, but rather by an IgE mediated milk allergy. To date, she is avoiding milk and carries an epinephrine autoinjector.
Abstracts: Medically Challenging Cases / Ann Allergy Asthma Immunol 121 (2018) S63−S134
bronchovascular markings without consolidation or hyperinflation have been investigated. Positive milk, egg, and mango specific IgE results have been detected in infant’s mother. Management: Antibiotics; cold medications including anti-histamines, bronchodilators, frequent nebulization; anti-reflux medications (Esomeprazole) have been used without positive response. After careful elimination of milk, dairy products, mango and egg, the condition had improved within 2 weeks. Anti-reflux medications have been eliminated 2 months later. Planned Food challenge has been tested with recurrence of the same symptoms. Elimination of these foods for 6 months was done. Planned re-introduction of milk, egg (at 1.3 year) and mango (at 2 years) has been done with successful results. Discussion: In this case, the challenge was to suspect the rule of the food as a trigger of cough, especially with the onset before start of weaning in an exclusively breast fed infant. We recommend careful food allergy testing in any unexplained chronic cough in infants.
M317 TREE NUT ALLERGY MASQUERADING AS FOREIGN BODY ASPIRATION: PISTACHIO ALLERGY PRESENTING WITH ISOLATED SUPRAGLOTTIC EDEMA M. Alpern*, E. O'Brien, R. Campbell, M. Park, J. Hagan, Rochester, MN Introduction: Foreign body aspiration and food allergy are common causes of respiratory distress in pediatric patients. We present a case of respiratory distress in a 19 month old male who reportedly choked on a pistachio. However, during evaluation for foreign body aspiration, he was found to have isolated supraglottic edema due to pistachio IgE mediated hypersensitivity. Case Description: A previously healthy 19 month old male presented with coughing and difficulty breathing immediately after reportedly choking on a pistachio. His respiratory rate was 50, heart rate was 180 bpm, and pulse oximetry was 30% which improved to 100% with supplemental oxygen. Exam was notable for grunting, accessory respiratory muscle use, and the absence of rash, angioedema, or wheezing. He was given intramuscular epinephrine 0.15mg without improvement. Chest X-ray revealed no radio-opaque foreign body or pneumothorax. Otolaryngology performed immediate laryngoscopy/ bronchoscopy that revealed significant supraglottic edema of epiglottis, aryepiglottic folds, and bilateral arytenoids (Figure 1). There was no evidence of foreign body, and he was intubated for airway protection. Intravenous epinephrine 9mcg, dexamethasone 7mg, and diphenhydramine 12.5mg were administered with improvement of edema. He was successfully extubated and discharged. Six weeks later, IgE to pistachio was 4.91 KU/L and cashew was 3.25 KU/L. IgE testing to other tree nuts was negative. He has subsequently had no further episodes with strict avoidance of tree nuts. Discussion: Food allergy must be considered in the differential diagnosis of foreign body aspiration, despite the lack of cutaneous findings and particularly following the ingestion of commonly allergic foods.
Supraglottic edema of epiglottis, aryepiglottic folds, and bilateral arytenoids.
M318 CASE SERIES OF INFANTS WITH CHRONIC FPIES M. Baker*, J. Flom, A. Nowak-Wegrzyn, New York, NY Introduction: Acute and chronic FPIES are two phenotypes that may occur in the same patient. Due to the heterogeneous presentation and lack of specific biomarkers, the FPIES diagnosis may prove elusive. At our practice, we recently saw three infants with chronic FPIES. Case Description: Patient A developed watery/mucousy stools and frequent spit-up on day 3 of life. She was switched from cow’s milk formula (CMF) to extensively hydrolyzed formula. Symptoms returned within 3 weeks, and she was hospitalized with dehydration, leukocytosis, thrombocytosis, and metabolic acidosis. Sepsis work-up was negative. Her symptoms resolved with an elemental formula, suggesting FPIES to milk. Patients B and C are twins who presented at 3 weeks. They were fed CMF and had chronic diarrhea, with Patient B requiring intensive care unit admission for dehydration. Symptoms resolved on elemental formula, and FPIES to milk was suspected. They remained asymptomatic until age 11 months, when they were fed cow’s milk yogurt and within 3 hours developed repetitive vomiting, followed by diarrhea and dehydration requiring intravenous fluids, confirming the FPIES diagnosis. They similarly developed vomiting and diarrhea one week after introducing soy yogurt, suggesting concomitant FPIES to soy. Discussion: Diagnosis of FPIES requires recognition of a constellation of symptoms. In these infants, the diagnosis of FPIES to milk was suggested by chronic diarrhea that resolved with dietary elimination. An acute FPIES reaction in the twins confirmed the diagnosis. The diagnosis of FPIES continues to require a detailed history and keen clinical acumen.
M319 URTICARIA, NAUSEA, AND VOMITING, IS THIS THE GALT ENZYME DEFICIENCY? R. Villarreal*,1, F. Tabatabaian2, D. Ledford1, 1. Tampa, FL; 2. St. Petersburg, FL Introduction: Galactosemia is a rare genetic disorder not frequently encountered in clinical practice. GALT enzyme deficiency is in the spectrum of non IgE mediated food intolerance. However, when encountered with symptoms consistent anaphylaxis, the possibility of an IgE mediated reaction should not be excluded. Case Description: Our patient is a 17-year old female who presented for evaluation of food intolerance after ingestion of milk. She was diagnosed as a carrier, or having a mild variant of galactosemia, at birth. She had been strictly avoiding milk since infancy, until her recent exposure to baked goods made with milk. After ingestion she developed immediate urticaria, nausea and vomiting. Physical exam was found to be unremarkable. Her in-vitro testing demonstrated a serum IgE to milk >100 kU/L (normal < 0.1 kU/L). Discussion: This is a unique case that demonstrates both non IgE mediated and IgE mediated intolerance to milk in the same patient. Classic galactosemia and Duarte variant galactosemia are autosomal recessive disorders that lead to the deficiency of the enzyme galactose-1-phosphate uridyltransferase. Diagnosis is made on the newborn screen. Classic galactosemia presents with feeding problems, failure to thrive, liver damage, bleeding, and infections. Duarte variant galactosemia presents as jaundice in infancy that resolves when switching to low-galactose formula. Our patient had symptoms upon introduction of milk later in life that was not fully explained by her diagnosis of Duarte variant of galactosemia, but rather by an IgE mediated milk allergy. To date, she is avoiding milk and carries an epinephrine autoinjector.