FPIES ANOTHER DISEASE ABOUT WHICH YOU SHOULD KNOW OBJECTIVES FPIES FPIES 11/10/2016. What is that? Robert P. Dillard, M.D.
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1 ANOTHER DISEASE ABOUT WHICH YOU SHOULD KNOW What is that? Robert P. Dillard, M.D. Food Protein Induced Enterocolitis Syndrome. OBJECTIVES 1: Awareness of this syndrome 2: Characteristics 3: Diagnosis 4: Treatment. NON IgE MEDIATED GASTROINTESTINAL FOOD HYPERSENSITIVITY ACUTE CHRONIC this is one type of adverse reaction to food. From Turnbull, Adams, Gorard Alimentary Pharmacology and Therapeutics 1
2 ADVERSE REACTION TO FOOD FOOD ALLERGY toxic reaction Infectious Contaminated NON IgE MEDIATED Pharmacological Caffeine Tyramine None immune Food intolerance Enzyme deficiencies Metabolic Errors of metabolism fructose intolerance Tyrosinemia Disaccharidase deficiency unexplained Food Protein Induced enterocolitis syndrome. Food Protein Induced proctocolitis Food Protein Induced enteropathy. Modified from Turnbull, et.al. PATIENT Hospital consult Recurrent vomiting and diarrhea 5 month old female infant Second hospitalization. Symptoms and Signs. vomiting gray, ashen, unresponsive diarrhea with specks of mucus and blood Treated with IV fluids and antibiotics CBC with increased polymorphonuclear cells Negative second sepsis evaluation Seemed well about 4 to 6 hours after episode Her mother reported baby was fine. Real, not composite. Continued Normal pregnancy, labor, delivery Exclusive breast feeding Rice cereal about 2 or so hours before each episode. Case 2 Real patient Office consult Complaint of recurrent severe vomiting and some diarrhea. 6.5 months old male Normal pregnancy, labor, delivery. 3 episodes of sudden vomiting, lethargy, seemed out of it Poor color, Slept 2 to 3 hours, awoke, fine. Breast fed. Rice cereal recently introduced. Plums, squash, sweet potatoes, green beans for three to four weeks. COMMON SYMPTOMS REVIEW OF 7 COHORT STUDIES. ONE ADULT. 6 PEDIATRICS ONE OFC ONE PROSPECTIVE POPULATION STUDY NUMBERS FROM 16 TO 66 Mixture of acute/chronic & mostly not stated. 2
3 COMMON SYMPTOMS VOMITING : almost all in pediatrics studies : 96% in OFC Diarrhea : from 7 ( OFC) to 58 % Lethargy : 7 (OFC) To 85% Pallor : 14 (mostly chronic type) to 67% Abd. Pain : 77% of adults. Hypotension: 77% in one, 19 % (OFC) Common Symptoms Prospective study in Israel 13, 019 infants 44 vomiting : all lethargy : 77% diarrhea : 25% ( bloody in 14%) pallor : 14% Main focus: cow s milk, all in first 6 months ACUTE Delayed onset after ingestion Classic is 2 to 4 hours. Predominate Symptoms repetitive vomiting. Shorter and longer durations have been described diarrhea ( up to 50%), may be delayed hours Pallor, lethargy Additional symptoms Irritability Loss of consciousness Clamminess Hypotonia Cyanosis Hematochezia Melena Malodorous, sticky, stools. Hypothermia ( 24%) Hypotonia ( 5 77%) SHOCK LIKE STATE CHRONIC Earlier age of onset Vomiting Reflux Chronic diarrhea ( may have blood ) FTT Irritable. Anemia Hypoalbuminemia Metabolic acidosis Methemoglobinemia Cho in stool Increased wbc ( overlap with other enteropathy). LABORATORY Increased WBC with L shift Thrombocytosis methemoglobinemia Fecal leukocytes CHO in stool Increased lymphocytes in gastric aspirate. THERE ARE NO DIAGNOSTIC LAB TESTS FOR. 3
4 NOT IgE All reports and studies eliminated IgE type reactions in their reports. SPT, blood, OFC. DIAGNOSIS No specific test. Clinical : History and presentation May occur on first exposure to food ( known). Differential : broad and frightening. It may be confusing THE USUAL CULPRITS COW S MILK SOY RICE OATS Reported Food Triggers Liquid: cow s milk, soy milk, goat s milk Grains : rice, oat, barley, wheat Veg. : sweet potatoes, squash, pumpkin, corn, carrots, white potato, spinach, cauliflower, cucumber Legumes: green peas, peanut, string bean, lentil, kidney beans. Fruits: banana, orange, pineapple, apple, more. MORE Animal protein: egg, chicken, turkey, beef, lamb, pork Seafood: fish, shellfish, mollusks Other: almond, other tree nuts, mushroom, Corn, Saccharomyces boulardi Adapted from Nowak, PCNA 4
5 Table V Characteristics of OFCs in subjects with There were no statistically significant differences between the characteristics of the OFCs to milk, soy, grains, and other foods. Food involved (no.) Milk (n = 72), soy (n = 44), grains[low *] (n = 48), others[dagger] (n = 16) No. of positive OFC results/no. of total OFCs (%) 74/180 (41) No. of patients 82 Age at OFC (y) Median 2.8 IQR[double dagger] 2 5 Sex, no. (%) Male 41 (50) Female 41 (50) Symptoms during the positive OFC, no. (%) Vomiting 70 (96) Abdominal pain 59 (80) Hypotension 14 (19) Diarrhea 5 (7) Altered state of consciousness/lethargy 5 (7) Timing of the reaction in positive OFC (min [range]) From the first dose 150 (35 370) From the last dose 120 (5 320) To complete recovery 50 (0 460) Treatment of positive OFC, no. (%) Intravenous normal saline bolus 70 (96) Intravenous corticosteroids 69 (94) None 3 (4) Hospitalization 0 (0) No. of reactions before OFC Median 2 Range 0 multiple Timing from the most recent reaction (mo) Median 13 IQR[double dagger] /10/2016 Food allergies in Children A shorter list: 85% are due to Cow s milk Soy Egg Wheat Peanut and tree nuts Fish and shell fish Other: rice and oats for. Reaction to Multiple Foods One third of cow s milk, soy develop solid food reaction Fifty per cent ( IN USA) react to more than one grain ( rice and oats) Other estimates ( 80%) in USA only one food. Multiples foods Oral Food Challenge Table III Rates of concomitant to multiple foods NA, Not applicable. If to: Milk Soy Solid Milk NA 38% 20% Soy 37% NA 14% Solid 20% 13% 44% More than 50% solid reacted to 2 or more foods. Those reacting to rice 44% to oats. From Caubet, et.al Time to resolution TREATMENT Most by the time they enter first grade BUT: Milk that develop IgE protracted May convert to acute reactions and anaphylaxis From Caubert, et.al IV fluids IV methylprednisolone ( 1 mg/kg) to 60/80 Ondansetron IV or IM (?oral) case reports Milder episodes may resolve at home with no treatment Long term, diet Most ( 75 80%) tolerate protein hydrolysate Formula. 5
6 TEAM ALLERGIST RD FEEDING TEAM SOCIAL /PSYCH GI ( WHY ALLERGIST FIRST) FOOD ALLERGIC CHILDREN: 35 TO 71% ARE ATOPIC 33 TO 40% ALLERIC RHINITIS 33 TO 49% EVIDENCE ASTHMA FOUNDATION. RESOURCES 6
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