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sIgE was determined to a-gal, bee venom, wasp venom, rApi m 1, rApi m 2, rApi m 5, rApi m 10, r Ves v 1, rVes v 5, and CCD MUXF3 (cross-reactive carbohydrate determinants). The a-gal sIgE value was 3.65 kUA/L (<0.10 kUA/L). sIgE to all insect venom allergens was negative. Total IgE and tryptase were in the normal range. In 2016, oral provocation tests were performed with hydrolyzed gelatin (0.5 g, 1 g, 5 g) and pork kidney (3 g, 5 g, 10 g). Hydrolyzed gelatin was tolerated, but the patient developed generalized erythema, throat tightness, and dyspnea 2 hours after consumption of 10 g of pork kidney. A basophil activation test (BAT) was performed in 2016 with a pork kidney extract (22.7 mg/mL to 0.00022 mg/mL), alpha-Gal-HSA (1000 ng/mL to 0.32 ng/mL) (BAG2-GAL; Bühlmann Laboratories AG), and Creon (2227 ng/mL to 0.0227 ng/mL) according to previous reports [1,2]. All substances activated the basophils. In 2018, oral provocation tests were performed with 3 capsules of Creon 25000 (first day), ASA (1000 mg) and 3 capsules of Creon 25000 (second day), and, finally, ASA, alcohol (20 mL), physical exercise, and Creon 25000 (third day). No symptoms were recorded. sIgE to a-gal showed a value of 0.77 kUA/L (<0.10 kUA/L). In 2018, the BAT performed 1 day after the oral provocation test series with Creon was positive to alpha-Gal-HSA and negative to Creon and bee and wasp venom. The BAT performed 1 month later was once again positive to Creon (Supplementary Table). This case shows that Creon, an a-gal–containing porcine pancreas extract can be tolerated at higher doses than usual and despite cofactors by patients with a-gal syndrome. This is particularly relevant in cases where the drug is necessary to treat exocrine pancreatic insufficiency. Two similar cases have been described [3], although this is the first case in which cofactors, which are well-known amplifiers of reactions to a-gal [4], were also tested with the a-gal–containing drug. The allergy work-up enabled us to rule out hymenoptera allergy as a cause or cofactor of the anaphylaxis. Several factors may enable patients to tolerate Creon despite sensitization. Provocation tests have generally shown that sensitivity to a-gal can be quite variable: some patients react only to pork kidney but routinely tolerate muscle meat, even in the presence of cofactors, whereas others, eg, patients with mastocytosis, experience biphasic immediate and delayed severe reactions [5-7]. Higher amounts of a-gal determinants in kidney than in meat may explain these observations. In the case we report, the patient also tolerated hydrolyzed gelatin. We do not know how much a-gal is present in Creon. In a recently published study [1] and in the present case, basophil activation was more pronounced with Creon than with pork kidney extract, thus pointing to a relevant amount. Inhibition experiments also revealed that the absolute reduction in a-gal sIgE binding by Creon could be reduced by up to 97% (median, 83.5%), thus confirming specific recognition of a-gal epitopes in Creon [3]. In the present case, the eliciting dose of pork kidney was 10 g, and the total amount of Creon ingested was 0.9 g (gel capsules), which might be below the threshold for eliciting symptoms. In the context of Creon protein extracts [1], a-gal may not be absorbed in the gastrointestinal tract. In vitro studies Negative Oral Provocation Test With Porcine Pancreatic Enzyme Plus Cofactors Despite Confirmed a-Gal Syndrome

Negative Oral Provocation Test With Porcine Pancreatic Enzyme Plus Cofactors Despite Confirmed α-Gal Syndrome