Abstract
The alpha-gal syndrome is an allergic syndrome that comprises two clinical pictures: an immediate hypersensitivity to drugs containing alpha-gal and a delayed hypersensitivity to the ingestion of red mammalian meat. This allergic syndrome is often under-recognized, and patients are mislabeled with diagnosis as spontaneous urticaria or idiopathic anaphylaxis. Even though less frequently, children could also be of interest, especially in tick-endemic areas. In most cases, a positive anamnesis for tick bites months before the onset of symptoms is recorded. The clinical manifestations could range from asymptomatic cases to severe anaphylaxis. The most frequently used diagnostic test is the determination of specific IgE for alpha-gal. Oral provocation test is usually reserved to unclear cases or to verify tolerance after diet. No long-term follow-up studies have been published, although an elimination diet could lead to a decrease of specific IgE for alpha-gal and a possible reintroduction of some avoided foods. This paper provides a literature review, focused on pediatric age, and an evaluation of available diagnostic tests. We analyze the correlation between tick bites and symptom onset and unfold the different clinical pictures to help clinicians to promptly recognized this syndrome. Lastly, we address unmet needs in this specific allergy.
Highlights
The alpha-gal syndrome (AGS) is an allergic syndrome comprising two scenarios: an immediate hypersensitivity to drugs containing alpha-gal and a delayed hypersensitivity due to the ingestion of red meat
The allergic response observed is due to the production of specific IgE for the disaccharide galactose-α-1,3-galactose, which is expressed on the surface of glycolipids and glycoprotein of mammalian cells and synthesized by the galactosyl-α-1,3-galactosyl-synthetase
Children seem to have a lower incidence of AGS [13% in the study by Wilson et al [32] and 35% in the study by Mabelane et al [31]], lower specific IgE (sIgE)-α-gal levels, a restricted number of trigger foods, a predominance of gastrointestinal symptoms, and exercise as the main allergic co-factor [7]
Summary
The alpha-gal syndrome (AGS) is an allergic syndrome comprising two scenarios: an immediate hypersensitivity to drugs containing alpha-gal and a delayed hypersensitivity due to the ingestion of red meat (intended as meat from non-primate mammals). It is still not clear what drives the IgE production for alpha-gal (sIgE-α-gal), but in most cases, there is a positive anamnesis for tick bites in the months before the onset of symptoms.
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