BackgroundDetermining physicians’ awareness about alpha-1 antitrypsin (AAT) deficiency (AATD) may help to explain the discrepancy between the observed and expected number of patients diagnosed with this disease.This study was designed to assess the opinions on knowledge, practice pattern and attitude regarding AATD among physicians in Spain and Portugal.MethodsAn online anonymous survey was performed on pulmonologists (n = 100), internal medicine specialists (IMS) (n = 100) and primary care physicians (PCP) (n = 176). Of the total number of physicians, 221 were from Spain, and 155 were from Portugal. Physicians answered 21 questions related to their personal and professional profile, knowledge regarding AATD and chronic obstructive pulmonary disease (COPD), performance and attitude about AATD, and use of augmentation therapy. Responses were ranked on a 4-point scale indicating the level of agreement. In addition, some of the responses were rated as either “low” or “high” indicating the level of knowledge the respondent felt he/she possessed.ResultsOnly 14 % of physicians reported to “know very well” about AATD (3.3 [SD 0.6] for pulmonologists vs. 2.64 [SD 0.60] for IMS and 2.48 [SD 0.71] for PCP; p < 0.001). Only 45.2 % of physicians correctly answered “<50 mg/dL” as the threshold value of serum AAT to be considered severe AATD (55.0 % of pulmonologists vs. 47.0 % of IMS and 38.6 % of PCP; p = 0.001). Choice of the correct answer did not agree with those physicians self-declaring a high level of AATD knowledge (51.2 %). A total of 43.9 % of physicians correctly identified all diseases or conditions in a list associated or not with AATD. A similar trend was detected when identifying which conditions would be responsive to augmentation therapy (<50 %). Only 15.8 % of specialists performed AATD testing in all patients with COPD (27.0 % pulmonologists, 12.6 % PCP; p = 0.001).ConclusionThe results suggest that a knowledge gap may be contributing to the underdiagnosis of AATD. Physicians in Spain and Portugal showed a marked lack of awareness of their shortcomings in knowledge about AATD, and in general did not follow guidelines and recommendations for AATD testing.

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RESUMENEl síndrome de enterocolitis inducida por proteínas alimentarias es una alergia alimentaria no mediada por inmunoglobulina E que se manifiesta clínicamente con vómitos profusos y repetitivos, en ocasiones, asociados a diarrea, y puede llegar a asociar deshidratación y letargia, con riesgo de desarrollo de shock. A pesar de su potencial gravedad, el índice de sospecha de este síndrome es bajo, lo que demora su diagnóstico, especialmente, en aquellos casos que son desencadenados por alimentos sólidos. La presencia de vómitos y la duración de más de un minuto son los datos clave que pueden diferenciarlo de los episodios breves, resueltos e inexplicados. Se presenta el caso de una lactante de 6 meses de vida con diagnóstico final de síndrome de enterocolitis inducida por proteínas alimentarias por ingesta de kiwi. Palabras clave: kiwi, síndrome de enterocolitis inducida por proteínas alimentarias, episodios breves, resueltos e inexplicados, vómitos, hipersensibilidad. ABSTRACTFood protein-induced enterocolitis syndrome (FPIES) is a non-IgE food allergy manifesting as profuse, repetitive vomiting, sometimes with diarrhea, leading to dehydration and lethargy that can be severe and lead to shock. Despite the potential severity, awareness of FPIES is low and diagnosis is often delayed, especially in those triggered by solid foods. Presence of vomits and duration of more than 1 minute are the key differential factors to distinguish FPIES from brief resolved unexplained events. We report a case of a 6-month-old infant finally diagnosed as having kiwi induced FPIES.