Nowak-Węgrzyn A, Jarocka-Cyrta E, Moschione Castro Apb
Jaffe Food Allergy Institute, Department of Pediatrics, Division of Allergy and Immunology, Kravis Children's Hospital, Icahn School of Medicine at Mount Sinai, New York, New York, USA.
Department of Pediatrics, Gastroenterology and Nutrition, Children's Hospital, University of Warmia and Masuria, Olsztyn, Poland.
J Investig Allergol Clin Immunol. 2017;27(1):1-18. doi: 10.18176/jiaci.0135.
Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE-, cell-mediated food allergy of unknown prevalence and pathophysiology. Onset is typically during the first year of life; seafood-induced FPIES may start in adulthood. Acute FPIES manifests within 1-4 hours after ingestion with repetitive emesis, pallor, and lethargy progressing to dehydration and hypovolemic shock in 15% of cases. Chronic FPIES manifests with intermittent emesis, watery diarrhea, and poor growth progressing to dehydration and hypovolemic shock over a period of days to weeks. Chronic FPIES has been only reported in infants aged less than 3 months fed with cow milk (CM) or soy formula. The most common triggers are CM, soy, rice, and oat. Diagnosis of FPIES relies on recognition of a pattern of clinical symptoms and may be missed owing to the absence of typical allergic symptoms (eg, urticaria, wheezing) and delayed onset in relation to food ingestion. Physician-supervised food challenge is recommended if diagnosis or the trigger food is not clear and to evaluate for resolution. Testing for food-specific IgE is usually negative, although a subset of patients, usually with CM-induced FPIES may develop sensitization to foods. Such atypical FPIES tends to have a more prolonged course. Despite the potential severity of the reactions, no fatalities have been reported, and FPIES has a favorable prognosis. In most cases, FPIES resolves by age 3-5 years, although persistence of CM-induced FPIES and soy FPIES into adulthood has been reported. The first international consensus guidelines on diagnosis and management of FPIES were published in 2017. Given that the pathophysiology of FPIES is poorly understood, there are no diagnostic biomarkers and no therapies to accelerate resolution. These unmet needs warrant future investigations to improve the care of patients with FPIES.
食物蛋白诱导的小肠结肠炎综合征（FPIES）是一种非IgE介导的细胞介导性食物过敏，其患病率和病理生理学尚不清楚。发病通常在生命的第一年；海鲜诱导的FPIES可能在成年期开始。急性FPIES在摄入后1 - 4小时内出现，伴有反复呕吐、面色苍白和嗜睡，15%的病例会发展为脱水和低血容量性休克。慢性FPIES表现为间歇性呕吐、水样腹泻和生长发育不良，在数天至数周内发展为脱水和低血容量性休克。慢性FPIES仅在小于3个月的婴儿中报道，这些婴儿喂养牛奶（CM）或大豆配方奶。最常见的诱因是CM、大豆、大米和燕麦。FPIES的诊断依赖于对临床症状模式的识别，由于缺乏典型的过敏症状（如荨麻疹、喘息）以及与食物摄入相关的延迟发作，可能会漏诊。如果诊断不明确或触发食物不清楚，建议在医生监督下进行食物激发试验，并评估症状是否缓解。食物特异性IgE检测通常为阴性，尽管一部分患者，通常是CM诱导的FPIES患者，可能会对食物产生致敏。这种非典型FPIES往往病程更长。尽管反应可能很严重，但尚未有死亡报告，FPIES预后良好。在大多数情况下，FPIES在3 - 5岁时缓解，尽管有报道称CM诱导的FPIES和大豆FPIES会持续到成年期。关于FPIES诊断和管理的首个国际共识指南于2017年发布。鉴于对FPIES的病理生理学了解不足，目前没有诊断生物标志物，也没有加速缓解的治疗方法。这些未满足的需求值得未来开展研究，以改善FPIES患者的护理。
