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食物蛋白诱导的小肠结肠炎综合征中的唐氏综合征:极高且意外的患病率。

Down Syndrome in FPIES: An Overwhelming and Unexpected Prevalence.

作者信息

Pecora Valentina, Mennini Maurizio, Valluzzi Rocco, Fierro Vincenzo, Villani Alberto, Valentini Diletta, Fiocchi Alessandro

机构信息

Translational Research in Pediatric Specialities Area, Division of Allergy, Bambino Gesù Children's Hospital, Istituto di Ricovero e Cura a Carattere Scientifico, Piazza Sant'Onofrio, 4, 00165 Rome, Italy.

Pediatric Unit, Pediatric Emergency Department (DEA), Bambino Gesù Children's Hospital, Istituto di Ricovero e Cura a Carattere Scientifico, Piazza Sant'Onofrio, 4, 00165 Rome, Italy.

出版信息

J Clin Med. 2022 Jul 13;11(14):4047. doi: 10.3390/jcm11144047.

Abstract

Down syndrome (DS) is one of the most common chromosomal anomalies. Gastrointestinal disorders in DS are predominantly related to anatomical anomalies and celiac disease. In 2015, the first two cases of non-IgE-mediated food allergy in patients with DS were described. However, gastrointestinal symptoms experienced by subjects with DS have never been related to a possible non-IgE-mediated food allergy and a Food Protein-induced Enterocolitis syndrome (FPIES). A retrospective descriptive single-center study was conducted. Subjects included were children with acute FPIES who entered our institutional follow-up protocol between January 2013 and January 2020. Among the 85 patients (forty-nine boys-57.6%), ten (11.76%) were children with DS. In our population, the FPIES triggers included different foods (such as milk, egg, fruit, fish, wheat, soy, beef, etc.). Nine patients with DS showed FPIES reactions after ingesting cow's milk (one even with beef and three with soy), while the last one was affected by FPIES to fish. Considering the subgroup of patients affected by cow's milk FPIES (40 subjects overall), 22.5% had a diagnosis of DS. Patients with DS experienced acute FPIES reactions with a severity degree slightly higher than that reported in other patients, ranging from mild-moderate to severe or very severe. During the acute reactions, the patients with DS showed increased white blood cell production, absolute neutrophil count and C-reactive protein levels. This series provides a starting point for novel hypothesis-testing clinical research and possible specific immunological alterations in FPIES children with or without DS.

摘要

唐氏综合征(DS)是最常见的染色体异常疾病之一。DS患者的胃肠道疾病主要与解剖结构异常和乳糜泻有关。2015年,首次报道了两例DS患者发生非IgE介导的食物过敏。然而,DS患者出现的胃肠道症状从未与可能的非IgE介导的食物过敏和食物蛋白诱导的小肠结肠炎综合征(FPIES)相关联。我们进行了一项回顾性描述性单中心研究。纳入的受试者为2013年1月至2020年1月期间进入我们机构随访方案的急性FPIES患儿。在85例患者(49名男孩,占57.6%)中,10例(11.76%)为DS患儿。在我们的研究人群中,FPIES的触发因素包括不同的食物(如牛奶、鸡蛋、水果、鱼、小麦、大豆、牛肉等)。9例DS患儿在摄入牛奶后出现FPIES反应(1例同时摄入牛肉,3例同时摄入大豆),而最后1例对鱼发生FPIES。考虑到受牛奶FPIES影响的患者亚组(共40名受试者),22.5%被诊断为DS。DS患者发生的急性FPIES反应严重程度略高于其他患者,范围从轻度-中度到重度或极重度。在急性反应期间,DS患者的白细胞生成、绝对中性粒细胞计数和C反应蛋白水平升高。本系列研究为开展新的假设检验临床研究以及探究有无DS的FPIES患儿可能存在的特定免疫改变提供了一个起点。

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