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2岁以下儿童的炎症性肠病及炎症性肠病模拟性小肠结肠炎

IBD and IBD mimicking enterocolitis in children younger than 2 years of age.

作者信息

Cannioto Z, Berti I, Martelossi S, Bruno I, Giurici N, Crovella S, Ventura A

机构信息

Department of Reproduction and Development Sciences, University of Trieste-IRCCS Burlo Garofolo, Trieste, Italy.

出版信息

Eur J Pediatr. 2009 Feb;168(2):149-55. doi: 10.1007/s00431-008-0721-2. Epub 2008 Jun 11.

Abstract

Inflammatory bowel disease (IBD) is uncommon in children younger than 2 years of age. The criteria for differentiating IBD from other diseases with similar clinical presentation is unclear. We describe 16 patients who, between 1984 and 2004, received a histological diagnosis of IBD during the first two years of life. Six patients presented with histological Crohn's disease, eight with ulcerative colitis and two with indeterminate colitis. The median age at diagnosis was 125 days (range 1 day to 18 months) and the medium follow up was 89 months (range 65 days to 20 years). The disease appeared to be very severe: four children (25%) underwent total parenteral nutrition (TPN), two received colectomy (12.5%) and three patients died. Many of the patients required an aggressive, multidrug, immunosuppressive approach (azathioprine [AZA], Infliximab, thalidomide, cyclosporine A). One child presented with a hypogammaglobulinaemia without any specific immunodeficiency, while in the other patients, Wiskott-Aldrich syndrome (WAS) (4 cases) and chronic granulomatous disease (CGD) (2 cases) were identified. In 6/16 cases, allergic colitis was first considered; these cases initially underwent cow's milk protein-free diet as the only therapy before IBD was finally diagnosed. In conclusion, early IBD has a severe prognosis and often needs an aggressive therapeutic approach. Furthermore, an improper diagnosis of allergic colitis might cause an important diagnostic delay. Some severe immunodeficiencies, such as WAS and CGD, may represent a problem in terms of differential diagnosis and might be wrongly diagnosed as very early onset IBD.

摘要

炎症性肠病(IBD)在2岁以下儿童中并不常见。将IBD与其他临床表现相似的疾病区分开来的标准尚不清楚。我们描述了16例在1984年至2004年间于生命的头两年接受IBD组织学诊断的患者。6例患者表现为组织学确诊的克罗恩病,8例为溃疡性结肠炎,2例为不确定性结肠炎。诊断时的中位年龄为125天(范围1天至18个月),中位随访时间为89个月(范围65天至20年)。该病似乎非常严重:4名儿童(25%)接受了全胃肠外营养(TPN),2名接受了结肠切除术(12.5%),3名患者死亡。许多患者需要积极的、多药联合的免疫抑制治疗方法(硫唑嘌呤[AZA]、英夫利昔单抗、沙利度胺、环孢素A)。一名儿童出现低丙种球蛋白血症且无任何特异性免疫缺陷,而在其他患者中,发现了维斯科特-奥尔德里奇综合征(WAS)(4例)和慢性肉芽肿病(CGD)(2例)。在16例中有6例,最初考虑为过敏性结肠炎;这些病例在最终确诊IBD之前,最初仅接受无牛奶蛋白饮食作为唯一治疗。总之,早期IBD预后严重,通常需要积极的治疗方法。此外,对过敏性结肠炎的不当诊断可能会导致重要的诊断延迟。一些严重的免疫缺陷,如WAS和CGD,在鉴别诊断方面可能是个问题,可能会被误诊为极早发型IBD。

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