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乳糜泻中选择性免疫球蛋白A缺乏症的患病率和临床特征:一项意大利多中心研究。意大利儿科学胃肠病学和肝病学会(SIGEP)以及乳糜泻“纤细俱乐部”工作组

Prevalence and clinical features of selective immunoglobulin A deficiency in coeliac disease: an Italian multicentre study. Italian Society of Paediatric Gastroenterology and Hepatology (SIGEP) and "Club del Tenue" Working Groups on Coeliac Disease.

作者信息

Cataldo F, Marino V, Ventura A, Bottaro G, Corazza G R

机构信息

Clinica Pediatrica R, Palermo, Italy.

出版信息

Gut. 1998 Mar;42(3):362-5. doi: 10.1136/gut.42.3.362.

Abstract

BACKGROUND

Selective immunoglobulin A (IgA) deficiency (SIgAD) is associated with coeliac disease (CD).

AIM

To make a retrospective study of the association of SIgAD with CD in Italy.

METHODS

Hospital medical records of 2098 patients consecutively diagnosed as having CD were reviewed.

RESULTS

Of 2098 patients with CD, 54 (2.6%) had SIgAD, representing a 10-16-fold increase over that in the population in general. This increase was not influenced by age or geographical factors. Patients with SIgAD had a higher incidence of silent forms (7/54, 13%), recurrent infections (16/54, 29.6%), and atopic diseases (7/54, 13%) than those without. The association with autoimmune and malignant diseases and the outcome after eating a gluten free diet were similar in patients with or without SIgAD. In all patients with SIgAD, antibodies for IgA gliadin and endomysium were absent, but serum levels of IgG anti-gliadin antibodies were high in almost all of them (51/54).

CONCLUSIONS

Serum IgA should be measured in order to be able to interpret negative results for IgA anti-gliadin antibodies and anti-endomysial antibodies in patients being screened for CD. Since some patients with CD and SIgAD may be negative for IgG anti-gliadin antibodies, an intestinal biopsy should be performed in all suspected cases.

摘要

背景

选择性免疫球蛋白A(IgA)缺乏症(SIgAD)与乳糜泻(CD)相关。

目的

对意大利SIgAD与CD的关联进行回顾性研究。

方法

回顾了2098例连续诊断为患有CD的患者的医院病历。

结果

在2098例CD患者中,54例(2.6%)患有SIgAD,相较于普通人群增加了10至16倍。这种增加不受年龄或地理因素影响。与无SIgAD的患者相比,患有SIgAD的患者隐匿型(7/54,13%)、反复感染(16/54,29.6%)和特应性疾病(7/54,13%)的发生率更高。有或无SIgAD的患者在自身免疫性疾病和恶性疾病方面的关联以及食用无麸质饮食后的结果相似。在所有患有SIgAD的患者中,不存在IgA麦醇溶蛋白和肌内膜抗体,但几乎所有患者(51/54)的血清IgG抗麦醇溶蛋白抗体水平都很高。

结论

为了能够解读筛查CD患者时IgA抗麦醇溶蛋白抗体和抗肌内膜抗体的阴性结果,应检测血清IgA。由于一些患有CD和SIgAD的患者可能IgG抗麦醇溶蛋白抗体呈阴性,所有疑似病例均应进行肠道活检。

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本文引用的文献

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Prevalence and diagnosis of celiac disease in IgA-deficient children.IgA 缺乏儿童乳糜泻的患病率及诊断
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Gluten enteropathy associated with selective IgA deficiency.与选择性IgA缺乏相关的麸质肠病。
Lancet. 1971 Jul 17;2(7716):121-4. doi: 10.1016/s0140-6736(71)92301-4.
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HLA antigens in IgA deficient paediatric patients.IgA 缺乏的儿科患者中的 HLA 抗原
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