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[原发性免疫缺陷导致抗体缺乏患者的胃肠道表现]

[Gastrointestinal manifestations in patients with primary immunodeficiencies causing antibody deficiency].

作者信息

Díez Rubén, García M Jesús, Vivas Santiago, Arias Laura, Rascarachi Gabriela, Pozo Elvira del, Vaquero Luis M, Miguel Aleida, Sierra Mónica, Calleja Sara, Ruiz De Morales José M

机构信息

Sección de Aparato Digestivo, Hospital Universitario de León, León, España.

出版信息

Gastroenterol Hepatol. 2010 May;33(5):347-51. doi: 10.1016/j.gastrohep.2009.12.012. Epub 2010 Mar 1.

Abstract

INTRODUCTION

Primary immunodeficiencies can lead to gastrointestinal manifestations that are still not well defined.

OBJECTIVE

To analyze gastrointestinal manifestations associated with primary immunodeficiencies.

MATERIAL AND METHODS

We performed a retrospective study that included patients diagnosed with primary antibody deficiencies in a third-level hospital. The patients were divided into two groups: isolated IgA deficiency and common variable immunodeficiency syndrome (CVIS). The timing of presentation and type of gastrointestinal symptoms were analyzed.

RESULTS

There were 57 patients: 20 with CVIS (35%) and 37 with isolated IgA deficiency (65%). Diagnosis was made in the pediatric age in 17 patients, of whom 13 had isolated IgA deficiency. In 84% of the patients, diagnosis of immunodeficiency was made before the development of gastrointestinal manifestations. Digestive symptoms were found in 74% of the patients, the most frequent being diarrhea. In 46% of the patients, digestive disease was confirmed, mainly through endoscopy. Celiac-like lesions, chronic atrophic gastritis, ulcerative colitis-like disease and Crohn's disease were more common in CVIS. In isolated IgA deficiency, Helicobacter pylori-positive chronic gastritis predominated. Mean age was significantly higher (36 vs. 24 years, p=0.02) and IgA titer significantly lower (17 vs. 34UI/ml; p=0.008) in patients with associated gastrointestinal disease.

CONCLUSIONS

Gastrointestinal symptoms are frequent and lead to endoscopic diagnosis in half of patients with primary immunodeficiencies. Ulcerative colitis, and celiac- and Crohn's-like disease are atypical entities that occur in CVIS.

摘要

引言

原发性免疫缺陷可导致胃肠道表现,但目前仍未完全明确。

目的

分析与原发性免疫缺陷相关的胃肠道表现。

材料与方法

我们进行了一项回顾性研究,纳入了一家三级医院中被诊断为原发性抗体缺陷的患者。患者分为两组:孤立性IgA缺乏症和常见变异型免疫缺陷综合征(CVIS)。分析了症状出现的时间和胃肠道症状的类型。

结果

共有57例患者,其中20例患有CVIS(35%),37例患有孤立性IgA缺乏症(65%)。17例患者在儿童期确诊,其中13例患有孤立性IgA缺乏症。84%的患者在出现胃肠道表现之前就已确诊免疫缺陷。74%的患者出现消化系统症状,最常见的是腹泻。46%的患者经内镜检查确诊为消化系统疾病。乳糜泻样病变、慢性萎缩性胃炎、溃疡性结肠炎样疾病和克罗恩病在CVIS中更为常见。在孤立性IgA缺乏症中,幽门螺杆菌阳性的慢性胃炎最为常见。伴有胃肠道疾病的患者平均年龄显著更高(36岁对24岁,p=0.02),IgA滴度显著更低(17对34UI/ml;p=0.008)。

结论

胃肠道症状在原发性免疫缺陷患者中很常见,半数患者经内镜检查确诊。溃疡性结肠炎以及乳糜泻样和克罗恩病样疾病是CVIS中出现的非典型病症。

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