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[爱泼斯坦-巴尔病毒活动性慢性感染的肝脏和结肠表现]

[Liver and colonic manifestation of active chronic infection by Epstein Barr virus].

作者信息

Sabillón-Mendoza Alejandra Maricela, Imbett-Yepez Sharon María, Ignorosa-Arellano Karen, Cadena-León Jose Francisco, Zárate-Mondragón Flora, Cervantes-Bustamante Roberto, Ramírez-Mayans Jaime

机构信息

Departamento de Gastroenterología y Nutrición, Instituto Nacional de Pediatría, Coyoacán, México.

出版信息

Andes Pediatr. 2022 Jun;93(3):416-422. doi: 10.32641/andespediatr.v93i3.3676.

DOI:10.32641/andespediatr.v93i3.3676
PMID:35857013
Abstract

INTRODUCTION

Chronic active Epstein Barr virus infection (CAEBV) is a rare condition, where the body is unable to counteract Epstein Barr viral replication (EBV), leading the patient to a chronic state with variable symptoms. Early recognition of infrequent or atypical clinical manifestations is relevant due to the particularities of their management and prognosis.

OBJECTIVE

to describe a case of CAEBV manifes ted with colitis and hepatitis, summarizing the clinical-pathological and endoscopic characteristics and their evolution.

CLINICAL CASE

A 6-year-old girl, previously healthy, presented recurrent episodes of jaundice, hepatosplenomegaly, and fever. EBV hepatitis was diagnosed with a blood viral load of 328,000 copies / mL. Her liver biopsy revealed Epstein-Barr virus-encoded small RNAs (EBER). She evolved with mucosanguineous diarrhea and weight loss; the colonoscopy showed loss of the haustral pattern, multiple aphthous ulcers covered with fibrin, and 7 million copies of EBV / gram of tissue were found in the colon. T-cell lineage infection was identified, therefore Rituximab was started, with a decrease in viral load, complete resolution of diarrhea, and improvement in liver function tests. The definitive treatment was bone marrow transplantation.

CONCLUSIONS

CAEBV is a serious disor der, little documented, and should be considered in the face of a prolonged or intermittent course of hepatitis, accompanied by general and gastrointestinal manifestations such as chronic diarrhea, hematochezia, and weight loss, since its outcome without treatment can be fatal.

摘要

引言

慢性活动性EB病毒感染(CAEBV)是一种罕见病症,机体无法抵抗EB病毒复制(EBV),导致患者处于具有多种症状的慢性状态。鉴于其治疗和预后的特殊性,早期识别罕见或非典型临床表现具有重要意义。

目的

描述一例表现为结肠炎和肝炎的CAEBV病例,总结临床病理及内镜特征及其演变过程。

临床病例

一名6岁以前健康的女孩出现黄疸、肝脾肿大和发热反复发作。通过血液病毒载量328,000拷贝/毫升诊断为EBV肝炎。她的肝脏活检显示EB病毒编码的小RNA(EBER)。她出现了黏液血性腹泻和体重减轻;结肠镜检查显示结肠袋消失,多个覆盖有纤维蛋白的阿弗他溃疡,并且在结肠中发现每克组织有700万拷贝的EBV。确定为T细胞谱系感染,因此开始使用利妥昔单抗治疗,病毒载量下降,腹泻完全缓解,肝功能检查改善。最终治疗是进行骨髓移植。

结论

CAEBV是一种严重疾病,文献记载较少,当出现肝炎的长期或间歇性病程,并伴有慢性腹泻、便血和体重减轻等全身及胃肠道表现时应考虑该病,因为未经治疗其后果可能是致命的。

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