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[普通可变免疫缺陷:两例报告及文献综述]

[Common variable immunodeficiency: report of two cases and literature review].

作者信息

Li Jian-ping, Wang Hui-jun, Zhang Lei, Yang Ren-chi, Zhang Feng-kui

机构信息

Institute of Hematology and Blood Diseases Hospital, CAMS & PUMC, Tianjin 300020, China.

出版信息

Zhonghua Xue Ye Xue Za Zhi. 2009 Feb;30(2):111-4.

Abstract

OBJECTIVE

To promote the awareness of common variable immunodeficiency (CVID).

METHODS

Report two cases of CVID and review related literature.

RESULTS

The two CVID patients were manifested recurrent infections of respiratory and digestive tract, splenomegaly, significantly reduced serum immunoglobulin, since adolescence. They were treated with intravenous gammaglobulin, anti-infection and symptomatic therapies and the diseases were well controlled. Case 1 was diagnosed as with spleen peripheral T cell lymphoma after splenectomy. Case 2 developed nutritional megaloblastic anemia resulting from repeated digestive tract infections.

CONCLUSION

CVID is a heterogeneous group of diseases with later-onset, characterized by hypogammaglobulinemia and recurrent bacterial infections. Early diagnosis and regular treatment may improve the prognosis.

摘要

目的

提高对普通可变型免疫缺陷病(CVID)的认识。

方法

报告2例CVID病例并复习相关文献。

结果

2例CVID患者自青春期起出现反复的呼吸道和消化道感染、脾肿大,血清免疫球蛋白显著降低。给予静脉注射丙种球蛋白、抗感染及对症治疗后病情得到良好控制。病例1脾切除术后诊断为脾外周T细胞淋巴瘤。病例2因反复消化道感染发生营养性巨幼细胞贫血。

结论

CVID是一组起病较晚的异质性疾病,以低丙种球蛋白血症和反复细菌感染为特征。早期诊断和规范治疗可改善预后。

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