普通变异型免疫缺陷病合并肝硬化1例并文献复习

摘要

目的：讨论普通变异型免疫缺陷病（common variable immunodeficiency disease，CVID）的发病机制、临床表现及治疗方法。方法：报道1例普通变异型免疫缺陷病合并肝硬化患者并复习相关文献。结果：普通变异型免疫缺陷病是临床少见病，易误诊，病因不清，临床表现复杂，部分患者病程中可出现肝功能异常，甚至并发肝硬化。结论：临床反复感染的患者应考虑 CVID 的可能，须进一步作血浆免疫球蛋白的检测确诊。对已经确诊患者定期补充静脉丙种球蛋白，同时还应定期检查肝功能。%Objective:To explore the pathogenesis,clinical manifestations and treatment of common variable immunodeficiency disease(CVID).Methods:A case of common variable immunodeficiency disease with cirrhosis was reported and related literature was reviewed.Results:CVID was rare,often misdiagnosed,unclear pathogeny and complicated clinical manifestations.Some patients may appear abnormal liver function,or even cirrhosis in the disease progression.Conclusion:Clinical recurrent infections should consider the possibility of CVID,which is confirmed by further detection of serum immunoglobulin.Patients who have been diagnosed CVID need intravenous gamma globulin periodically and liver function examination regularly.