目的：探讨遗传性出血性毛细血管扩张症（ HHT）家系的临床特征、诊断及治疗。方法：对先证者进行家系调查、体格检查及胃肠镜检查，经过沙利度胺治疗后复查胃肠镜。结果：本家系4代包括先证者在内共7例患者。先证者经沙利度胺系统治疗后，症状缓解，胃镜复查出血灶明显减少。结论：遗传性出血性毛细血管扩张症家系以反复胃出血为主要表现，沙利度胺能够很好地控制患者的出血症状。%Objective:To learn more the clinical features,diagnosis and treatment of Hereditary hemorrhagic telangiectasia ( HHT). Methods:The records before and after thalidomide therapy,including family investigation,physical examination and endoscopic examination,were reviewed of the patient who was presented with recurrent gastrointestinal bleeding,and later was diagnosed as HHT. Results:In the four-generation pedigree,including the proband himself,a total of 7 patients were suffered from HHT. After treatment by thalidomide,the patient's gastrointestinal bleeding was effectively controlled. Conclusion:This family of HHT patients are characterized by repeated gastric bleeding,and thalidomide therapy is proved to be effective.
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