患者男,26岁。因反复鼻腔黏膜出血7年、活动后气短10余年、手指青紫2个月入院。体检:消瘦,面部、唇部、手指及趾尖可见红紫色毛细血管扩张,口唇发绀,杵状指(趾)。实验室检查:白细胞6.61×109/L,红细胞7.03×1012/L,血红蛋白233g/L,血小板127×109/L,总胆红素55μmol/L,直接胆红 Male patient, 26 years old. Due to repeated nasal mucosal hemorrhage 7 years after the event shortness of breath more than 10 years, finger cyanosis 2 months admission. Physical examination: weight loss, face, lips, fingers and tip to see purple-red capillaries dilate, lips cyanosis, clubbing (toe). Laboratory tests: white blood cells 6.61 × 109 / L, red blood cells 7.03 × 1012 / L, hemoglobin 233g / L, platelet 127 × 109 / L, total bilirubin 55μmol / L, direct bile red