目的 探讨血友病A病人行内镜逆行胰胆管造影术（endoscopic retrograde cholangiopancreatography, ERCP）治疗相关肝胆胰疾病的有效性和安全性。方法 回顾性分析2013年12月至2017年3月在我院外科行ERCP治疗的7例血友病A病人临床资料,其中5例轻型,2例中型。有5例为胆总管结石,其中1例伴感染,2例为胰头恶性肿瘤引起胆总管下端梗阻。对血友病病人围术期充分补充抗血友病球蛋白（anti-hemophilic globulin, AHG）,严密监测相关凝血指标和生命体征。结果 2例行十二指肠乳头球囊扩张+网篮取石,2例行括约肌切开+网篮取石,2例行金属支架,1例单独行鼻胆管引流。4例胆总管结石病人予鼻胆管引流术。7例病人均顺利出院,平均住院时间为（9.6±3.2）（7~16） d。术前病人FⅧ:C为(18.7%±11.1%)（1.2%~28.2%）,术前输AHG（2 743±964）（2 000~4 000） U/d。所有病人术后血红蛋白（100±30）（51~131） g/L和红细胞比容（0.293±0.084）（0.160~0.396）,与术前[（105±35）（50~150） g/L和（0.307±0.097）（0.161~0.423）相比差异无统计学意义（P>0.05）。术后平均血淀粉酶为（105±80）（28~237） U/L。所有病例均无围术期出血、消化道穿孔、胰腺炎等ERCP相关并发症发生。结论 在补充AHG等治疗下,对血友病A病人行ERCP治疗安全有效。

Objective To investigate the effectiveness and safety of ERCP in treatment of hemophilia A patients. Methods The data of 7 cases with hemophilia A including 5 cases mild type and 2 cases moderate type in our hospital performed ERCP from December 2013 to March 2017 were retrospectively analyzed. Five cases had choledocholithiasis including 1 case combined with biliary infection and other 2 cases with biliary obstruction of pancreatic head cancer. Anti-hemophilic globulin (AHG) was given perioperatively combined with accurate monitor of coagulation factor level as well as vital signs. Results There were 2 cases with endoscopic papillary balloon dilation and bile duct stone removal, 2 cases with endosco-pic sphincterotomy and bile duct stone removal, 2 cases with endoscopic metal biliary drainage and 1 case with endosco-pic nasobiliary drainage (ENBD) only. Totally, ENBD was performed for 4 cases of bile duct stone. All patients discharged with hospital stay (9.6± 3.2) (7-16) d. The preoperative FⅧ:C was (18.7%± 11.1%) (1.2%-28.2%) and AHG dose was (2 743± 964) (2 000~4 000) U/d. There was no significant difference in the postoperative hemoglobin (100± 30)(51~131) g/L and hematocrit (0.293± 0.084) (0.160-0.396) when compared with those preoperatively (105± 35) (50-150) g/L and (0.307± 0.097) (0.161-0.423). The postoperative serum amylase was (105± 80)(28-237) U/L. No any ERCP perioperative complications were found including bleeding, gastronintestinal perforation and acute pancreatitis. Conclusions ERCP could be effective and safe for patients with hemophilia A after infusion of AHG and careful monitoring and perioperative treatment.