A型血友病合并左肾积水伴出血围手术期凝血因子Ⅷ替代治疗1例报告  

A case report of perioperative coagulation factorⅧreplacement therapy for hemophilia A with left hydronephrosis and hemorrhage

作　　者：陈爽 强紫阳 黄禹栋 任承德[1] 陈国俊[1] Chen Shuang;Qiang Ziyang;Huang Yudong;Ren Chengde;Chen Guojun(Department of Urology,Affiliated Hospital of Qinghai University,Xining 810000,China)

机构地区：[1]青海大学附属医院泌尿外科,西宁810000

出　　处：《中华泌尿外科杂志》2022年第8期620-621,共2页Chinese Journal of Urology

摘　　要：血友病是一种X染色体连锁的隐性遗传性出血性疾病。青海大学附属医院2020年7月28日收治1例A型血友病合并左肾重度积水、左肾急性出血患者。患者因左侧腰背部疼痛6 d入院,既往有A型血友病家族遗传史。术前活化部分凝血活酶时间95.0 s(参考值22.7~31.8 s),Ⅷ因子活性(FⅧ∶C)3.2%(参考值70%~150%)。B超检查:左肾重度积水。肾图:左肾无灌注、无功能、无排泄;右肾灌注可,功能代偿性增高。全腹CT平扫检查:左肾内见不规则斑片状稍高密度影,考虑出血。诊断为左肾重度积水并出血,A型血友病。经多学科会诊,于围手术期应用人凝血因子Ⅷ治疗。全麻下行后腹腔镜左肾切除术,术中、术后均无大出血。术后随访20个月,无血尿和腰痛症状,肾功能和凝血功能正常。Hemophilia is a recessive inherited disorder linked to the X chromosome.On July 28,2020,a case of hemophilia A with severe left kidney hydronephroses and acute left kidney hemorrhage was admitted to the Affiliated Hospital of Qinghai University.The patient had a family history of hemophilia A and activated partial thromboplastin before surgery.The APTT was 95.0s(reference value 22.7-31.8s),factorⅧactivity(FⅧ∶C)was 3.2%(reference value 70%-150%),and B-ultrasonic examination revealed severe hydronephroses in the left kidney,and the nephrogram indicated that the left kidney was absent of perfusion.On the 9th day of admission,the pain in the left lower back was aggravated than before.The CT scan of the whole abdomen revealed irregular patches with slightly higher density in the left kidney.Considering bleeding,through multidisciplinary consultation and perioperative infusion of human coagulation factorⅧ,the patient was underwent retroperitoneal laparoscopic left nephrectomy.There was no bleeding during and after operation.After 20 months of follow-up,the renal function and coagulation function were normal,and there were no symptoms of hematuria and low back pain.

关 键 词：血友病A 凝血因子 腹腔镜 肾切除术 

分 类 号：R699.2[医药卫生—泌尿科学] R554.1[医药卫生—外科学]