新生儿血友病七例  

Clinical features of seven neonates with hemophilia

作　　者：刘靖媛[1] 刘红[1] 吴润晖[2] 靳绯[1] 李耿[1] 翁景文[1] 吴海兰[1] 

机构地区：[1]首都医科大学附属北京儿童医院新生儿重症监护病房,100045 [2]首都医科大学附属北京儿童医院血液中心,100045

出　　处：《中国新生儿科杂志》2013年第3期159-162,共4页Chinese Journal of Neonatology

摘　　要：目的探讨新生儿血友病的临床特点及诊治经验。方法选择本院新生儿重症监护病房2010年4月至2012年3月收治的新生儿血友病患儿,回顾性分析其临床特点及诊疗过程,并复习国内外相关文献。结果研究期间收治的7例新生儿血友病患儿均为男性,有出血表现,首次出血日龄平均3.7天,2例有明确血友病家族史。3例入院时有休克,出血部位包括头颅血肿、颅内、皮下、肌肉、肾脏出血等。7例患儿活化部分凝血活酶时间均延长,凝血酶原时间、血小板计数等正常,5例因子Ⅷ促凝活性降低诊断血友病A,2例因子Ⅸ促凝活性降低诊断血友病B。入院后予对症治疗,确诊后,血友病A予以静脉输注Ⅷ因子,血友病B予以凝血酶原复合物。6例好转,1例家长放弃。结论新生儿期发病的血友病多起病急,对有出血表现者,尽早进行血友病筛选及确诊试验,以提高该病在新生儿期的诊断率、改善预后。Objective To discuss clinical features, diagnosis and management of hemophilia in neonates. Methods All seven neonates participated in this study were identified from NICU admissions of April 2010 - March 2012. Clinical features, diagnostic work ups and treatments of these patients were analyzed. Associated publications were reviewed. Results All patients were male presented with hemorrhage. The average chronological age at initial onset of bleeding was 3.7 days old. Two patients had family history of hemophilia, three patients presented with hemorrhagic shock at the time of admission. Organ involvements include scalp hematoma, intracranial hemorrhage, sub cutaneous hematoma, intra-muscular and renal hemorrhage. All patients had prolonged activated partial thromboplastin time （APTT）, however, prothrombin time （PT） and platelets count remained normal. Five neonates with decreased activity of factor VIII were diagnosed with hemophilia A and were treated with factor VIH infusion. Prothrombin complex were given to two hemophilia B neonates with decreased activity of factor IX. Six patients had improved symptoms with treatment; one patient was unable to complete treatment due to parental refusal. Conclusions Patients with hemophilia most often present with acute onset of bleeding during neonatal period. Early screening and confirmative laboratory tests are important in order to facilitate early diagnosis and to improve clinical outcomes of these infants.

关 键 词：血友病 出血性疾病 因子Ⅷ 因子IX 婴儿 新生 

分 类 号：R722.1[医药卫生—儿科]