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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 investigate the clinical features and diagnosis and treatment of hemophilia in neonates. Methods Neonatal children with hemophilia admitted to our neonatal intensive care unit from April 2010 to March 2012 were retrospectively analyzed for their clinical features and diagnosis and treatment process. Relevant literatures at home and abroad were reviewed. Results All the 7 neonates with hemophilia admitted during the study were male, with bleeding. The average age of the first bleeding was 3.7 days, and the other 2 had a clear family history of hemophilia. 3 cases of shock at admission, bleeding sites, including head hematoma, intracranial, subcutaneous, muscle, kidney bleeding. 7 cases of children with activated partial thromboplastin time were prolonged, prothrombin time, platelet count was normal, 5 cases of factor Ⅷ procoagulant activity to reduce the diagnosis of hemophilia A, 2 cases of factor Ⅸ procoagulant activity to reduce the diagnosis of hemophilia B . After admission to symptomatic treatment, diagnosed, hemophilia A intravenous infusion of factor VIII, hemophilia B to prothrombin complex. 6 cases improved, 1 case of parents give up. Conclusions In the neonatal period, hemophilia is more and more emergent. For persons with bleeding, hemophilia screening and confirmatory tests are conducted as soon as possible to improve the diagnosis rate of the disease in the neonatal period and improve the prognosis.