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目的:提高临床医师对新生儿期发病血友病患儿诊断及治疗的早期认识。方法:选取我院新生儿科2014年诊治的2例血友病患儿,结合临床资料及相关文献,比较其临床表现、诊治经过及预后,讨论血友病新生儿期发病的诊疗经验及教训。结果:2例患儿均为男性,均为血友病A(重型);2例均为剖宫产出生,但病例2为顺产转剖宫产,2例均无器械分娩等辅助分娩史。2例患儿入院时间分别为生后4天和1天,确诊年龄分别为出生后6天及24天。2例患儿早期均有高胆红素血症,均无颅内出血。其中病例1还表现为皮肤瘀斑及医源性穿刺部位血肿,出生后第6天确诊血友病,给予凝血因子或血浆治疗后随访短期内预后良好;病例2出生后第1天因黄疸、头颅血肿住院,经退黄、换血等治疗效果不佳,继发胆汁淤积性肝炎,于出生后24天转入我院,经检查确诊血友病,给予相应治疗,家长放弃治疗,出院1周后继发严重颅内出血合并脑疝死亡。结论:血友病目前尚无特异的根治方法,强调对新生儿期发病的血友病患儿早期认识及早期预防性治疗对改善预后,降低致残率及致死率有较大意义。
Objective: To improve clinicians early diagnosis and treatment of children with hemophilia in the neonatal period. Methods: Two children with hemophilia who were diagnosed and treated in neonatal department of our hospital in 2014 were enrolled in this study. The clinical manifestations, diagnosis, treatment and prognosis of hemophilia were compared with clinical data and related literatures to discuss the experience and lessons in the diagnosis and treatment of hemophilia in neonatal period. Results: Two children were all male, all of whom were hemophilia A (heavy type). Two patients were born with cesarean section. However, case 2 was delivered cesarean section by cesarean section and no delivery was performed in two cases. The admission time of 2 children was 4 days and 1 day respectively after birth, and the diagnosed age was 6 days and 24 days after birth respectively. 2 cases of children with early hyperbilirubinemia, no intracranial hemorrhage. Case 1 also showed skin ecchymosis and iatrogenic puncture site hematoma, hemophilia 6 days after birth confirmed, given clotting factor or plasma follow-up after a short period of good prognosis; Case 2 1 day after birth due to jaundice, Head of hematoma hospitalized, the back yellow, exchange of blood and other treatment ineffective secondary cholestatic hepatitis, 24 days after birth into our hospital, the diagnosis of hemophilia, given the appropriate treatment, parents to give up treatment, discharged a week Subsequent severe intracranial hemorrhage with cerebral hernia death. CONCLUSION: There is no specific radical cure for hemophilia at present. It emphasizes that early recognition and early prophylaxis of children with hemophilia in neonatal period are of great significance to improve prognosis, reduce morbidity and mortality.