小儿血友病93例临床分析

目的分析93例血友病患儿的诊断及治疗经验。方法对患儿的发病年龄、病例特点、实验室检查进行分析。结果血友病甲78例(84%),血友病乙15例(16%),其中重型45例(48%),中型33例(35%),轻型14例(15%),亚临床型1例(1%)。各种类型的出血为小儿血友病的主要表现,重型多见于婴幼儿,其中6例发生颅内出血(6%);关节病变及严重关节畸形多见于年长儿,共6例(6%)。初次出现出血症状的中位年龄为1.5(0～12.0)岁,但初次诊断的中位年龄为5.4(0.4～12.0)岁。延迟诊断的原因多种多样。结论延迟诊断、延误因子替代治疗是导致脏器出血和关节畸形的主要原因。

Clinical analysis in 93 childhood hemophilia

Objective To summarize the diagnosis and treatment of children with hemophilia.Methods The onset age,clinical features and laboratory examination of 93 cases with hemophilia were analyzed.Results Seventy-eight cases were hemophilia A(84%),and 15 cases were hemophilia B(16%).Among them,45 cases were severe hemophilia(48%),33 were medium hemophilia(35%),14 were light hemophilia(15%) and 1 was subclinical hemophilia(1%).Bleeding was the main clinical manifestation of children with hemophilia.Severe hemophilia was more common in infants and younger children,of which,six cases occurred intracranial hemorrhage(6%).Arthropathy and severe joint deformity were more common in older children(six cases,6%).The median age of the first bleeding was 1.5 years old(aged 0~12 years),while the median age of the initial diagnosis was 5.4 years old(aged 0.4~12 years).The reasons for delayed diagnosis were diverse.Conclusions Delayed diagnosis and delays in replacement therapy were the main causes leading to organ bleeding and joint deformities.