儿童血友病429例临床回顾分析

目的探讨血友病患儿确诊时间、症状及其关节畸形等情况。方法天津血液学研究所血液病医院于1986年7月至2004年8月，对就诊的429例血友病患儿临床症状出现时间、诊断时间及关节畸形出现的时间进行分析。结果429例血友病患儿，均为男性，血友病A共390例，占909%，其中亚临床型5例(13%),轻型43例(110%)，中型181例(464%)，重型161例(413%)；血友病B共39例，占91%，其中亚临床型2例(51%),轻型6例(154%)，中型26例(667%)，重型5例(128%)。患儿初次出现出血症状的中位年龄为16岁(0~12岁)；但初次诊断年龄03~120岁，中位年龄为52岁(03~120岁)，两者之间差异具有显著性意义(P<001)。结论目前血友病患儿的诊断存在延迟，替代治疗疗程和因子剂量的不足是关节畸形高发生率的原因。

Clinical retrospective analysis of 429 childhood patients with haemophilia.

AbstractObjectiveTo analyze the data of diagnosis,symptom and joint deformity of childhood patients with haemophilia.MethodsA total of 429 childhood patients with haemophilia were referred to our hospital and diagnosed in our hospital from July 1986 to August 2004,and were retrospectively analyzed.ResultsThe 429 childhood patients(male,median age 5) were analyzed,390 of whom (909%) had haemophilia A (subclinical 5,mild 43,moderate 181,severe 161),and 39(91%) had haemophilia B(subclinical 2,mild 6,moderate 26,severe 5).The mean age at initial bleeding symptoms was 16 years (0~12years),and that of initial diagnosis was 52 years (03~12years).It was significantly different between them (P＜001).ConclusionThe delay of diagnosis,insufficiency of period of replacement treatment and the dose of factor concentration lead to high prevalence of joint deformity in childhood patients in China.