柳州地区血友病患者临床表现及生活现状分析

ClinicalCharacteristics and Living Conditions of Patients With Hemophilia in The District of Liuzhou

目的调查分析柳州地区登记在册的41例血友病患者的临床表现特点及生活状况,为制定本地区公共卫生政策提供依据。方法查阅登记信息、发放调查问卷、电话访问,对收集到的资料进行分析。结果41例血友病患者全部为男性,成年患者占56.1%(23/41),未成年患者占43.9%(18/41)。汉族占48.8%(20/41),壮族占24.4%(10/41),其他少数民族占26.8%(11/41)。血友病A(HA)占82.9%(34/41),其中轻型11.8%(4/34),中间型23.5%(8/34),重型64.7%(22/34);血友病B(HB)占17.1%(7/41),其中中型42.3%(3/7),重型57.7%(4/7)。成年组和未成年组的中位确诊年龄及诊断延迟时间分别为8岁(0.25~50)VS 0.71岁(0.42~7),7年(0~49)VS 0.08年(0~6)(P<0.05)。成年患者均接受按需治疗,未成年患者33.3%(6/18)接受小剂量预防治疗。25例患者出现关节畸形,其中成年患者21例,未成年患者4例,成年关节畸形患者中42.9%(9/21)为低保户。40%(22/55)的一级和二级医疗机构医务人员知晓血友病,从未接受过血友病相关知识专业培训。结论柳州地区血友病患者存在诊断延迟、儿童患者预防治疗不足、残疾血友病患者生活状况差、基层医疗机构防治能力弱的特点,急需建立本地区血友病分级诊疗体系,重点加强和规范血友病儿童预防治疗,从而降低患者致残率,改善生活质量。

Objective To analysis the clinical characteristics and family living conditions of patients with hemophilia in the district of Liuzhou and develop some public health policies.Methods A retrospective study was conducted with 41 hemophilia patients by consulting medical records,issuing questionaires and telephone follow-up.Results All the 41 patients were males including 56.1%(23/41)adults and 43.9%(18/41)children.The han nationality accounted for 48.8%(20/41),the zhuang nationality 24.4%(10/41),and other ethnic minorities 26.8%(11/41).During these 41 patients,34 of whom(82.9%)had haemophilia A(HA,mild 4,moderate 8,severe22),and 7(17.1%)of haemophilia B(HB,moderate 3,severe 4).21 cases had hemophilia family history(51.2%).The median age at initial bleeding symptoms was 1.0 years,the time of initial diagnosis was 1.5 years and delay time of diagnosis was 0.66 years.In adults and children,the median time of initial diagnosis and delay time of diagnosis were 8 years versus(VS)0.71 years and 7 years VS 0.08 years,respectively.All adult patients received on-demand treatment,and 33.3%of children patients received low-dose prophylaxis.25 cases had joint deformity,including 21 adults and 4 children.Among the adult patients with joint deformity,42.9%are low-income families.In HA and HB,inhibitor occurrence were 8.8%(3/34)and 14.3%(1/7)respectively.HBV infection were found in 3 patients(7.3%),HCV in 1 patients(2.4%).40%(22/55)medical staffs in primary hospital are aware of hemophilia and have never received professional training in hemophilia.Conclusion Patients with hemophilia in liuzhou have delayed diagnosis,inadequate prophylaxis for children,low quality of life for adults,weak prevention and control capacity of primary hospital.It is urgent to establish a hierarchical diagnosis and a treatment system for hemophilia in the region,with emphasis on strengthening and standardizing the prophylaxis of hemophilia children,so as to reduce the disability rate of patients and improve their living quality.