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四川省儿童血友病临床资料及生活质量分析 被引量:2

Evaluation of Clinical Data and Quality of Life of Hemophilic Children in Sichuan Province
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摘要 目的 收集和分析儿童血友病的临床资料以及生活质量情况,了解目前四川省儿童血友病的现状,并作相关因素分析。方法 收集 2008 年 1 月 1 日- 2015 年 5 月 30 日成都市妇女儿童中心医院注册登记的儿童血友病患者资料,并进行统计分析。结果 共收集到 92 例患者资料,均来自四川省内,年龄 3.6 ~ 18.0 岁,中位年龄9.6岁;血友病A 87例(94.6%),血友病B 5例(5.4%);轻型4例(4.3%),中间型67例(72.8%),重型21例(22.9%);18 例(19.6%)有家族史。首次出血时月龄 0 ~ 48 个月,中位月龄 11 个月;首次出血为轻度出血 23 例(25.0%),中度出血 31 例(33.7%),重度出血 38 例(41.3%);首次出血部位多为皮肤黏膜,其次为关节肌肉。首次关节出血时月龄2 ~ 107个月,中位月龄18个月;46例在病程进展中有靶关节形成(59.0%)。4例(4.3%)患儿曾发生过颅内出血,其中 3 例临床治愈,1 例有后遗症。获得诊断时月龄为 0 ~ 120 个月,中位月龄 12 个月;近期诊断 62 例(67.4%),中期诊断 9 例(9.8%),远期诊断 21 例(22.8%)。40 例(43.5%)一直足剂量因子替代治疗,其余患儿未得到足量和充分的治疗。56 例患儿接受过预防治疗(60.9%),首次开始预防治疗的月龄为 1 ~ 199 个月,中位月龄 36 个月,27例(48.2%)患儿在治疗过程中终止预防治疗。共收集到29份疾病家庭负担量表,评分为4 ~ 43分,平均(22.7±11.6)分,其中 11 例(37.9%)日常活动无法自理。23 例患儿评估了参与社会活动能力,其中 2 例处于失学状态,6 例在校园不参与活动。相关性分析显示获得诊断时机与有无家族史及患儿所处地区无关(P=0.795、0.495),而与首次出血程度呈正相关(r=0.392,P=0.035)。疾病家庭负担与患儿靶关节数呈正相关(r=0.370,P=0.048),与所处地区及疾病程度、出血频率、医疗保险报销比例、日常活动能力均无相关性(P > 0.05)。结论 四川省儿童血友病诊断治疗仍相对滞后,关节病变发生率高,患儿生活质量差,疾病家庭负担重。 Objective To collect and analyze the clinical data and quality of life of hemophilic children, understand the present condition of these patients in Sichuan Province, and analyze related influencing factors. Methods We retrospectively analyzed the clinical data of hemophilic children treated in our hospital from January 1, 2008 to May 30, 2015. Results There were 92 child patients from Sichuan Province with a median age of 9.6 years old (ranging from 3.6 to 18.0). There were 87 cases (94.6%) of hemophilia A and 5 (5.4%) of hemophilia B; the number of light cases was 4 (4.3%), of moderate cases was 67 (72.8%), and of severe cases was 21 (22.9%); eighteen (19.6) of the patients had family history. First bleeding episode occurred at a median age of 11 months (0-48 months). Mild bleeding occurred in 23 cases (25.0%), moderate bleeding in 31 cases (33.7%), and severe bleeding in 38 cases (41.3%). First bleeding site was mainly the mucous membrane of the skin, followed by the joint muscles. Fist joint bleeding occurred at a median age of 18 months (2-107 months). Forty-six patients (59.0%) had severe joint damage during the course of the disease. Intracranial hemorrhage occurred in 4 patients (4.3%), among whom 3 were cured and discharged from the hospital, and 1 had neurological sequelae. Median diagnostic age was 12 months (0-120 months). Sixty-two (67.4%) were diagnosed in a shortperiod of time, 9 (9.8%) in a mediate period of time, and 21 (22.8%) in a long period of time. Forty (43.5%) of the patients had been given sufficient coagulation factors, while all the others had not received sufficient replacement therapy. Fifty-six (60.9%) children had received prophylactic treatment. First prophylaxis was administrated at a median age of 36 months (1-199 months), but 27 (48.2%) discontinued. The median score of the 29 retreated Disease Burden Scale was 22.7±11.6 (4-43), and among them, 11 (37.9%) could not care for themselves. Twenty-three participated in the assessment of social activity ability, among whom, 2 did not attend school, and 6 could not take part in the assessment because of school learning. Correlation analysis showed that there was no significant relationship between diagnostic timing and family history (P=0.795) or between diagnostic timing and areas they came from (P=0.495). However, significant association was found between diagnostic timing and the severity of first bleeding (r=0.392, P=0.035). Disease burden of family was significantly correlated with the number of target joints (r=0.370, P=0.048), and was not dosely related with area, severity of bleeding, frequency of hemorrhage, medical insurance, or physical and social activities. Conclusions The general diagnosis and treatment condition of child hemophilia in Sichuan is relatively under-developed with a high prevalence of joint damage, poor quality of life, and high disease burden to the family. Improvement in the care of hemophilia children is urgently needed.
出处 《华西医学》 CAS 2015年第9期1662-1665,共4页 West China Medical Journal
关键词 血友病 儿童 诊断 治疗 疾病家庭负担 Hemophilia Children Diagnosis Treatment Disease burden of family
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参考文献15

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