48例儿童血友病A低剂量预防治疗的效果评价

Low dose Prophylactic Treatment Effect Evaluation of 48 Cases on Children Hemophilia A

目的讨论分析低剂量预防治疗在儿童血友病A中的临床应用效果。方法选取新疆地区48例血友病A患儿,均在2013年6月到2015年2月在我院进行检查和实验室确诊为血友病A,对所有患儿均进行低剂量预防治疗,比较治疗前后患儿的临床表现、关节功能以及生活质量改善情况。结果治疗后患儿的出血次数(2.03±0.84)次、急诊次数(1.65±0.64)次以及严重出血事件发生率(4.16%)与治疗前患儿的出血次数(25.00)次、急诊次数(4.63±1.74)次以及严重出血事件发生率(25.00%)相比显著减少;治疗后患儿的自我照顾评分(9.45±3.16)分、转移功能评分(6.34±2.14)分、移动功能评分(5.98±1.85)分显著高于治疗前患儿的自我照顾评分(4.23±1.43)分、转移功能评分(3.21±1.07)分、移动功能评分(3.65±1.21)分;治疗前、后患儿生活质量评分存在显著性差异,具统计学意义(P<0.05)。结论低剂量预防治疗在儿童血友病A的治疗中具有显著的优势,降低了出血频率,改善了患儿的生活质量,值得临床推广使用。

Objective To discuss the low dose prevention and treatment effect in children with hemophilia A. Methods 48 cases of hemophilia A patients,from June 2013 to February 2015 in our hospital for examination and laboratory diagnosis for hemophilia A,were low dose prophylactic treatment of all patients before and after the treatment,clinical manifestation,joint function and life quality improving condition. Results The frequency of hemorrhage in children after treatment（ 2. 03 ± 0. 84） times,the emergency number（ 1. 65± 0. 64） times and major bleeding event rate（ 4. 16%） and the number of children with bleeding before treatment（ 25）,the emergency number（ 4. 63 ± 1. 74） and serious bleeding events（ 25%） were significantly decreased in comparison after the treatment of children; self care score（ 9. 45 ± 3. 16） points,activity score（ 6. 34± 2. 14）,mobile score（ 5. 98 ± 1. 85） were significantly higher than before treatment in children with self care score（ 4. 23 ± 1. 43） points,activity score（ 3. 21 ± 1. 07）,mobile score（ 3. 65 ± 1. 21）; before and after the treatment,the quality of life in children with significant difference score,with statistical significance（ P〈0.05）. Conclusion Low dose prophylactic treatment has obvious advantages in the treatment of children with hemophilia A,reduce the frequency of bleeding,improve the quality of life of the patients,is worthy of clinical use.