目的 调查中国A型血友病4～18岁患儿家庭治疗和护理状况。方法 2006年、2010年两次对血友病家庭治疗和护理状况进行调查。结果 A型血友病患儿诊断年龄和治疗年龄晚于首次出血年龄;凝血因子用量不足;血友病性骨关节病患病率高;开展了家庭治疗的血友病患儿从55例上升到119例,血友病患儿及家庭对发病原因、遗传规律、出血严重程度的评估和正确服用止痛药物认知率分别从80.8％、78.5％、37.4％和48.4％上升到94.7％、94.1％、52.7％和85.5％;在开展家庭康复训练、保持治疗记录完整两方面没有明显改善。结论 A型血友病患儿诊断治疗延迟,凝血因子用量不足,骨关节病发病率高,家庭护理水平仍然有待于进一步提高。%Objective To investigate the current status of home care for children with hemophilia A. Methods Twice investigation was conducted with a self-designed questionnaire about the status of home care in children with hemophilia A in 2006 and 2010. Results The diagnosis confirming and initial treatment were implemented after initial hemorrhage in children with hemophilia A. Moreover, the dosage of coagulation factor was insufficient and the incidence of joint deformity was high. The number of hemophilia A children received family therapy increased from 55 in 2006 to 119 in 2010. The mastery rates of knowledge on the etiology,inheritance rule,assesment of bleeding and proper use of analgesics were improved from 80.8 percent,78.5 percent,37.4 percent,and 48.4 percent in 2006 to 94.7 percent,94.1 percent,52.7 percent,and 85.5 percent in 2010,respectively. The status of home rehabilitation training and the completeness of medical records was not unproved. Conclusion Delayed diagnosis and treatment,insufficient dosage of coagulation factor,and high incidence of joint deformity are prevalent in the children with hemophilia A. The level of family therapy and home care is still need to be further improved.
展开▼
