论文部分内容阅读
目的通过评价对苯丙酮尿症(PKU)患儿不同方式免费医疗补助的干预效果,探索解决PKU患儿治疗和管理的最佳模式。方法募集45例PKU患儿接受免费医疗补助,其中27人只接受免费特殊奶粉补助,18人接受免费特殊奶粉和特殊米面补助。定期随访患儿的体格和智力发育情况,并且定期检测患儿血液苯丙氨酸(Phe)浓度。结果只接受免费特殊奶粉补助的患儿中体格发育正常的比例为75%,智力发育正常的比例为81.25%,患儿血Phe浓度控制在4mg/dl以内的次数占总检测次数的比例能达到80%的人数仅占18.52%。接受免费特殊奶粉和特殊米面补助患儿中体格发育正常的比例为88.89%,智力发育正常的比例为94.44%,患儿血Phe浓度控制在4mg/dl以内的次数占总检测次数的比例能达到80%的人数占44.44%)。结论同时给予免费特殊奶粉和特殊米面补助能有效提高PKU患儿规范治疗依从性,可提高患儿家长对该疾病的认知度,降低血Phe浓度在4mg/dl以内的次数比例,改善PKU患儿预后。
Objective To explore the best mode of treatment and management for children with PKU by evaluating the effect of different modes of free medical aid on children with phenylketonuria (PKU). Methods 45 cases of PKU children were recruited for free medical assistance, of which 27 received only free special milk powder subsidies and 18 received special free milk and special rice subsidies. The physical and mental development of the children were regularly followed up, and the blood Phe concentration of the children was measured regularly. Results Only children with special special milk powder subsidies had a normal physical development rate of 75% and a normal intelligence development rate of 81.25%. The percentage of children with Phe concentration within 4mg / dl could reach the total number of tests 80% of the population accounted for only 18.52%. The proportion of normal physical development in children receiving free special milk powder and special rice subsidy was 88.89%, the normal rate of mental development was 94.44%, and the proportion of children whose blood Phe concentration was controlled within 4mg / dl could reach the total number of tests 80% of the population accounted for 44.44%). Conclusions At the same time, giving free special milk powder and special rice noodles subsidy can effectively improve the standard treatment compliance of children with PKU, can improve the awareness of parents in children with this disease, reduce the proportion of blood Phe concentration in the number of 4mg / dl, improve PKU Prognosis.