肠内营养粉剂治疗苯丙酮尿症患儿的疗效和安全性分析

Efficacy and safety analysis of enteral nutrition powder in the treatment of phenylketonuria in children

目的分析肠内营养粉剂治疗苯丙酮尿症患儿的疗效性和安全性。方法选取本院2016年9月至2018年1月收治的116例苯丙酮尿症患儿作为研究对象,持续32周给予肠内营养粉剂,评估治疗前、治疗8周及32周后患儿的苯丙氨酸浓度、身高、体质量、头围、智力水平、各项血清营养学指标变化及不良反应发生情况。结果治疗8周及32周后患儿的苯丙氨酸浓度明显低于治疗前,差异有统计学意义(P<0.05)。治疗8周及32周后患儿的身高、体质量、头围与治疗前相比均有明显的增长,差异具有统计学意义(P<0.05)。治疗8周后患儿的智力水平明显优于治疗前,治疗32周后患儿的智力水明显优于治疗8周时,差异有统计学意义(P<0.05)。治疗后患儿的血清蛋白、白蛋白、尿素氮、总胆红素及前白蛋白水平较治疗前,差异无统计学意义。本次研究过程中,共有10例患儿,32例不良反应发生,其中腹泻7例,上呼吸道感染15例,肠胃炎1例,其他反应9例。所有出现不良反应的患儿,均经有效处理后恢复正常。结论肠内营养粉剂治疗苯丙酮尿症患儿,可以有效的控制患儿的苯丙氨酸水平,避免中枢神经受损,改善患儿的智力发育,具有较好疗效性和安全性,值得在临床大力推广。

Objective To analyze the efficacy and safety of enteral nutrition powder in the treatment of phenylketonuria in children. Methods 116 children with phenylketonuria admitted to our hospital from September 2016 to January 2018 were selected as the study subjects, Enteral nutrition powder was administered for 32 weeks. Phenylalanine concentration, height, weight, head circumference, intelligence level, serum nutritional indexes and adverse reactions were evaluated before treatment, 8 weeks after treatment and 32 weeks after treatment. Results The concentration of phenylalanine in 8 and 32 weeks after treatment was significantly lower than that before treatment (P<0.05). After 8 and 32 weeks of treatment, the height, weight and head circumference of the children increased significantly compared with those before treatment, and the difference was statistically significant (P<0.05). After 8 weeks of treatment, the children's intelligence level was significantly better than that before treatment. After 32 weeks of treatment, the children's intelligence level was significantly better than that after 8 weeks of treatment, with statistical significance (P< 0.05). There was no significant difference in serum protein, albumin, urea nitrogen, total bilirubin and prealbumin levels after treatment compared with those before treatment. In this study, there were 10 cases of children, 32 adverse reactions occurred, including 7 diarrhea, 15 upper respiratory tract infections, 1 gastroenteritis and 9 other reactions. All children with adverse reactions recovered to normal after effective treatment. Conclusion Enteral nutrition powder in the treatment of phenylketonuria in children can effectively control the level of phenylalanine in children, avoid central nervous system damage, improve the intellectual development of children, with good efficacy and safety, it is worth vigorously promoting in clinical practice.