苯丙酮尿症女性孕期的饮食治疗  

Dietary therapy for women with phenylketonuria during pregnancy

作　　者：赵德华[1] 麻宏伟[2] 张立琴 宋肪[4] 强荣 贾晨路[1] 王丽德[6] 

机构地区：[1]郑州大学第三附属医院,河南郑州450057 [2]中国医大盛京医院 [3]青岛妇女儿童医院 [4]首都儿科研究所 [5]陕西省妇幼保健院 [6]北京大学医学部

出　　处：《中国妇幼保健》2013年第5期763-766,共4页Maternal and Child Health Care of China

摘　　要：目的:探讨饮食治疗对苯丙酮尿症(PKU)育龄妇女获得良好的妊娠结局并防止母源性苯丙酮尿症(MPKU)发生的效果。方法:对3例PKU育龄妇女均采用课题组特定食谱进行治疗并定期监测其血苯丙氨酸(Phe)浓度,使之控制在5～7 mg/dl,观察并随访其妊娠结局及对妊娠的影响。结果:3例PKU育龄妇女既往均有过自然流产或胎儿异常的病史,采用课题组特定食谱严格控制血苯丙氨酸浓度后,其中2例已成功产下正常婴儿,另1例经过6个半月饮食治疗现孕7周。结论:初步认为采用课题组制定的食谱,控制并定期监测血苯丙氨酸浓度,可使苯丙酮尿症育龄妇女获得良好的妊娠结局。Objective： To explore the effect of dietary therapy on pregnancy outcome of women with phenylketonuria （PKU） of childbearing age and prevention of maternal PKU. Methods： Three women with PKU of childbearing age were treated with special recipes designed by research group, and the blood concentration of phenylketonuria （Phe） was examined regularly and controlled within 5 - 7 mg/dL, the pregnancy outcome and the effect of dietary therapy on pregnancy were observed and followed up. Results： Three women with PKU of childbearing age had medical histories of spontaneous abortion or fetal abnormalities. After dietary therapy, two pregnant women had given birth to healthy babies respectively, and the other pregnant woman had conceived for 7 weeks after controlling diet for 6 months. Con- elusion： It is elementarily considered that special recipes designed by research group, controlling and regularly monitoring blood concentra- tion of Phe can obtain good pregnancy outcome among women with PKU of childbearing age.

关 键 词：母源性苯丙酮尿症 苯丙氨酸 特定食谱 预防 

分 类 号：R173[医药卫生—妇幼卫生保健]