Purnell H
Early Childhood Centre, Westmead Hospital and Community Health Services, 39 Bridge St Epping, NSW 2121.
Breastfeed Rev. 2001 Jul;9(2):19-21.
Phenylketonuria is a genetic disease affecting 1:10,000 to 14,000 live births. In NSW there is an average of nine cases diagnosed each year (Dietitians Working Party 1996). This paper discusses the management of phenylketonuria, and in particular the value of breastfeeding, complemented with a low phenylalanine infant formula, in facilitating easier maintenance of satisfactory phenylalanine blood levels. The 'diet for life' approach to managing phenylketonuria is to avoid long-term neurological deficits and, in particular, the risk that maternal PKU, which is not under strict dietary control, will have adverse effects on infants born of mothers with the disease. There have been 31 successful pregnancies to 1997 managed by the Nutrition and Dietetics Department of The Children's Hospital at Westmead, Sydney. The Maternal PKU diet is presented with the case of a client with phenylketonuria who has achieved two normal pregnancies and breastfed her second child for six months.
苯丙酮尿症是一种影响每10000至14000例活产儿中的1例的遗传性疾病。在新南威尔士州，每年平均诊断出9例病例（营养师工作小组，1996年）。本文讨论了苯丙酮尿症的管理，特别是母乳喂养结合低苯丙氨酸婴儿配方奶粉在更轻松地维持令人满意的苯丙氨酸血水平方面的价值。管理苯丙酮尿症的“终身饮食”方法是避免长期神经功能缺损，特别是避免未严格饮食控制的母体苯丙酮尿症对患病母亲所生婴儿产生不利影响。到1997年，悉尼韦斯特米德儿童医院营养与饮食科已成功管理了31例妊娠。本文通过一位患有苯丙酮尿症的客户的案例介绍了母体苯丙酮尿症饮食，该客户已成功孕育了两胎，并且对第二个孩子进行了六个月的母乳喂养。
