Fisch R O, Matalon R, Weisberg S, Michals K
Department of Pediatrics, University of Minnesota, Minneapolis-St. Paul, USA.
J Am Coll Nutr. 1997 Apr;16(2):147-51. doi: 10.1080/07315724.1997.10718665.
A survey of treatment centers for phenylketonuria (PKU) in the United States and Canada was undertaken regarding current practices of dietary treatment of PKU.
A total of 111 centers, who follow more than 6,950 patients with PKU responded to the survey.
The majority of the centers, 87%, favor life-long dietary control of phenylalanine intake. The survey found lack of uniformity regarding acceptable range of blood phenylalanine levels. The frequency of clinic visits varied and became less frequent as patients got older. Although most of the clinics recommend diet for life, only one-third of the clinics follow patients beyond the age of 18 years, therefore, it is unclear who manages these patients beyond that age. The survey also showed a high number of families with children who were reported for medical neglect (3.0% compared to < 0.06% in the general population). Because of dietary noncompliance, 1% of the children were removed from the home.
The survey points to the common treatment goal of diet for life for patients with PKU and underscores the need for uniform guidelines for achieving this goal.
对美国和加拿大的苯丙酮尿症（PKU）治疗中心进行了一项关于PKU饮食治疗当前实践的调查。
共有111个治疗中心对该调查做出回应，这些中心随访了6950多名PKU患者。
大多数中心（87%）赞成对苯丙氨酸摄入量进行终身饮食控制。调查发现，血液苯丙氨酸水平的可接受范围缺乏一致性。门诊就诊频率各不相同，且随着患者年龄增长而降低。虽然大多数诊所建议终身饮食控制，但只有三分之一的诊所对18岁以上的患者进行随访，因此，不清楚18岁以上的患者由谁管理。调查还显示，有大量有孩子的家庭被举报存在医疗疏忽（3.0%，而普通人群中这一比例小于0.06%）。由于饮食不依从，1%的儿童被带离家庭。
该调查指出了PKU患者终身饮食治疗的共同目标，并强调了实现这一目标需要统一指南。
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