PHENYLKETONURIA - CURRENT DIETARY-TREATMENT PRACTICES IN THE UNITED-STATES AND CANADA

Objective: A survey of treatment centers for phenylketonuria (PKU) in the United Stales and Canada was undertaken regarding current practice s of dietary treatment of PKU. Methods: A total of 111 centers, who fo llow more than 6,950 patients with PKU responded to the survey. Result s: The majority of the centers, 87%, favor life-long dietary control o f phenylalanine intake. The survey found lack of uniformity regarding acceptable range of blood phenylalanine levels. The frequency of clini c visits varied and became less frequent as patients got older. Althou gh 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 u nclear who manages these patients beyond that age. The survey also sho wed a high number of families with children who were reported for medi cal neglect (3.0% compared to <0.06% in the general population). Becau se of dietary noncompliance, 1% of the children were removed from the home. Discussion: The survey points to the common treatment goal of di et for life for patients with PKU and underscores the need for uniform guidelines for achieving this goal.