Abstract
Forty-one patients with phenylketonuria (PKU) whose dietary therapy was discontinued at an average age of 6 years were placed on a phenylalanine restricted diet after 1 to 16 years of unrestricted diet. Resumption of diet was prompted by decline in academic achievement, behavior problems, loss of I.Q., abnormal changes in the EEG, tremors, eczema and body odor. Twenty-three patients failed to comply with diet restrictions even after as much as one year of counseling and their blood phenylalanine levels remained greater than 25mg/dl. Eighteen patients maintained some diet restrictions but they still reported excessive intake of phenylalanine, an average of 1370mg daily in the first two years and 700mg daily after 4 years. Average blood phenylalanine levels in this group were 17.8mg/dl after 2 years and 14.8mg/dl after 4 years indicating poor compliance. The limited degree of success with diet seemed to be related to the length of time off diet. The 23 patients who failed were on an unrestricted diet an average of 9 years compared to 4 years for the remaining 18 patients. This study indicates the difficulty in returning to a phenylalanine restricted diet, particularly in adolescents and young adults. Since the female PKU patients must return to diet prior to conception this problem may have serious implications for this population who is at risk for unplanned pregnancies.
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Michals, K., Matalon, R. & Dominik, M. DIFFICULTIES RETURNING TO DIET THERAPY IN PATIENTS WITH PHENYLKETONURIA. Pediatr Res 18 (Suppl 4), 98 (1984). https://doi.org/10.1203/00006450-198404001-00032
Issue Date:
DOI: https://doi.org/10.1203/00006450-198404001-00032