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June 1967

Ten-Year Follow-Up of Treatment of Two Phenylketonuric Brothers

Author Affiliations

Kansas City, Mo
From the Division of Neurology and the Mental Retardation Diagnostic Laboratory, University of Missouri School of Medicine, and Kansas City General Hospital and Medical Center, Kansas City.

Arch Neurol. 1967;16(6):658-663. doi:10.1001/archneur.1967.00470240096014

Relatively few long-term studies of the treatment of phenylketonuria have been published in spite of the fact that the low phenylalanine diet was introduced in 1954.1 Much controversy still exists in regard to the time when the special diet, when it is prescribed, may be discontinued. This controversy is undoubtedly related to the fact that the exact manner in which the biochemical abnormality affects mental development is still unclear.

The low phenylalanine dietary regimen poses great problems for both the family and the patient, particularly when near normal or normal intelligence is attained, since the child is constantly exposed to phenylalanine-containing foods both at home where this situation can be controlled and outside the home where such control is nearly impossible. The question then arises about the necessity for weighing the presumed beneficial physiologic effect of the diet against the psychologic disturbances that its administration frequently creates.


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