PEDIATRICS Vol. 46 No. 6 December 1970, pp. 881-890
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RESULTS OF TREATMENT AND TERMINATION OF THE DIET IN PHENYLKETONURIA (PKU)

Ellen Song Kang M.D.1, Natalie D. Sollee Ph.D.1, and Park S. Gerald M.D.1

1 Division of Clinical Genetics, Department of Medicine, the Department of Psychiatry, Children's Hospital Medical Center, and the Department of Pediatrics, Harvard Medical School, Boston, Massachusetts

The mean I.Q. of 27 PKU patients at the mean age of 3 years 10 months treated before 3 weeks of age was comparable to that of their unaffected siblings. The mean I.Q., of 12 patients at the mean age of 5 years 6 months treated between 3 and 6 weeks of age fell significantly below the mean I.Q. of their unaffected siblings. Seventeen patients treated after 8 months of age did not differ in their mean I.Q. at the end of treatment from 11 untreated patients, despite the observation that more than half of the late-treated patients achieved significant gains during the course of their treatment. Patients off dietary therapy did not appear to deteriorate over a follow-up duration of 2 to 3frac12 years. Eleven were followed for 6 years without evidence of deterioration. Thirteen atypical PKU's showed normal intellectual development. An unexplained predominance of males was observed in our group of atypical PKU patients.

Submitted on April 22, 1970
Accepted on September 9, 1970




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K. H. Schulpi and A. Scarpalezou
Triglycerides, Cholesterol, HDL, LDL, and VLDL Cholesterol in Serum of Phenylketonuric Children Under Dietary Control
Clinical Pediatrics, October 1, 1989; 28(10): 466 - 469.
[Abstract] [PDF]