Data from forty-eight medical records of phenylketonuria children under treatment were investigated to determine if growth, as measured by stature, was normal; and to determine if the state of control or the age at beginning of treatment had an effect on growth. Mean intakes of phenylalanine, protein, and calories were obtained on the nutritional data only if the simultaneous serum phenylalanine was equal to or less than 6 milligrams per cent. These means were grouped according to the age of the children into 7 groups (0 The height at beginning of treatment and the last measured height was compared with established norms of the Brush Foundation Study. To determine whether the state of control or age of beginning treatment were factors in growth, the children were divided into groups according to (1) age dietary treatment was instituted (equal to or less than six months and over six months), and (2) state of control. "Good” control for this part of the study was having serum phenylalanine levels equal to or less than 4 milligrams per cent 75 percent of the time, and "poor" control, those having levels over 4 milligrams per cent 75 per cent of the time. The mean height of the parents compared favorably with the mean height for the white American male and female. At beginning of treatment the children were approximately half an inch shorter than estimated norms but at their last measured height they were 1,40 inches shorter. When the last recorded heights of the phenylketonuric children were analyzed considering time of beginning of treatment and state of control during treatment, there was no significant difference between the groups. The mean phenylalanine intake ranged from 227 to 488 milligrams each day. On a pound basis Group 1 consumed 19.3 milligrams per pound, with a gradual decrease to a low of 9.7 milligrams per pound in the 73 to 108 month age group. Protein intakes ranged from 20.8 grams a day for Group 1 to 45.7 grams each day for Group 7. The protein intake of the youngest group was above that recommended by the National Research Council, Groups 2, 3, 4, and 6 intakes were only slightly above the recommendations, Groups 5 and 7 had a somewhat lower intake than recommended. Mean caloric intake ranged from 611l to 1817 calories each day, increasing with increasing age. Calorie intake in Group 1 was 53.6 calories per pound of body weight. This was slightly higher than the recommended allowances. All other groups had mean calorie intakes less than those the National Research Council recommends. It was concluded that treated phenylketonuric children are significantly shorter than established norms but that age at beginning of treatment and control during treatment had no influence on the last heights of the children.
Congenital, Hereditary, and Neonatal Diseases and Abnormalities, Dietetics and Clinical Nutrition, Human and Clinical Nutrition, Phenylketonuria; Diet Therapy
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