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January 12, 1970

Growth and Nutrition in Treated Phenylketonuric Patients

Author Affiliations

From the Children's Hospital Research Foundation, Institute for Developmental Research and the Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati.

JAMA. 1970;211(2):270-276. doi:10.1001/jama.1970.03170020034007

Nutritional, clinical, and laboratory data collected over a period of years were examined to explain growth rate changes in children treated for phenylketonuria. The data suggest that periods of inconstant growth, associated clinical symptoms, and elevations of serum phenylalanine levels were related to an imbalance between essential amino acids in a commercial, low-phenylalanine, protein substitute (Lofenalac) and phenylalanine in natural food sources. Growth stability, clinical well-being, and stable levels of serum phenylalanine were obtained by providing both the protein substitute in amounts adequate to meet the recommended protein requirement for growth, and sufficient phenylalanine from natural sources to balance the other essential amino acids in the protein substitute.