To assess the effects of nutritional therapy with a medical food on growth and protein status of patients with a urea cycle enzyme defect.
A 6-mo multicenter outpatient study was conducted with infants and toddlers managed by nutrition therapy with Cyclinex-1 Amino Acid-Modified Medical Food with Iron (Ross Products Division, Abbott Laboratories, Columbus, OH). Main outcome variables were anthropometrics and plasma amino acids (selected), albumin, and transthyretin concentrations.
Seventeen patients completed the study. Mean (b1;SE) baseline age was 11.30 b1; 3.20 months (median 4.40 months; range 0.22–38.84 months). Length and weight z-scores increased significantly during the 6-month study. Head circumference increased, but not significantly. Three patients were stunted and two were wasted (−2.0 z-score) at baseline while at study end, only one patient was both stunted and wasted. The majority of patients increased in length, head circumference, and weight z-scores during study. Mean (b1;SE) plasma albumin concentration increased from 34 b1; 2 g/L at baseline to 38 b1; 1 g/L at study end. Plasma transthyretin increased from a mean (b1;SE) of 177 b1; 13 mg/L at baseline to 231 b1; 15 mg/L at study end. No correlation was found between plasma NH
Intakes of adequate protein and energy for age result in anabolism and linear growth without increasing plasma NH