title: Nutritional therapy improves growth and protein status of children with a urea cycle enzyme defect.
abstract: BACKGROUND
  Poor growth has been described in patients with urea cycle enzyme defects treated with protein-restricted diets, while protein status is seldom reported.
  
  
  OBJECTIVE
  To assess the effects of nutritional therapy with a medical food on growth and protein status of patients with a urea cycle enzyme defect.
  
  
  METHODS
  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.
  
  
  RESULTS
  Seventeen patients completed the study. Mean (+/-SE) baseline age was 11.30+/-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 (+/-SE) plasma albumin concentration increased from 34+/-2g/L at baseline to 38+/-1g/L at study end. Plasma transthyretin increased from a mean (+/-SE) of 177+/-13 mg/L at baseline to 231+/-15 mg/L at study end. No correlation was found between plasma NH(3) concentrations and medical food intake. Plasma NH(3) concentration was positively correlated with the percentage of Food and Agriculture Organization/World Health Organization/United Nations recommended protein ingested.
  
  
  CONCLUSIONS
  Intakes of adequate protein and energy for age result in anabolism and linear growth without increasing plasma NH(3) concentrations. Medical food intakes did not correlate with plasma NH(3) concentrations.
authors.affiliation: Ross Products Division, Abbott Laboratories, Columbus, OH, USA. pja1933@aol.com
authors.forename: Phyllis B
authors.initials: PB
authors.lastname: Acosta
authors.forename: Steven
authors.initials: S
authors.lastname: Yannicelli
authors.forename: Alan S
authors.initials: AS
authors.lastname: Ryan
authors.forename: Georgianne
authors.initials: G
authors.lastname: Arnold
authors.forename: Barbara J
authors.initials: BJ
authors.lastname: Marriage
authors.forename: Magda
authors.initials: M
authors.lastname: Plewinska
authors.forename: Laurie
authors.initials: L
authors.lastname: Bernstein
authors.forename: Joyce
authors.initials: J
authors.lastname: Fox
authors.forename: Vyonne
authors.initials: V
authors.lastname: Lewis
authors.forename: Marvin
authors.initials: M
authors.lastname: Miller
authors.forename: Antonio
authors.initials: A
authors.lastname: Velazquez
mesh_terms: D000596:Amino Acids / Q000008:administration & dosage / Q000097:blood; D001798:Blood Proteins / Q000378:metabolism; D001827:Body Height; D002675:Child, Preschool; D004044:Dietary Proteins / Q000008:administration & dosage; D002149:Energy Intake; D005260:Female; D006128:Growth; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008661:Metabolism, Inborn Errors / Q000178:diet therapy* / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D009752:Nutritional Status; D011228:Prealbumin / Q000378:metabolism; D012709:Serum Albumin / Q000378:metabolism; D014508:Urea / Q000378:metabolism*; D015430:Weight Gain
chemical_list: D000596:Amino Acids; D001798:Blood Proteins; D004044:Dietary Proteins; D011228:Prealbumin; D012709:Serum Albumin; D014508:Urea