Nutrition support of inborn errors of amino acid metabolism.

Programs for nutrition support of patients with phenylketonuria, maternal phenylketonuria, branched chain ketoaciduria and vitamin B-6 non-responsive homocystinuria were written in BASIC. These programs plan diets to fill diet prescriptions using natural foods, available amino acid-free or restricted elemental products, milk or infant proprietary formulae and protein-free fat and carbohydrate sources. Emphasis is placed on satisfying the amino acid and protein prescriptions simultaneously. The final semisynthetic formula is evaluated for vitamins, minerals, renal solute load and renal net acid excretion. Fluid requirement is estimated. The paper describes how the conventional protocols are enhanced by computerization and details the requisite calculations.
AuthorsB Kennedy, K Anderson, P B Acosta
JournalInternational journal of bio-medical computing (Int J Biomed Comput) Vol. 17 Issue 1 Pg. 69-76 (Jul 1985) ISSN: 0020-7101 [Print] ENGLAND
PMID3840460 (Publication Type: Journal Article)
Chemical References
  • Keto Acids
  • Pyridoxine
  • Amino Acid Metabolism, Inborn Errors (diet therapy)
  • Computers
  • Drug Resistance
  • Homocystinuria (diet therapy, drug therapy)
  • Humans
  • Infant
  • Infant Nutritional Physiological Phenomena
  • Keto Acids (urine)
  • Microcomputers
  • Phenylketonurias (diet therapy, genetics)
  • Pyridoxine (therapeutic use)
  • Software

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