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Phenylketonuria Scientific Review Conference: state of the science and future research needs.
Nutrient intakes and physical growth of children with phenylketonuria undergoing nutrition therapy.
Nutrition therapy of organic acidaemias with amino acid-based formulas: Emphasis on methylmalonic and propionic acidaemia
- S. Yannicelli
- MedicineJournal of Inherited Metabolic Disease
- 1 April 2006
Although protein requirements in patients with organic acidaemias have not been elucidated, providing an adequate balance of protein, energy and other nutrients will help promote growth.
A re-evaluation of life-long severe galactose restriction for the nutrition management of classic galactosemia.
The management of breast feeding among infants with phenylketonuria
Breast feeding may be continued in the newly diagnosed phenylketonuric infant without any apparent adverse nutritional consequences and supplementation with sufficient phenylalanine for normal growth and development.
Regulation of dietary supplements.
Protein intake affects phenylalanine requirements and growth of infants with phenylketonuria
Growth and metabolic status of 25 infants with PKU were evaluated based on protain intake and mean protein intake 24% greater than Recommended Dietary Allowances was associated with better PHE tolerance and growth than was found when meanprotein intake was 9% greaterthan RDA.
Improved growth and nutrition status in children with methylmalonic or propionic acidemia fed an elemental medical food.
Nutritional therapy improves growth and protein status of children with a urea cycle enzyme defect.