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Recommendations for the nutrition management of phenylalanine hydroxylase deficiency
Evidence-based guidelines for managing phenylalanine hydroxylase deficiency, optimizing outcomes, and addressing all available therapies are lacking and recommendations for nutrition management were developed using evidence from peer-reviewed publications, gray literature, and consensus surveys.
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 Management of Patients With Inherited Metabolic Disorders
- P. Acosta
- 17 August 2009
This book describes problems and helps medical food manufacturers, medical geneticists, nutritionists/dietitians, and other health care providers find alternative forms of nutrients that would provide optimal nutrition and health for the patients.
The effect of dietary fruits and vegetables on urinary galactitol excretion in galactose-1-phosphate uridyltransferase deficiency
- G. Berry, M. Palmieri, S. Segal
- Medicine, BiologyJournal of Inherited Metabolic Disease
- 1 January 1993
The measured intake in total foodstuffs matched the theoretical content of galactose in the patient's diet based on amounts in fruits and vegetables alone, thus supporting the concept thatruits and vegetables are primarily responsible for galactOSE intake in a lactose-free diet.
Role of nutrition in pregnancy with phenylketonuria and birth defects.
The data indicate that blood Phe control and how soon it is attained during pregnancy with PKU is important, and normal pregnancy weight gain should be encouraged to reduce microcephaly.
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.
Hidden sources of galactose in the environment
A galactose-restricted diet free of lactose is lifesaving in patients with GALT deficiency, but does not prevent long-term complications such as developmental delay, abnormal speech, poor growth and, in females, ovarian failure.
The therapeutic effect of fiber in treating obesity.
Preliminary data on the influence of ingestion of 15 g of dietary fiber daily for 4 weeks on weight change and serum iron concentrations in obese children shows mean weight loss of subjects was greater during fiber ingestion than during placebo ingestion.