Dietary management of inborn errors of metabolism.
Indian J Pediatr
; 2002 May; 69(5): 421-6
Article
in En
| IMSEAR
| ID: sea-82069
ABSTRACT
Inborn errors of metabolism are individually rare but are an important cause of mortality and morbidity in infants and children. Dietary therapy is the mainstay of treatment in phenylketonuria, maple syrup urine disease, homocystinuria, galactosemia and glycogen storage disease (Type I/III). Some disorders like urea cycle disorders and organic acidurias require dietary modification in addition to other modalities. Certain basic principles of dietary management should be clearly understood for proper management of these disorders. Commercially available diets are very expensive and modification in routine Indian diet may be tried based on content of different nutrients but the desirable fine control is not achieved.
Full text:
1
Index:
IMSEAR
Main subject:
Phenylketonurias
/
Humans
/
Glycogen Storage Disease
/
Child
/
Diet Therapy
/
Galactosemias
/
Homocystinuria
/
Maple Syrup Urine Disease
/
Metabolism, Inborn Errors
Language:
En
Journal:
Indian J Pediatr
Year:
2002
Type:
Article