Dietary management of inborn errors of metabolism.
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.
Kabra
Madhulika
M
Department of Pediatrics, All India Institute of Medical Sciences, New Delhi. madhulikakabra@hotmail.com
D002648:Child; D004035:Diet Therapy / Q000379:methods; D005693:Galactosemias / Q000178:diet therapy; D006008:Glycogen Storage Disease / Q000178:diet therapy; D006712:Homocystinuria / Q000178:diet therapy; D006801:Humans; D008375:Maple Syrup Urine Disease / Q000178:diet therapy; D008661:Metabolism, Inborn Errors / Q000175:diagnosis / Q000178:diet therapy*; D010661:Phenylketonurias / Q000178:diet therapy