[Dietary treatment of maple syrup urine diseases].
Principles of dietary management and needs in maple syrup urine disease are well defined. However, long-term results show that there is a need for an improvement of the maintenance of a low plasma leucine level and in monitoring episodes of metabolic decompensation in order to ameliorate the survival and psycho-intellectual outcome.
Service de pédiatrie et génétique, centre d'étude des maladies héréditaires du métabolisme hôpital Debrousse (faculté Lyon-Nord), France.
P
P
Guibaud
L
L
de Parscau
N
N
Guffon
M C
MC
Laval
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases / Q000178:diet therapy; D008375:Maple Syrup Urine Disease / Q000150:complications / Q000178:diet therapy*; D009751:Nutritional Requirements; D011247:Pregnancy; D011248:Pregnancy Complications; D012307:Risk Factors
Le traitement nutritionnel des leucinoses.