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[Dietary treatment of maple syrup urine diseases]
P Guibaud1, L de Parscau, N Guffon
1Service de pédiatrie et génétique, centre d'étude des maladies héréditaires du métabolisme hôpital Debrousse (faculté Lyon-Nord), France.
Pediatrie
|January 1, 1993
Abstract:
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.