1.
Pediatrie
; 48(10): 681-6, 1993.
Article
in French
| MEDLINE
| ID: mdl-8015866
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.