Branched-chain amino acid-free parenteral nutrition in the treatment of acute metabolic decompensation in patients with maple syrup urine disease.
Berry
G T
GT
Division of Biochemical Development and Molecular Diseases, Children's Hospital of Philadelphia, PA 19104.
Heidenreich
R
R
Kaplan
P
P
Levine
F
F
Mazur
A
A
Palmieri
M J
MJ
Yudkoff
M
M
Segal
S
S
D000138:Acidosis / Q000628:therapy; D000597:Amino Acids, Branched-Chain / Q000097:blood; D002648:Child; D002675:Child, Preschool; D003681:Dehydration / Q000628:therapy; D004750:Enteral Nutrition; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D008375:Maple Syrup Urine Disease / Q000628:therapy*; D010288:Parenteral Nutrition / Q000379:methods*
D000597:Amino Acids, Branched-Chain