Different outcome in isovaleric acidemia might be related to unsatisfactory diet compliance.
Mario
M
Castorina
Donato
D
Rigante
Daniela
D
Antuzzi
Giada
G
Sciascia Cannizzaro
Roberta
R
Ricci
D000592:Amino Acid Metabolism, Inborn Errors / Q000178:diet therapy / Q000378:metabolism*; D002648:Child; D018753:Diet, Protein-Restricted; D045782:Hemiterpenes; D006801:Humans; D050769:Isovaleryl-CoA Dehydrogenase / Q000172:deficiency*; D007930:Leucine / Q000378:metabolism*; D008297:Male; D010349:Patient Compliance; D010421:Pentanoic Acids / Q000378:metabolism*
D045782:Hemiterpenes; D010421:Pentanoic Acids; C008216:isovaleric acid; D050769:Isovaleryl-CoA Dehydrogenase; D007930:Leucine