Medical nutrition therapy in patients with HIBCH and ECHS1 defects: Clinical and biochemical response to low valine diet

• J.E. Abdenur,
• M. Sowa,
• M. Simon,
• M. Steenari,
• J. Skaar,
• S. Eftekharian,
• R. Chang,
• S. Ferdinandusse,
• J. Pitt

Affiliations

J.E. Abdenur

Division of Metabolic Disorders, CHOC Children's, Orange, CA, USA; Department of Pediatrics, University of California Irvine, Orange, CA. USA; Corresponding author at: Division of Metabolic Disorders, CHOC Children's, Orange, CA, USA.

M. Sowa

Division of Metabolic Disorders, CHOC Children's, Orange, CA, USA

M. Simon

Division of Metabolic Disorders, CHOC Children's, Orange, CA, USA

M. Steenari

Division of Neurology, CHOC Children's. Orange, CA, USA; Department of Pediatrics, University of California Irvine, Orange, CA. USA

J. Skaar

Division of Metabolic Disorders, CHOC Children's, Orange, CA, USA

S. Eftekharian

Division of Metabolic Disorders, CHOC Children's, Orange, CA, USA

R. Chang

Division of Metabolic Disorders, CHOC Children's, Orange, CA, USA; Department of Pediatrics, University of California Irvine, Orange, CA. USA

S. Ferdinandusse

Departments of Clinical Chemistry and Pediatrics, Laboratory Genetic Metabolic Diseases, Amsterdam, the Netherlands

J. Pitt

Victorian Clinical Genetics Services, Murdoch Children's Research Institute, Melbourne, AU, Australia