Skip to main content

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 89.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Acosta PB. Nutrition management of patients with inherited metabolic disorders. In: Acosta PB, editor. Jones and Bartlett Publishers, Sudbury, Massachusetts. LLC; 2010. p 476.

    Google Scholar 

  2. Pusti S, et al. A treatable neurometabolic disorder: glutaric aciduria type 1. Case Rep Pediatr. 2014;2014:256356.

    CAS  PubMed Central  PubMed  Google Scholar 

  3. Hedlund GL, Longo N, Pasquali M. Glutaric acidemia type 1. Am J Med Genet C: Semin Med Genet. 2006;142C(2):86–94.

    Article  CAS  Google Scholar 

  4. Kölker S, et al. Diagnosis and management of glutaric aciduria type I–revised recommendations. J Inherit Metab Dis. 2011;34(3):677–94.

    Article  PubMed Central  PubMed  Google Scholar 

  5. Harting I, et al. Dynamic changes of striatal and extrastriatal abnormalities in glutaric aciduria type I. Brain. 2009;132(Pt 7):1764–82.

    Article  PubMed  Google Scholar 

  6. Strauss KA, et al. Type I glutaric aciduria, part 1: natural history of 77 patients. Am J Med Genet C: Semin Med Genet. 2003;121C(1):38–52.

    Article  Google Scholar 

  7. Thomas JA, et al. Apparent decreased energy requirements in children with organic acidemias: preliminary observations. J Am Diet Assoc. 2000;100(9):1074–6.

    Article  CAS  PubMed  Google Scholar 

  8. Strauss KA, et al. Safety, efficacy and physiological actions of a lysine-free, arginine-rich formula to treat glutaryl-CoA dehydrogenase deficiency: focus on cerebral amino acid influx. Mol Genet Metab. 2011;104(1–2):93–106.

    Article  CAS  PubMed  Google Scholar 

  9. Boy N, et al. Low lysine diet in glutaric aciduria type I–effect on anthropometric and biochemical follow-up parameters. J Inherit Metab Dis. 2013;36(3):525–33.

    Article  CAS  PubMed  Google Scholar 

  10. Zempleni J, Galloway JR, McCormick DB. Pharmacokinetics of orally and intravenously administered riboflavin in healthy humans. Am J Clin Nutr. 1996;63(1):54–66.

    CAS  PubMed  Google Scholar 

  11. Winter SC, et al. Plasma carnitine deficiency. Clinical observations in 51 pediatric patients. Am J Dis Child. 1987;141(6):660–5.

    Article  CAS  PubMed  Google Scholar 

  12. Brandt NJ, et al. Treatment of glutaryl-CoA dehydrogenase deficiency (glutaric aciduria). Experience with diet, riboflavin, and GABA analogue. J Pediatr. 1979;94(4):669–73.

    Article  CAS  PubMed  Google Scholar 

  13. Chalmers RA, Bain MD, Zschocke J. Riboflavin-responsive glutaryl CoA dehydrogenase deficiency. Mol Genet Metab. 2006;88(1):29–37.

    Article  CAS  PubMed  Google Scholar 

  14. McMenamy RH, Oncley JL. The specific binding of L-tryptophan to serum albumin. J Biol Chem. 1958;233(6):1436–47.

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Laurie E. Bernstein MS, RD, FADA, FAND .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2015 Springer International Publishing Switzerland

About this chapter

Cite this chapter

Bernstein, L.E. (2015). Nutrition Management of Glutaric Acidemia Type 1. In: Bernstein, L., Rohr, F., Helm, J. (eds) Nutrition Management of Inherited Metabolic Diseases. Springer, Cham. https://doi.org/10.1007/978-3-319-14621-8_19

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-14621-8_19

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-14620-1

  • Online ISBN: 978-3-319-14621-8

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics