Skip to main content
SpringerLink
Log in
Book cover

Nutrition Management of Inherited Metabolic Diseases pp 229–237Cite as

Nutrition Management During Pregnancy: Maple Syrup Urine Disease, Propionic Acidemia, Methylmalonic Acidemia, and Urea Cycle Disorders

  • Chapter

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

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

Learn about institutional subscriptions

References

  1. Van Calcar SC, et al. Case reports of successful pregnancy in women with maple syrup urine disease and propionic acidemia. Am J Med Genet. 1992;44(5):641–6.

    Article  PubMed  Google Scholar 

  2. Grünewald S, Hinrichs F, Wendel U. Pregnancy in a woman with maple syrup urine disease. J Inherit Metab Dis. 1998;21(2):89–94.

    Article  PubMed  Google Scholar 

  3. Yoshida S, Tanaka T. Postpartum death with maple syrup urine disease. Int J Gynaecol Obstet. 2003;81(1):57–8.

    Article  CAS  PubMed  Google Scholar 

  4. Wessel AE, et al. Management of a woman with maple syrup urine disease during pregnancy, delivery, and lactation. J Parenter Enteral Nutr. 2014;doi:10.1177/0148607114526451.

  5. Tchan M, et al. The management of pregnancy in maple syrup urine disease: experience with two patients. JIMD Rep. 2013;10:113–7.

    PubMed Central  PubMed  Google Scholar 

  6. Pessel C, Tsai MC. The normal puerperium. In: DeCherny A et al., editors. Current diagnosis and treatment: obstetrics and gynecology. New York: McGraw Hill; 2013.

    Google Scholar 

  7. Langendonk JG, et al. A series of pregnancies in women with inherited metabolic disease. J Inherit Metab Dis. 2012;35(3):419–24.

    Article  PubMed  Google Scholar 

  8. Wasserstein MP, et al. Successful pregnancy in severe methylmalonic acidaemia. J Inherit Metab Dis. 1999;22(7):788–94.

    Article  CAS  PubMed  Google Scholar 

  9. Lubrano R, et al. Pregnancy in a methylmalonic acidemia patient with kidney transplantation: a case report. Am J Transplant. 2013;13(7):1918–22.

    Article  CAS  PubMed  Google Scholar 

  10. Boneh A, et al. Metabolic treatment of pregnancy and postdelivery period in a patient with cobalamin A disease. Am J Obstet Gynecol. 2002;187(1):225–6.

    Article  PubMed  Google Scholar 

  11. Deodato F, et al. Successful pregnancy in a woman with mut- methylmalonic acidaemia. J Inherit Metab Dis. 2002;25(2):133–4.

    Article  CAS  PubMed  Google Scholar 

  12. Raval DB, et al. Methylmalonic acidemia (MMA) in pregnancy: a case series and literature review. J Inherit Metab Dis. 2015; doi:10.1007/s10545-014-9802-8.

  13. Brunel-Guitton C, et al. Treatment of cobalamin C (cblC) deficiency during pregnancy. J Inherit Metab Dis. 2010;33 Suppl 3:S409–12.

    Article  PubMed  Google Scholar 

  14. Enns GM, et al. Postpartum “psychosis” in mild argininosuccinate synthetase deficiency. Obstet Gynecol. 2005;105(5 Pt 2):1244–6.

    Article  PubMed  Google Scholar 

  15. Peterson DE. Acute postpartum mental status change and coma caused by previously undiagnosed ornithine transcarbamylase deficiency. Obstet Gynecol. 2003;102(5 Pt 2):1212–5.

    Article  PubMed  Google Scholar 

  16. Eather G, et al. Carbamyl phosphate synthase deficiency: diagnosed during pregnancy in a 41-year-old. J Clin Neurosci. 2006;13(6):702–6.

    Article  CAS  PubMed  Google Scholar 

  17. Potter MA, et al. Pregnancy in a healthy woman with untreated citrullinemia. Am J Med Genet A. 2004;129A(1):77–82.

    Article  PubMed  Google Scholar 

  18. Lamb S, et al. Multidisciplinary management of ornithine transcarbamylase (OTC) deficiency in pregnancy: essential to prevent hyperammonemic complications. BMJ Case Rep. 2013;149.

    Google Scholar 

  19. Arn PH, et al. Hyperammonemia in women with a mutation at the ornithine carbamoyltransferase locus. A cause of postpartum coma. N Engl J Med. 1990;322(23):1652–5.

    Article  CAS  PubMed  Google Scholar 

  20. Kotani Y, et al. Carbamyl phosphate synthetase deficiency and postpartum hyperammonemia. Am J Obstet Gynecol. 2010;203(1):e10–1.

    Article  PubMed  Google Scholar 

  21. Mendez-Figueroa H, et al. Management of ornithine transcarbamylase deficiency in pregnancy. Am J Perinatol. 2010;27(10):775–84.

    Article  PubMed  Google Scholar 

  22. Institute of Medicine (U.S.). Panel on Macronutrients. Standing Committee on the Scientific Evaluation of Dietary Reference Intakes. Dietary reference intakes for energy, carbohydrate, fiber, fat, fatty acids, cholesterol, protein, and amino acids. Washington, DC: National Academies Press; 2005. xxv, 1331 p.

    Google Scholar 

  23. Hadden DR, et al. Normal and abnormal maternal metabolism during pregnancy. Sem Fetal Neonatal Med. 2009;14:66–71.

    Google Scholar 

  24. Scott-Schwoerer JA, et al. Use of gastrostomy tube to prevent maternal PKU syndrome. JIMD Rep. 2012;6:15–20.

    Google Scholar 

  25. Lee PJ. Pregnancy issues in inherited metabolic disorders. J Inherit Metab Dis. 2006;29(2–3):311–6.

    Article  PubMed  Google Scholar 

  26. Institute of Medicine. Weight gain during pregnancy: re-examining the guidelines. 2009. [cited 2014 May 14]; Available from: http://iom.edu/Reports/2009/Weight-Gain-During-Pregnancy-Reexamining-the-Guidelines.aspx.

  27. Siega-Riz, AM, Evenson KR, Dole N. Pregnancy-related weight gain–a link to obesity? Nutrition Reviews. 2004;62(suppl 2):S105–S111.

    Google Scholar 

  28. Abrams B, Selvin S. Maternal weight gain pattern and birth weight. Obstetr Gynecol. 1995;86(2):163–9.

    Google Scholar 

  29. Carmichael S, Abrams B, Selvin S. The pattern of maternal weight gain in women with good pregnancy outcomes. Am J Pub Health. 1997;87(12):1984–88.

    Google Scholar 

Download references

Acknowledgements

The author wishes to thank the metabolic team at the University of Wisconsin-Madison and the patients followed by this clinic for allowing publication of their experiences.

Author information

Authors and Affiliations

  1. Department of Molecular and Medical Genetics and Graduate Programs in Human Nutrition, School of Medicine, Oregon Health and Science University, 3181 S.W. Sam Jackson Park Rd., Portland, OR, 97239-3098, USA

    Sandy van Calcar PhD, RDN, LDN

Authors
  1. Sandy van Calcar PhD, RDN, LDN
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Sandy van Calcar PhD, RDN, LDN .

Editor information

Editors and Affiliations

  1. Clinical Genetics and Metabolism, Children’s Hospital Colorado, University of Colorado Denver – Anschutz Medical Campus, Aurora, Colorado, USA

    Laurie E. Bernstein

  2. Division of Genetics and Genomics, Boston Children’s Hospital, Boston, Massachusetts, USA

    Fran Rohr

  3. Clinical Genetics and Metabolism, University of Colorado Denver, Children’s Hospital Colorado, Aurora, Colorado, USA

    Joanna R. Helm

Rights and permissions

Reprints and permissions

Copyright information

© 2015 Springer International Publishing Switzerland

About this chapter

Cite this chapter

van Calcar, S. (2015). Nutrition Management During Pregnancy: Maple Syrup Urine Disease, Propionic Acidemia, Methylmalonic Acidemia, and Urea Cycle Disorders. 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_21

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-14621-8_21

  • 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

Search

Navigation