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Neonatal Hyperammonemia

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Abstract

This chapter describes the clinical approach and dialytic management of neonatal hyperammonemia. Newborns with hyperammonemia typically present as a metabolic emergency between the ages of 24 and 48 h. Early management including glucose infusion, protein restriction, and pharmacologic intervention with nitrogen scavengers is important to limit neurological damage. Dialysis is required to urgently remove ammonia if pharmacologic intervention is insufficient to remove the ammonia load, with high-dose continuous venovenous hemodiafiltration (CVVHDF) the modality of choice in most cases. Intermittent hemodialysis may be required initially in the presence of severe hyperammonemia to rapidly reduce ammonia levels, followed by CVVHDF to prevent ammonia rebound. The severity of the neurological outcome appears to be related to the duration of severe hyperammonemia and not to peak ammonia levels.

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References

  1. Gropman AL, Summar M, Leonard JV. Neurological implications of urea cycle disorders. J Inherit Metab Dis. 2007;30:865–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  2. Auron A, Brophy PD. Hyperammonemia in review: pathophysiology, diagnosis, and treatment. Pediatr Nephrol. 2012;27:207–22.

    Article  PubMed  Google Scholar 

  3. Häberle J, Boddaert N, Burlina A, Chakrapani A, Dixon M, Huemer M, Karall D, Martinelli D, Crespo PS, Santer R, Servais A, Valayannopoulos V, Lindner M, Rubio V, Dionisi-Vici C. Suggested guidelines for the diagnosis and management of urea cycle disorders. Orphanet J Rare Dis. 2012;7:32. doi:10.1186/1750-1172-7-32.

    Article  PubMed  PubMed Central  Google Scholar 

  4. Emms GM. Nitrogen sparing therapy revisited 2009. Mol Genet Metab. 2010;100:565–71.

    Google Scholar 

  5. Summar M. Current strategies for the management of neonatal urea cycle disorders. J Pediatr. 2001;138:S30–9.

    Article  CAS  PubMed  Google Scholar 

  6. Brusilow SW, Valle DL, Batshaw M. New pathways of nitrogen excretion in inborn errors of urea synthesis. Lancet. 1979;2(8140):452–4.

    Article  CAS  PubMed  Google Scholar 

  7. Schaefer F, Straube E, Oh J, Mehls O, Mayatepek E. Dialysis in neonates with inborn errors of metabolism. Nephrol Dial Transplant. 1999;14:910–8.

    Article  CAS  PubMed  Google Scholar 

  8. Fakler CR, Kaftan HA, Nelin LD. Two cases suggesting a role for the L-arginine nitric oxide pathway in neonatal blood pressure regulation. Acta Paediatr. 1995;84:460–2.

    Article  CAS  PubMed  Google Scholar 

  9. Summar M, Pietsch J, Deshpande J, Schulman G. Effective hemodialysis and hemofiltration driven by an extracorporeal membrane oxygenation pump in infants with hyperammonemia. J Pediatr. 1996;128:379–82.

    Article  CAS  PubMed  Google Scholar 

  10. Picca S, Dionisi-Vici C, Bartuli A, De Palo T, Papdia F, Montini G, Materassi M, Donati MA, Verrina E, Schiaffino MC, Pecoraro C, Iaccarino E, Vidal E, Burlina A, Emma F. Short-term survival of hyperammonemic neonates treated with dialysis. Pediatr Nephrol. 2015;30:839–47.

    Article  PubMed  Google Scholar 

  11. Bunchman TE. The complexity of dialytic therapy in hyperammonemic neonates. Pediatr Nephrol. 2015;30:701–2.

    Article  PubMed  Google Scholar 

  12. Bunchman TE, Barletta GM, Winters JW, Gardner JJ, Crumb TL, McBryde KD. Phenylacetate and benzoate clearance in a hyperammonemic infant on sequential hemodialysis and hemofiltration. Pediatr Nephrol. 2007;22:1062–5.

    Article  PubMed  Google Scholar 

  13. Kido J, Nakamura K, Mitsubuchi H, Ohura T, Takayanagi M, Matsuo M, Yoshino M, Shigematsu Y, Yorifuji T, Kasahara M, Horikawa R, Endo F. Long-term outcome and intervention of urea cycle disorders in Japan. J Inherit Metab Dis. 2012;35:777–85.

    Article  CAS  PubMed  Google Scholar 

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Correspondence to Hui-Kim Yap .

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Yap, HK. (2017). Neonatal Hyperammonemia. In: Warady, B., Schaefer, F., Alexander, S. (eds) Pediatric Dialysis Case Studies. Springer, Cham. https://doi.org/10.1007/978-3-319-55147-0_41

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  • DOI: https://doi.org/10.1007/978-3-319-55147-0_41

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-55145-6

  • Online ISBN: 978-3-319-55147-0

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