REVIEW: Practical strategies to maintain anabolism by intravenous nutritional management in children with inborn metabolic diseases

Kimberly A. Kripps, Peter R. Baker, Janet A. Thomas, Heather E. Skillman, Laurie Bernstein, Sommer Gaughan, Casey Burns, Curtis R. Coughlin, Shawn E. McCandless, Austin A. Larson, Aaina Kochar, Chelsey F. Stillman, Erica M. Wymore, Ellie G. Hendricks, Michael Woontner, Johan L.K. Van Hove

Research output: Contribution to journalReview articlepeer-review

Abstract

One of the most vital elements of management for patients with inborn errors of intermediary metabolism is the promotion of anabolism, the state in which the body builds new components, and avoidance of catabolism, the state in which the body breaks down its own stores for energy. Anabolism is maintained through the provision of a sufficient supply of substrates for energy, as well as critical building blocks of essential amino acids, essential fatty acids, and vitamins for synthetic function and growth. Patients with metabolic diseases are at risk for decompensation during prolonged fasting, which often occurs during illnesses in which enteral intake is compromised. During these times, intravenous nutrition must be supplied to fully meet the specific nutritional needs of the patient. We detail our approach to intravenous management for metabolic patients and its underlying rationale. This generally entails a combination of intravenous glucose and lipid as well as early introduction of protein and essential vitamins. We exemplify the utility of our approach in case studies, as well as scenarios and specific disorders which require a more careful administration of nutritional substrates or a modification of macronutrient ratios.

Original languageEnglish (US)
Pages (from-to)231-241
Number of pages11
JournalMolecular Genetics and Metabolism
Volume133
Issue number3
DOIs
StatePublished - Jul 2021

Keywords

  • Anabolism
  • Caloric requirements
  • Catabolism
  • Essential amino acid deficiency
  • Inborn errors of metabolism
  • Intravenous
  • Ketogenic diet
  • Protein restriction
  • Treatment approach

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Biochemistry
  • Molecular Biology
  • Genetics
  • Endocrinology

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